,

1963 SEMINAR HOUSTON SOCIETY OF CLINICAL PATHOLOGISTS

PATHOLOGIC-RADIOGRAPHIC CORRELATION IN LESIONS OF LUNG, BONE, GASTROINTESTINAL TRACT,

BREAST AND SOFT TISSUES

RAFFAELE LATTES, M.D.

Presented by

ARTHUR PURDY STOUT, M.D. Emeritus Professor of Surgical Pathology

College of Physicians and Surgeons Columbia University

New York City

1P /1!1

Professor of Surgica l Pathology

College of Physicians and Surgeons Columbia UniVersity

BENJAMIN FELSON, M.D. Professor of Radiology

University of Cincinnori College of M edicine

Cincinnati, Ohio New York City

Houlton. Texas

March 2, 1963

'

A SEMINAR Olf SURGICAL PATHOLOGY

Houston Soci ety of Clinical Pathologists

~larch 2 , 1963

9:00 A.M. - 5:00 P.M.

at

ST. LUKE 1 S EPISCOPAL HOSPITAL

.Auditorium~-Texas Children 1 s Hospi t al

Texas Medical Center

Houston, Texas

Conducted by

AR'1'HllR PURDY STOUT, M. D. Emeritus Professor of Pathology

College of Physicians and Surgeons Columbia University

New Y.ork City

RAFFAELE LA!r:PFS, M. D. BENJAMIN FEI.SON, M.D. Professor of surgical Pathol ogy

College of Physicians and Surgeons Columbia University

New York City

Professor of Radiology University of Cincinnati

College of Medicine Cincinnati, Ohio

cASE 1.

C~E 2.

CASE 3·

CASE 4.

CASE 5·

CA!lE 6.

CASE 7·

CASE 8.

CASE 9.

CASE. 10.

CI\SE 11.

CASE 12.

CASE 13.

CASE 14 .

HOUSTON SOCIETY OF 'CL.INICAL )'ATHOLOGISTS

:1963 Seminar

PathOlogic -Radiogra;phic Corre;Latio.n in Lesions of Lung, Bone, Gastrointestinal Tract,

Breas t and Soft Tissues

DIAGNOSES

Fibr osarcoma of Lung

Hodgkin's Disease of Mediastinum

Carcinosarcoma. of Lung

Neurobl ast oma (Sympathoblastoma)

Pseudosarcoma.tous Fas ciitis

Inflammatory Fibroid Polyp of Stomach (Vanek's Tilmbr)

Benign Giant Gastric Ulcer with Eosinophilic Infiltration

Osteogenic Sarcoma. of Rib

Neuri lemmoma of Retroperitoneum

Giant Rugal Hypertrophy (Menetrier 's Disease)

Granuloma- of Stomach, Sarcoid l'YPe.

iramartomatosis of Lung, Cystic Dise.ase of L:ung

Cystosarcoma Phylloides ( ?lo!a.lignant)

Pseudoneoplastic Granulomas with Inclusion Bodies

I '2 • t. r · 'S ,

CASE 1. I! & E

CASE L Retioul1n

CASE 1 SL-59-5628

Contributed by: Howard T. Barkley, Sr., M.D., and John A . Webb, M.D. 1 St. Luke's Episcopal Hospi tal

This 69 year old 1 white 1 male was seen in December 1 1959. He

bad bad an abdominal. perineal resection for carcinoma of the rectum in 19521 •

and bad been in good health until May, 1959 when be developed discomfort

and irritation i n his throat with productive persistent cough and occasional

episodes of hemoptysis .

Chest x-ray 1n May 1 1959 was negative. Cough continued until

the time of admission with production df thick yellow sputum occasionally

st reaked with blood . There was a l oss of 25 pounds in the year before

admission. Dyspnea, angina and other symptoms were denied.

On examination there was some dullness anteriorly over the

left upper lobe with reduced vocal fremitus .

Chest x-ray showed a large mass in the parenchyma of the left

upper lobe . Lef t thoracotomy and l eft upper lobect omy were performed .

~ton S9ciety or Clinical Pathologists Seminar ltlrch 2, 1963

CASE 1 P&S 70886

St. Luke's SL- 59-5628

We are told that the operation performed consisted of a left upper lobectomy and that the specimen weighed about 700 grams and measured 18 x l2 x 10 em. Most of the portion of lung was firm in consistency and on sec~ion it appeared to be repl aced by large, fleshy, yellowish-white necrotic tuoor tissue.

Sections of this tumor show that it is definitely different trom the usual primary carcinoma of the lung. The over-all pattern is that of predominantly spindle-shaped cells with irregularly oval- shaped nuclei which show finely subdiVided chromatin and displaying an arrangement in interlacing bundles . There is fairly high mitotic activity. With ordinary hematoxylin 1111d eosin stain there appears t o be l ittle recognizable ground substance between the tumor cells except for occasional areas where eosinophilic fibrillar mater ial can be recognized. An interesting feature of this tumor i s that on low pewer it has a so-called peritheliomatous pattern. Thi s actually i s due to the fact that at a certain dist ance from the blood vessels the tumor cells undergo necrosis so that you are left with cuffs of tumor cells around blood vessels . This pattern obViously should not be confUsed with that of hemangio­pericytoma. There is no obVious carcinoma of the mucous D'.embrane of the bronchi which is singularly well preser ved except for patches of atrophy. I have not seen any squamous metaplasia. The tumor must have been partly fungating into the bronchi since this can be seen also on histological study. Reticulin stains show abundant reticulin. fibers, arranged between cell and cell in a rich mesh­vorl!:. The pattern is strongly suggestive of a mesenchymal tumor of fibrous origin.

Obviously then, the diagnosis here is between an unusual carcinoma of t he lung which masquerades as a mesenchymal tumor of sarcomatous type and, second, primary or secondary sarcoma of the lung. Assuming t hat the tumor is primary, that is, assuming that there i s no evidence or history of an analogous tumor elsewhere, I believe that this case falls in that group t hat has generally been referred to as fibrosarcoma or leiomyosarcoma of the lung. There are several reports in the literature. The impression one gains from the litera­ture available is that these tumors are not always as malignant as the more common bronchogenic carcinoma.

•

Houston Society of Clinical Pathologists Seminar !·larch 2 , 1963

CASE 1 P&S 70886

St. Luke 's SL· 59· 5628

I see no reason to consider this tumor as a leiomyosarcoma. !leither the cell type nor the reticulin pattern suggest that inter­pretation. I am going, therefore, to classify this case as a poorly differentiated fibrosarcoma.

DIAGNOSIS: Fibrosarcoma of Lung .

References : Bolinger, P. H. , et aJ..: Primary Fibrosarcoma of Bronchus.

Diseases of Chest 37: 2, 1960. Iverson, L.: Bronchopulmonary Sarcoma. J. Thor. Surg. 27:

130- 148, 1954.

Dr. John R. McDonald, Harper Hospital., Det roit, Michigan:

1liAGNOSIB: Primary Pulmonary Fibrosarcoma (sarcoma)

Dr. Lauren v. Ackerman, Barnes Hospite.J,. , St. Louis, Missouri:

DIAGNOSIS: Left ·Upper Lob~, Excision· Fibrosarcoma

Follow-UP: February 28, 1963 • Patient is alive and well, without clinical roentgenological evidence of recurrent or persistent tumor.

CASE 2 .

CASE 2 SL-59-3951

Contributed by: William D. Seybold, M. D. and William T. Hi ll, !1. D., .St. Luke 's Episcopal Hospital

This 30 year ol d , white, female was in good .healt h unt il two

veeks prior to admis$1on, when she noticed sore neck and s l i&ht diffi-

culty in swallowi ng •

. Chest x-ray showed a ni!!>SS in the supe·rior medias t inum. I - 131

uptake was normal. Gammagram showed a normal symmetrical p~ttern wi th

no activity over the mass .

CBC and urinalys·is were nor mal.

A r i ght posterolat eral thoracotomy was performed t nrough the

bed of the fifth. rib with t he pleural space being f ree and the lung

apparently normal . A mass was removed .

Houston Society of Clinical Pathologists SeiDinar lllrch 2' 1963

CASE 2 P&S 7o851

St. Luke's SL· 59· 395l

We are told that the gross specimen removed from this woman's anterior mediastinal region consisted essentially of two lobulated fragments vith a total weight of 150 grams. These fragments appear to be enveloped in a fibrous capsule. The cut surfaces were homogeneous grayish-white, showing one yellowish-brown well demarcated nodule 3.8 em. in diameter. We are also told that several months later a cervical lymph node was removed from the same patient.

Microscopic study of this case brings up once again the problem of differentiating between Hodgkin's disease and the so-called granulomatous thymoma. I had seen this case some time ago when I was requested to express

.. an opinion on it by Dr. Hill (our P&S 64425). At that time, I expressed the opinion that this was a lesion of the thymus of the so-called granulomatous typa resembling Hodgkin's disease but different from it. The sections avail­able can be described as follows; There are multiple poorly outlined nodular masses some of which are predominantly lymphoid, others predominantly fibrous. In all of them there is a mixture of lymphocytes with granulomatous foci in which are epithelioid cells, fibroblasts, eosinophile, and larger reticulo­endothelial type cells with bizarre nuclei and, sometimes, with conspicuous acidophilic nucleoli. This description applies not only to the histological findings of the sections being distributed for this seiDinar but also of the sections that I saw originally about two years ago. At that time I did not notice two features ; one, that nowhere is it possible to recognize thymic tissue in the diseased areas; two, that frequently in the lymphoid tissue containing these granulomas of the Hodgkin's type there is anthracotic pigment, vhich I believe is a good proof that we are looking at mediastinal lymph nodes and not at thymus. Under these circumstances my present thoughts are that we have not proven that this is a lesion originating in the thymus. If to that you add the fact that a cervical lymph node removed subsequently showed si.milar features, I believe that it is more fair to consider the whole thing as an example of Hodgkin's disease of the mediastinum rather than an

Houston Society or Clinical Pathologists Seminar March 21 1963

CASE 2 P&S 70851

St. Luke's SL- 59· 3951

eXB.JllPle of that mysterious thymic lesion which is, by some pathologists including myself, considered an entity different from Hodgkin's disease.

Reference:

DIAGNOSIS: Hodgkin's Disease of Mediastinal and Cervical Lymph Nodes .

Lattes, R.: Thymoma and other Tumors of the Thymus . Cancer, November-December, 1962.

Dr. John R. McDonald, Harper Hospital, Detroit, Michigan:

DIAGNOSIS: Thymus, Hodgkin 's Disease Lymph Node, CerVical, Ifodgkin 's Disease

or. Lauren V. Ackerman, Barnes Hospital, St . . Louis, Missouri:

DIAGNOSIS: Thymus, Hodgkin 's Gra!).uloma Lymph Node,. Cervical, Hodgkin's Granuloma

Follow-up: In September, 1959, t he patient received 4,000 r. in a period of 4 weeks, to mediastinum. The course was uneventful until February of 1961, a year and a half later, when the node in the right supraclavicular region appeared as a recurrent disease. It is our custom to treat to a half tolerance dose. She received approximately 2,500 r. in ten days. Seen repeatedly at follow-up. On February 14, 1963, there is enlargement or the mediastinum. We instituted treatment on the 22nd or February·, and she has received 1,800 r. in the past week. She is asymptomatic.

•

c~ J. fq:ot.lc:u.un

CASE 3 SL-61-1933

Contributed by: William. D. Seybol d, M.D. and Carl J. Lind, Jr., M.D., St. Luke's Episcopal Hospital

This 66 year old, 'White, male 'Was first admitted in 196o, when

a cholecyst.ectomy was performed for lithiasis. Second admission - 1961,

foz- repair of ve)ltral hernia, following chol ecystectomy.

He had bee.n a heavy smoker sinc.e the 'age of 10, and had al'Ways

had a productive cough.

Routine "Pre-operative ches t x-ray revealed infiltration of t he

right lower lobe. ~ploratory. right thoracotomy was performed with right

• !ower and middle lobectomy.

Houston Society oi' Clinical Pathologists Seminar March 2, 1963

CASE 3 P&S 7o853

St. Luke's SL-61-1933

We are told that a right middle and lower lobe lobectomy was done. The over-all weight of the specimen was 470 grams and the over-all measure­ments 14 x 13 x 7 em. There was a large tumor involving the lung parenchyma and indurating the pleural surface. On sectioning, it is stated that there vas a tumor "derived from the bronchial lining." It wa.s also stated that from the bronchus it extended into the lung parenchyma and that the total diameter was 5 x 3 em. , and that it was partly necrotic. The tumor was located in the lower lobe. The pleural surface posteriorly was grossly involved by tumor and a portion of. the lei't atrium was resected with the lung. In addition, a statement is made that there was invasion of the chest vall.

Histological sections of this tumor show that it apparently consists of two different tissue types; namely, carcinoma which is predominantly squamous, and a neoplastic stroma which consists of poorly differentiated spindle- shaped cells arranged in bundles. The definitely epithelial portion shows solid cords and nests of cells which often show differentiation into squamous pavement type cells and occasional concentric keratinized pearls but also with occasional droplets of mu~nous material, as can be demonstrated with special stains. The stromal component is composed of elongated or irregularly shaped cells which, with trichrome stains, often show a strongly acidophilic cytoplasm and perhaps also intracellular fibrils which could well be myofibrils. Mitotic activity is fairly h.igh in these "sarcomatous" areas and foci of necrosis are seen here and there. From sections available to me it is not clear whether the tumor was polypoid or fungating in the bronchial lumen. However, it certainly involved the l amina propria of the mucosa of the bronchi which did not show any obvious squamous metaplasia or atypism. In one of the sections there is a granulomatous area associated with multinucleated giant cells and abundant anthracotic pigment. This could well be old healed tuberculous scar. Reti­culin stains are quite useful in this case because they show beautifully the two tissue types; name~~, the epithelial carcinomatous islands without any reticulin fibers, and the sarcomatous stroma with abundant reticulin between the tumor cells. Some hilar lymph nodes show anthracosis and old i'ibrosed granulomatous foci and one of them appears to be involved by the tumor 1 but probably by direct extension rather than by metastasis. A section of myo­cardium made available to me shows an area of :lnfiammation and pericardia! adhesions but no clear-cut evidence of neoplastic invasion. Throughout the lung parenchyma there are many obliterated branches of the pulmonary veins and arteries which are surrounded by tumor and probably also invaded by it.

We are obviously dealing here with that variant of malignant tumor of the l ung which goes under the name carcinosarcoma. Whether the sarcomatous port ion is purely fibroblastic or is in reality leiomyosarcomatous is hard to t o decide, especially since for reasons unknown to me many reactive connective tissue proliferat i ons in the lung result in a cell type whi ch can easily be !Dis taken for sD!Ooth muscle type .

Houston Society of Clinical Pathologists Seminar t.l.arch 2, 1963

CASE 3 P&S 70853

St . Luke's SL-61-1933

A good review of the problem of carcinosarcoma of the lung can be found in a paper published by Bergman and Acker man in Cancer in 1951. At that t ime only eight simil ar lung tumors had been recorded according to the authors. Two. new cases were .added in that article and both of them were histologically ver y similar to thi s current case. The authors of that ~ticle emphasi zed the relatively l ow malignancy of their cases , one of which was a long survival. This has oeen also our experience, especially including a spect acular case in which the patient is still alive ·e~en after the sarco­~~~ous portion of the tumor metastasized t o the small i ntestine ~hich was then resected with what was thought to be a palliative procedure several years ago.

DIAGNOSIS: Carcinosarcoma of Lung .

• Reference:

Ber gman, M., Ackerman, L., Keinler, R.: Carcinosarcoma of t he Lung. Cancer ·~: 919-929, 1951.

Dr . Lauren v. Acker man, Barnes Hospital, St. Louis, Missouri:

DIAGNOSIS: Lung, Right - Carcinosarcoma.

Follow-Up: This patient's initial surgery ·was April 26, 1961- a right lower lobectomy. The patient expired May 13, 1961. There were two l esions i n the cerebrum, just beneath the pi a a r achnoid, measuring 1 em. and 1 .5 em. which were basically squamous cell carcinoma. There was one lar ge mass in t he anterior abdomi nal wall , 3~ em. in transverse diamet er, whi ch was composed predominantly of fibrosarcoma . Ther e 1<1aS no other eVidence of metastasis. The patient had bilateral bronchopneumonia.

Ck3E 4. H & E

CASE 4 'l'OH s-62-936

Contributed by: Harvey S. Rosenberg , M.D. Texas Children 1 s Hospi t al

This 7~ year old, white, male developed asymptomatic swelling

of the l eft upper eyelid and a soft spongy swelling of t he lert temple.

For a period o f two weeks, the temple swelli ng increased in size to 3 em.

It ws smooth, firm, and easily outlined.

c:BC was reported as normal. Skin test for cat-scratCh disea se

and T.B. were negative. Within the next veek, the !!18SS enlarged and be-

cam$ more firm, and the second . mass appeared adjacent to it over the

left cheek.

X-rays revealed opacif icati on of the left maxillary antrum.

An operati on was performed.

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 4 P&S 7o854

Texas Children's S-62-936

This is a tumor involving the maxillary bone and the maxillary sinus in a 7~ year old boy.

Here we are up against the familiar problem of a differential diagnosis in poorly differentiated small cell malignant tumors in children. Microscopically, the findings can be summarized as follows: In the better preserved portions of the tumor the neoplastic cells show round or slightly oval shaped vesicular nuclei which have small nucleoli. The cytoplasm is generally poorly outlined. 1be tumor cells te.nd to 'be arranged in nests and in the centers of some of these nests there are ~ooser areas that resemble neuro-epithelial rosettes. These individual nests which have a somewhat alveolar pattern are separated from each other by fairly regularly arranged slender connective tissue trabeculae contain­ing thin-walled blood vessels . Apparently there is no stroma within the indi­Vidual cell clusters. One additional feature that might help in a correct evaluation of the case is that occasionally these neoplastic alveolar nests have Wldergone central necrosis and calcification. Here and there are granules of hemosiderin pigment but nowhere did I see pigment of the melanin type. In some instances t .he stroma is extremely vascular.

Summarizing the findings, as can be observed, with a routine hema­toxylin and eosin stain we had to consider the possibilities of Ewing's sarcoma, reticulum cell sarcoma, and metastatic neuroblastoma, while other possibilities are more remote. Against Ewing's sarcoma would 'be the unusual topographY and the epithelial-like arrangement of the tumor ce~ls in alveoli and nests. The latter features as well as the foci of calcification are more suggestive of a neuroblastoma. Whether or not the loose spaces seen occasionally are true rosettes is debatable. I think that reticulum cell sarcoma is much less likely, not only 'because of the histological findings, which in my opinion do not suggest this, 'but also because of the patient 's age, since IIDSt reticulum cell sarcomas of the bone have occurred in adult age.

•

Uouston Society of Clinical Pathologists Seminar March 2, 1963

CASE 4 P&.S 7o854

Texas Children's S-62-936

The most important histological feature is shown by the reticulin stains: there are no reticulin fibrils within the nests of tumor cells, but only in the trabeculae containing blood vessels. Th2s pattern is so definitely epithelial in nature that I think we are most likely dealing with a malignant neuro-epithelial tumor, probably a metastatic sympathicobla.stoma.

DIAGNOSIS: Neuroblastoma ( sympa.thicoblastoma) of ;ra.w, following sympathicobla.stoma of unknown origin. vs ,

Reference: • Jaffe, H.: Tumors and Tumorous Conditions of Bones and Joints.

Lea and Febriger, Philadelphia, 1958.

/

Dr. Harvey s. Rosenberg, Texas Children's Hospit~ Houston, Texas:

DIAGNOSIS: Ewing's Tullier '-'

Follow-Up: The patient received cytoxan 5 mgm/Kilo/day for two weeks, and then received 5,000 r., with cobalt therapy t o the ·maxilla. The patient is alive and well at the present time, with irradiation change in the maxilla.

CASE 5.

CASE 5 P&S 70496

Contributed by: Arthlll' Purdy Stout, M.D. and Rat"t"aele Lattes, M.D., Presbyterian Hospital

This 15 year o1d gir1 gives a short, 2-3 week history or a

subcutaneous nodule developing on the lateral. aspect of the lower right

thign in an i mmediate supracondyl ar position.

At examination the nodu1e vas subcutaneous and painl.ess. A

wide surgical excision disclosed a firm, dark-reddish-brown nodule whi.ch

"popped out" immediately after the skin incis i on was pert"ormed. The

surgeon states a mass was 1ocated anterior to the insertion of the biceps

femoris and attached to the fascia lata • ..

Houston Society of Clinical Pathologists Seminar J.Brch 2, 1963

CASE 5 P&.S 70496

The case is that of a 15 year old girl with a history of a sub­cutaneous nodule present only two to three weeks . Apparently it was attached to the fascia lata and was easi.ly shelled out after the skin was incised.

Histologically the tissue i s somewhat pleomorphic, in that it shows myxoid loose areas in which are a few fibroblastic t ype cells and little collagen separated by abundant mucinous ground substance and other denser areas in which again the cell t ype and intercellular subst ance are obviously fibrous but whi ch are also associated with considerable vascularity, occasional lllllltinucleated gi ant cells, and a sprinkling of inflanmatory cells . Sections taken at the periphery do not show e true capsule . In feet, the tumor seems to merge with 1..-,.e surrounding f i brous and :fatty tissue . Mitotic activity is minimal to moderate and one could perhaps describe the over-all findings as suggesting a t i ssue of r eparati ve type.

This case was originally seen by Dr. Stout, and I believe I should quote here the opinion which he wrote to the pathologist who submitted the l esion to him: "This t ulnor is indeed a pseudosarcomatous fasciitis. Since I have now accumulated some 200 cases I am quite familiar with its appearance . ibis one is somewhat larger and more cellular than many, but it i s so character­i stic that I have no hesitancy about the diagnosis . Tbe features of importance are a kind of myXOid appearance to the fibroblastic proliferation, an iilfil­t ration of inflammatory cells which is quite marked in this case, and a pro­liferation of' capillaries about the margins of the tumoromere it infiltrates fat . I t i s not surpx·ising to find it in a child since about 23 of our cases have been i n children, t he youngest being three mont hs old. In no instance have any of the tumors displayed any evidence of malignancy. If incompletely excised they may recur locally, but none has metastasized . "

This is a lesion which is very fashionable in these days. It is referred to as fasciitis, pseudosarcomatous fasciitis, subcutaneous pseudo­sarcomatous fasciitis , nodular fasciitis, prol iferative fasciitis, etc.

Houston Society o·:r Clinical Pathol ogists SeDlinar Maz:ch 2, 1963

CASE 5 P&S 70496

Basically, these terms refer to a tumor-like nodule generally occurring in the subcutaneous, generally growi ng fair ly rapidly ·and seldom reaching a large size. This nodule is probabil.y not a true tumor, but some kind of tumor-like· raparative pr ocess which is triggered by .unknown factor s . The most important point to keep in Dlind :!;s that certainly in the past many of these le-sions were wrongl'Y classified as either fibrosarcoma or lipo­s~coma, or myxol iposarcoma, etc. Conver sely, it i s my bpinion that we have 'now the ·opposite danger , namely, that in the enthusi asm for a new entity some true liposarcomas or fibrosarcomas may be wrongly classified with the benign label of pseudosarcomatous fasciitis. I have ·already seen a couple of instances of this .sort .

DIAGNOSIS: Pseudosarcomatous Fasc11tis, of Lower ·Extremity

References: Hutter, R. V. ,. et al.: Fasc11tis. Cancer 15: 992-10031 1962. Konwa:ler, B. E. , et al. : Subcutaneous P~eudosarcomatous

Fibr.omatosis (Fasc11tis), etc . Am. J. Clin. Path. '2~~ 241-252, 1955-

Price, E. B., et al.: NOdular Fasciitis, etc. Am. J. Clin. Path. 35: 122-136, 1961.

Stout, A. P.: Pseudosarcomatous Fasciitis in Children. Cancer 14: 1216-1222, 1961,

CA,SE 6 • P&S B-597L

.Contributed by: Arthur Purdy Stout, M.D. a:nd Raffaele Lattes 1 .M.D. , Pres=t;>yterian Hosp;i tal

This 18 y.e.a'X' old, white, male was admitted to the hospital with

a chie1' complaint of tarry s.tool - intermittently one week. PRE!SEN'l'

D:J,NESS~ _Excellent health most of his life, exc.ept for emotional problems

which were being treated by psycho-analysis ami psychic energizer pills .•

Three to four mont hs pr ior to admission he developed substernal burning

after meal s , relieved by antacids. One weeJt Prior to admission he com-

plained of moder.ate generalized discom:f'ort wit hout · nausea or vomit ing,

l~ting ·several hours. This was followed by pas.sage of tarry stool s which

persisted until the time of admission. On the day of admi'$sion he noted • marked dizziness and had a near s>~copal epi sode. PHYSI CAL EXAM!UATION:

Temperature was 99, pulse 112, blood pressure 110/50. A pallid young man1

·somewhat l ethargic with a soft non-tender abdomen and in no particular distres.s.

The stools were st ill dark black. LABORATORY DA'llA: Hemoglobin was 1>.8, hemato-

c~rit 14.5, white bl ood cel.ls 11,700 with 46 polys, 54 lymphs. Sedimel:itation

rate was ll. Stool gua:;iac 4+. Aci d was present in t he gastric aspirate.

Liver function test~ were negative. Prothrombin time was 15 seconds. The

pl atelets normal. SGOT and SGPT normal.

Chest x-ray negative. Gastroi nt est inal x-ray study showed a

filling defect on t he lesser curvat ure of the stomac)l .

OPERATION: S~mental resection of stomach.

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 6 B- 5971

The gross description of this specimen is that of a partial gastric resection. There was a tumor located within the gastric wall in the lesser curvature measuring 1.5 em. in greatest dimension. The overlying mucosa shows an 8 mm. linear ulcer. On sectioning, the tumor appeared somewhat yellowish and somewhat gelatinous. It was not well circumscribed so that one observer suggested the possibility of either a leiomyoma or ectopic pancreatic tissue.

We found some difficulty in the interpretation of this tumor. The grossly described linear ulceration is probably not a true ulcer. At least in the sections studied there is a slit lined by an intact row of mucous secreting cells. Deep to it there is a multilobulated fairly cellular tumor which is not c.ircumscribed but which everywhere interdigitates with the smooth muscle cells o:f\ the IIIU8cularis propria of the stomach. This tumor is made up of cells which are generally elongated and have a clear somewhat granular cytoplasm and oval shaped nuclei with blunted ends . In some places it is quite vascular and in other places it shows a myxoid gr01.md substance. Some of the vascular foci contain also collections of mononuclear inflammatory cells and apparently proliferating fibroblasts suggesting that perhaps an ulceration did occur and that inflammation and repair were superimposed on the pre-exist-ing lesion. Trichrome stains did not show satisfactorily myofibrils. We were then left with a choice between intramural leiomyoma in which inflammatory and reparative processes became superimposed due to ulceration, and one of the pseudoneoplastic granulomatous lesions of the gastric wall that go under vary-ing names such as eosinophilic granuloma, Vanek's tumor 1 etc. If one restudies this lesion with those alternate diagnoses in mind, one perhaps is more influenced by the reparative and inflammatory aspects of the lesion. There are also some scattered eosinophile besides the small and large mononuclear inflammatory cells .

Even though originally I was one of those who decided for the diagnosis of leiomyoma, I now believe that we are dealing essentially with an inflammatory and reparative pseudoneoplastic mass.

DIAGNOSIS: Inflammatory pseudo-tumor of stomach (Vanek's tumor) (Inflammatory fibroid pplyp of stomach)

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 6 B- 5971

References: Cohen, et al.: Inflammatory Fibroid Polyp (Hemangioper ±cytoma)

of Stomach. Am. J. Dig. Pi s. 4: 549- 555, 1959 Helwig, E. B. , et al.: Inflammatory Fibroid Polyps of the

St omach. S. G. and 0 . 96: 355-367, 1953 Vanek, J.: Gastric Submucosal Granuloma wi tli Eosinophilic

Infiltration. Am. J. Path. 25: 397- 411, 1949

Follow-Up: Patient last seen in November 1962, i.e. eight months· post­operatively. There were no gastric complaints; the chart made· no ·mention of t he stat~ of this patient's psychiatric condition (treated by a private psychiatri st} • •

CASE 7 P&S B-7668

Contribut ed by: .1\,rthur Purdy Stout, M.D. and Rai"fa:el.e La.ttes 1 M.D. , Presbyterian Hospital

This 77 yea:r olcl, vhite., male ~1e.s admitted to the hosJ?ital

i•!ay, l-9621 with a .. chi ef' complaint "ulcer" - i"i ve months durat ion. PRESEJIIT

ILLNESS : Seen in the outpatient clinic about April. , 1.96o. Symptoms of

mild epi gastric pain sin ce ea:rly 196o. The pain was postpra:nclial, nocturnal;

wi th some r elief f r om antacids. Blood pressure 218/105. G.I. Ser ies1 ,A l.arge

antral ulcer with a deformed bulb. Treated with medical regimep, apparently

r e sponded until September , 1961. He returned to the clinic with lllO:tre severe

epi g13.st r i c pai n, loss of appetite, nausea and vomiting. G.I. Series: The

antral ulcer •,,ras larger than the one seen pr e viously. PHYSICAL EXAMINATI ON:

• Temperat ure 98, pulse 84, respirations 18, blood pressure 194/120. Grade

II arterioscl er otic changes of the ~~da.l vessels. Chest: Clear. Heart~ NSR;

no murmurs . Abdomen: Tendernes s in the epigastrium. Liver , spl een and

kidneys we r e not palpab l e . Firm prostate gland. LABORATORY DATA: Blooci:­

Hemoglobin 12.2, ~IBC 9 ,600 with normal different ial. Urine: 1+ albumin and

occasi onal r ed blo0d cells per lower po•rer field. Stool gua:i ac ><as negative.

F'aati ng blood suga:r 91, BONt,l6, Total. protein 7. 6, albumin 4, globulin 3. 6.

The other l aboratory t ests were wit hin nor~l limits.

Chest films were normal.

OPERATION: .May 11, 1962 - A :partial gastrectomy performed.

-7-

Houston Society of Clinical Pathologists Seminar J~ch 2, 1963

CASE 7 B-7668

At the time of operation a large area of induration was palpated in the region of the antrum. Because of this, t he stomach was opened and biopsies were taken from an irregular ulcer. Frozen section study revealed them as benign. A subtotal gastrectoi!IY was subsequently performed. 'llle stomach specimen measured 9 em. in length in the lesser curvature and 22 em. along the greater curvature. A large shallow ulcer 10 em. in maximum diameter occupied the distal fundus and practically t he entire antrum. There was sligj'lt elevation and minimal induration of t he margins of the ulcer. In the base of the ulcer there were multi ple firm nodules, the largest of which was about 4 em in length . When these were secti oned, they showed no obvious neoplastic tissue but only an apparent diffUse thickening of the muscularis propria and of the submucosa. The lymph nodes in the greater and lesser omentum appeared enlarged but soft and edematous . The largest measuring about 1.7 i n len~th.

The microscopic findings are those of diffUse extreme thickening and fibrosis of the submucosa, ulceration of the mucosal surface, and diffUse and focal infiltration with lymphocytes, plasma cells, and some eosinophils t hroughout all the coats of the gastric wall . Lymphoi d follicles with germinal centers can be seen in the submucosa and also in the muscular coats. There are no striking vascular changes except for apparently non-specific medial and intimal thickening in some smaLl arteries and t here are areas of marked hyper­trophy of the neural elements of the IIIYenteric plexus not unlike what is seen i n many ulcerated lesions of stomach end intestine. Tne regional lynqL~ nodes showed marked follicular hyperplasia, but no granulomatous lesions nor any other obvious specific changes . Away from the ulcerated area, the gastric mucosa did not show the features of chronic atr ophic gastritis but presented the usual pattern or the i'undic and antral mucosa.

One interesting feature is that in some places away from the heaVily fibrosed areas, the muscularis of t he stomach showed foci of almost pure eosinophilic infiltration .

This case was introduced i n this seminar in order to present not only the difficult radiological problem of differentiating between ulcerated

-13-

Houston Society of Clinical P~thologists Seminar March 2, 1963

CASE 7 B-7668

carcinoma and atypical benign ulceration, but al:so in order to suggest that not aU benign ulcers of the' stomach have necessarily the same pathogenesis. While we do not know almost anything about the pathogenesill · of the standard tYPe of chronic penetrating peptic ulcer, this case certainly does not blllong to that group. Essentially we apparently have here a diffuse inflammatory and fibrosing process associated with microscopic areas which could qualify for the term eosinophilic granuloma and which is associated with an irregular ulcerating process . Whether the pathogenesis of this lesion was based on some hypersensitivity mechanism and also whether it is in any way analogous to the so-called eosinophilic granuloma of the stomach is purely ·a matter for specu­lation.

DIAGNOSIS:· Benign giant gastric ulcer with eosinophilic , infiltrat,on

References: Ruzic, J , P., et al.: Gastric Lesions of Loeffler's Syndrome.

J,A.M.A. '149: 534·537, 1952 . . Ureles, A. L., et al.: Idiopathic Eosinophilic Infiltration

of the Gastro-Intestinal Tract, Diffuse and Circumscribed. Am. J, Med. 30: 899-909, 1961

(Excellent review of bibliography)

Follow-Up: No history of allergy. Gastric symptoms go back for two years. A frozen section was made at the time of the operatiqn, and the ulcer was diagnosed benign.

Patient was last seen five months postoperatively: lie "feels great."

-14-

CASE 8 P&B B-5191

Cont r ibuted by: .Arthur Purdy Stout, M.D. and Raffaele Latt e·s , M. D., Presbyteri an Hospit al.

This 66 year· old , white, f emale was admitted t o the hos pital

February 12, 1962.. Her ·chief complaint ~ "lump on t he ch~st'' - f our months .

This l ump i s asymptomatic.. It has not changes i n s i ze or s hape . Ph'YSICAL

EXAMINATION: Negative, except f or t he f,ixed mass about 6 em. i n diamet er

over t h e right fifth and s iXth r ibs anter i or l y . LABORATORY DATA: Normal.

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 8 B-5191

The operation performed on t his 66 year old woma.~ consisted of an extensive resection of the chest wall. The total weight of the specimen was 620 grams . It consist ed of a portion of the cheat wall including segments of 6th, 7th, 8th and 9th ribs, and it included a tumor mass measuring 11 x 7 x 12 em. The tumor mass was found to extend from the parietal pleura to about 2 em. from the epidermal surface . On the pleural aspect there were several nodular elevations . Except for the 6th rib that seemed to be free of tumor, the other ribs were found to disappear into the main body of the tumor which was firo, yellowish-white and gritty on section. Superficially, the tumor appeared to infiltrate the skeletal muscle of the a~terior and lateral chest wall.

111croscopically, this is an anaplastic poorly dirferentiated tumor composed of cells with poorly outlined cytoplasm and with pl eomorphic nuclei which vary in s~ze and shape from elongated to rounded, and which is generally associated with abundant ground substance . The ground substance in most places appears to be fibrillar of the collagen type but here and there becomes more abundant and homogeneous and strongly eosinophilic, and the tumor cells are scattered in it in a way somewhat similar to that seen in osteoid . There is also a tendency of this ground substance to be arranged in trabecular fashion . Here and there are multinucleated giant cells of the osteoclastic type . ~mnants of costal cartilage and of costal bone are seen in many of the sections taken and can be easily differentiated from the neoplastic tissue. No neoplastic cart i lage was seen in any of the many secti ons prepared. Only occasionally the tumor cells are bizarre and multinucleated . At the peri­phery the t umor is seen to infiltrate the intercostal spaces and to surround the intercostal nerves.

This is an osteogenic sarcoma -which i s partly undifferentiated and partly of the so- called osteoblastic variety. The site of origin is quite unusual. In the book on Neoplasms of Bone by Dr. Bradley Coley, osteogenic sarcomas of the rib were observed 42 t i mes out of 985 cases. In the files of our Laboratory df Surgical Pathology there are only three cases classi ­fied as osteogeni c sarcoma of the rib , and only one of these , namely the case under discuss i on, occurred in a Presbyterian hospital patient. The

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 8 B-5191

other two were consultation cases submitted from outside sources. This includes all bone tumors seen by us for the past 50 years. We are then dealing with a fairly rare site for osteogenic sarcoma.

Reference:

Follow-up:

DIAGNOSIS: Osteogenic sarcoma of rib

Coley, B. L.: Neoplasms of Bone, Second Edition. Paul B. Roeber, New York 1 196o.

No postoperative radiation or other treatment given.

Patient was O.K. one year postoperatively.

CASZ 9· .Antoni type A CASE 9 . .Antoni t}'P" ll

..

CASE 9 P&S B-9666

Contributed bJ': Arthur Purdy Stout, M. D. and Raffaele I.attes, M .. D., Presbyteri an Hospital

Thi s 47 year old, white, female was admitted to the hospital,

JUly, 1962. PRESENT ILLNESS: On r outine physical examination, an abdominal

mass was discovered three weeks prior to her admission. She had no bistocy

of change in b owel habit·s , no melena , no hematemesis. There were no urinary

symptoiDB, no pain, no weight loss . PAST HISTORY: Unremarkable, except for

an automobil e accident in 1937, at which time she had a blunt abdominal

trauma. PHYSI CAL EXAMINATION: The onl.y s i gnificant finding was a f irm,

non- tender, smooth mass judged to be about 10 em. in ma.x:i.e1um dimension and

situated in retroperitoneal position in the right iliac fossa. LABORATORY

DATA: Normal.

X- rays : G. I . Series.

An operation "as performed.

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 9 B-9666

This case was of interest not because of any difficulty in hist­ological diagnos is, but because of the unexpected nature of this clinically asymptomatic retroperitoneal mass . A number of possibili ties were considered pre-operatively but no definite decision was made and, for this reason, t he patient was explored . The surgeon described the findings as follows : The mass proved to be encapsulated, somewhat firt:1 and rubbery in consistency and was s ituated in the retroperitoneal. area in the upper reaches of the right iliac fossa, just lateral to the psoas muscle . It measured 8 x 6 x 5 em. It had a smooth capsule and t he gross specimen was described as ovoid, some­what cystic, measuring 6. 5 x 5 em. On sectioning, it showed areas of cystic appearance conta ining a sero- sanguineous fluid . The tissue was otherwise gelatinous and yellowish with hemorrhagic areas . Grossly, a connection with the fourth lumbar nerve was noted. The surgeon stated t hat he had to tran­sect a nerve branch in order to f ree the mass .

Microscopically, this tumor was found t o contain medium and small sized nerve trunks in its capsule . The tumor i tself showed the typical. architecture of the neuri l emmoma, namely areas of Antoni A and Antoni B type wi th the former showing whorls of Schwann cells with palisading and the latter showing myxoid and cystic pattern. There is a sprinkling of lympho­cytes and there are numerous blood vessels surrounded by a hyalin cu:ff which is also characteristic of this lesion . Reticulin stains showed what is the usual pattern of the reticulin fibrils in these tumors; namely, that in the individual bundles of Schwenn cells . The predominant direction of the reti­culin fibrils i s parallel to the long axis of t he bundles. 01\e l ast comment has to do with t he presence of lipid i n 1h ese t umors . Exactly where the l ipid comes from is not too clear . However, not infrequently apparently non­degenerated tumor cells contain lipid drople~s and this , of course, does not mean that we are dealing with lipoblastic tumor . The lesion is t o be considered benign.

Reference :

DIAGNOSIS: Neurilemmoma of retroperitoneal area

Ackerman, L. V.: Tumors of Retriperitoneum, Mesentery, and Peritoneum. Atlas of Tumor Pathology, Section VI, Fascicles 23 and 24, · AFIP, 1954.

Fol low-Up: Pati ent was O,K. six months postoperatively.

CASE 10. Low power

CASE 10 P&S A-47430

Contributed by: Arthur PIL"<'iy Stout, ~!.D . and Raffaele Lattes, M.D. , Pres'byteria;p Hospital

Thi s patient, a 54 year old, white male , was admitted to the

hospital April, 1955 · His chief complaint was black tar:ry stools of :four

days duration. PRESENT ILLNESS: 'llhe patient was well until four days

prior to admission, when without warning be had a 'black stool. Eight

hours later, had another similar episode. His physician hospitalized him.

Tl;enty hours later~, he had another episode of melena. He never had hema-

temesis and was never Jaundiced . PHYSICAL EXAMINATION: Essentially

negative, including abdomen . LABORA'roRY DATA: Hemoglobin 11.8 gms .,

\IBC 10 ,6oo.

X-rays: Two gastrointestinal series were made.

A total gastrectomy was performed on Apr:Ll 21, 1955

Houston Society of Clinical Pathologists Seminar Milrch 2, 1963

CASE 10 A-47430

As you have seen, the gastrointestinal radiological studies showed multiple filling defects in the stomach. The impression of t he radiologist at that time was in favor of a fungating or polypoid carcinoma. The patient vas operated upon in April, 1955 . The surgeon descri bed the stomach as being WlUSually large. The pars media and the lesser curvature presented a "big tumor" bulging into the lumen of the stomach and extending upward close to the esophageal-gastric junction. The surgeon also described a few enlarged lymph nodes and decided to do a total gastrectomy. The gross specimen was a stomach measuring 21 em. along the lesser curvatUre and 32 em. along the greater curvature. The specimen was bulky but otherwise, externally, it had a normal appearance. When the stomach was opened, the over-all architecture appeared to be maintai ned, but the rugal folds were greatly thickened, measuring up to l. 5 em. in width and presenting a cobblestone surface pattern. The thick­ness of the gastric wall and the fundus was up to 2 . 5 em. In the lesser curva­ture there were two supert'icial ulcers, one 2 em. in length and • 3 em. in width, and the other about .5 em. in greatest dimension.

This i s a good example of the true diffuse giant rugal hypertrophy of the stomach, the so- called Men~trier's disease . 1-lenetrier did in fact describe two different gastric lesions which now go under the terms Ml!netrie.r ' s disease # 1 and Men~trier's disease # 2 . They were described in a paper written in the year 1888, only one of t he two lesions bearing on the present case. The other l esion is that of a diffuse polyposis of the stomach.

Microscopically, our case shows big gastric folds including actual increase in thickness and in number of glands of the mucous membrane with occasional cyst formation, increase in thi ckness of the muscularis mucosae and apparent edema of the submucosa. Here and there small extensions of these cystic glands could be seen in the submucosa. Also here and there superficial erosions of the mucosa could be easily identified. Studying these sections one gains the impression that there is an actual increase in bulk of the acid secreting portions of the gastric mucosa . Of course these things are very hard to evaluate. At any rate, most of the true hypertrophy i nvolved the fundic and midgastric portion rather than the antral portion of the stomach. Only one of the ulcers had the histological appearance of a true chronic peptic ulcer extending down through the submucosa into the fibrosed muscularis .

This lesion has been enjoying quite ~ bit of popularity recently since it was discovered that most of these patients have also profound hypo-

Houston Society of Clinical Pa t hologists Seminar ~!B.rch 2, 1963

CASE 10 A-47430

:Qroteinemia. I bel ieve it has been proven beyond. doubt that the prote i n loss occurs through the gastric secretions which are high in proteins . In this patient , a protein ser um determination done pre-operatively showed tot al protei n 5.2 (L21'G/2-9 A).

DIAGNOSIS: Giant r ugal hypertrophy ( l~netrier's disease) with pepUc ulcer

References: Butz, H. C.: Giant Hypertrophic Gast ritis. Gastroenterology-39:

183-190, 1960. Cit rin, Y., e t a l.: The 1-techanism of Hypoproteinemia Associated

with Giant Hypertrophy of the Gastric Mucosa. New Eng. J. Med. 257: 906-912, 1957.

Kenney, F. n:, e t a l. : Giant Hypertrophy o f Gastric Mucosa. Cancer 7: 671- 681 , 1954 .

Wm~trier, P. : Des Poly-Adenomes Gastriques et Leur Rapports Avec l.e Cancer de l'Estomac. Arch. de Physiol. Nor m. et Path. 1: 32-55; 236-262, 1888.

Follow-Up: Patient probably has esophageal varices and no liver cir rhosis.

Alive seven years postoperatively.

CASE ll . Lymph node

•

r CASE 11 P&S B-9466

Contri buted by : Arth ur Purdy Stout , t4.D. and Raff aele" Latt es, !If. D. , Presbyterian Hospi tal

Thi s pati ent, a 58 year ol d, whit e f emale, wa s admit ted t o t he

hospital J uly, 1962 . Her chi ef complaint on admiss ion wa,s having vomit ed abotti;

three-quart ers o f a cup of bl ood, and having 11assed, t hree ta:rrY" s t eo,ls. The

past h i s t ory wa s not r emarkabl e. PHYSI C,AL EXA!(!JjNrJ:ON: Temperature normal,.

blood pressure 130/ 8o, pul se normal, An elde rly ap:pearing , pale l ady

with no appar ent distress . Ther e 'Wa s a 3 em. movabJ.e lymph node i n r i ght

axilla · The abdomen was not tende r and t her e wer e n o palpable masse s .

The l i v:er, spleen and kidneys wer e not pal pable. LA:BORATORY DATA: Hemo-

gl ob in was 7 .4 gms. , hematocrit was 22. 3; di f f erential count was normal.

Sedimentation rate was 63 mm. , t ot a l prote i n 6. 5, 3 .2 gms . al bumin and

1.3 gms . globulln . HOSPITAL COURSE: Since the hemoglob in :failed t o r i se ,

t wo uni ts of blo"od were g iven the day of admiss i on. Two days after admis:;fi on,

t he patient had anot ?;: -r ep isode of hemat emesis , followed b-y tarry stools .

On. July 12t h, after haying r e cei v ed 5 uni t s o f blood, t"he pati ent was treated

wi,tll gastric hypothermia (balloon) , Improvement in bot h hemoglobin and hemato-

crit f a iled to materialize.

X- ray: Gastroint estinal B"eri e s were perf ormed, but the f ilms

are not available.

A s ubtotal gastrectomy was done .

Houston Society of Clinical fatl;lo-logists Seminar March 2, 1963

CASE ll B-9466

The subtotal gastrect0my specj,men measured 14 em. in length. There were no gross serosal lesio!ls det~cta'!:>le', •Wl;len th!,! sto~ch was opened, the mucosal surface presented the usual rugal pattern. with ;f'ocal areas of' hyper­emia. No gross ulcers were seen. An ulct;!r, ho'lle_ver, was resected eeparately from the region of the- pylorus. The specimen was 2 em. in maximum diameter but was otherwise not b~tter described by the patholo-gist, except that he could not see any definitive muco~a.

Microscopically,. :tP.e so-called ulcer showed. onl.y what appeared to be a mass of moderateiy inflamed scar ·tissue. T!le ~expected findings consisted of the presence of mUltiple tubercle-like or sarcoid-like granulomatous foci in the mucous membrane of the sto~ch, as wel l as i n some· of the regional +ymph nodes from the lesser omentum. The les-ion was in fact much more conspicuous in the lymph nqdes than in tne gas.tric muc.osa. Casee.~ion was not observed and all bacterial ~tains were n~gative.

The findings in the stomacb ·were Unexpected. There was evidence of a healing ulceration of the pyloric duodenal region and probably the symptoms of gastric bleedlng originated there. The. un~xpected findings consisted of multiple microscopic granulomata ~n the mucosa and in the -regional !VmPh nodes. Microscopically -they ·are consistent with the lesiE!Ds seen in Boeck's ~arcoid. This patient, however, has no known systemic sarcoidosis. There are a few reports in the l i terature of so.cal1ed sarcoidosis of the stomach, and references to ·them will be appended below. Some of these cases !lppa.rently were mistaken clinically and radiologically for carcinoma of the linitis plastica type·. This diagnosis was not considered in the case under discussion now. Other similar cases reported in the literature present.ed with symptoms of profuse gastric hem9rxhage . O'ne could also commen.t that granulomata of this type are found in regional enteritis and that re_gione.l ent'eriti·s-like lesions of stomach and duodenum have occasionally been descri~ed. Since we do not know what is -the cause of regional enteritis, it is useless to argue here whether the lesions are, or are not, 'the same as those se,en in regional enteritis,

Houston Soc iety of Clinical Pathologists Seminar March 2 , 1963

DIAGNOSIS : Granuloma of stomach and of superior gastric lymph node (sarcoid type)

References:

CASE 11 B-9466

Comfort, 1-1. ~/. , et al.: Non- Specific Granulomatous Inflammation of Stomach and Duodenum: I ts Relation to Regional Enteritis. Am. J. ~led. Sc . 220: 616-632, 1950.

McKusick, V. A.: Boeck' e Sarcoid of Stomach with Comments on Etiology of Regi onal Enteritis. Gastroenterology 23: 103-113, 1953-

Palmer, E. D.: Note on Silent Sarcoidosis of Gastric Mucosa. Journal Lab. Med . 52: 231-234, 1958 .

Scott, N. 1-1., et al . : Sarcoid and Sarcoid-Like Granulomas of t he St omach . Arch . Int . Med. 92: 741-749, 1953.

Follow- Uo: P~tient needed several blood transfusions before and during surgery. Following this, homologous serum jaundice developed, from which patient recovered uneventfully.

Kveim teat was done, and read as negative. (pr e -operatively, the patient had received large doses of

aspirin and butazolidin for "leg pains '.') Total proteins were 6 .3 (Alb. 3.1; Glob . 3 )

CASE 12.

CASE 1.2 P&S A-39647

Contribt~ted by: Arthur Purdy Stout ,, M. D. and Raffaele Lattes , M. D. , Presbyteri an !los pi t al

~is 43 year old, white, female was admitted to the hospital

February, 1954 . Her chief complaint was dyspnea and pain in the right chest

which bad been present for at l east 18 years . PRESENT n.LNESS: A vague

history of l eft anterior chest pain first noted 18 years sec 1 occurring

initially with a so-called pleurisy of one week' s duration . The pain was of

rather mild degree and wa s described as dull , aching, and not localized, but

a;ggra.ve:ted by long hours of work and relieved by rest. The dyspnea is ex:ert-

ional and has progressed to a point where she is unable to walk one block or

climb one :flight of stairs without stopping . PAST HISTORY: Non- contributory .

X-ray 1 two months prior to admission, was interpreted as e!IIPhysematous bullae

in the left lung with a marked m...odiastina.l shift to the right am a moderately

severe dorsal kyphos is. PHYSICAL EXAMINATION: Temperature 100 , pulse 86 and

regular, blood pressure 145/75, respirations 20. 'Ihe trachea in the neck

' deviated to th~ right . Chest: ~ne left s i de appeared more prominent than

the right side, ' with decreased respi ratory excursions on the left. Breath

sounds were markedly diminished on the left with decrease in tactile fremitus

on the left, and i mmobility of the diaphragm on the l eft, where there was

tympany to percussion . The right side seemed to be within normal limits .

LAllORATORY !>ATA: CBC and urinalys i s were normal. Pulmonary function studies :

Ilorma.l values for ventilation, arterial oxygen saturation, and C02 tension a t

rest "!Jld following exercise. Respiratory breath space was within normal limits,

as was the index of intra-pulmoi\ary miXing. The residual. volume was normal.

Total capacity was reduced to 6o percent. Vital capacity wa.s 55 percent. Maxi-

mum breathing capacity was 70 percent. The right lung function was presumably

good.

A left pneumonectomy was done on February 2, 1954.

- 12-

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 12 A-39647

The left pneumonectomy specimen showed that there were multi l ocular cystic changes which were confined to a greatly enlarged upper lobe, while the l ower lobe could be seen as a compressed and atrophic structure apparent ly not involved by the pathology to be described below. There was an area of the lung measuring about 21 em. from the apex and 16 em. in medial to lateral di mension, which appeared to be a multiloculated cyst. The bronchi appeared t o be distorted but not definitely narrowed by this lesion. The outer surface of the lung was smooth and varied from a light pink to black in color. The compressed lower lobe was light pink and showed crepitation on palpation. On s,ectioning, the cystic area mentioned above was found to be divided in count­less smal ler cystic compartments separated by t hin transparent s epta which contained small, yellow, hard nodules. Air was apparently contained in many of the small er cysts, although it was not easy to demonstrate communication between the bronchi al tree and the cystic cavities.

Microscopic study of this exceptionally unusual specimen shows changes which one cou1d summarize by defining them as cystic disease of lung associated with multiple hamartomas of lung. In fact, there are innumerable cystic spaces lined by either flattened or low col umnar epithelium in which cilia are seldom recognizable. In the septa and walls of these cystic areas, there are innumerable nodules of well differentiated hyaline cartilage which sometimes has undergone calci fication . In addition to the cartilage , there are l obules of adult fat tissue and a l oose, myxoid stroma resembling undifferentiated mesenchyma. Smooth muscle is seen in t he trichrome stain as i nconspicuous small bundles. Normal lung parenchyma is not seen i n the area with multicystic changes and multiple hamartomata, but elsewhere, that is in the left lower lobe, the lung parenchyma is apparently normal except it appear s somewhat compressed. Thi s case i s unique i n our experience . It was already used once in 1954, as one of the cases discussed by Dr. Stout 1n the yearly meeting of t he Arthur Purdy Stout Society. At that t ime, Dr. Stout suggested a diagnosis of 90ngenital cystic disease of the left upper lobe of l ung and hamart omatosis of the l eft upper l ung. I was also curious to l earn "what Dr. Liebow 's interpretation would be and I have sent him some sections of t his case and he too said that the case was unique in his experience . He accepted our i nterpretation that t he lesion had a congenital

- 22-

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 12 A-396~7

basis, but was not entirely happy about it. He referred me to the case i llustrated in his fascicle of Tumor s of the Lower Respiratory Tract, plate 3-B and figures 166-172, which illustrate a case of osteochondro­matosis which has some resemblance to the case i n question except that it was not associated with multiple cysts. The only other possible reference to a similar lesion is a paper published in the Journal of Thoracic Surger y, Vol. 31, page 504, in 1956, with the title "Massive Cys.tic Hamartoma," by Jackson, et al.

DIAGNOSIS: 1) Hamartomatosis of lung

2) Cystic disease of lung, with hamartomatosis

References : Jackson, R. c. , et al.: Massive Cystic Pulmonary Hamartoma.

Journal Thor. Surg. 31: 504-510, 1956. Liebow~ A. A.: TUmors of the Lower Respi ratory Tract. Atlas

of Tumor Pathology, Section V, Fascicl e 17, AFIP, 1952.

Follow-Up: Patient is l iving and well, nine years postoperatively.

C~S~ 13 . Reticulin

CA;:!E 13 l.fi>NI s-62-9655

Centributed by: Wi l l iam o. Russell, M.D. , ~. D. Anderson Hospital & Tumor Institute

T'ni s 63 year old, whi t e housewi fe wa s admitted .complaining

of a lU!liJ! in the right breas t Whi cll had b e en pre s ent f or 12 months . The

mass was .about 4 ·cm. in diameter, firm, s harply marginated, and attached

to the skin and t .o the pectoral fascia. The ni pple ;,a s · not devi at ed or

retracted. A few small, soft, disc r et e n odes were palpable· iii the

axilla. History and phys ical examination were otherlli se not r evealing.

X- r ay : Mammogra m.

Houston Society o~ Clinical Pathologists Seminar March 2, 1963

CASE 13 P&S 72195

M. D. Anderson's S-62-9655

This is a lesion o~ the breast which was pre-operatively studied vith mammography.

I do not want to enter into a discussion on the diagnostic value of mammography since I have practically no personal eXperience . I will, therefore, limit myself to describing and interpreting the microscopic findings . These show a highly ceilular tumor composed essentially of spindle­shaped cells arranged in bundles which take interlacing pattern. These cells vary considerably in size . Their nuclei are often irregularly shaped out tend to be oval. Mitotic activity varies in the different f ields out in some places it is high. Trichrome stains show no obvious myof ibrils and a fairly diffuse amount of pale staining collagenous material. Reticulum stains show abundant reticulin between the tumor cells arranged in bundles with a tendency to be parallel to the direction of the bundle. In addition, they shqw better than any other stain t hat this is not a purely "sarcomatous" tumor but that it is in fact a tumor i n which there are epithelial elements represented by cuboidal, columnar, and flattened cells vhich line gland- like spaces and slits. In rare areas, the ground substance of this tumor has a vaguely osteoid pattern, and areas of myxoid appearance are seen here and there. It is hard to judge the method of growth of the tumor which was grossly described as well circumscribed. There is, ho~ever, some evidence t hat i t is not a perfectly encapsulated tumor since it appears to infiltrate the surrounding fat .

The diagnosis seems to me fairly obvious, namely we are dealing here with a lesion belonging in the group of cystosarcoma phylloides . The problem in such instances i s always to evaluate t he probable cl i nical behavior. We are told here that the lesion was attached to the skin and to the pectoral fascia . Microscopically, the lesion is not definitely encapsulated even though it obvi ously grew more by expansion than by infiltration. Mitotic activity is moderately high and there is an absolute predominance of the sarcomatous com­ponent . In spite of all of these features, it is possible that thi s lesion will behave as a local non-malignant problem. In our experience there are no reliable histological criteria to predict the clinical behavior of a cysto­sarcoma phylloides. Among the rare metastasizing cases that we have seen, there was one which kil led the patient i n a fev months with multiple hemato­genous metastases, and which histologically had been considered as definitely benign by all those who had seen the sections.

Houston Society of Clinical Pathologists Seminar March 2, 1963

CASE 13 P&S 72195

M. D. Anderson's S-62-9655

Because of this difficulty in predi cting the outcome of any individ­ual case of cystosarcoma phylloides and because of the fact that the greatest majority of them, as stated above, are essentially local problems without any tendency to behave as malignant lesions, it has been our practice to discourage any radical approach unless there was obvious evidence of a local aggressive behavior such as infiltration of t he pectoralis muscle (which, incidentally, I have seen only once).

DIAGNOSIS: Cystosarcoma phylloides (?malignant), of female mammary gland

References : Jackson, A. v.: Metastasizing Liposarcoma of the Breast Arising

in a Fibroadenoma. J ournal Path. Bact. 83: 582-584, 1962. Lester, J , and Stout, A. P.: Cystosarcoma Phyllodes. Cancer 7:

335-353, 1954. Treves, N. , et al.: Cystosarcoma Phyllodes of the Breast:

A l·lalignant and a Benign Tumor, etc. Cancer 4: 1286-1332, 1951.

Follow-Up: The patient was seen last week with no evidence of local recurrence or distant metastases . A chest x-ray was negative . The diagnosis was made on frozen section. The patient had a simple and not a radical mastectomy.

The patient is alive and well 6 months postoperatively .

CAS! l~. Voo Kooaa CASE lll . H & P. I I CASE 14. H & E (high)

CASE 14 P&S A-90430

Contributed b)': Arthur Purdy Stout, M.D. and Rafi'aele Lattes, M.D., Presbyterian Hospital

This 12 year old, '<lhite, 1'enale was admitted to the hospital 1n

1Jecember, 1959· PHYSICAL EXAMINATION: General appearance : A very thin

woman of 80 pounds, '<~hO shows e vidence of weight loss and dehydration. The

abdomen was tender on the right s i de with rebound tenderness r eferred to the

right . There was a large bulging in t he abdominal wall on the ri@llt side

which was quite tender. There was a ques tionable mass palpable in R. L.Q.

and R.u. Q. Liver, spl~n, and kidneys are not palpable. PR&3ENT TI.J..NESS :

Admitted December 1959, with cr ampy abdominal pain apparently due to obstruction .

An exploratory laparotomy was performed and a large right- s ided mass in the

in the right upper quadr~t was found. This pr~ed to be an abscess with no

obvious perforation of the bowel. A cardiac arrest occurred with a success-

ful cardiac massage. Post-operative pneumonda due to Staphylococcus aureus.

In March 196o, the pat i ent was admitted for a recurrent intraperitoneal abscess.

This was drained and adhesions of the small intestine were lysed. Following

di schar ge, the wound c ontinued to drain small amounts of dark materia l. She

experienced bouts o f diarrhea ·and abdominal pain, with fever . PAST HISTORY:

Hospital admissions : Urinary stones and hematuria - 1941>, Bheumatoi d

erthriti s - 1952. Thymoma removed 1952. Subtotal ~strectomy 1951 i'or

chronic gastri c ulcer and obstructi on. 1·956 - Marginal ulce r and. bronchial

asthma. 1955-1959 - Bronchial asthma, several times. LAllORA'IDRY DATA:

Urine: 1+ albumin. Blood: Hemoglobin 9.8 gms.; hematocrit 33. 5; WBC 11,750.

Sedimentation r ate 61 ll!lll· BUN 29 mgm<f.. , SOOT 16.

X-ray: Flat plate of abdomen and G. I . Series.

A laparotomy was performed September 8, 196o.

-14-

.. i

Houston Society or Clinical Pathologists Seminar March 2, 1963

CASE 14 A-90430

This one of the most unusual and confusing cases that we have had t o cope with in a long time. Pre-operatively it was f elt t hat the mass felt in the right abdominal quadrant could well be of carcinomatous nature. Some of the barium studies suggested the presence of an intrinsic mass in the lumen of the colon end another study done a rew days later seemed to suggest an intussusception of the cecum and terminal ileum into the ascend-ing colon . When the patient was operated upon there were dense adhesions of the terminal ileum to the abdominal wall and to a "tumor mass" involving the wall of the cecum and ascending colon . Other intraperitoneal structures, including the duodenum, wer e adher ent to this mass . Post eri or to the main tumor ~Ass, the retroperi tonea l and periaortic areas showed what was i nterpreted to be invasion by tumor. It was decided to do an ileocol ectomy, which re­sulted in a specimen of terminal ileum, cecum, and ascending colon i nclud-ing a mass of adherent omentum attached to the medial wall of the cecum and of ascendi ng colon and mesocolon. There was a f i st-sized firm mass i n the wal l of the cecum at i ts point of at tachment to t he ileocol ic mesentery. There were polyps identi f ied within the lumen of the cecum. One of them measured 1 .2 x 1 em. in t he upper portion of the cecum. Another was a sessile mass measuring 3. 4 x 1 .6 x 2.8 em., and was found in t he poster ior wall of the cecum. Another one measured 1 .4 x 2 .2 x 2 . 5 em. The surfaces of these polyps were granular and reddish-tan and apparently covered with necrotic material. On sectioning, they showed a dull white-gray tissue flecked with yellowish areas . An important point is that these polypoid projections were continuous w:!th extraluminal masses situated outside of the cecum and extend­ing into retroperitoneal fat . These resulted in a lobulated tumor mass 10 x 8 x 15 em.

The microscopic findi ngs were unexpected and most puzzling. The t umor-like masses described as protruding in polypoi d manner in the lumen of t he ascending colon as well as the tumor-like masses i nvol ving the retro­peritoneal area are similar i n type . Essentially, they appear to be of granulomatous nature cont aini ng enormous numbers of cells of histiocytic type which in turn contain intracytoplasmic inclusions . These inclusions ar e r ound, approximately the size of a small lymphocyte , but vary somewhat in size. They are strongly basophilic and they appear to be made of concentric layers . Special stains show that these bodies are faintly mucicarm1noph111c,

-26-

. , .. Houston Society of Clinical Pathologists Seminar t-!.arch 2, 1963

CASE 14 A-90430

strongly positive with the calcium stain of Von Kosse , strongly PAS positive even after diast ase digestion, positive with Prussian blue stain for iron, and negative with Gomori silver methenamine stain.

At first we thought that we were looking at micro-organisms but apparently it is impossi ble for micro-organisms to show this positive calcium and iron stain . There is a striking resemblance or rather identity between the inclusion bodies observed in this case and those that can be seen in the so-celled malakoplakia of the bladder (Michaelis -Guttman bodies). This latter disease is also a mysterious condition in spite of a good number of published cases . We are still at a loss in the interpretation of these find ­ings, especially since there is no analogous or similar case that we could find in the literature as having involved the colon . This case was studied extensively by Dr . Jacob Terner with histochemical techni que . Dr. Terner agrees that these inclusi on bodies are not micr o-or ganisms but could well be induced by an infectious agent . A paper is being pr epared by Dr . Terner and will probably be publ ished i n the near future .

Reference :

DIAGNOSIS: Granuloma, multiple, pseudoneoplastic, of colon and retroperitoneum with inclusion bodies of un­determined origin (analogous to malakoplakia of bladder)

Melicow, M. M.: Malacoplakia; report of a case, review of the literature. J ournal Urol. 78: 33-40, 1957 .

Fol low-UP: Patient died t wo days postoperatively, possibly due to coronary disease . No autopsy was obtained.

Pre-operative diagnosis was carcinoma of cecum with intussusception .

-27-