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PATHOLOGISTS' CLUB OF NEW YORK

DATE:

PLACE:

HOST:

Meeting

Thursday- November 7, 1996

The Long Island Coll ege Hospita l 357 Ricks Street Brookl yn, N. Y. 11201

./ { ~ Joh n K . Li , M. D.

INFORMATION: Mrs . Myrta Ramos ( 718 ) 780-1005

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Rece ption and Di nner : 5:15 - 7 :00 p.m. Othmer Conference Room B

Scientif i c Session: 7 : 00 - 9:00p . m. Othmer ~onference Room A

Directions :

P-arki ng :

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By car and subway: See map on reverse.

Parking i s available i n Pier 7 on Columbia Stree t. You may park after identifying yourself as a member or guest of the Pathologists ' Club.

After parking, wait for the s huttle van t o t a ke y.ou to the hospital, or walk the 3 short b locks to the Hicks Street entr ance .

NEXT MEETING

December 5 at Wi nthr op University Hosp ital.

DIRECliONS TO THE LONG ISLAND COLLEGE HOSPITAL:

.Qs!: Brooklyn-Queens Expressway to Exit 27 Atlantic Avenue. Parking available at Pier Seven, entered from Columbia Street, opposite BQE Exit 27.

There will be a hospital shuttle van at Pier Seven to take you the 3 short blocks to the hospital. Enter the hospital through the main entrance on Hicks Street. The meeting w ill be in the Othmer Conference Center.

Subway: F & G t rains to Bergen Street (corner of Smith Street), 5 blocks away; or A and C trains to Jay Street-Borough Hall, transfer to F across the platform, ride one stop to Bergen Street. 2, 3, and 5 trains to Borough Hall, R and M t rains to Court Street, 8 blocks away.

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PATHOLOGISTS' CLUB M EETING

The Long Island College Hospital November 7, 1996

CASE HISTORIES:

Case #1 595·9919 This 10 year-old girl initially developed cholestasis in 1992. at the age of 6 , with total bilirubin of 3.4, direct bilirubin 3.2. At that time, ANA was positive, as well as antibody to smooth muscle.

There is a past medical history of congenital heart disease (AV canal defect) that was treated by pulmonary artery banding. At this time, the patient has intermittent jaundice and elevations of LFTS:

SGOT · 300 Bilirubin • normal GGT • 230

Ceruloplasmin · normal ALP · 670 Alpha. AT - normal Viral Hepatitis markers- all negative

CT shows multiple discrete nodules occupying most of the liver. Open biopsies of liver were performed.

Case #2 596·3165

Invited discussant: Elsa Valderrama. M.D. long Island Jewish Medical Center

Host discussant: Richard Alexis, M.D.

This 36 year -old Black woman presented with a vague complaint of abdominal pain. C. T. showed a predominantly solid tumor mass which appeared to be in continuity with the posterior wall of the stomach, adjacent to the ta il of pancreas. At laparotomy, the mass was noted to involve only the tail of pancreas and measured 10 x 8 x 7 em. No enlarged lymph nodes were noted. A distal pancreatectomy was performed.

Case #3 596-9084

Invited discussant: Constantine Axiotis, M.D. Kings County Hospital

Host discussant: Bojana Krgin, M.D.

This 27 year-old Black man underwent subtotal resection of the right posterior maxilla for a lesion involving the alveolar ridge and extending into the maxillary antrum. Radiographs reveal a radiolucent lesion with smooth borders. The specimen measured 4 x 3 x 2 em and was tan· brown and firm.

Invited discussant: Host discussant:

Harry Lumerman, D. D. S. Roosevelt Torno, M.D.

Case #4 S96-6921 This 23 year-old Hispanic male underwent a liver biopsy for elevated alkaline phosphatase (376 u/L), abnormal SGOT (1 064 u/L) and total Bilirubin 1.4 mg/dL. The patient is a photographer and is not taking any hepatotoxic drugs or other medications. Serum AMA and ANA are negative. A cholangiogram was reported as normal.

Case #5 S96-6127

Invited discussant: Kathryn Tanaka, M.D. Montefiore Medical Center

Host discus.sant: Richard Alexis, M.D.

This 71 year-old female with past history of CV!, and hypertension, underwent a left modified radical mastectomy. A 4.5 x 3.5 em-pinkish grey to yellow and focally necrotic tumor was present.

Invited discussant: Ibrahim Hitti, M.D. North Shore University Hospital at

Forest Hills Host discussant : John Li, M.D.

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PATHOLOGISTS' CLUB OF NEW YORK

MINUTES OF MEETING

LONG ISLAND COLLEGE HOSPITAL Thursday, November 7, 1996

Dr. John Li, our host, welcomed members and visitors to the second meeting of the year and chaired the sci'entific session. A record number

of new members were unanimously voted in: Drs. Paula Caracia, Shanthi Govindaraj, Debra S. HeUer,' C. Stephan Kwon, Vasiliki Saitas, Manjula Vara, Elizabeth Wu and Pan Zheng.

:;m,'~5-9919). At age 6 a girl, whose AV canal defect had been treated with pulmonary artery banding in infancy, presented with total bilirubin 3.4, direct bilirubin 3.2, ANA and smooth muscle antibodies. Now, at 10 years, she has multiple discrete liver nodules, intermittent jaundice, normal bilirubin, ceruloplasmin and alpha-1 AT, GGT 230, SGOT 300. She has no serologic evidence of viral hepatitis. Dr. Valderrama began by noting that at age 3 the pulmonary artery band was reabsorbed and a new band was emplaced. The liver nodules now measure up to 1 Ocm. In some parts of the liver, central veins are normal, therefore congestive heart failure as a cause of cirrhosis can be excluded. Some portal tracts are also normal. A variety of causes of cirrhosis from the neonatal to childhood period needs to be considered Biliary atresias include Alagille' s syndrome and various non-syndromic forins, some of which are-associated with other diseases. Several types of hepatitis, including viral, Wilson's disease, Indian childhood cirrhosis, and autoimmune , as well as primary sclerosing cholangitis, primary biliary cirrhosis and cirrhosis associated with total parenteral nutrition are possibilities. In this case, arteries persist but intralobar bile ducts are lacking. The question is the etiology of this irregular cirrhosis with absence of bile ducts. The absence of portal hypertension is surprising. There is no history of inflammatory bowel disease, and no antimicrosomal antibodies. Sclerosing cholangitis may be idiopathic, or it may be associated with chronic ulcerative colitis, and a variety of immunologic disorders, including Riedel's thyroiditis, sicca syndrome , and idiopathic fibrosis. The history of ANA suggests autoimmune hepatitis. Dr. Valderrama concluded that the patient's cirrhosis with absence of bile ducts was the result of sclerosing cholangitis secondary to an autoimmune disorder, or the result of an autoimmune hepatitis.

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Dr. Alexis reviewed histologic findings, noting tbe presence .of focal hemorrhage and thrombosis, and of some portal tracts which are normal. He discussed non-cirrhotic parenchymal nodules, also known as

focal nodular hyperplasia or solid hyperplastic nodule. This is most common in females in the third to fourth decade and is an incidental finding. It may occur in association with vascular malformations in various sites, and CNS neoplasms. It is usually solitary and has a central scar. Vascular malformations may be essential to the development of the liver nodule. His diagnosis was focal nodular hyperplasia and focal ischemic hepatic necrosis with chronic congestion. An opinion from the AFIP was in agreement.

Dx: Focal Nodular Hyperplasia L Wanless IReta!: Multiple Focal Nodular Hyperplasia ... Mod Pathol2: 456-462, 1989 2. Sadowski DC et al: Progressive Type ofFocal Nodular Hyperplasia .. . Hepatol 21: 970-975, 1995

..

2. (S96-3165) A 36 year old black woman with vague abdominal pain underwent resection of a large solid tumor of the tail of the pancreas. Dr. Axiotis began by noting this unusual presentation for common pancreatic tumors. Solid and papillary epithelial pancreatic tumors may indeed present in this manner. In this case, there are nuClear grooves, nucleoli are not prominent and mitoses are not found . Some foci show micropapillary pattern, in others there is sclerosis with nesting reminiscent of neuroendocrine tumors. Degenerative changes also are evident. Hyaline globules, both intra- and extracytoplasmic are PAS­diastase positive. Based on these observations the differential diagnosis includes solid and papillary tumors, neuroendocrine, microcystic adenoma, acinar carcinoma and pancreatoblastoma, although the absence of glands is against the latter two possibilities.

Slow growth is characteristic in the approximately 300 cases reported to date. The tumor presents with upper abdominal discomfort in young women. Immunohistochemical stains demonstrate keratin, vimentin, desmoplakin and alpha-1-antitrypsin. There may be focal staining for NSE, insulin and glucagon as well as for progesterone receptors. Thre is no staining for chromogranin, synaptophysin and Leu-7. It has been suggested that the tumor may derive from a totipotential primordial cell. Its behavior cannot be predicted from morphology, and mya be either benign or of very low malignancy. Dr. Ku showed the gross appearance of the tumor which was encapsulated, yellow, soft, with cystic and hemorrhagic foci. Degenerative changes resulted in the fonnation of pseudopapillary structures. Marked edema led to cystic changes. Cytokeratin stains were negative. Ther.e are no characteristic immunohistochemical stains for this tumor. Dr. Ku concluded that this was a solid and cystic papillary neoplasm. In those cases that progressed to local recurrence and metastasis there were venous invasion, high nuclear grade and necrobiotic rests.

Dx: Solid and papillary cystic tumor of pancrea.s Pettinato Get al: Papillary cystic tumor of the pancreas. Am J Clio Pathol 98: 478, 1992 Nishihara K et al: Papillary cystic tumors of the pancrea.s. Cancer 71 : 82, 1993 Stommer Petal: Solid and cystic pancreatic tumors. Cancer 67: 1635, 1991

Case 3. (S96-9084) A firm, tan brown 4x3x2cm tumor was resected from the right posterior maxilla of a 27 year old black man. Dr. Lummennan revealed that two years earlier this man had a radiographic diagnosis of radicular cyst and underwent excision. But, another look at the Xrays showed wide spacing in the maxilla between teeth # 1 and 2. A larger lucent lesion developed somewhat later, and curettings showed a cystic lesion lined by several layers of parakeratinized epithelium. The appearance was consistent with odontogenic keratocyst, but it also had solid foci with intercellular edema. When the patient returned six months later tissue was growing out of the socket and it had destroyed bone, extending into the maxillary sinus. At present the tumor is mostly solid, without a capsule. Epithelium shows palisading and is edematous. Some foci show increased epithelial component. There is a prominent basal layer of squamous cells. In the differential diagnosis one must consider: Odontogenic keratocyst, Gorlin syndrome ( basal. ceU nevus syndrome ),calcified odontocyst (similar to pilomatricoma), squamous odontogenic tumor (has solid nests) and the two major types of keratoameloblastoma (follicular and plexiform). Dr. Torno showed the subtotal resection specimen and pointed out focal staining by PAS-diastase.

Dx. Keratoameloblastoma. Norval EJG: An unusual variant ofkeratoameloblastoma. J Oral Path Med 23: 465, 1994 Siar CH: Combined ameloblastoma and odontogenic keratocyst ... Brit J Oral Maxill Surg

31 : 183, 1993

Case 4. (896-6921) Liver biopsy was perfonned on a 23 year old Hispanic photographer with elevated alkaline phosphatase, SGOT and total bilirubin, and negative ANA and AMA. Dr. Tanaka began by pointing to the widened portal tracts , resulting from fibrosis, and to the presence of

,. 3 .

inflammatory cells and piecemeal necrosis with ductular proliferation associated with neutrophilic infiltrate. She emphasized the absence of bile ducts in some but not all portal tracts. Within the lobules she noted necrosis, cholestatic liver cell rosetttes and Kupffer cell increase. Ductopenia, as found here, is the presence of ducts in fewer than 50% of portal tracts, while normally 80 to 90% of tracts should have ducts. Granulomas and periductal fibrosis were not found. Among the many causes of ductopenia, one can probably exclude developmental and genetic categories (stain for alpha-I-AT was negative), as well as neoplastic. Among immunologic disorders, primary biliary cirrhosis is unlikely in a young man with negative ANA and AMA, but primary sclerosing cholangitis (PSC) of the small duct variety remains a possibility. Infectious, drug, toxin and ischemic categories lack historic support, however, Dr. Tanaka reminded that Wilson's disease cannot be ignored even though it is not usually included in causes of ductopenia. Idiopathic adulthood ductopenia must be seriously considered. This and small duct PSC are the two leading diagnostic contenders . It is possible that in some cases they are actually one entity. Drug and infectious background need to be kept in mind, because a history of these might emerge later on. Idiopathic adulthood ductopenia is a diagnosis of exclusion, and it is mor-phologically indistinguishable from non-syndromatic paucity of intrahepatic bile ducts. Some patients will go on to cirrhosis. The literature seems to suggest that there may exist a spectrum of small to large duct PSC, and in some cases it may be associated with inflammatory bowel disease. The elevated SGOT is difficult to explain in the absence of infection, drug, toxic or ischemic conditions. Dr. Tanaka concluded that we have a case of inflammatory bowel disease. Dr. Alexis agreed with these observations and called attention to a portal tract with constriction of a bile duct by a fibrous collar, and reiterated the finding of a normal cholangiogram. The elevated SGOT remained unexplained. Chronic hepatitis C should not be forgotten, however there was no serologic evidence for it.

Dx: Chronic cholestatic syndrome consistent with idiopathic adult ductopenia. I. Bruguera Metal: Nonsyndromic paucity ... Hepat<>l15: 830, 1992 2. Zafrani ES eta!: Idiopathic biliary ductopenia ... Gastroenterol 89: 1823, 1990 3. Ludwig Jet a!: Idiopathic adulthood ductopenia. J Hepatol pp 193-199, 1988

Case 5. (S96-6127) A 4.5 em focally necrotic tumor was found in the mastectomy of a 71 year old woman. Dr. Hitti showed a cellular tumor within a capsule, with intraductal carcinoma external to the capsule, and lobulation with papillary structures within. At low magnification the tumor appears to be an invasive papillary carcinoma, hardly the kind of case for this sophisticated audience. Closer inspection reveals that the papillary projections do not have vascular cores, in fact they are acinar formations with frequent mitoses and with vascular supply on the outer surface. Clear cell tumors, especially of myoepithelial origin, come to mind, as well as the glycogen-rich and lipid-rich cell cancers. Recently described forms resembling this tumor are solid papillary and invasive micro papillary carcinoma, and a very rare type of tumor with micropapillae within clear spaces, which most closely resembles the present case. An important point is not to mistake the clear spaces for vascular lumens. Dr. Li empbasized the sharp circumscription and relative softness of the tumor, features which on gross inspection would easily suggest a medullary carcinoma. He agreed with the presence of micropapillary type ofDClS at the periphery. Invasive micropapillary carcinoma is an impOrtant variant because of its very aggressive behavior. Papillary clusters invade the fibrous stroma without eliciting much desmoplasia. Dr. Rosen reviewed the case and agreed, and his new book includes a chapter on micropapillary carcinoma.

Dx: Invasive micropapillary carcinoma. I. Siriaungkgul S et al: Invasive micropapilliuy carcinoma .. Mod Pathol6: 660, 1993 2. Middleton LP et al: Infiltrating micropapillary carcinoma. .. Abstract, US-Canadian Acad Patho~

March 1996.