Hersheychapter 34

8/9/2019 Hersheychapter 34

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Chapter 34: SOFT TISSUETUMORSSoft Tissue Tumors


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SOFT TISSUE TUMORSSoft tissue tumors are those originating in the supportive and connectivetissues of the body, other than bone or cartilage. Benign and malignant formsoccur. In addition, there are tumor-like lesions (pseudotumors) that mayclinically suggest a neoplasm. The difference is based upon a good history,examination and biopsy

Soft Tissue Tumors1. Scar (cicatrix), Hypertrophic scar, Keloid:a. Reactive connective tissue lesions, secondary to penetrating trauma, andtherefore secondary lesions (see Chapter 13, Dermatology)

2. Fibroma:a. A benign, self-limiting proliferation of fibroblasts, producing collagen, mostbeing subcutaneous single lesionsb. They occur at any agec. Koenen's Periungual Fibromas are seen on the hands and feet of aboutone-third of patients with tuberous sclerosis

3. Fibromatosis:This is a group of diseases demonstrating infiltrativegrowth that makes them less discreet than fibromasa. Lesions are frequently multiple, and patients may have more than onetypeb. Plantar fibromatosis usually affects the medial fascial slip, but any areamay be affected. This is correctly called Ledderhose's or Dupuytren's diseasec. A cure may be impossible because the lesion is infiltrative so that residualunseen lesion is always left behind after excisiond. Variants affecting the feet may include juvenile hyalin fibromatosis, extra-abdominal desmoid tumors, infantile digital fibromatosis, and juvenile

aponeurotic fibromatosis of Keasbey (which also occurs in adults)e. Desmoids also occur in Gardner's syndromef. Fibromas may histologically resemble well diferentiated fibrosarcomag. Older Keasbey lesions may become chondrified or ossified. Fibroosseouspseudotumor of the digits affects the fingers and toes, showing soft tissueossifications. It is not the same as a Keasbey lesion

NOTE* Patients with tuberous sclerosis show an acneform facial rash whoselesions are called adenoma sebaceum, but which are angiofibromas. Somepatients also are epileptic due to intracranial calcifications. This form oftuberous sclerosis is called Epiloia. Fibromas may develop independent ofother diseases and are firm, discrete les ions. Sometimes otherelements are involved, e.g. fibrolipoma, fibrokeratoma, angiofibroma,fibromyxoma, xanthofibroma, neurofibroma, etc. Lesions may recur afterexcision


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4. Myxomas:a. Are soft tumors of fibrous origin that show a loose fibrous stroma andmuch mucin and mucopolysaccharide within

b. This group includes digital mucus cysts and the synovial cysts such asganglion (only when the tendon sheath is present, hence, never distal to thetarsus), and bursac. Synovial cysts can also be intraosseous lesionsd. This group are pseudotumors

5. Fibrosarcoma:a. A malignant, collagenous, metastatic tumor that can occur at any age andwhich is uncommon in the footb. They may be deep or intradermalc. They also may be well differentiated or poorly differentiated

d. They may show a histologic "herringbone" pattern of cellular arrangement

6. Histiocytomas:a. Are similar to fibromas, but show less collagenb. The histiocyte is considered a facultative fibroblast, a cell state (ratherthan a true cell type)c. There are 3 major groups:i. Fibroblastic fibrous: includes the following types

Dermatofibroma

Xanthomas (some)

Myxomas

Dermatofibrosarcoma protuberans (malignant): show a storiformpatternhistologicallyii. Histiocytic fibrous:

Giant cell tumor of soft tissue (and bone)

Xanthogranuloma

Pigmented villonodular synovitis

There are also malignant forms recognizediii. Pleomorphhic fibrous: A group of unusual xanthomas,xanthogranulomas, and atypical fibroxanthomas

7.Synovial Chondromatosis (loose bodies, joint mice, joint rice,osteochondritis dissecans):

NOTE* A differential diagnosis of fibrosarcoma vs. plantar fibromatosis shouldbe kept in mind on initial examination of the foot

NOTE* Tendon xanthomas may suggest lipoproteinemia. Necrobioticxanthogranulomas may be associated with multiple myeloma. Multicentricreticulohistiocytosis may be a cause of polyarthritis


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a. A rare condition that follows trauma to synovium of large joints such as theankleb. Some of the bodies may ossifyc. X-rays and scans are helpful in the diagnosis

8. Synovial sarcoma (tendosynovial sarcoma):a. A highly malignant tumor of synovial tissues, often not arising from a jointb. Young people are affectedc. They may be slow or fast in growing and are very metastatic, especially if5 cm or larger in sized. Radiographic techniques and biopsy aid in the diagnosise. Treat aggressively (chemotherapy, surgery, irradiation)f. This is one of the few tumors for which there is no benign equivalent (i.e.there is no synovioma)

9. Subcutaneous Nodular Fat Necrosis (pancreatic panniculitis):a. Consists of reddish, tender nodules on the feet and ankles oftenaccompanied by ankle arthralgiasb. This accompanies pancreatitis and pancreatic carcinomac. Elevated blood levels of trypsin increase vascular permeability so thatelevated serum lipase escapes and damages the fat, causing inflammatorytumor-like nodulesd. If biopsy proven, these lesions suggest need for immediate consultation

10. Lipogranulatomatosis (Farber's disease):a. Very rare, recessively inherited sphingolipidosis, showing periarticular andtendon masses, especially at the wrists and anklesb. Other organs are affected and patients rarely live beyond the first yearc. The masses may, therefore, be diagnostic, requiring immediateconsultation

11. Piezogenic Papules:a. Represent cystic herniations of subcutaneous heel fat into the dermisb. They are seen on weightbearing in obese patientsc. They may be painful and can undergo necrosisd. Control with heel cupping devices and diet

12. Lipoma:a. A benign tumor of adipose tissue and multiple lesions may occur

b. These may be present independently or as part of Gardner's syndrome andneurofibromatosis-1c. Lipomas do not reduce in size as fat is lost due to diet or illnessd. Variants include fibrolipoma, angiolipoma, and myelolipoma (producingmyeloid hematopoietic cells)e. They can be excised locally without recurrencef. Tendon sheath lipomas (endovaginal lipomas) may occur in the feet and/orhandsg. Lipoblastoma may affect the feet of children


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13. Liposarcoma:a. Is a malignant, metastatic tumor of fatty tissueb. It is probable the most common lower extremity sarcoma in adults, but it isuncommon in the feetc. There are gradations from well to poorly differentiatedd. They may be slow growing and asymptomatic until a nerve or other tissueis sufficiently affected to result In pain or altered function e. These are deepseated tumors that should be excised followed by chemotherapy andirradiation

14. Myositis Ossificans (Munchmeyer's disease):a. A benign reactive lesion deep in striated muscle resulting from traumab. Early stages may be hard to tell on x-ray and histologically from theparosteal or extraosseous forms of osteogenic sarcomac. Older lesions appear fully ossified and may be excised if they impedefunction or cause paind. Myositis ossificans progressiva is a rare hereditary disease of children andyoung adults in which ossified lesions affect many tissues and are not relatedto trauma (patients may show shortened digits, absent thumbs and greattoes, or hallux valgus bilaterally)

15. Rhabdomyoma:a. Is a benign tumor of striated muscle and has not been reported in the footb. Patients showing signs and symptoms of tuberous sclerosis (see Fibromasection this chapter) may have cardiac rhabdomyoma and should, be sent forexamination

16. Supernumerary Muscles:a. Have been reported in the ankle region and may simulate tumors

17. Rhabdomyosarcoma:a. Very malignant metastatic tumor of striated muscleb. Rare in the foot, there have been reported casesc. There are 2 main types:i. Embryonal: seen in mostly younger personsii. Pleomorphic: seen in older personsd. These tumors are fast-growing and they erode bonee. Amputation, chemotherapy, and irradiation are all treatment methods

18. Leiomyoma:a. Is a benign tumor arising from smooth muscle

NOTE* Probably the most common lower extremity malignant tumor ofchildren


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b. The tumors are pilar leiomyomas and angioleiomyomasc. These lesions are often painful

19. Leiomyosarcoma:a. A malignant smooth muscle tumor rare (but does occur) in the ankle orfoot areab. Lesions may be superficial (dermal in origin) or deep (subcutaneous)c. Metastases from other sites may occur in the foot

20. Neuromas:a. Are not tumorsb. They are reactive lesions arising as a result of traumac. Rudimentary (supernumerary) digits may contain neuromatous tissues

21. Schwannoma:a. Is the benign peripheral nerve sheath tumor seen in the lower extremitiesb. It is considered a more accurate term than the older "neurilemoma" or"neurinoma"c. Slow growing, sometimes painful and mainly occurring on the flexorsurfacesd. They may fluctuate due to cystic changese. These fall into the peripheral nerve sheath tumor (PNST) groupf. Histologically one sees Verocay bodies that may be well developed (Antonitype A) or that may show myxoid degeneration (Antoni type B)g. The "ancient schwannoma" is one that shows cystic degeneration and/or

calcificationh. These lesions can be excised and are not recurrenti. In association with neurofibromatosis-1 there may be multipleschwannomasj. The neurothekeoma is a benign peripheral nerve sheath tumor

22. Neurofibroma:a. A benign, slow-growing, solitary, superficial, usually asymptomatic noduleb. Excision is curativec. Most cases occur in womend. Neurofibromatosis-1 (von Recklinghausen's disease, multiple

neurofibromatosis) is a hereditary malformation involving neural, cutaneous,and fibrous elementsi. Many organ systems may be involvedii. There are strict criteria for diagnosis of neurofibromatosis-1 that involvecafe-au-lait spotsiii. Patients show a high rate of development of unrelated malignant tumorsiv. There is a high risk of transformation of neurofibromas toneurofibrosarcomas in neurofibromatosis-1v. Diseases associated with neurofibromatosis-1 include:

NOTE* The sources of smooth muscle in the feet are the arrector pill musclesassociated with pilosebaceous units in the dorsal skin of the feet and toes,and blood vessels (frequently veins) anywhere in the foot.


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Scoliosis

Erosions of bone (due to neurofibromas)

Frohlich's syndrome

Addison's disease

Endocrine tumors

Increased incidence of malignant melanoma

Connective tissue tumors Multiple lipomas

Meningocele and syringomyelia

23. Malignant Peripheral Nerve Sheath Tumors (MPNST):a. These include malignant schwannoma and malignant neurofibrosarcomab. These are extremely rare in the feet but are a risk in the patient withneurofibromatosis-1 (when present there is a high mortality ratec.These tumors may also arise as a result of overexposure to irradiation(therapeutic or occupational)d. These tumors are metastatic and may be painful

e. Treatment consists of amputation (improvements in chemotherapy andirradiation are still being assessed)f. Malignant triton tumor is a malignant schwannoma with evidence ofconcurrent rhabdomyosarcoma

24. Malignant Neuroepithelioma (adult peripheral neuroblastoma,primative neuroectodermal tumor):a. Rare in the feet but has been reportedb. Neuroectodermal tumor of infancy (pigmented neuroectodermal tumor ofinfancy, melanotic progonoma) is also extremely rare in the foot but hasbeen reported

25. Granular Cell Tumor (Abrikossoff tumor):a. Occurs in benign and malignant forms and is uncommonb. It was once thought to be a tumor of muscular origin, but has been shownto be of peripheral nerve originc. It does occur in the foot

26. Angiomas:.a. May arise from the endothelium of blood vessels and/or lymphatic vesselsb. Predominant picture based on the presence of blood cells determineswhether the lesion is a hemangioma or a lymphangioma

27. Congenital Hemangiomas:a. May be localized or may involve large areas of limb and or other organsb. Most are capillary hemangiomas and after what appears to be a spurt ofrapid growth (in the first 6 months of life) most involute within a year or twoc. Patients should be checked to determine whether a larger (venous)hemangioma underlies the surface lesiond. There are rare cases in which capillary hemangiomas in infants areaccompanied by thrombocytopenia (Kasabach-Merritt syndrome)


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28. Acquired Tufted Hemangioma:a. Is the adult form of congenital capillary hemangiomab. Verrucous hemangioma (angiokeratoma circumscriptum) underlieshyperkeratoses and may clinically resemble warts or angiokeratomac. Cherry angiomas (De Morgan spots, senile angiomas) are tiny papularangiomas that arise (mostly on the trunk) after adolescenced. Nevus flammeus (nevus telengiectaticus, port wine stain) is a dermallesion with many dilated capillaries that usually suggests an underlyingvascular anomaly such as arteriovenous malformation and/or varicosities inthe Parkes-Weber syndrome (osteohypertrophic hemangiectasia, Klippel-Trenauney syndrome). The A-V malformations usually produce bruits audiblewith a stethoscope, local temperature changes, limb hypertrophy, and a riskof high output heart failure

29. Cavernous Hemangiomas:a. Composed of arterial and venous channels and are deep, large, diffuselesions that may clinically feel like a bag of wormsb. X-rays may show intravascular calcifications called phleboliths (associatedwith thrombi)c. Venous hemangiomas are clinically similar but do not show the arterialelementsi. These types of hemangiomas are seen in the blue rubber bleb nevus,Maffuci (Kast-Maffuci) syndrome, ataxia telangiectasia, von Hippel-Lindaudisease, and Sturge-Weber syndrome

30. Angiokeratoma:a. The name given to a number of syndromes that affect the feet (andother areas) showing dilated capillaries (ectasias) with secondary epidermalhyperplastic changesb. There are various types, all (except the Fordyce type) affecting the feeti. Mibelli typeii. Papular typeiii. Fabry type

c. Cobb's syndrome involves a cutaneous hemangioma in a dermatomal

distribution resembling angiokeratoma and associated with a spinal cordangioma

31. Angioma Serpiginosum:a. Consists of red, flat, grouped, asymptomatic, dilated capillaries, formingnet-like or macular patternsb. Most occur in postadolescent women

32. Pyogenic Granulomas:

NOTE* Angiokeratoma of Fabry is a storage disease whereas the others arenot


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a. Eruptive hemangiomas that are friable, easily traumatized, and that maybecome secondarily infectedb. Most are on a stalk

33. Glomus Tumors (glomangiomas, angiomyoneuromas):a. Arise in the digits as a tiny, painful and tender, frequently subungualtumorsb. They arise from the Suquet-Hoyer canal, the shunts arising from arteriolesthat bypass the capillary network to the venulesc. They may be red to purple and may be accompanied by thrombocytopenia(hence a variant of the Kasabach-Merritt syndrome)d. Most occur as single lesionse. Glomangiosarcoma is extremely rare, but has been reported

34. Synovial Hemangiomas:a. Arise in joints and tendon sheathsb. Rare and difficult to excisec. There is pain, swelling, joint effusion and hemarthrosis

35. Hemangiopericytoma:a. Rarely occurs in the feetb. There are benign and malignant forms, and histologic characteristics donot necessarily correlate with the benign or malignant nature of the lesionc. The patients must be watched for recurrence and/or metastases

36. Lymphangiomas:a. As a group are rareb. They may arise in the context of chronic and extensive lymphedema, e.g.Milroy's disease, elephantiasis due to Wucheria infestation, etc.c. Cystic hygroma represents a cavernous lymphangioma

37. Papillary Endothelial Hyperplasia (Masson'spseudoangiosarcoma):a. An intravascular proliferation of endothelial cells in association with athrombus, and usually follows traumab. It is not commonc. Most cases show single lesions and these may be difficult to differentiatehistologically from angiosarcoma

38. Ectasias (vascular dilatations):a. May occur in the feet and be mistaken for angiomasb. The most common is the spider nevus (spider angioma, nevus araneus)

39. Angioendothelioma:a. The name given to group of lesions that are considered by some to be ofintermediate malignancyb. Types:i. Spindle cell type:


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Is most apt to occur on the lower extremities

Uncommon, seen frequently in young malesii. Reactive proliferating angioendotheliomatosis:

A vascular, benign lesion in response to infection, (especially subacutebacterial endocarditis) that truly involves endothelial proliferation

Usually clears n 6-24 months

AIDS patients may develop this lesions n response to cat scratch fever

organisms (Rochalimaea henselae)iii. Malignant angioendothelioma:

A malignant intravascular lymphoma and not an angioma orangiosarcoma

40. Angiosarcomas:a. Very rare in the lower extremitiesb. Very malignant endothelial tumors, usually occurng in the elderlyc. They may be associated with chronic edemad. They may grow rapidly and may metastasize

e. Most cases are fatalf. Amputation a good distance proximal to the tumor is the surgical approachg. Irradiation has not been very effectiveh. Some superficial forms (not the deep forms) have been treatedsuccessfully with carbon dioxide laseri. Patients must be checked for recurrences and metastases

41. Kaposi's Sarcoma (multiple idiopathic hemorrhagic sarcoma):a. Is related to immunodeficiency, occurs n older patients, AIDS patients, andpatients on chemotherapy or post-transplant immunosuppression therapyb. May occur n the presence of leukemias and lymphomas

c. Lesions may appear as purpuric macules, papules, patches, nodules,plaques, and tumorsd. Silent visceral lesions may be presente. It is presently divided into the following types:i. Classic (sporadic) epidemic (non AIDS)

Attributed to the elderly eastern and southern European men

Skin associated lesions frequently begin on the feetii. African- affects adults and children and not restricted to the feetiii. Allograft-associated (immunosuppression)iv. Epidemic (AIDS-associated)

42. Nodules of Metastatic Lymphoma (non-Hodgkins) or MyeloidLeukemia (granulocytic sarcoma, chloroma):a. May occur n the dermis as metastatic site to feetb. Biopsy is diagnostic

NOTE* On occasion, malignant lower extremity tumors may develop thatcannot be clearly identified as to tissue of origin. These include thealveolar soft-part sarcoma, the mesenchymoma, and anyundifferentiated malignant soft tissue tumor


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c. Mycosis fungoides is a T-cell lymphoma originating n the skini. Woringer-Kolopp disease: Is the localized epidermotropic form formerlycalled Pagetoid reticulosisii. Sezary syndrome: is the systemic form

Circulating abnormal lymphoid cells

Peripheral lymphadenopathy

Erythroderma Intense pruritus

43: Mastocytomas:a. Are tumors formed by mast cellsb. Some localize to the dermis others are systemicc. Dermal lesions urticate (Darier's sign)d. Pruritis and flushing may occur due to local vasodilatory effects of mastcellderived histamine shocke. A single local lesion is known as urticaria pigmentosa

44. Metastatic Cancers:a. Metastatic cancers from visceral organs may occur in the dermisb. They are usually rapidly growing, multiple (various sites) and otherwisenonspecific clinicallyc. A history if malignant tumor (even years ago) is a clue, but not alwayspresentd. Even with such a history, a new lesion may be an independent entity

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Hersheychapter 34