A Hand Mass In A 31 Year Old Male



“A 31 y/o Man with a Mass in the Hand”

California Tumor Tissue Registry’s

Case of the Month

CTTR COTM Vol 8(3), December, 2005

A 31-year-old male presented with a mass in his hand. His physician thought that it was likely a foreign body reaction and the mass was removed.

It microscopically consisted of multiple nodules separated by fibrous stroma (Fig. 1). The nodules were generally discrete but tended to coalesce (Fig. 2). Central necrosis was present in most. A surrounding rim or “collar” of lining cells consisted mostly of polygonal cells with eosinophilic cytoplasm (Fig. 3). In some regions the cells were loosely arranged (Fig. 4a) and in others they were solid and infiltrative (Fig. 4b). Although most of the tumor lacked mitotic activity (Fig. 5) there were regions of active proliferation including atypical mitotic figures (Fig. 6).

Diagnosis: “Epithelioid Sarcoma, necrobiotic variant”

Lawrence B. Willes, MPH, and Donald R. Chase, MD

Loma Linda University, Loma Linda, California

Epithelioid sarcoma (ES) was first described by Enzinger in 1970 as a malignant soft tissue tumor which tends to occur in adolescents and young to middle-aged adults. It has a 2:1 male predominance. The tumor most frequently occurs on the extremities, especially fingers, hands, forearm, knee and lower leg. Less common sites include the shoulder, arm and feet. The tumor is rare in truncal sites but has been reported in thigh, buttocks, penis and vulva. It usually arises in subcutaneous tissue or in fascia; however it may also develop in deeper soft tissue. Bone is involved only secondarily. The tumor ranges from 3-6 cm but has a reported range of several mm to 15 cm.

ES usually presents as a slow growing painless mass that can be solitary or multinodular. Tumoral ulceration of the overlying skin may mimic a necrobiotic inflammatory process or an ulcerating squamous cell carcinoma. Deeper tumors are usually ill-defined and tend to spread along tendon sheaths and/or fascial planes.

Grossly ES is described as a solitary or multinodular mass with irregular outlines. The cut surface of the tumor is usually grey/white or grey/tan. There may be areas of red or yellow representing hemorrhage or necrosis.

Microscopically ES is defined by epithelioid appearing tumor cells with eosinophilic cytoplasm and a nodular arrangement. The nodules may undergo central degeneration and/or necrosis. Fusion of the necrotic centers results in a characteristic “geographic” pattern with an undulating nodular rim having scalloped edges. Interestingly, ES has a proclivity to “track” along blood vessels, nerves and fascial edges. It is now recognized that ES occurs in many phenotypes including:

o Classic types

▪ Solid variant

▪ Necrobiotic variant

o Recently described types:

▪ Fibrotic variant

▪ Angiomatoid variant

▪ Large cell variant (Proximal, Rhabdoid)

The Solid type is multinodular with little or no necrosis. The cells are polygonal or spindle-shaped and may show cording similar to epithelioid hemangioendothelioma. The Necrobiotic type consists of coalescent nodules with central areas of necrosis or hylanized collagen. Polygonal cells appear centrally and spindled cells peripherally. The Fibrotic variant is less epithelioid and mostly composed of spindled cells. It mimics either a fibroma or a benign fibrous histiocytoma. The Angiomatoid Variant shows cystic blood filled spaces bordered by epithelioid and spindled cells. The Large Cell Variant has a truncal location and shows larger polygonal cells with increased pleomorphism and mitotic activity.

Epithelioid sarcoma was one of the first mesenchymal neoplasms reported to co-express both vimentin and cytokeratins. Virtually all mark positively for EMA. Among the cytokeratins, ES marks for CK8 (94%), CK19 (78%), 34BEH12 (48%) and occasionally for CK7 (22%). About half stain for CD34 and 41% stain for MSA.

The main differential diagnoses include:

o Carcinoma

o Synovial sarcoma

o Epithelioid hemangioendothelioma

o Melanoma (vs. large cell variant)

o Epithelioid angiosarcoma (vs. angiomatoid variant)

o Extra-renal rhabdoid tumor (vs. large cell variant)

Carcinoma and ES may have similar cytokeratin staining patterns but carcinoma tends to occur in an older population and in proximal sites of the body. ES does not show epithelial dysplasia or CIS of adjacent structures or the overlying epidermis. The inclusion of a CD34 stain is important because it is negative in carcinoma. Synovial sarcoma (SS) occurs in a more proximal location usually around weight-bearing joints, and in deeper tissues rarely involving skin or subcutis. SS may stain for cytokeratins and EMA, but does not mark for CD34. Interestingly, SS may also stain for calretinin. Epithelioid hemangioendothelioma (EH) may show a cording growth pattern similar to solid type ES and stains for keratin and CD34. However unlike ES, EH usually contains vacuoles, shows no geographic necrosis and stains for CD31 and HFVIII. Melanoma (as compared to the large cell variant of ES) should stain for S100 protein and less uniformly for HMB45. Epithelioid angiosarcoma (EA) may look like the Angiomatoid Variant of ES and may stain for keratin but EA is usually more pleomorphic and should stain for CD31 and HFVIII. Extra-renal rhabdoid tumor shares the same rhabdoid phenotype as the large cell variant of ES and may also stain for keratin, EMA and CD34 thus making distinction from ES difficult, if not impossible.

Numerous lines of differentiation have been suggested including fibrohistiocytic, fibroblastic, fibrocytic, myoepithelial and endothelial, however the cell of origin has remained elusive.

Epithelioid sarcoma has a high risk of local recurrence and metastasis. In the study of over 240 cases by Chase and Enzinger (1985) recurrence occurred in 77%, metastasis in 45%, and 32% of patients died of disease. Metastases most frequently involved regional lymph nodes, lung, scalp, bone, brain and liver. Because of the high rate of multiple recurrences and metastasis, therapy should include early and complete excision of the tumor “at all costs.” Although limb-sparing surgery is generally preferred in other soft tissue sarcomas, early amputation is the preferred treatment of choice for ES.

Recommended Reading:

Arber DA, Kandalaft PL, Mehta P, Battifora H. Vimentin-negative epithelioid sarcoma. The value of an immunohistochemical panel that includes CD34. Am J Surg Pathol 1993; 17: 302-307.

Bos GD, Pritchard DJ , Reiman HM, Dobyns JH, Ilstrup DM, Landon GC. Epithelioid sarcoma. An analysis of fifty one cases. J Bone Joint Surg 1988; 70-A; 862-870.

Chase DR, Enzinger FM , Weiss SW, Langloss JM. Keratin in epithelioid sarcoma. An immunohistochemical study. Am J Surg Pathol 1984; 8: 435-441.

Chase DR, Enzinger FM. Epithelioid sarcoma. Diagnosis, prognostic indicators and treatment. Am J Surg Pathol 1985; 9: 241-263.

Chase, DR. Rhabdoid versus epithelioid sarcoma (lttr), Am J Surg Pathol 1990, 14(8); 792-794.

Chase, DR. Do “rhabdoid features” impart a poorer prognosis to proximal-type epithelioid sarcomas? Advances Anat Pathol 1997; 4(5); 293-299.

Enzinger FM. Epithelioid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-1041.

Evans HL, Baer SC. Epithelioid sarcoma: A clinicopathologic and prognostic study of 26 cases. Sem Diag Pathol 1993, (10)4: 286-291.

Fisher C. Epithelioid sarcoma: the spectrum of ultrastructural differentiation in seven immunohistochemically defined cases. Hum Pathol 1988; 19: 265-275.

Gerharz CD, Moll R , Meister P, Knuth A, Gabbert H. Cytoskeletal heterogeneity of an epithelioid sarcoma with expression of vimentin, cytokeratins and neurofilaments. Am J Surg Pathol 1990; 14: 274-283.

Guillou L, Wadden C, Coindre J-M, Krausz T, Fletcher CDM. "Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Am J Surg Pathol 1997; 21: 130-146.

Kudo E, Hirose T, Fujii Y, Hasegawa T, Ino H, Hizawa K. Undifferentiated carcinoma of the vulva mimicking epithelioid sarcoma. Am J Surg Pathol 1991; 15: 990-1001.

Kodet R, Smelhaus V, Newton WA, Hamoudi AB, Qualman SJ, Singley C, Jacobs Dl. Epithelioid sarcoma in childhood: An immunohistochemical, electron microscopic, and clinicopathologic study of 11 cases under 15 years of age and review of the literature. Ped Pathol 1994; 14: 433-451.

Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF. Epithelioid Sarcoma: An Immunohistochemical Analysis of 112 Classical and Variant Cases and a Discussion of the Differential Diagnosis. Hum Pathol 1999, 30:934-942.

Mirra JM, Kessler S , Bhuta S, Eckardt J. The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors. Cancer 1992; 69: 1382-1395.

Molenaar WM, DeJong B, Dam-Meiring A, Postma A, DeVries J, Hoekstra HJ. Epithelioid sarcoma or malignant rhabdoid tumor of soft tissue? Epithelioid immunophenotype and rhabdoid karyotype. Hum Pathol 1989; 20: 347-351.

Perrone T, Swanson P E, Twiggs L, Ulbright T M, Dehner L P. Malignant rhabdoid tumour of the vulva: is distinction from epithelioid sarcoma possible? Am J Surg Pathol 1989; 13: 848-858.

Rose DSC, Fisher C, Smith MEF. Epithelioid sarcoma arising in a patient with neurofibromatosis type 2. Histopathology 1994;25:379-380.

Sirgi KE, Wick MR, Swanson PE. B72.3 and CD34 immunoreactivity in malignant epithelioid soft tissue tumors. Adjuncts in the recognition of endothelial neoplasms. Am J Surg Pathol 1993; 17: 179-185.

Von Hochstetter AR, Meyer VE, Grant JW, Honegger HP, Schreiber A. Epithelioid sarcoma mimicking angiosarcoma: The value of immunohistochemistry in the differential diagnosis. Virchows Archiv A Pathol Anat 1991; 418: 271-278.

Wick MR, Manivel JC. Epithelioid sarcoma and isolated necrobiotic granuloma: a comparative immunocytochemical study. J Cutan Pathol 1986; 13; 253-260.

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