epithelioid sarcoma

epithelioid sarcoma

malignant mesenchymal tumor showing nodular, granulomalike growth of eosinophilic epithelioid and spindle cells palisading around the centrally located necrotic areas. Two subtypes are recognized: a classical distal and a fibromalike form. Tumors typically occur in the subcutaneous tissue along the tendons and fascia.

epithelioid sarcoma

A rare, low-grade sarcoma, which primarily affects the distal extremities, especially the hand and fingers. Male:female ratio, 2:1, affecting patients age 10–35 if distal and older if proximal.
 
DiffDx-benign
Benign fibrous histiocytoma, fibromatosis, granuloma annulare, infectious granuloma, myositis ossificans, necrobiosis lipoidica, necrobiotic granuloma, nodular fasciitis, rhabdoid tumour, rheumatoid nodule.
 
DiffDx-malignant
Adnexal carcinoma, clear-cell carcinoma, epithelioid vascular neoplasm, fibrosarcoma, melanoma, metastatic carcinoma, squamous cell carcinoma, synovial sarcoma.

Management
Wide local excision or amputation.
 
Prognosis
Recurrence in 35–75%; 40–45% metastasise—e.g., to lung, regional lymph nodes, scalp; 5-year survival in 80%; 10-year survival in 50%.

Adverse prognosticators
Deep, > 5 cm, high mitotic rate, vessel and nerve invasion, recurrence, nodal metastasis.

Epithelioid sarcoma types
Conventional (diffuse) form
Affects distal extremities, especially hand and fingers; male:female ratio, 2:1; affects patients age 10–35 if distal and older if proximal.

Proximal type
Older patients; more aggressive than conventional (distal) type; located deep in the pelvis, perineum, pubis, vulva and penis.

Micro
Large epithelioid cells with marked atypia and prominent nucleoli, varying amounts of rhabdoid features.

epithelioid sarcoma

A low-grade sarcoma of the upper extremity, ♂:♀ ratio, 2:1, of Pts age 10-35 Treatment Wide local excision or amputation DiffDx-Benign Fibromatosis, fibrous histiocytoma, nodular fasciitis, infectious granuloma, necrobiosis lipoidica, rheumatoid nodule DiffDx-malignant Synovial sarcoma, fibrosarcoma, melanoma Prognosis Recurrence is common; 45% metastasize–eg, to lung, regional lymph nodes, scalp
References in periodicals archive ?
Epizyme, Inc, a late-stage biopharmaceutical firm focusing on new epigenetics therapies has recently released new data on on tazemetostat from the epithelioid sarcoma cohort of its active Phase 2 research in patients with molecularly defined solid tumours.
'Our vision is to bring tazemetostat to both epithelioid sarcoma and follicular lymphoma patients, while also expanding its development in the future to additional tumor types and combinations, to advance research efforts to bring EZM8266 and other early programs into the clinic, and to further enhance our leadership in the field of epigenetic drug discovery and development.
The company has proposed an indication of metastatic or locally advanced epithelioid sarcoma not eligible for curative surgery.
The name epithelioid sarcoma-like hemangioendothelioma was used to emphasize the similarity of this tumor to epithelioid sarcoma and because 6 of the 7 patients (86%) in that series had a prior diagnosis of epithelioid sarcoma.
Net proceeds from these offerings, together with its existing cash, cash equivalents and marketable securities, may be used by the company to fund global development and commercialisation costs of tazemetostat for epithelioid sarcoma and follicular lymphoma, to fund research and development costs to identify and develop other product candidates, including EZM8266 for sickle cell disease, working capital as well as other general corporate purposes.
Pseudomyogenic hemangioendothelioma (PHE)--previously referred to as a fibroma-like variant of epithelioid sarcoma, given its morphological similarity to epithelioid sarcoma--is a rare and recently described endothelial tumor.
Epithelioid sarcoma is an uncommon soft tissue tumor that occurs frequently in the forearm and hand.
Epithelioid sarcoma: clinical, MR imaging and pathologic findings.
The morphology and immunophenotype provided no support for a reactive process, melanoma, lymphoma, carcinoma, epithelioid sarcoma, epithelioid malignant peripheral nerve sheath tumor, rhabdoid tumor, or alveolar soft part sarcoma.
Certain types of STSs (synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, rhabdomyosarcoma, and angiosarcoma) have a tendency to spread via the lymphatic system, thereby warranting palpation of local lymphatics for other masses [7].
Epithelioid sarcoma (ES) on the other hand is a soft tissue tumor composed of large polygonal cells resembling carcinomas [5].
Epithelioid sarcoma (ES) is a rare high-grade malignancy identified by Enzinger in 1970 [1].