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 ?
In broad terms, the differential diagnosis of epithelioid MPNST includes melanoma, clear cell sarcoma, epithelioid sarcoma and carcinoma.
2],[3],[4] SS can lead to diagnostic confusion with the following neoplasms: myoepithelial carcinoma, mesothelioma, leiomyosarcoma, epithelioid sarcoma, malignant peripheral nerve sheath tumor, and carcinosarcoma.
After a biopsy I was told I had a rare soft tissue cancer called epithelioid sarcoma, which only affects one in two million people.
It is also typically expressed by solitary fibrous tumor, gastrointestinal stromal tumor, DFSP, epithelioid sarcoma, spindle cell lipoma, synovial sarcoma, and vascular tumors.
And last August - three years after Henry was diagnosed with a brain tumour at the age of just five months - doctors discovered Edward had rare cancer on the thigh - epithelioid sarcoma proximal.
However, the case referred for consultation at yet another facility, which instead diagnosed the lesion as an epithelioid malignant neoplasm, favoring carcinoma over epithelioid sarcoma, according to the study.
The differential diagnosis includes synovial sarcoma or epithelioid sarcoma.
Differential diagnosis of GCT-ST includes soft tissue mesenchymal tumour which are rich in giant cell, villonodular sinuvitis, cellular dermatofibroma, atypical fibroxanthoma, plexiform fibrohistiocytic tumour, malignant fibrous histiocytoma, leiomyosarcoma, extraskeletal osteosarcoma and epithelioid sarcoma.
Other epithelioid neoplasms, including epithelioid sarcoma, melanoma, mesothelioma, and anaplastic large cell lymphoma, should also be considered in the differential diagnosis.
Bryony, 11, was diagnosed with proximal epithelioid sarcoma two years ago.
Most common diagnosis was that of Spindle cell type, which accounted for 26 of the 50 cases (52%) Followed by Round cell type accounted for 15 of 50 cases (30%), Pleomorphic type 7 cases (14%), Epithelioid sarcoma 1 case (2%).
15) The clinical presentation of epithelioid sarcoma includes penile nodularity or mass, urethral stenosis, penile curvature, dysuria and pain during erection.