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 ?
Loss or alteration to PTEN, a common tumor suppressor gene, was observed in up to 80 percent of subtypes, including osteosarcoma, rhabdomyosarcoma, epithelioid sarcoma, Ewings sarcoma, alveolar soft part sarcoma and chordoma Clinical trials are options for patients with absent or altered PTEN in these subtypes.
Bryony, 11, was diagnosed with proximal epithelioid sarcoma two years ago.
Then, they finally got the results - epithelioid sarcoma - a rare form of cancer that affects soft tissue.
For instance, epithelioid hemangio-endothelioma, epithelioid angiosarcoma, epithelioid sarcoma, desmoplastic small round cell tumor, and synovial sarcoma may arise in body cavities and may sometimes be relevant to the differential diagnosis.
15) The clinical presentation of epithelioid sarcoma includes penile nodularity or mass, urethral stenosis, penile curvature, dysuria and pain during erection.
The histologic differential diagnosis is predicated on whether the tumor is biphasic or monophasic; it includes adenocarcinoma, spindle cell carcinoma, fibrosarcoma, malignant peripheral nerve sheath tumor, glomangiopericytoma (hemangiopericytoma), leiomyosarcoma, malignant melanoma, epithelioid sarcoma, and angiosarcoma.
Sergeant Profitt was suffering from epithelioid sarcoma, a type of cancer that typically develops in tissues under the skin of the hands, forearms, feet or lower leg.
The other soft tissue sarcomas include epithelioid sarcoma (Figure 7) and angiosarcoma (Figure 8).
About six weeks later, the Diana, Princess of Wales Children's Hospital confirmed within two hours of Mariam's visit that she had epithelioid sarcoma - a cancer which is seen in children approximately once every ten years.
Special attention is needed to rule out malignant neoplasms that can commonly mimic RDD, including melanoma (HMB45 and MART-1 positive) and epithelioid sarcoma (epithelial membrane antigen, cytokeratin, vimentin, CD34, and actin positive; most often, S100 negative).
Bryony, who lost an arm to proximal epithelioid sarcoma, said: "I don't know much about football but I can't wait to see Nicholas sing and have a chat with him.