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Review Question - QID 213923

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QID 213923 (Type "213923" in App Search)
Which of these soft tissue sarcomas is most likely to spread via lymphatics?

Liposarcoma

7%

119/1677

Fibrosarcoma

3%

46/1677

Leiomyosarcoma

11%

189/1677

Epithelioid sarcoma

67%

1119/1677

Undifferentiated pleomorphic sarcoma

12%

193/1677

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Epithelioid sarcoma is associated with metastases to local lymph nodes.

Epithelioid sarcoma is a rare malignant tumor of unknown cell origin that most commonly present in the hands and wrist. It most commonly is found in males between the ages of 10-40 years of age. Typically, it presents as a firm and painless mass, possibly with skin ulceration. Key histologic features include a nodular growth pattern with central areas of necrosis. H&E staining will demonstrate mixed polygonal cells with eosinophilic cytoplasm mixed with spindle cells and epithelioid cells. There will be immunohistochemistry reactivity for keratin, epithelioid membrane antigen (EMA), vimentin, and CD34. Classically these will metastasize to local lymph nodes, and a sentinel node biopsy may be performed at the time of excision. Other soft-tissue sarcomas that spread via lymphatics include rhabdomyosarcoma, angiosarcoma, synovial sarcoma, and clear cell sarcoma. The mnemonic to remember these is RACES (Rhabdomyosarcoma, Angiosarcoma, Clear cell, Epithelioid, and Synovial sarcomas). The treatment for epithelioid sarcoma includes wide local excision with adjuvant radiation.

Maduekwe et al looked at 29 patients with a diagnosis of synovial, epithelioid, and clear cell sarcomas without radiographic evidence of lymph node metastases. 28 of these patients subsequently underwent successful sentinel lymph node biopsy (SLNB) and all patients had a primary tumor resection. 3 patients had histologic evidence of metastases of which one develops later lung metastasis, while one patient with a negative SLNB later developed a lymph node metastasis. They concluded that in the absence of radiologic evidence of lymph node metastasis, occult lymph node metastases are relatively low and SLNB may not hold significant utility.

Thway et al reviewed the diagnostic features of epithelioid sarcoma. Clinical features of the sarcoma include young adult male predominance, typically presenting in the upper extremities distally, and appearing as slow-growing firm nodules that develop into chronic nonhealing ulcers. 30-50% of cases have metastases, and there is a local recurrence in 87-93% of cases. Histopathology shows nodular aggregates of plump epithelioid cells and abundant eosinophilic cytoplasm. There is no consistent cytogenetic anomaly yet identified.

Incorrect answers:
Answer 1: Liposarcoma is not associated with lymph node metastases.
Answer 2: Fibrosarcoma is not associated with lymph node metastases.
Answer 3: Leiomyosarcoma is not associated with lymph node metastases.
Answer 5: Undifferentiated pleomorphic sarcoma is not associated with lymph node metastases.

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