Macrocephaly Capillary Malformation
Heather Borders, MD
over 7 years ago
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Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 5 years ago
|Updated: over 5 years ago
Teenage male with thigh mass.
MR – Intramuscular T1 isointense and T2 and post-gadolinium hyperintense mass with minimal perilesional edema.
Epithelioid sarcoma is a soft-tissue sarcoma usually presenting as a subcutaneous or deep dermal mass lesion in the distal portions of the extremities of young adults. Histologically, epithelioid sarcoma commonly presents as a multinodular proliferation of epithelioid and spindle-shaped cells with cytologic atypia . Proximal type epithelioid sarcomas show significant clinical and histologic differences from conventional epithelioid sarcoma. The proximal type demonstrates a large cell epithelioid cytomorphology, marked cytologic atypia, and frequent rhabdoid features. Survival rates for proximal-type epithelioid sarcoma at 5-10 years range between 50% and 80%. Metastases develop in about 40% of patients. Proximal-type sarcoma has a predilection for appearing in the genitalia, especially the vulva, penis, pelvis, and buttocks, however, proximal extremities, including the trunk, head and neck, can also be involved. The proximal type appears to be slightly more aggressive and appears to metastasize earlier than conventional epithelioid sarcoma.
Tateishi U, Hasegawa T, Kusumoto M, et al. Radiologic Manifestations of Proximal Type Epithelioid Sarcoma of the soft Tissues. AJR (2002); 179: 973 – 977.
I. Rekha Meesa, MD