| 20055602 |
Alaggio R, Collini P, Randall L, Barnette P, Smith L, Coffin CM: High Grade Pleomorphic Undifferentiated Sarcomas In Children: A Clinicopathologic Study Of 10 Cases and review of literature. Pediatr Dev Pathol. 2010 Jan 7.
Abstract UHGPS is a sarcoma of debated nosology affecting adults, with rare cases reported in children. In order to investigate the clinicopathologic and prognostic features of pediatric UHGPS, 10 UHGPS occurring before 18 years (mean age 8.9 years) were analyzed. All were localized at diagnosis (head: 4, lower extremities: 4, trunk: 2), with a mean diameter of 4.5 cm. Mean follow-up was 6 years. Six patients were in complete remission (CR), one after a relapse; 2 died of metastatic disease; one was alive with metastasis. Histologically 8 tumors showed spindle cells with a focal or diffuse storiform pattern and scattered aggregates of epithelioid cells in 2. Two tumors displayed a prominent epithelioid component. Cellular pleomorphism, high mitotic rate with atypical mitoses were found in all tumors; necrosis in 6, vascular invasion in 2. CD68 and desmin were positive in 2 cases each, SMA in 4, S100 in one. Five tumors in first CR and 1 in second CR were superficial, showed a spindle cell morphology with epithelioid foci in one, necrosis in 3; Five were grade 3, 1 grade 2. Three metastatic tumors (2 in the dura, 1 in the leg) displayed either a prominent epithelioid morphology (2) or scattered aggregates of epithelioid cells (1), with a myxoid background in 1. All were grade 3 and showed foci of necrosis. In summary, UHGPS are rare in children and frequently located in the head. A more favorable outcome is associated with superficial location. Foci of epithelioid cell may portend an aggressive behavior. |
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