Dense Pattern of Embryonal Rhabdomyosarcoma, a Lesion Easily Confused With Alveolar Rhabdomyosarcoma A Report From the Soft Tissue Sarcoma Committee of the Children%26apos;s Oncology Group

作者：Rudzinski Erin R^*; Teot Lisa A; Anderson James R; Moore Julie; Bridge Julia A; Barr Frederic G; Gastier Foster Julie M; Skapek Stephen X; Hawkins Douglas S; Parham David M

来源：American Journal of Clinical Pathology, 2013, 140(1): 82-90.

DOI：10.1309/AJCPA1WN7ARPCMKQ

• Alveolar rhabdomyosarcoma
• Embryonal rhabdomyosarcoma
• Histologic classification
• Rhabdomyosarcoma
• Myogenin

Objectives: To examine whether the frequency of fusion-negative alveolar rhabdomyosarcoma (ARMSn) increased coincident with changes in the definition of alveolar histology. %26lt;br%26gt;Methods: We re-reviewed alveolar rhabdomyosarcoma (ARMS) in the Children%26apos;s Oncology Group study D9803, comparing histopathology with fusion status. %26lt;br%26gt;Results: Our review of 255 original ARMS cases (compared with a control group of 38 embryonal rhabdomyosarcomas [ERMS] cases) revealed that many had an ARMS-like densely cellular pattern with cytologic features and myogenin expression more typical of ERMS. Following re-review, 84 (33%) cases of original ARMS were rediagnosed as ERMS. All reclassified ERMS, including dense ERMS, were fusion negative, whereas 82% of confirmed ARMS cases were fusion positive. Total ARMS diagnoses returned to historic rates of 25% to 30% of all rhabdomyosarcomas, and ARMSn decreased from 37% to 18% of ARMS cases. The outcome of reclassified ERMS was similar to confirmed ERMS. %26lt;br%26gt;Conclusions: To address the role of fusion status in risk stratification, pathologists should include both a histologic diagnosis and an evaluation of fusion status for all new ARMS diagnoses.

• 出版日期2013-7