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Am J Clin Pathol. 2013 Jul;140(1):82-90. doi: 10.1309/AJCPA1WN7ARPCMKQ.

Dense pattern of embryonal rhabdomyosarcoma, a lesion easily confused with alveolar rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

Author information

1
Seattle Children's Hospital, Department of Laboratories, M/S OC.8.720, 4800 Sandpoint Way NE, Seattle, WA 98015, USA. erin.rudzinski@seattlechildrens.org

Abstract

OBJECTIVES:

To examine whether the frequency of fusion-negative alveolar rhabdomyosarcoma (ARMSn) increased coincident with changes in the definition of alveolar histology.

METHODS:

We re-reviewed alveolar rhabdomyosarcoma (ARMS) in the Children's Oncology Group study D9803, comparing histopathology with fusion status.

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.

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.

KEYWORDS:

Alveolar rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Histologic classification; Myogenin; Rhabdomyosarcoma

PMID:
23765537
PMCID:
PMC4624292
DOI:
10.1309/AJCPA1WN7ARPCMKQ
[Indexed for MEDLINE]
Free PMC Article

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