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Rhabdomyosarcoma Arising in the Hand or Foot: A Clinicopathologic Analysis

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Pediatric and Developmental Pathology

Abstract

Rhabdomyosarcomas (RMS) arising at different anatomic sites have distinct clinicopathologic features that affect prognosis and outcome. RMS arising in the extremities generally have a worse prognosis. Case reports and anecdotal experience suggests the possibility that RMS of the hand or foot (RMSHF) may behave even more aggressively than RMS elsewhere in the extremities. This study describes clinicopathologic features and outcome of RMSHF. Charts and pathologic material from RMSHF cases from institutional files over a 32-year interval were reviewed. Additional cases from published reports and series over a 42-year interval were identified by a MEDLINE search. Pertinent clinicopathologic features for each case were compiled. Survival analysis and other data from these patients were compared with previously reported statistics in the literature for RMS at other anatomic sites, including elsewhere in the extremities. Six patients from the institutional files and 46 from published reports were identified. A number of adverse prognostic indicators were observed in the majority of these patients including alveolar morphology (86%) and distant metastasis at presentation (63%). Despite the aggressive nature of these tumors, the majority were smaller than 5 cm (61%). A propensity was observed for metastases to unusual sites such as breast, ovary, testis, pancreas, and kidney. The median survival was 19 months, and the 5-year survival was 27%. While these may reflect a constellation of findings related to site, it is also possible that alveolar histology is the main influence. RMSHF can present as a very small primary tumor with clinically evident or occult metastatic disease. Whether this reflects the location, the predominantly alveolar histologic type, or a molecular genetic subtype will require further analysis. Patients presenting with RMSHF should be thoroughly evaluated for occult metastases at the time of diagnosis and followed closely throughout treatment for subsequent involvement of unusual sites.

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Acknowledgments

Krista Spilker assisted with manuscript preparation. This material was presented in part as an abstract at the Society for Pediatric Pathology, Annual Meeting, New Orleans, LA, March 2000 (Zhou H, Smith L, Coffin CM. Mod Pathol 2000;13:4P).

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Authors and Affiliations

  1. Division of Anatomic Pathology, Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, MN, 55905, USA

    Dylan V. Miller

  2. Division of Pediatric Pathology, Department of Pathology, University of Utah School of Medicine and Primary Children’s Medical Center, 100 North Medical Drive, Salt Lake City, UT, 84113, USA

    Cheryl M. Coffin & Holly Zhou

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  1. Dylan V. Miller
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  2. Cheryl M. Coffin
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Correspondence to Cheryl M. Coffin.

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Miller, D., Coffin, C. & Zhou, H. Rhabdomyosarcoma Arising in the Hand or Foot: A Clinicopathologic Analysis. Pediatr. Dev. Pathol. 7, 361–369 (2004). https://doi.org/10.1007/s10024-003-6071-1

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  • DOI: https://doi.org/10.1007/s10024-003-6071-1

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