Rhabdomyosarcoma Arising in the Hand or Foot: A Clinicopathologic Analysis

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.

Soft-tissue sarcomas of the extremities in patients of pediatric age

“…Our analysis also shows that most recurrences occurred with distant metastases (5-year LRFS was 78.6%, while MFS was 41.1%), unlike the situation in RMS at other sites, where local progression or recurrence is the main cause of treatment failure. Our study also confirms a particularly poor outcome for RMS of the foot or hand (5-year OS 33.3%) [18][19][20].…”

Orthopaedic Case of the Month: A 14-Year-Old Boy With a Medial Thigh Soft Tissue Mass

“…Rhabdomyosarcoma is the most common type of soft tissue sarcoma in the pediatric population [35,43], although adult-type soft tissue sarcomas become more predominant in the adolescent age group [39]. As in most other soft tissue tumors, MRI features of rhabdomyosarcoma can be nonspecific [28,29].…”

Improved Survival for Sarcomas of the Wrist and Hand

“…2 In addition it has been estimated that 0.1% of skeletal metastases from carcinomas involve the bones of the hand and that 16% to 25% of these lesions represent the first sign of an underlying malignancy. [3][4][5][6] It generally has been believed that benign tumors occurring in the hand and wrist behave more aggressively than those arising in other musculoskeletal sites [7][8][9][10][11][12][13][14][15] whereas malignancies seem to behave less aggressively (with the exception of rhabdomyosarcoma 16,17 ). Favorable outcomes for this group of tumors have been associated with attainment of wide margins at the time of surgical resection, 18 -23 prompt institution of multimodal therapy, 22,24 -28 tumor size smaller than 5 cm, and Musculoskeletal Tumor Society stage I or II lesions.…”