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Adult-type Rhabdomyosarcoma: Analysis of 57 Cases With Clinicopathologic Description, Identification of 3 Morphologic Patterns and Prognosis

Stock, Nathalie MD* †; Chibon, Frederic PhD*; Nguyen Binh, Matthieu Bui MD* †; Terrier, Philippe MD; Michels, Jean Jacques MD§; Valo, Isabelle MD; Robin, Yves Marie MD; Guillou, Louis MD; Ranchère-Vince, Dominique MD**; Decouvelaere, Anne-Valérie MD**; Collin, Francoise MD††; Birtwisle-Peyrottes, Isabelle MD‡‡; Gregoire, Fleur MD*; Aurias, Alain MD, PhD§§; Coindre, Jean-Michel MD* †

The American Journal of Surgical Pathology: December 2009 - Volume 33 - Issue 12 - p 1850-1859
doi: 10.1097/PAS.0b013e3181be6209
Original Articles

Adult-type rhabdomyosarcoma (RMS) has been classically defined as a pleomorphic sarcoma with desmin expression occurring in adult patients. To reevaluate this entity, we analyzed a series of 57 cases using immunohistochemistry for desmin, myogenin, alpha smooth muscle actin, h-caldesmon, pankeratin AE1/AE3, epithelial membrane antigen (EMA), S100 protein, CD34, MDM2, and CDK4. In this series, there were 36 men and 21 women aged from 22 to 87 years (median: 59). Tumors were mainly located in the lower limbs (27 cases), trunk wall (15 cases), and upper limbs (10 cases). Most tumors were deeply located (51/54) with a size from 1 to 30 cm (median: 8 cm). Cases were classified in 3 histologic categories: spindle cell RMS (25 cases), pleomorphic RMS (16 cases), and mixed type (16 cases). Forty-one tumors were grade 3 and 16 grade 2. Immunohistochemistry showed that every case was positive for desmin and myogenin. Alpha smooth muscle actin was positive in 21%, pankeratin AE1/AE3 in 20%, and CD34 in 13.2%. Treatment modalities and follow-up were available in 46 cases. Median follow-up was 60.9 months. Eight patients developed a local recurrence and 16 a distant metastasis with a 5-year overall survival rate of 52.6% and a 5-year metastasis-free survival of 62.9%. The only predictive factor for metastasis was histologic grade. In conclusion, adult-type RMS is a rare sarcoma occurring mainly in the extremities and trunk wall with 2 main histologic patterns, spindle cell, and pleomorphic patterns, which represent the end of the spectrum of a single entity.

*Bergonié Institute and INSERM U 916

Department of Pathology, University Victor Segalen, Bordeaux

Gustave Roussy Institute, Villejuif

§François Baclesse Center, Caen

Paul Papin Center, Angers

Oscar Lambret Center, Lille

**Léon Bérard Center, Lyon

††Georges-François Leclerc Center, Dijon

‡‡Antoine Lacassagne Center, Nice

§§Institut Curie, Paris, France

University Institute of Pathology, Lausanne, Switzerland

Correspondence: Jean Michel Coindre, MD, Department of Pathology, 229 Cours de l'Argonne, Bordeaux Cedex 33076, France (e-mail:

© 2009 Lippincott Williams & Wilkins, Inc.