Access Keys:
Skip to content (Access Key - 0)



  • Juvenile types
    • Embryonal rhabdomyosarcoma (70-80%): mainly affects children < 15 years of age occurring in the head and neck, GU tract, retroperitoneum and extremities
    • (Botryoid type: a variant of the embryonal type characterized by a grapelike growth pattern not occurring in the extremities, but in hollow visceral organs)
    • Alveolar rhabdomyosarcoma (10-20%): usually occurs between ages 10-25, responsible for a second incidence peak
    • Occurs with greater incidence in the extremities
  • Adult type
    • Pleomorphic rhabdomyosarcoma: peak incidence >45 years of age, occurring most commonly in the thigh
    • Pretreatment staging system
      • Stage    Site*   Invasiveness    Size    Nodes    Metastases
      • 1       favorable   T1 or T2       a or b    N0, N1        M0
      • 2     unfavorable T1 or T2     a (<5cm)     N0           M0
      • 3     unfavorable T1 or T2     b (>5cm)  N0, N1        M0
      • 4        any site   T1 or T2     a or b      N0, N1        M1
        * all extremity sites unfavorable
    • Intergroup Rhabdomyosarcoma Study Clinical groups
    • I Localized, complete resection, no LNs
      • a) confined to nuscle/organ or origin
      • b) tumor infiltration beyond muscle/organ of origin
    • II Localized/regional disease, complete macroscopic resection
      • a) resection with microscopic margin, no LNs
      • b) complete resection of tumor and LNs
      • c) tumor and LNs resected with microscopic margins
    • III < wide resection with macroscopic residual
    • IV Distant metastases at dx


  • The most common soft tissue sarcoma of children, addolescents, and young adults, although only 14.6% occur in the extremities
  • Accounts for 4-8% of all pediatric malignancies
    • 20% involve the extremities


  • Rare >45 years of age (except pleomorphic type which occurs 40-80 yrs of age)
  • May present at birth
  • M:F = 1.5:1
  • Discovery frequently associated with a traumatic episode (falsely diagnosed as a hematoma or benign tumor)
  • Most are deep-seated intramuscular masses
  • Often asymptomatic
  • Alveolar subtype reported in a neonate with Beckwith-Wiedemann Syndrome with multiple nodular and vesicular lesions on the face, limbs, and abdomen, resembling "blueberry muffin baby"
  • Head/neck locations
  • Unusual locations


  • Erosion of bone in hand or foot lesions
  • MRI: deep-seated intramuscular mass


  • Poorly defined, infiltrating into surrounding structures in extremity lesions
  • Firm, rubbery, and grayish-white in appearance ± necrosis or cystic degeneration


  • Rhabdomyoblasts have eosinophilic cytoplasm that is filamentous and granular ± transverse striations, contractile elements
  • Alveolar types: embryonal types = 4:3
  • Embryonal types (46-60%)
    • Poorly-differentiated to well-differentiated looking like fetal muscle tissue with variation in cellularity denseness and alternating cellular and myxoid areas
    • Scant interstitial collagen
    • Elongated and rounded rhabdomyoblasts with hyperchromatic nuclei, eosinophilic cytoplasm, and fibrillary material around the nucleus ± cross striations (50-60%)
    • + PAS ± diastase for intracellular glycogen in well-differentiated tumors("spider-web" cells are rhabdomyoblasts with glycogen washed out by the fixative and strands extending to the cellular periphery)
    • Iron hematoxylin, PTAH, Masson trichrome shows cross striations
    • "Broken-straw" sign = angulation of narrow, ribbon-shaped muscle fibers
  • Alveolar types (20-41%)(50% of extremity locations)
    • Aggregates of tumor cells with "alveolar" spaces separated by loose fibrous septa are typical but areas may be solid cells, especially at the periphery of a tumor
    • Rounded nuclei and indistinct cytoplasm
    • Rhabdomyoblasts in only 30% ± cross striations (20-30%)
    • Multinucleated giant cells are prominant
    • Entrapment of normal muscle tissue
  • Pleomorphic types (~13%)(adult-type)
    • Difficult to distinguish from other pleomorphic sarcomas (eg, MFH)
    • Rarely with cross striations
  • Undifferentiated (15-20%)
  • Botryoid
  • Spindle cell
  • Rhabdomyosarcoma with rhabdoid features
  • Immunohistochemistry and special stains
    • Desmin and myoglobin (beware of false + in entrapped normal muscle)
    • + PAS for glycogen
    • Myogenic transcriptional regulatory proteins
    • + myogenin
    • + MyoD1
  • Cytogenetics
    • Li-Fraumeni syndrome associated with rhabdomyosarcoma
    • Reported in Noonan syndrome due to a SOS1 mutation
    • Metastatic alveolar rhabdomyosarcoma reported in Multiple Endocrine Neoplasia Type 2A
    • Expression of MyoD1 geneLoss of 11p15 heterozygosity in embryonal subtype
    • Translocation t(2;13) or t(1;13) with fusion gene Pax 3-FKHR or Pax 7-FKHR fusion genes respectively in alveolar subtype
  • EM
    • Variable appearance resembling stages of embryonal muscle tissue development
    • Myofilaments and polyribosomes
    • Golgi apparatus is prominent


  • Poorly differentiated round or spindle cell sarcomas
  • Neuroblastoma
  • Neuroepithelioma
  • Ewing’s sarcoma
  • PNET
  • Poorly differentiated angiosarcoma
  • Synovial sarcoma
  • Malignant melanoma
  • Granulocytic sarcoma
  • Lymphoma
  • Small cell carcinoma
  • Benign lesions
    • Granular cell tumor


  • Alveolar subtype with more aggressive clinical behavior/worse outcome than embryonal subtype
  • Chemotx + wide resection + chemotherapy ± XRT after careful staging
    • Dictated by Intergroup Rhabdomyosarcoma Study (IRS) protocols
  • Aggressive regional LN sampling, if +, further proximial node evaluation
  • Response to chemotx overall is good; "second look" surgeries have been advocated
    • Vincristine, actinomycin-D, cyclophosphamide, ± adriamycin, ifosfamide
    • De novo multiple drug resistance has been reported
  • Metastases
    • Lung (67% when metastases are present)
    • LNs(depends on location and type--alveolar > embryonal)
    • Bone marrow (aspirate as part of staging work-up)
    • Heart
    • Brain
    • Abdominal organs
  • Prognosis depends on stage at time of diagnosis with up to 83% 5 year survival with localized disease and with histological type (alveolar type has a worse prognosis), and with ability to gain a wide margin and complete resection
  • Unresectable lesions will have <10% long term survivors
  • Embryonal histologic subtype has the most favorable prognosis
  • More distally located tumors generally have a better prognosis
  • Extemity lesions have a worse prognosis than orbital or GU lesions
  • 12% 5-yr risk of regional failure
    • Poor prognostic indicators
      • Female gender
      • LN involvement at dx


  • A

    • y
  • A

    • A


Andrassy RJ, Corpron CA, Hays D, Raney RB, Wiener ES, Lawrence W Jr, Lobe TE, Bagwell C, Maurer HM: Extremity sarcomas: an analysis of prognostic factors from the Intergroup Rhabdomyosarcoma Study III: J Pediatr Surg 31:191-6,1996.

Ariel IM, Briceno M: Rhabdomyosarcoma of the extremities and trunk: analysis of 150 patients treated by surgical resection. J Surg Oncol 7:269-87,1975.

Boulad F, Karnan NA, LaQuaglia MP, Heller G, Lindsley KL, Rosenfield NS, Abramson SJ, Gerald WL, Small TN, Gillio AP, Gulati SC, O’Reilly RJ, Ghavimi F: High-dose induction chmoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing’s sarcoma, and undifferentiated sarcoma. J Clin Oncol 16:1697-706,1998.

Cessna MH, Zhou H, Perkins SL, Tripp SR, Layfield L, Daines C, Coffin CM: Are myogenin and MyoD1 expression specific for rhabdomyosarcoma? A study of 150 cases. with emphasis on spindle cell mimics. Am J Surg Pathol 25:1150-7,2001.

Chen B, Dias P, Jenkins JJ, III, Savell VH, Parham DM: Methylation alterians of the MyoD1 upstream region are predictive of subclassification of human rhabdomyosarcomas. Am J Pathol 152:1071-9,1998.

Coffin CM: The new International Rhabdomyosarcoma Classification, its progenitors, and considerations beyond morphology. Adv Anat Pathol 4:1-16,1997.

Coffin CM, Rulon J, Smith L, Bruggers C, White FV: Pathologic features of rhabdomyosarcoma before and after treatment: a clinicopathologic and immunohistochemical analysis. Mod Pathol 10:1175-87,1997.

Cohen M, Ghosh L, Schafer ME: Congenital embryonal rhabdomyosarcoma of the hand and Apert’s syndrome. J Hand Surg 12A:614-7,1987.

Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, : The Third Intergroup rhabdomyosarcoma study. J Clin Oncol 13:610-30,1995.

Cui S, Hano H, Harada T, Takai S, Masui F, Ushigome S: Evaluation of new monoclonal anti-MyoD1 and anti-myognein antibodies for the diagnosis of rhabdomyosarcoma. Pathol Int 49:62-8,1999.

Dias P, Chen B, Dilday B, Palmer H, Hosoi H, Singh S, Wu C, Li X, Thompson J, Parham D, Qualman S, Houghton P: Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. Am J Pathol 156:399-408,2000.

Dias P, Parham DM, Shapiro DN, Webber BL, Houghton PJ: Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. Am J Pathol 137:1283-91,1990.

Engel ME, Mouton SC, Emms M: Paediatric rhabdomyosarcoma; MyoD1 demonstration in routinely processed tissue sections using wet heat pretreatment (pressure cooking) for antigen retrieval. J Clin Pathol 50:37-9,1997.

Enzinger FM, Weiss SW: Rhabdomyosarcoma. In Soft Tissue Tumors FM Enzinger and SW Weiss (Eds), C.V. Mosby Co., St. Louis (3rd ed) 1995, pp 539-578.

Eusebi V, Rilke F, Ceccarelli C, Fedeli F, Schiaffino S, Bussolati G: Fetal heavy chain skeletal myosin. An oncofetal antigen expressed by rhabdomyosarcoma. Am J Surg Pathol 10:680-6,1986.

Furlong MA, Mentzel T, Fanburg-Smith JC: Pleomorphic rhabdomyosarcoma in adults: a clincopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol 14:595-603,2001.

Ghavimi F, Mandell LR, Heller G, Hajdu SI, Exelby P: Prognosis in childhood rhabdomyosarcoma of the extremity. Cancer 64:2233-2237,1989.

Hastings R, Newbury-Ecob R, Ng A, Taylor R: A further patient with Noonan syndrome due to a SOS1 mutation and rhabdomyosarcoma. Genes Chromosomes Cancer. 2010 Jul 6.

Hays DM: Rhabdomyosarcoma. Clin Orthop 289:36-49,1993.

Hays DM, Lawrence W, Jr, Wharam M, Newton W Jr, Ruymann FB, Beltangady M, Maurer HM:Primary reexcision for patients with "microscopic residual" tumor following initial excision of sarcomas of the trunk and extremity sites. J Pediatr Surg 24:5-9,1989.

Hays DM, Raney RB, Crist WM, Lawrence WW Jr, Ragab A, Wharam MD, Webber B, Gehan E, Johnston J, Maurer HM: Secondary surgical procedures ot evaluate primary tumor status in patienst with chemotherapy-responsive stage III and IV sarcomas: a report from the Intergroup Rhabdomyosarcoma Study. J Pediatr Surg 25:1100-5,1990.

Hays DM, Soule EH, Lawrence W, Jr, Gehan EA, Maurer HM, Donaldson M, Raney RB, Tefft M: Extremity lesions in the intergroup rhabdomyosarcoma study (IRS-I): a preliminary report. Cancer 49:1-8,1982.

Hays DM, Soule EH, Lawrence W, Jr, : Rhabdomyosarcoma: surgical therapy in extremity lesions in children. Orthop Clin North Am 8:883-902,1977.

Jones AE, Albano EA, Lovell MA, Hunger SP: Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A. Pediatr Blood Cancer. 2010 Jun 8.

Jones TR, Norton MS, Johnstone PA, Kane E, Shin AY: Pleomorphic rhabdomyosarcoma in an adult forearm: a case report. J Hand Surg 27A:154-9,2002.

Jung JH, Lee JE, Shin JH, Lim YT, Choi HY: Lower eyelid rhabdomyosarcoma ina 3-month-old infant. J AAPOS. 2010 Jun;14(3):285-287.

Kumar S, Perlman E, Harris CA, Raffeld, M, Tsokos M: Myogenin is a specific marker for rhabdomyosarcoma: an immunohistochemical study in paraffin-embedded tissues. Mod Pathol 13:988-93,2000.

La TH, Wolden SL, Rodeberg DA, Hawkins DS, Brown KL, Anderson JR, Donaldson SS: Regional nodal involvement and patterns of spread along in-transit pathways in children with rhabdomyosarcoma of the extremity: A report from the Children's Oncology Group. Int J Radiat Oncol Biol Phys. 2010 Jun 11.

LaQuaglia MP: Extremity rhabdomyosarcoma: biological principles, staging, and treatment. Sem Surg Oncol 9:510-9,1993.

LaQuaglia MP, Ghavimi F, Penenberg D, Mandell LR, Healey JH, Hadju SI, Exelby PR: Factors predictive of mortality in pediatric extremity rhabdomyosarcoma. J Ped Surg 25:238-43,1990.

LaQuaglia MP, Heller G, Ghavimi F, Casper ES, Vlamis V, Hajdu S, Brennan MF: The effect of age at diagnosis on outcome in rhabdomyosarcoma. Cancer 73:107-17,1994.

Lawrence W, Jr, Gehan EA, Hays DM, : Prognostic significance of staging factors of the UICC staging system in children rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study (IRS III). J Clin Oncol 5:46-54,1987.

Lawrence W Jr, Hays DM, Heyn R, Tefft M, Crist W, Beltangady M, Newton W Jr, Wharam M: Lymphatic metastases with childhood rhabdomyosarcoma. A report from the Intergroup Rhabdomyosarcoma Study. Cancer 60:910-5,1987.

Lawrence W, Jr, Hays DM, Moon TE: Lymphatic metastasis with childhood rhabdomyosarcoma. Cancer 39:556-61,1977.

Little DJ, Ballo MT, Zagars GK, Pisters PW, Patel SR, El-Naggar AK, Garden AS, Benjamin RS: Adult rhabdomyosarcoma: outcome following multimodality treatment. Cancer 95:377-88,2002.

Lobe TE, Wiener ES, Hays DM, Lawrence WH, Andrassy RJ, Johnston J, Wharam M, Webber B, Ragab A: Neonatal rhabdomyosarcoma: the IRS experience. J Pediatr Surg 29:1167-70,1994.

Maurer HM, Beltangady M, Gehan E, : The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 61:209-20,1988.

Maurer HM, Moon T, Donaldson M, : Intergroup Rhabdomyosarcoma Study. Cancer 40:2015-25,1977.

McMulkin HM, Yanchar NL, Fernandez CV, Giacomantonio C: Sentinel lymph node mapping and biopsy: a potentially valuable tool in the managment of childhood extremity rhabdomyosarcoma. Pediatr Surg Int 19:453-6,2003.

Milanovic R, Zganjer M, Cizmic A, Pajic A: Rhabdomyosarcoma of the hand. Med Pediatr Oncol 37:481-2001.

Newton W, Jr, Gehan EA, Webber BL, Marsden HB, van Unnik AJ, Hamoudi AB, Tsokos MG, Shimada H, Harms D, Schmidt D, : Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study. Cancer 76:1073-85,1995.

Newton W, Jr, Soule E, Hamoudi A, : Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: Clinico-pathological correlation. J Clin Oncol 6:67-75,1988.

Ortega JA, Wharam M, Gehan EA, Ragab AH, Crist W, Webber B, Wiener ES, Haeberlen V, Maurer HM: Clinical features and results of therapy for children with paraspinal soft tissue sarcoma: a report of the Intergroup Rhabdomyosarcoma Study. J Clin Onocl 9:796-801,1991.

Pappo AS, Etcubanas E, Santan VM, Rao BN, Kun LE, Fontanesi J, Roberson PK, Bowman LC, Crist WM, Shapiro DN: A phase II trial of ifosfamide in previously untreated children and adolescents with unresectable rhabdomyosarcoma. Cancer 71:2119-25,1995.

Parham DM, Webber B, Holt H, Williams WK, Maurer H: Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasm: reults of an Intergroup Rhabdomyosarcom study project. Cancer 67:3072-80,1991.

Philippe PG, Rao BN, Rogers DA, : Sarcomas of the flexor fossae in children: is amputation necessary? J Ped Surg 27:964-7,1992.

Piersigilli F, Danhaive O, Auriti C: Blueberry muffin baby due to alveolar rhabdomyosarcoma with cutaneous metastasis. Arch Dis Child Fetal Neonatal Ed. 2010 Jun 14. 

Pittore B, Fancello G, Cossu Rocca P, Ledda GP, Tore G: Rhabdomyosarcoma: a rare laryngeal neoplastic entitiy.

Raney RB, Jr, Ragab AH, Ruymann FB, : Soft tissue sarcoma of the trunk in childhood: results of the Intergroup Rhabdomyosarcoma Study. Cancer 49:2612-6,1982.

Ranson JL, Pratt CB, Shanks E: Childhood rhabdomyosarcoma of the extremity: results of combined modality therapy. Cancer 40:2810-6,1977.

Tabrizi P, Letts M: Childhood rhabdomyosarcoma of the trunk and extremities. Am J Orthop 28:440-6,1999.

Takahashi T, Machida K, Honda N, Hosono M, Kashimada A, Murata O, Osada H, Nishimura K: Adult embryonal rhabdomyosarcoma in axilla. Radiat Med 21:135-7,2003.

Tallini G, Parham DM, Dias P, Cordon-Cardo C, Houghton PJ, Rosai J: Myogenic regulatory protein expression in adult soft tissue sarcomas: a sensitive and specific marker of skeletal muscle differentiation. Am J Pathol 144:693-701,1994.

Xarchas K, Paparassiliou N, Tsoutseous N, Burke FD: Rhabdomyosarcoma of the hand. Two case reports and a review of the literature. J Hand Surg 21B:325-9,1996.


Peer Review

OrthopaedicsOne Peer Review Workflow is an innovative platform that allows the process of peer review to occur right within an OrthopaedicsOne article in an open, transparent and flexible manner. Learn more

Instructions for Authors

Read our Instructions for Authors to learn about contributing or editing articles on OrthopaedicsOne.

Content Partner

Learn about becoming an OrthopaedicsOne Content Partner.

Academic Resources

Resources on Rhabdomyosarcoma from Pubget.

The license could not be verified: License Certificate has expired!
Orthopaedic Web Links

Internet resources validated by

The license could not be verified: License Certificate has expired!
Related Content

Resources on Rhabdomyosarcoma and related topics in OrthopaedicsOne spaces.

Page: Rhabdomyosarcoma (OrthopaedicsOne Articles)
Page: Myositis ossificans (OrthopaedicsOne Articles)
Page: Glomus tumor (OrthopaedicsOne Articles)
Page: Pigmented villonodular synovitis (PVNS) (OrthopaedicsOne Articles)
Page: Synovial sarcoma (OrthopaedicsOne Articles)
Page: Giant cell tumor of tendon sheath (OrthopaedicsOne Articles)
Page: Fibromatosis (OrthopaedicsOne Articles)
Page: Dermatofibrosarcoma protuberans (DFSP) (OrthopaedicsOne Articles)
Page: Angiosarcoma (OrthopaedicsOne Articles)
Page: Desmoplastic fibroma (OrthopaedicsOne Articles)
Showing first 10 of 149 results