Other tumors

Topic Completed: 1 June 2014

Minor changes: 11 March 2020

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PubMed Search: Rhabdomyosarcoma [title] ovary

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Cite this page: Gupta N. Rhabdomyosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorrms.html. Accessed September 29th, 2020.
Definition / general
  • First described by Virchow et al in 1850 (Proc R Soc Med 1929;22:320)
  • Rare and aggressive ovarian tumor
  • Presents as disseminated tumor in neonates or young children, with difficulty determining primary tumor site (other pediatric tumors with similar presentations include infantile myofibromatosis, neuroblastoma, lymphoma, malignant rhabdoid tumor)
  • Subtypes in children and 5 year survival: embryonal (49% of cases, 66%), alveolar (31% of cases, 88%), botryoid (6% of cases, 95%) and spindle cell (3% of cases, 88%)
  • Head and neck, genitourinary, extremities
  • Rhabdomyoblasts in the ovary may arise from either uncommitted stromal fibroblasts or from fibroblasts of endometriotic stroma (Gynecol Oncol 1983;15:325)
Clinical features
  • Abdominal pain or swelling
  • Final diagnosis by histology and immunohistochemistry
Radiology description
  • USG: solid cystic adnexal mass
Prognostic factors
Case reports
  • Aggressive combination therapy including surgery, radiation and chemotherapy [VAC Regimen-vincristine (V), actinomycin D (A) and cyclophosphamide (C)]
Gross description
Microscopic (histologic) description
  • Mostly embryonal, rarely alveolar rhabdomyosarcoma
  • Embryonal subtype is characterized by spindled cells with a stroma rich appearance
  • Alveolar RMS has small, round, densely appearing cells lined up along spaces reminiscent of pulmonary alveoli
Microscopic (histologic) images

AFIP images

Metastatic alveolar rhabdomyosarcoma

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Embryonal and alveolar subtypes

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Embryonal (site unspecified)

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H&E, desmin (fig 3A) and myogenin (fig 3B)

Cytology description
  • Highly cellular, with background reactive mesothelial cells and lymphocytes
  • Tumor cells are bizarre, discohesive giant cells with pleomorphic hyperchromatic nuclei, variable eosinophilic cytoplasm with occasional vacuoles and frequent mitotic figures
Positive stains
Negative stains
Electron microscopy description
  • Rhabdomyogenesis recapitulates skeletal muscle differentiation during early fetal life
Molecular / cytogenetics description
  • t(2;13)(p36;q14) found in 60% of alveolar subtypes (PAX3 and FKHR genes); poor prognosis
  • t(1;13)(p36;q14) involves PAX7 and FKHR genes, associated with better prognosis
Differential diagnosis
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