Heart

Malignant tumors

Rhabdomyosarcoma



Topic Completed: 1 December 2013

Minor changes: 4 January 2021

Copyright: 2007-2021, PathologyOutlines.com, Inc.

PubMed Search: Rhabdomyosarcoma [title] heart pathology

Related Topics: Alveolar rhabdomyosarcoma, Embryonal rhabdomyosarcoma

Nat Pernick, M.D.
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Cite this page: Pernick N. Rhabdomyosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/hearttumorrms.html. Accessed October 22nd, 2021.
Definition / general
  • Sarcoma with striated muscle differentiation
  • Rare, 4 - 7% of cardiac sarcomas but most common cardiac malignancy in infants and children
  • Occurs throughout heart, not just in left atrium as with other sarcomas
  • Usually children / young adults
  • Metastases to lung, regional nodes, CNS, GI tract, kidney, other
Case reports
Treatment
  • Resection with atrial reconstruction; possibly chemotherapy
Gross description
  • Bulky, invasive, central necrosis
  • Often 10 cm or more
  • Almost always myocardial involvement
  • May involve mitral valve or atrial wall
Microscopic (histologic) images

AFIP images
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Tumor of 1 year old child studded
epicardial surfaces and infiltrated both
atria; rhabdomyoblasts were scarce but
have abundant eosinphilic cytoplasm
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Desmin+



Images hosted on other servers:
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Metastatic tumor to brain

Cytology description
  • Tumor cells have cross striations
Positive stains
Electron microscopy description
  • Thick and thin filaments
  • Well formed Z bands
  • Frequent glycogen granules in clumps and mitochondria
  • Nuclei are lobulated with variable condensed chromatin
  • Variable A and I bands
Electron microscopy images

AFIP images
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Z bands indicate myofibrils within tumor cells

Differential diagnosis
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