Heart & vascular pathology
Malignant tumors
Rhabdomyosarcoma
Author: Nat Pernick, M.D.
Last author update: 1 December 2013
Last staff update: 4 January 2021
Copyright: 2007-2023, PathologyOutlines.com, Inc.
PubMed Search: Rhabdomyosarcoma [title] heart pathology
Related Topics: Alveolar rhabdomyosarcoma, Embryonal rhabdomyosarcoma
Table of Contents
Definition / general | Case reports | Treatment | Gross description | Microscopic (histologic) images | Cytology description | Positive stains | Electron microscopy description | Electron microscopy images | Differential diagnosisCite this page: Pernick N. Rhabdomyosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/hearttumorrms.html. Accessed September 26th, 2023.
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
- 20 year old man with hypereosinophilia (Am J Hematol 2003;74:64)
- 28 year old man with huge biatrial tumor (Cardiovasc Pathol 2006;15:354)
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
epicardial surfaces and infiltrated both
atria; rhabdomyoblasts were scarce but
have abundant eosinphilic cytoplasm
Desmin+
Images hosted on other servers:
Metastatic tumor to brain
Cytology description
- Tumor cells have cross striations
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
Z bands indicate myofibrils within tumor cells
Differential diagnosis
- Leiomyosarcoma: spindle cells with dispersed glycogen, although also desmin+
- Undifferentiated sarcoma: desmin negative
