Rhabdomyoma

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Rhabdomyoma
Diagnosis in short

Rhabdomyoma. H&E stain. (WC)

LM spider cells (large polygonal cells (~10-20x RBC diameter), abundant cytoplasm with clearing)
Subtypes fetal rhabdomyoma, adult rhabdomyoma
LM DDx granular cell tumour, hibernoma, oncocytoma
IHC desmin +ve, actin +ve, myoglobin +ve, S-100 -ve
Site heart (see cardiac tumours), head and neck

Prevalence rare
Prognosis benign

Rhabdomyoma a benign muscle tumour. Often seen in the context of tuberous sclerosis.

General

Gross

  • Solid, white/tan colour.

Image:

Microscopic

Features - cardiac:[3]

  • Spider cells:
    • Large polygonal cells (~10-20x RBC diameter):
      • Abundant cytoplasm filled with glycogen.

Note:

DDx:

Images

www

IHC

Features:[1]

  • Desmin +ve (21 of 21 adult cases[4]).
  • Myogoblin +ve.
  • Actin +ve (21 of 21 adult cases[4]).
  • Vimentin -ve/+ve.
  • S-100 -ve.
    • Positive in granular cell tumour and hiberoma.

See also

References

  1. 1.0 1.1 1.2 Hansen, T.; Katenkamp, D. (Nov 2005). "Rhabdomyoma of the head and neck: morphology and differential diagnosis.". Virchows Arch 447 (5): 849-54. doi:10.1007/s00428-005-0038-8. PMID 16133368.
  2. Neri, M.; Di Donato, S.; Maglietta, R.; Pomara, C.; Riezzo, I.; Turillazzi, E.; Fineschi, V. (Dec 2012). "Sudden death as presenting symptom caused by cardiac primary multicentric left ventricle rhabdomyoma, in an 11-month-old baby. An immunohistochemical study.". Diagn Pathol 7: 169. doi:10.1186/1746-1596-7-169. PMID 23206573.
  3. URL: http://www.brown.edu/Courses/Digital_Path/systemic_path/cardio/rhabdomyoma.html. Accessed on: 19 October 2011.
  4. 4.0 4.1 Kapadia, SB.; Meis, JM.; Frisman, DM.; Ellis, GL.; Heffner, DK.; Hyams, VJ. (Jun 1993). "Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study.". Hum Pathol 24 (6): 608-17. PMID 8505039.