Soft tissue

Skeletal muscle

Neuromuscular hamartoma



Topic Completed: 1 November 2012

Minor changes: 2 December 2020

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Neuromuscular hamartoma

Vijay Shankar, M.D.
Page views in 2020: 1,077
Page views in 2021 to date: 1,139
Cite this page: Shankar V. Neuromuscular hamartoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueneuromuscularhamartoma.html. Accessed October 16th, 2021.
Definition / general
  • Rare developmental lesion of mature skeletal muscle and nerve
Terminology
  • Not a WHO diagnosis
  • Also called benign triton tumor (malignant triton tumor refers to rhabdomyosarcoma plus MPNST), neuromuscular choristoma
Clinical features
  • Usually < 2 years old, affects brachial plexus or sciatic nerve
  • May involve large cranial nerve trunks and present as intracranial mass
  • Very rarely involves peripheral nerve and presents as small nodules
  • Aggressive behavior
Case reports
Treatment
  • Biopsy for diagnosis plus observation, may develop fibromatosis after biopsy or complete excision
  • May recur, mandating regular followup after excision
Gross description
  • Circumscribed, firm, gray-brown-white, multinodular, attached to nerve
Microscopic (histologic) description
  • Multiple nodules, each 3 - 5 mm, separated by narrow bands of connective tissue
  • Nodules are composed of fascicles of striated muscle of varying size with nerve fibers (myelinated or not) within same perimysial fibrous sheath
  • Stroma may be more cellular with bland spindle cells and resemble fibromatosis
Microscopic (histologic) images

AFIP images

Nodules of
skeletal muscle
and neural
elements

Nodules contain
fascicles and nerve
fibers in perimysial
fibrous sheath

Rare case with haphazardly distributed skeletal muscle and nerve fibers, cellular stroma and bland spindle cells with scant cytoplasm and oval nuclei

Nerve fibers are S100+

Positive stains
Differential diagnosis
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