Soft tissue

Peripheral nerve

Other benign

Benign triton tumor / neuromuscular choristoma



Last author update: 1 November 2012
Last staff update: 25 January 2023

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PubMed Search: Neuromuscular hamartoma

Vijay Shankar, M.D.
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Cite this page: Shankar V. Benign triton tumor / neuromuscular choristoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueneuromuscularhamartoma.html. Accessed February 3rd, 2023.
Definition / general
  • Rare developmental lesion of mature skeletal muscle and nerve
Terminology
  • Also called neuromuscular hamartoma
  • Malignant triton tumor refers to rhabdomyosarcoma plus MPNST)
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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