Congenital Smooth Muscle Hamartoma

Congenital Smooth Muscle Hamartoma
Steven D. Billings, MD
Hematoxylin & eosin shows a haphazard arrangement of smooth muscle bundles in the dermis.
Hematoxylin & eosin shows a fascicular arrangement of smooth muscle with characteristic elongated blunt-ended nuclei.
TERMINOLOGY
Synonyms
  • Smooth muscle hamartoma
Definitions
  • Rare benign smooth muscle tumor presenting in infants
  • Characterized by disorganized proliferation of smooth muscle bundles
CLINICAL ISSUES
Epidemiology
  • Age
    • Infants
Site
  • Lumbosacral area and proximal extremities
Presentation
  • Macule or plaque
    • Indurated
    • Often hyperpigmented
    • May have coarse hairs
  • Pseudo-Darier sign
    • Piloerection induced by mechanical stimulation
  • Occasionally presents as multiple lesions
  • Occasional familial cases
Treatment
  • Surgical approaches
    • Simple excision

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Congenital Smooth Muscle Hamartoma

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