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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