Congenital Smooth Muscle Hamartoma
Steven D. Billings, MD
Key Facts
Terminology
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Rare lesion presenting in infants, characterized by disorganized proliferation of benign smooth muscle bundles in dermis, often involving subcutis
Clinical Issues
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Infants
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Most common in lumbosacral area
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Macule or plaque
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Often hyperpigmented, may have coarse hairs
Microscopic Pathology
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Benign smooth muscle bundles
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Haphazard arrangement
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Thin fascicles rather than solid growth pattern
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No atypia
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No mitoses
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No necrosis
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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
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Smooth muscle hamartoma
Definitions
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Rare benign smooth muscle tumor presenting in infants
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Characterized by disorganized proliferation of smooth muscle bundles
CLINICAL ISSUES
Epidemiology
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Age
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Infants
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Site
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Lumbosacral area and proximal extremities
Presentation
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Macule or plaque
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Indurated
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Often hyperpigmented
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May have coarse hairs
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Pseudo-Darier sign
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Piloerection induced by mechanical stimulation
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Occasionally presents as multiple lesions
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Occasional familial cases
Treatment
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Surgical approaches
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Simple excision
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