RHABDOMYOMATOUS MESENCHYMAL HAMARTOMA

By Gary M. White, MD

Rhabdomyomatous Mesenchymal Hamartoma


Rhabdomyomatous Mesenchymal Hamartoma (RMH) is an extremely rare congenital lesion made up of striated muscle. Because of this, it may move or contract when the baby feeds.

Clinical

Initially described as striated muscle hamartoma, this rare disease presents as a solitary or multiple, congenital, facial papulonodule which may be elongated or snake-like in appearance. It contains striated muscle which may cause movement or contraction when the infant feeds or when the older child contracts his facial muscles. Midline lesions of the face and neck are the most common presentations.

Case reports describe lesions appearing in a variety of ways including a purple "vascular" lesion on the lip, a bilobed pedunculated tag on the neck, a papule in the tongue, a subcutaneous nodule, and a 2 cm red polyploid mass emanating from the vagina of a newborn. Multiple lesions have been described. Rhabdomyomatous mesenchymal hamartoma is usually an isolated finding, but various congenital abnormalities have been associated in case reports.

Associations

Associated congenital anomalies, including ocular abnormalities, thyroglossal duct sinus, and amniotic band syndrome, occur in approximately 40% of cases [Peds Derm 2016;33;e36].

Treatment

Spontaneous regression has occurred but is uncommon [West Indian Med J 2009:58;607]. Surgical excision, if feasible, may be done. Since no cases of malignant transformation have been reported, observation is appropriate [Clin and Exp Derm 2013;38;439].

References

A 15-day-old boy born at 38 weeks of gestation presented with a skin-covered, smooth, polypoid papule measuring 0.7 × 0.5 × 0.5 cm protruding from the chin. JAAD Case Rep. 2016 May; 2(3): 222–223

Peds Dermatology 1986;3;153

Picture of the Month. Am J Dis Child. 1989; 143(8):963-964

Am J Dermatopath 1990;12;485

Am J Dermatopath 1989;11;568

Additional Photos

Rhabdomyomatous Mesenchymal Hamartoma Rhabdomyomatous Mesenchymal Hamartoma

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.