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Muscular Tumour

Pathology of Rhabdomyomatous Mesenchymal Hamartoma

Dr Sampurna Roy MD  

 

                                                                                                                      

 

Rhabdomyomatous mesenchymal hamartoma is a rare congenital lesion of the dermis and soft tissues which was first reported in 1986.

This entity exists under various names including striated muscle hamartoma, congenital midline hamartoma, and hamartoma of cutaneous adnexa and mesenchyme.

Rhabdomyomatous mesenchymal hamartoma is often associated with congenital abnormalities, such as craniofacial defects, Goldenhar syndrome, Dellemane syndrome, and amniotic band syndrome.

Age: Usually presents in neonates or infants.

Cases have been reported in adults.

Site: Presents as one or more small cutaneous polyps in the head and neck region.

Microscopic features:  

White LR, Agrawal V, Sutton L, Balbosa AC. Rhabdomyomatous Mesenchymal Hamartoma of the Face Causing Trigeminal Neuralgia. The American Journal of Case Reports. 2015;16:338-340. doi:10.12659/AJCR.893719.

The lesion consists of a disordered and varied collection of mature adipose tissue, skeletal muscle, adnexal elements and nerve bundles.

Bundles of skeletal muscle is present in the reticular dermis which may extend into the subcutis.

This lesion may be associated with other congenital anomalies and anomaly syndromes hence systemic evaluation of patients diagnosed with this entity has been recommended.

 

Further reading:

Rhabdomyomatous mesenchymal hamartoma: a plaque-type variant in an adult.

Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst.

Rhabdomyomatous mesenchymal hamartoma: an unusual dermal entity with a report of two cases and a review of the literature.

Rhabdomyomatous mesenchymal hamartoma of the eyelid: report of a case and literature review.

Rhabdomyomatous mesenchymal hamartoma. 

Rhabdomyomatous mesenchymal hamartoma of skin. 

Multiple rhabdomyomatous mesenchymal hamartomas of skin.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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