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Infantile myofibromatosis in Infant/Neonate
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Infantile myofibromatosis in Infant/Neonate

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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Infantile myofibromatosis (Solitary Type) : Head/neck, Skin ulcer, Trunk, Solitary lesion
Solitary Type
Infantile myofibromatosis is a rare mesenchymal disorder in which benign fibroblasts proliferate and invade adjacent structures. A majority of cases present within the first 2 years of life (90%), and 60% present at birth. There are two forms of the disease, solitary and multicentric; the latter can have visceral involvement. The solitary form accounts for a majority of cases (70%). The multicentric form appears more often at birth and in females.

In the solitary form, lesions are seen in the skin, subcutaneous tissue, or muscle. In the multicentric form, lesions can be seen in the bone and viscera. Skeletal lesions occur in more than half the cases, with the skull, femur, tibia, spine, and ribs involved most commonly.

Without visceral involvement the prognosis is favorable: it is typically benign and self-limited and has a low recurrence rate. The multicentric form with visceral involvement is less favorable, with a 73% fatality rate, typically due to cardiopulmonary failure.

Familial cases are rare, but autosomal dominant and recessive inheritance have been reported.

Most of these lesions in the past were called hemangiopericytomas.

Codes

ICD10CM:
Q89.8 – Other specified congenital malformations

SNOMEDCT:
254146000 – Infantile myofibromatosis

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Last Updated:01/17/2022
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Infantile myofibromatosis in Infant/Neonate
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A medical illustration showing key findings of Infantile myofibromatosis (Solitary Type) : Head/neck, Skin ulcer, Trunk, Solitary lesion
Clinical image of Infantile myofibromatosis - imageId=72924. Click to open in gallery. caption: 'A tiny pink papule with scant overlying scale on the finger.'
A tiny pink papule with scant overlying scale on the finger.

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