Tumors of the muscle
MYOFIBROMATOSIS
Definition:
Infantile myofibromatosis is a mesenchymal disorder of infancy characterized
by the formation of tumors in skin, subcutaneous tissue, muscle,
bone and viscera.
Prevalence:
Until now 175 cases of Infantile Myofibromatosis have been published in the
English literature. The incidence of soft tissue tumors in newborns has been
estimated to be 1 in 150,000 live births.
Etiology: Unknown. An association to excess estrogen
production has been proposed.
Diagnosis: The diagnosis relies
on identification of solid tumor masses of variable size at different
locations, who are well circumscribed and sometimes well encapsulated.
Prognosis: The prognosis depends
on the presence of visceral involvement. Of the 175 collected cases in the
literature, 27 (15.5%) died. All deaths were associated with visceral
involvement.
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