Infantile Myofibromatosis: Report of Nine Patients

Abstract

Infantile myofibromatosis is a rare fibrous tumor of infancy that can be solitary or multiple. Although most of the cases are limited to the skin, in some instances systemic involvement can be present. Solitary tumors limited to the skin usually present a good prognosis with spontaneous regression. We performed a retrospective observational review of the clinical and pathologic characteristics of nine patients diagnosed as having infantile myofibromatosis, followed during a 10-year period in a Pediatric Dermatology Department.

Whole-body magnetic resonance imaging in the diagnosis and follow-up of multicentric infantile myofibromatosis: A case report

“…Solitary or multicentric disease without visceral involvement is usually associated with a very good prognosis with spontaneous regression, whereas the multi- centric form with visceral spread requires aggressive surgery and/or radiotherapy and chemotherapy; however, the prognosis is often poor, particularly when the lungs are involved (1,4,5). A solitary visceral form has rarely been reported, and its prognosis and treatment depend on the involved organ and associated symptoms (7).…”

“…Myofibromatosis clinically presents as single mass or diffuse multiple nodules that may develop anywhere, from the skin to subcutaneous tissues or viscera, characterized by spindle-shaped cells, SMA and vimentin positivity, related to myofibroblast proliferation; the lesions are often associated with richly vascular central areas, hemangiopericytomalike, frequently displaced by sheets of hyalinization or necrosis and calcification due to extensive apoptosis (3)(4)(5). According to several authors, hemangiopericytoma and infantile myofibromatosis are a continuous spectrum of a single myofibroblast disease (8).…”

“…The clinical behavior of myofibromatosis may vary between a benign solitary or multicentric form, with involvement of muscles, bones and subcutaneous tissues, and a diffuse multicentric form with visceral involvement and a worse prognosis (1,4). Conservative management and observation are preferred for the solitary or multicentric forms without visceral involvement, as they usually undergo spontaneous regression; occasionally, surgery is suggested to reduce eventual mass effect symptoms.…”

“…Examination of a pathological specimen is mandatory for ruling out malignancies that may require different management (4).…”

“…Conservative management and observation are preferred for the solitary or multicentric forms without visceral involvement, as they usually undergo spontaneous regression; occasionally, surgery is suggested to reduce eventual mass effect symptoms. Aggressive treatment with radiotherapy, chemotherapy and radical surgery is used for disseminated forms with visceral involvement (1,4,5).…”