Infantile Myofibromatosis: A Review of Clinicopathology with Perspectives on New Treatment Choices

Abstract

The fibromatoses are a heterogeneous group of disorders characterized by proliferation of fibroblasts. Infantile myofibromatosis is a variant that is distinctive because of its multicentric origin, appearance at birth, and cellular composition, which is predominantly myofibroblasts. We treated a patient with infantile myofibromatosis with the interesting clinical presentation of a linear lesion involving the left arm and shoulder, and aggressive hepatomegaly with jaundice secondary to fibroblastic infiltration of the common bile duct and gallbladder. Diagnosis was confirmed histologically and ultrastructurally. Excision of the cutaneous lesion was facilitated by tissue expansion of uninvolved regional tissue.

Spontaneous Remission of Solitary-Type Infantile Myofibromatosis

“…In the case of solitary-type infantile myofibromatosis, spontaneous regression can be expected [3, 4]. In contrast, a quarter of the cases with the multicentric form may have visceral involvement and can be life-threatening [2, 4, 5]. The solitary type of infantile myofibromatosis is usually benign and is typically found in the dermis, subcutis, or deep soft tissues.…”

“…The prognosis is excellent in the solitary type, which is limited in the skin, muscle, and subcutaneous lesions [2, 3, 4]. In contrast, the multicentric form of infantile myofibromatosis, which has visceral involvement, can be life-threatening [4, 5]. The solitary type is usually benign and the recurrence rate is low at 10%.…”

Non-Malignant Fibrosing Tumors in the Pediatric Hand: A Clinicopathologic Case Review

“…Infantile myofibromatosis is one of the most commonly occurring tumors of the neonatal period [14,28], presenting in both solitary and multicentric forms.…”

Solitary infantile myofibroma compromising the airway

“…The prognosis is excellent if limited to skin and bone but some may have an aggressive clinical presentation. Patients with visceral involvement usually have multiple lesions involving internal organs and have an 80% mortality [7].…”