Fibromatosis Arising in Association with Neuromuscular Hamartoma of the Mandible

Abstract

Aggressive fibromatosis is a benign but locally-aggressive tumor, which most often affects the muscles of the shoulder, the pelvic girdle, and the thigh. It usually affects adolescents and young adults. Desmoplastic fibroma, considered the bone counterpart of soft tissue fibromatosis, is a rare tumor that usually affects the metaphyseal or diaphyseal portions of long bones or, less commonly, the jaw. Neuromuscular hamartoma, a rare developmental lesion composed of mature elements of both striated muscle and nerve, is usually diagnosed in infants and children and affects large nerve trunks. Rarely, it can affect the head and neck region. Occasional cases showing an association between aggressive fibromatosis and neuromuscular hamartoma have been reported in the literature. Here we present a unique case of an adult patient with desmoplastic fibroma of the mandible in association with neuromuscular hamartoma.

A multicentric case study of fibroblastic and myofibroblastic oral spindle cell lesions

“…have reported immunopositivity for beta-catenin in DF cases. [ 17 18 19 ] Similar to one of our cases, Hauben et al , Wood et al . and Kahraman et al .…”

Desmoplastic Fibroma of the Mandible: A Series of Three Cases and Review of Literature

“…In 2005, Hauben et al [10] reported two cases of mandibular DFs that stained positively with b-catenin; however only one demonstrated nuclear positivity and thereby deemed true positive labeling. Multiple cases of gnathic DF have since been shown to be reactive to b-catenin [10,17,18]. Most recently, Gondak et al [19] reported a maxillary lesion with negative reactivity to b-catenin (Table 1).…”

Periosteal reaction as a crucial radiographic finding for desmoplastic fibroma of the jaw bone in children: A case report

“… 20 21 Desmoid-type fibromatoses and adenocarcinoma have positive results for β-catenin. 20 Multiple cases of desmoplastic fibromas of the jaw bones have been reactive to β-catenin, 22 23 24 while some have not been. 12 22 25 …”