Regional Lymph Node Enlargement in Clinically Severe Cherubism.

ABSTRACT: Cherubism is a rare benign autosomal dominant disorder characterized by progressive, painless, bilateral enlargement of the mandible and/or maxilla because of bone replacement by fibrotic stromal cells and osteoclast-like cells forming multilocular cysts. The lesions typically stabilize and regress after puberty. We present a 14-year-old male with severe familial cherubism. Bilateral mandibular enlargement began around age 4 and progressed until puberty, affecting his speech and mastication without subsequent involution. Composite mandibulectomy and mandible reconstruction with fibula free flap technique improved functionality and cosmesis. Histology was consistent with the diagnosis of cherubism, showing large areas of bland spindle-cell fibrous tissue and moderately abundant collagen and

SUBMITTER: Chen Wongworawat Y 

PROVIDER: S-EPMC6611013 | biostudies-literature | 2019 Jan-Dec

REPOSITORIES: biostudies-literature