Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management.

A case is reported of a 8-year- old girl with desmoid fibroma in the mandible who presented at the Department of Pediatric Dentistry with a swelling measuring 4×4 cm on the lower edge of the right mandible which had appeared a few months earlier and slowly developed.

Desmoid-type fibromatosis (DF) is a rare locally aggressive monoclonal proliferation of myofibroblasts lacking metastatic capacity. It may be observed in nearly every part of the body. Considering the unpredictable behavior and the heterogeneity of this disease, we propose a treatment algorithm approved by the French and the Italian Sarcoma Group, based on a front-line wait and see approach and subsequent therapy in the case of progression.

This paper describes a case of a sporadic cystic DT of the pancreas managed successfully with central pancreatectomy, with no signs of recurrence 40 mo after surgery.

Dermatofibrosarcoma protuberans (DFSP) is a locally invasive soft tissue tumor with rare malignant potential, but a high tendency for local recurrence. We present 2 cases of DFSP involving the head and neck requiring tissue expansion for reconstruction.

We report a case of familial adenomatous polyposis( FAP) presenting with thyroid cancer. A 24-year-old woman, on histological examination, was diagnosed as having the cribriform-morular variant of papillary thyroid carcinoma, which is known to be associated with FAP. After total thyroidectomy, total proctocolectomy and excision of the tumor arising from the mesentery of the small intestines were performed. The histological diagnosis was FAP with a desmoid tumor arising from the mesentery.

Desmoplastic fibroma is a rare benign primary bone tumor that is histologically similar to the soft tissue desmoid tumor. It most often involves the mandible, large long bone or iliac bone. Desmoplastic fibroma in a toe has been extremely rarely reported.