Case report Desmoplastic fibroma of the mandible associated with pathological fracture in a pediatric patient Ben-Zion Joshua a , Eldad Silberstein b , Victor Diomin c , Lipa Bodner d, * a Department of Otolaryngology, Head and Neck Surgery, Soroka Medical Center and Ben Gurion University of the Negev, Beer-Sheva, Israel b Department of Plastic and Reconstructive Surgery, Soroka Medical Center and Ben Gurion University of the Negev, Beer-Sheva, Israel c Institute of Pathology, Soroka Medical Center and Ben Gurion University of the Negev, Beer-Sheva, Israel d Department of Oral and Maxillofacial Surgery, Soroka Medical Center and Ben Gurion University of the Negev, Beer-Sheva, Israel 1. Introduction Desmoplastic fibroma (DF) is a rare, benign, locally aggressive tumor of bone. Any bone can be affected, but the mandible is the most frequently affected site. It is recognized as the osseous manifestation of the soft tissue fibromatosis. The involvement of multiple bone sites, such as the femur, pelvic bones, radius, and tibia have been reported [1–4]. The first report on mandibular involvement is attributed to Griffith and Irby in 1965, and since then numerous cases have been described [5–7]. DFs occur most frequently in young adults and present as a slow-growing, painless swelling. It is considered rare in children. The surgical approach to DF is controversial. Variable treatment modalities, ranging from conservative enucleation to a complete en bloc resection, with different outcomes, have been implemen- ted [6]. Radiotherapy or chemotherapy is indicated, for patients with non-resectable tumors [4]. The purpose of this article is to report on a case of DF occurring in the mandible of a 12-year-old female patient, its diagnostic work-up, initial conservative surgical management, complication by a pathological fracture and the reconstruction by a vascularized free fibula flap. 2. Case report A 12-year-old female presented with a swelling on her face which was first noticed two months previously. Extra-oral examination revealed a mild facial asymmetry and swelling of the left mandible. Oral examination showed some tenderness in the floor of the mouth adjacent to the left mandible. All the permanent teeth were intact and stable. The remainder of the orofacial examination was within normal limits. The patient’s medical history was noncontributory. However, her mother had many abortions and died during pregnancy for an unknown reason. There is no other family history of cancer or skin disease. A panoramic radiograph revealed a unilocular radiolucent lesion with disruption of the left inferior cortex of the mandible. The lesion extended from the inferior border in the premolar- molar region (Fig. 1A). Axial CT of the mandible demonstrated a lesion at the posterior mandible that is extending toward the soft tissue adjacent to the lingual side of the mandible (Fig. 1B). Under general anesthesia, a biopsy from the center of the lesion was obtained and submitted for histological evaluation. Microscopic examination revealed an ill defined spindle cell proliferation in a collagenous background with varying degrees of myxoid changes. The arrangement of the spindle cells varied with International Journal of Pediatric Otorhinolaryngology Extra 9 (2014) 60–63 A R T I C L E I N F O Article history: Received 25 September 2013 Received in revised form 9 January 2014 Accepted 11 January 2014 Keywords: Mandible Neoplasia Bone tumor Desmoplastic fibroma Children A B S T R A C T Desmoplastic fibroma (DF) is a benign, locally aggressive, bone tumor that rarely presents in the facial bones. It is considered an intraosseous counterpart of the soft tissue fibromatoses. It is very rare in children. The present report describes the clinico-pathological and radiographic features of a desmoplastic fibroma of the mandible in a 12-year-old female who presented with a few months history of an expanding, painless mass along the left inferior border of the mandible. The patient underwent a resection with wide margins of the inferior border of the mandible that was followed by a pathological fracture. The fracture was reduced and the mandible was reconstructed by a vascularized free fibula flap. Conservative surgical approach and long term follow up is an effective treatment modality for mandibular DF in children. ß 2014 Elsevier Ireland Ltd. All rights reserved. * Corresponding author at: Department of Oral and Maxillofacial Surgery, Soroka Medical Center, P.O. Box 151, Beer-Sheva 84101, Israel. Fax: +972 8 6403651. E-mail addresses: lbodner@bgu.ac.il, benbat7@yahoo.com (L. Bodner). Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology Extra jo ur n al ho m ep ag e: ww w.els evier .c om /lo cat e/ijp o r l 1871-4048/$ – see front matter ß 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.pedex.2014.01.004