ABSTRACT
This paper reports one case, of an ameloblastic fibro-odontosarcoma (AFOS) affecting the mandible, in a 12-year-old girl. This neoplasm is a rare odontogenic neoplasm. To the authors' knowledge this is the fifteenth case of AFOS reported in English. The patient's chief complaint was a swelling in the face for 6 months. An incisional biopsy was performed diagnosing the case as an ameloblastic fibroma. After radiography ameloblastic fibro-odontoma was diagnosed. Computed tomography was performed and a stereolithography model made to plan the surgical procedures. A hemimandibulectomy followed by a vascularized fibular flap was then proposed. The surgery was uneventful. Microscopic features diagnosed an AFOS. After 23 months of close follow-up there is no sign of recurrence or metastasis. Dental implants were recently placed in the fibular flap.
Subject(s)
Ameloblastoma/pathology , Mandibular Neoplasms/pathology , Odontoma/pathology , Sarcoma/pathology , Ameloblastoma/surgery , Child , Dental Implants , Female , Humans , Jaw, Edentulous, Partially/rehabilitation , Mandible/surgery , Mandibular Neoplasms/surgery , Odontoma/surgery , Sarcoma/surgery , Surgical Flaps , Treatment OutcomeABSTRACT
OBJECTIVE: To discuss the diagnosis and the etiology of a bilateral urologic anomaly in a patient with Soto's Syndrome (cerebral gigantism). To alert the pediatric physicians and urologists about the coexistence of these two malformations. RESULTS: A three month old boy, born of a 35 week uncomplicated first pregnancy by cesarian, with phenotypic, radiologic and metacarpophalangeal profile of Soto's Syndrome was admitted to the Hospital because of hyperpnea, tachycardia and 39o C recurrent fever. The culture of urine disclosed 500.000 col/ml. Cistouretrographic study showed bilateral megaloureter and hydronephrosis. Vesico-ureteral bilateral reflux was diagnosed and antibiotic therapy was administered. Patient's follow up was excellent and was discharged taking preventive nitrofurantoin. CONCLUSIONS: As in other cases of urinary tract infection, pediatric physicians must consider urologic anomalies in children with cerebral gigantism who present the clinical presentation of Soto's Syndrome. Autonomic Nervous System development failure in ureteral muscles could be an alternative etiology.