Human ; Male ; Adult ; Young Adult ; Hemophilia A ; Neoplasms ; Thigh ; Lower Extremity

Primary sinonasal ameloblastoma is an extremely rare odontogenic epithelial tumor histomorphologically identical to its gnathic counterparts but with distinct epidemiologic and clinicopathologic characteristics. We present a case of a 46-year-old female with a one year history of recurrent epistaxis, nasal obstruction and frontonasal headache. Clinical examination, CT scan and subsequent surgical excsion revealed an intranasal mass attached to the lateral nasal cavity with histomorphologic features of ameloblastoma and was signed out as extragnathic soft tissue ameloblastoma of the sinonasal area. Extraosseous extragnathic primary sinonasal ameloblastoma are rare but do occur and should be distinguished from infrasellar craniopharyngiomas.

Human ; Female ; Middle Aged ; AMELOBLASTOMA ; ODONTOGENIC TUMORS ; CALCIFYING EPITHELIAL ODONTOGENIC TUMOR ; NEOPLASMS BY HISTOLOGIC TYPE ; NEOPLASMS ; EPISTAXIS ; RECURRENCE ; DIAGNOSTIC IMAGING ; MEDICAL IMAGING ; DIAGNOSTIC TECHNIQUES AND PROCEDURES ; TOMOGRAPHY SCANNERS, X-RAY COMPUTED

Objective. Classic high-grade osteosarcoma is uncommon in preadolescents (less than or equal to 10 years of age). The possibilities of clinicopathologic differences from the typical adolescent osteosarcoma patient have been raised. We sought to compare the presentation, treatment and survival of this subgroup of patients with published rates in order to determine if there is a need to use a treatment regimen different from that for regular adolescent osteosarcoma patients. Methods. Records of the University of the Philippines-Musculoskeletal Tumor Unit (UP-MuST) over a 15-year period (1993-2008) were reviewed and data collected on patients 10 years and younger with biopsy-proven classic high-grade intramedullary osteosarcoma who underwent complete treatment by the Unit. Demographics and survival rates were then compared with published rates for preadolescent and regular adolescent osteosarcoma cases. Results. There were fourteen patients; (6M:8F; age: 4-10 years). The most common presentation was a painful mass in the distal femur (8); the tumors most commonly had osteoblastic histology (12). Treatment consisted of neoadjuvant chemotherapy, wide surgical excision through ablation (9) or limb-saving surgery (5), and postoperative chemotherapy. There was a good histologic response (over 90% tumor necrosis) in four patients. Seven patients are ANED (alive no evidence of disease) 25 to 186 months after diagnosis. Five-year survival estimate is 52%, compared to a dismal 5 to 10% 15 years ago. Conclusion. Clinicopathologic presentation, clinical course, and overall survival in this subgroup of patients are comparable with published results for both preadolescent and adolescent osteosarcoma patients. There is no need to alter the present treatment regimen for this group of young patients.
Human ; Male ; Female ; Child ; Child Preschool ; OSTEOSARCOMA ; THERAPEUTICS ; THERAPY ; NEOPLASMS ; NEOPLASMS BY HISTOLOGIC TYPE ; NEOPLASMS, CONNECTIVE AND SOFT TISSUE ; NEOPLASMS, CONNECTIVE TISSUE ; NEOPLASMS, BONE TISSUE ;