Low-grade central osteosarcoma of the mandible: a case study report.
ABSTRACT Low-grade central osteosarcoma is a rare type of osteosarcoma with peculiar clinical radiographic and microscopic features. The aim of this article is to report and discuss a case of low-grade central osteosarcoma in the mandible of a 42-year-old woman. The patient reported sensing mild pain and tooth mobility for a period of 4 years, despite continuous dental treatment. Radiographic evaluation showed a mixed radiopaque/radiolucenct lesion in the body, ramus, coronoid process, and condyle of the left side of the mandible. Destruction of the mandibular cortex in the area was also observed. After incisional biopsy, the patient underwent hemimandibulectomy. Microscopic findings showed a tumor exhibiting spindle cells with nuclear hyperchromasia and no mitotic activity, irregular osteoid formation, and soft tissue infiltration. The immunohistochemical analysis of the expression of Ki-67, Cyclin B1, and PCNA proteins (cellular proliferation markers) revealed a very low Ki-67+ and Cyclin B1+ cell index (mean 7% and 3%, respectively), but a moderate number of PCNA+ cells (mean 49%). The 2 years of clinical and imaging postoperative follow-up showed no evidence of recurrence. Clinicians should be aware of these lesions, because histopathologicially low-grade central osteosarcoma may be misinterpreted as fibrous dysplasia.
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ABSTRACT: Cemento-ossifying fibromas are benign tumors, and, although cases of an aggressive type have been reported, no cases of cemento-ossifying fibroma transforming into osteosarcoma have been documented previously. Low-grade osteosarcoma is a rare type of primary bone tumor, representing 1%-2% of all osteosarcomas. A 45-year-old female patient was diagnosed with cemento-ossifying fibroma, treated with mass excision several times over a period of two years and eight months, and followed up. After biopsy gathered because of signs of recurrence, she was diagnosed with low-grade osteosarcoma. The patient underwent wide excision, segmental mandibulectomy, and reconstruction with fibula free flap. The aim of this report is to raise awareness of the possibility that cemento-ossifying fibroma can transform into osteosarcoma and of the consequent necessity for careful diagnosis and treatment planning.
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ABSTRACT: Low-grade central osteosarcoma is a rare type of osteosarcoma, representing fewer than 2% of all osteosarcomas. Approximately 80% of these tumors are located in the long bones, and they are particularly common in the distal and proximal tibia. However, they are rare in the craniofacial bones, and this tumor type has never been reported to occur in the orbit. In this report, a case of low-grade central osteosarcoma arising from the orbit is presented. The differential diagnoses of this tumor and the surgical results of radical resection and reconstruction are also presented.The Journal of craniofacial surgery 05/2012; 23(3):e178-80. DOI:10.1097/SCS.0b013e31824de0ef · 0.68 Impact Factor
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ABSTRACT: Fibrous dysplasia (FD) is a non-malignant condition caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The phenotype is variable and may be isolated to a single skeletal site or multiple sites and sometimes is associated with extraskeletal manifestations in the skin and/or endocrine organs (McCune-Albright syndrome). The clinical behavior and progression of FD may also vary, thereby making the management of this condition difficult with few established clinical guidelines. This paper provides a clinically-focused comprehensive description of craniofacial FD, its natural progression, the components of the diagnostic evaluation and the multi-disciplinary management, and considerations for future research.Orphanet Journal of Rare Diseases 05/2012; 7 Suppl 1(Suppl 1):S2. DOI:10.1186/1750-1172-7-S1-S2 · 3.96 Impact Factor