Low-grade central osteosarcoma of the mandible: a case study report

Department of Stomatology, School of Dentistry, Federal University of Goiás, Goiás, Brazil.
Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology (Impact Factor: 1.46). 03/2007; 103(2):246-52. DOI: 10.1016/j.tripleo.2005.11.006
Source: PubMed


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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    • "Typically the malignancy is a sarcomatous lesion, most often osteosarcoma but fibrosarcoma, chondrosarcoma, and malignant fibrohistiocytoma have also been reported [16,20,28,42-45]. The diagnosis may be difficult, particularly in cases of low-grade osteosarcoma [46,47]. In such cases, immunohistochemical analysis with MDM2 and CDK4 may assist in distinguishing FD from a malignancy as a malignancies will often express MDM2 or CDK4 while FD will not [48,49]. "
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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 · 3.36 Impact Factor
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    ABSTRACT: Osteosarcoma (OS), a rare malignant bone tumour arising from primitive bone forming mesenchyme, most often arises in the metaphyses of long bones of the extremities. Bone or osteoid formation within the tumour is characteristic of an osteosarcoma. Craniofacial osteosarcoma (CFOS), most often located in the mandible or maxilla, accounts for only 5-13% of all osteosarcomas. In general, OS of the jaw is a high-grade lesion. Low-grade lesions are rare and represent less than 2% of all osteosarcomas reported in the literature. Because of its rarity and well differentiation, Low-grade OS is usually misdiagnosed as a benign lesion. The clinical and radiographic presentation does not correlate well with the subtle histology picture of a low-grade osteosarcoma which makes the diagnosis difficult.
    Journal of Maxillofacial and Oral Surgery 06/2010; 9(2):186-90. DOI:10.1007/s12663-010-0057-0
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    ABSTRACT: Well-differentiated osteosarcoma of the jaw bones is rare, and is often confused with a variety of benign fibroosseous lesions. To better understand its clinicopathologic features, we reviewed our experience with 15 cases. The patients included 7 males and 8 females (age range 14 to 66 y; mean 42.8 y). Six of the tumors arose in the mandible, and 9 in the maxilla. Microscopically, the tumors were infiltrative, relatively hypocellular, and consisted of monomorphic, minimally atypical spindle cells that were usually arranged in fascicles. The tumor cells were intimately related to the surfaces of elongated trabeculae of neoplastic woven bone that lacked osteoblastic rimming. Nine patients were treated with wide excision, 2 patients with marginal excision, and 2 patients with excision and radiation therapy. After definitive therapy, there were no reports of recurrence or metastasis over an average follow-up of 34 months (range 1 to 80 mo). Well-differentiated gnathic osteosarcoma is a low-grade malignancy that may be successfully treated with wide local excision. The prognosis in our series was excellent.
    The American journal of surgical pathology 11/2010; 34(11):1647-55. DOI:10.1097/PAS.0b013e3181f7dac6 · 5.15 Impact Factor
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