BackgroundSmall cell osteosarcoma is an extremely rare histopathological variant of conventional osteosarcoma. Due to nonspecific symptoms, most osteosarcomas of the jaws are misdiagnosed as periapical abscesses and mistreated by teeth extraction and drainage.Case presentationWe report, to our knowledge, the seventh case of small cell osteosarcoma in gnathic sites affecting the mandible of an old female with history of a large painful swelling related to the right mandibular molar area for 2 months. Cone-beam computed tomography scan showed an osteolytic lesion related to the lower molar area with involvement of the inferior alveolar nerve. An incisional biopsy was taken, and after histopathological examination and immunohistochemical staining, a diagnosis of small cell osteosarcoma was reached. Hemi-mandibulectomy was performed by a maxillofacial surgeon. No clinical evidence for recurrence was noted until manuscript writing.ConclusionAccurate diagnosis is very important, and general practitioners should be aware of this entity considering that small cell osteosarcoma has a poor prognosis when compared to conventional osteosarcoma.
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