Meningiomas are benign tumours common in the central nervous system. Primary extracranial meningiomas in the jaws are extremely rare. We performed a systematic review of all the primary extracranial meningioma arising in the jawbones reported in the English-language literature including indexed and non-indexed journals and we report a unique case in our hospital of a man with two primary extracranial meningiomas, the first frontal intradiploic and 16 years later, a second located in the mandible. We found 14 cases with primary jaws meningioma and we report another one. A mandibular predominance was observed with only four cases in the maxilla. Most of them occurred in women, with a mean age of 35.9 years. It is the first published case of a patient with two primary extracranial meningiomas one of them in the mandible, which the gender, age and laterality of the lesion are less frequent.

Ewing's sarcoma is a rare and aggressive neoplasm that typically affects the long bones. The presence of a primary tumor in the facial bones is extremely uncommon. Here, we present a case of a 21-year-old male with Ewing's sarcoma of the zygoma. To date, only a few such cases have been reported worldwide in the literature.

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Mesenchymal chondrosarcoma (MCS) is a rare histological variant of chondrosarcoma, with aggressive behaviour. Due to the unique nature of this disease, management strategies are not well established. Li-Fraumeni syndrome (LFS) is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with TP53 germline mutations. We report a case of MCS of the maxilla, treated with surgical excision and adjuvant chemotherapy, in a patient with a past medical history of choroid plexus papilloma and a family history of early age first-degree cervical uterine cancer, that led to the clinical suspicion of a cancer predisposition syndrome and the subsequent diagnosis of LFS. This is the first MCS described in a LFS case. It demonstrates that adjuvant chemotherapy should be considered, in conjunction with surgical excision, in MCS and that cancer predisposition syndromes should be suspected in patients with multiple neoplasms and a strong family history of cancer.