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Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5-10% of cases, and commonly affecting extremities. Diagnosis, treatment, and prognosis remain challenging especially when localized in uncommon areas, such as intracranial lesions. Case Description: A 13-year-old male patient with a clinical history of neurofibromatosis Type I (NF1) presenting holocranial headache with jet vomiting and apathy 2 days before admission, without neurological deficits and/or focal findings. On magnetic resonance imaging: an extra-axial infiltrative lesion with contrast uptake at the base of the skull in the olfactory groove topography. After total tumor resection, the anatomopathological examination showed monophasic SS. The patient returned after 6 months with similar symptoms, and the lesion recurred and was reoperated. Unfortunately, 7 months after the second surgery, the patient died. Conclusion: SS can occur extraarticulously and with a variable clinical presentation and poor prognosis despite adjuvant therapies with radiotherapy and chemotherapy. In individuals with clinical history of NF1, there is still no direct correlation between the two manifestations, although current descriptions are suggestive of a possible interaction.
RESUMO
Introduction Mucoepidermoid carcinoma (MEC) is a tumor originated from the epitheliumof the glandular excretory ducts and has highly variable biological potential. It is the most prevalent cancer of the salivary glands. The present report aims to describe a case of nasal mucoepidermoid carcinoma that developed after adjuvant radiotherapy (RT) treatment of a recurrent pituitary macroadenoma. Case Report Male patient, 62 years old, presented with recurrent nasal epistaxis on the right, associated with intense pulsatile headache, visual analogical scale (VAS) 10/10, with improvement only with the use of opioids andmorphine. After undergoing oncological screening and study by imaging exams, the presence of an expansive seal lesion with suprasellar extension was seen, involving the medial wall of the cavernous segment of the right carotid artery and the anterior cerebral artery, as well as the presence of a new expansive lesion in the right nasal cavity, with ethmoid bone invasion superiorly and medial orbit wall invasion laterally, compressing the ipsilateral optic nerve canal. Discussion Sinonasal neoplasms represent a small portion of all malignancies of the upper aerodigestive tract, accounting for<5% of these neoplasms. The development of MEC involves risk factors such as occupational issues, history of trauma and surgery involving the nasal area, and radiation exposure, as in previous RT. Conclusion Mucoepidermoid carcinoma is an uncommon neoplasia and can be associated with RT treatment, as used in cases of recurrent pituitary macroadenoma. In general, surgical resection to obtain free margins of neoplastic tissue is the aimed treatment, seeking better prognosis.
