Endocrine Abstracts

Paragangliomas (PG) originating from the nasal cavity are very rare and usually have an indolent growth. Surgery offers the only possibility of cure for these patients while radiotherapy can only slow their growth rate. Intracranial or leptomeningeal dissemination is very rare, with only 7 cases described in the literature. A 77 year old man with a 4-years history of nasal obstruction. He underwent a nasofibroscopy with a biopsy that revealed a nasosinusal neuroendocrine tumour. The patient underwent Weber-Fergusson surgery with frontal craniotomy. Histological examination confirmed the diagnosis of nasosinusal PG. Bone and lymphovascular invasion and intracranial growth were present. Two years later due to epistaxis, the imagiological study showed extension to the soft tissues of the left orbit and nasopharynx. In this context, the patient was proposed for stereotactic radiotherapy. Eighteen months later, the patient presented psychomotor slowing, unbalance and crural hemiparesis. MRI showed the appearance of multiple cystic dural nodules, leading to a mass effect and cerebral oedema. The patient was started on dexamethasone and oral chemotherapy with capecitabine and temozolomide. Despite the stability of dural lesions, the neurological status. This case represents one of the few malignant nasosinusal PG described in the literature. Although the most appropriate initial therapeutic approach was applied, the metastatic presentation in these cases offers a massive challenge, where therapeutic options are truly limited.