Endocrine Abstracts

Introduction: Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a malignant neuroendocrine tumor of the nasal fossa that arises from neurosensory receptor cells of the olfactory epithelium. It represents 3% of all nasal tumors. It presents a slow and progressive evolution that primarily affects the upper nasal mucosa and cribriform plate, with subsequent extension to the entire cavity and paranasal sinuses and can invade the orbit and cranial base.

Objective: We present the first case described in the literature of ENB with intracranial invasion as a cause of panhypopituitarism.

Case report: A 64-year-old man was assessed for bilateral hearing loss secondary to recurrent otitis, bilateral nasal respiratory failure (without associated epistaxis), holocranial headache, and sensation of stuffiness with Valsalva maneuvers. In the imaging tests (nasofibroscopy and CT of the paranasal sinuses) asymmetry was observed in the cavum, as well as a 35x30x30mm solid mass originating from the left sphenoid sinus, expansive, remodeling and rupturing the posterior wall of the sinus, clivus and sella turcica (invading the suprasellar region), affecting the pituitary stalk, with partial invasion of the cavernous sinuses, all suggestive of neoplasia of the sphenoid sinus. The laboratory study showed moderate hyponatremia, severe hyperprolactinemia (derived from compression of the pituitary stalk), central hypothyroidism, GH deficiency, and hypogonadism. The lesion was biopsied under general anesthesia (subsequently surgically removed by joint ear, nose, and throat intervention) with an anatomopathological diagnosis indicative of olfactory neuroblastoma. He required complementary radiotherapy treatment (64Gy).

Conclusion and discussion: As a neuroendocrine tumor, there are descriptions of paraneoplastic ectopic secretion of ACTH and Vasopressin in relation to olfactory neuroblastoma. No case of panhypopituitarism related to parasellar invasion of the pituitary stalk by this neoplasia has been described to date. Early diagnosis and treatment are necessary to achieve a favorable result, requiring close multidisciplinary collaboration.