A primary sphenoclival skull base neuroendocrin tumor: A rare case report and literature review.

Autor:	Amine KM; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco. Electronic address: gaminos70@gmail.com., Bouzoubaa Y; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco., Oukessou Y; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco., Rouadi S; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco., Abada RL; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco., Mahtar M; ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco.
Jazyk:	angličtina
Zdroj:	International journal of surgery case reports [Int J Surg Case Rep] 2024 Aug; Vol. 121, pp. 109750. Date of Electronic Publication: 2024 May 10.
DOI:	10.1016/j.ijscr.2024.109750
Abstrakt:	Introduction and Importance: Primary intracranial neuroendocrine tumors (NETs) are exceedingly rare, often posing diagnostic challenges, particularly in non-secreting variants. These tumors may initially present with nonspecific symptoms, leading to delayed diagnosis and potential neurological complications. Case Report: We present the case of a 33-year-old male admitted with a one-year history of progressively worsening headache accompanied by acute left ptosis and diplopia. Initial examination revealed left eye ptosis and hypotropia, indicative of third and fourth cranial nerve paralysis. Cavoscopy revealed a mild anterior wall bulge of the sphenoid with normal mucosa. MRI imaging unveiled an expansive clival process extending towards the sellar region and left cavernous sinus, completely occupying the sphenoid sinus and exerting mass effect on the pituitary stalk. Hormonal assays were within reference ranges, ruling out a hormonally-active tumor. Endoscopic endonasal surgery for biopsy revealed a low-grade neuroendocrine tumor positive for cytokeratin AE1/AE2, chromogranin A, synaptophysin, and beta-catenin, with a Ki-67-labeling index <2 %. Somatostatin receptor scintigraphy confirmed intense hyper fixation of the tracer in the sphenoidal tumor, supporting its neuroendocrine origin. The patient declined surgical intervention after informed consent, opting for C1 somatostatin analogs prior to radiotherapy. One-year follow-up demonstrated symptom stability with no tumor progression. Clinical Discussion: To this day, no consensus among reports concerning the optimal management of these cases. Imaging assessment is crucial to validate the primary nature of the tumor and to exclude any distant localization. Various therapeutic modalities, such as surgery, radiotherapy, and somatostatin analogs, should be considered based on the specific characteristics and extent of the tumor. Conclusion: Our case is a clear reminder that neuroendocrine tumors should be considered as a differential diagnosis for skull base neoplasms. Competing Interests: Declaration of competing interest None. (Copyright © 2024. Published by Elsevier Ltd.)
Databáze:	MEDLINE
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