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DOI: 10.1055/s-0033-1336741
Neuroendocrine carcinoma arising in the sella: Diagnosis and treatment
Introduction: Neuroendocrine carcinomas arising in the sella region are extremely rare.
Methods: A 69-year-old woman presented with left-sided eye-pain. Magnetic resonance imaging demonstrated a subsellar lesion with invasion of the clivus and extensions to the cavernous sinuses. Endocrine evaluation was unremarkable. A transsphenoidal pituitary biopsy with partial resection of the tumor was undertaken. Histologically a chondrosarcoma was initially suspected. Staging performed with total body computed tomography (CT) scan was negative. Given the extension of the tumor to the cavernous sinus, radiation of the tumor was performed.
Results: The final immuno-histopathologic examination of the biopsies revealed a low-grade neuroendocrine carcinoma. Somatostatin-receptor-based imaging with Ga-68-DOTA-TATE PET/CT demonstrated focally increased uptake in the sellar region, but no evidence of tumors elsewhere.
Conclusion: Whereas metastases of neuroendocrine tumors to the pituitary have been described as very rare neoplasms, this is a report of a neuroendocrine tumor originating in the sella region as an exceptionally rare entity. To the best of our knowledge, this is the first published case so far.