Neuroendocrine carcinoma arising in the sella: Diagnosis and treatment

CHM Jaspers; R Salbeck; C Kahl; V Hans; G Berding; UJ Knappe

doi:10.1055/s-0033-1336741

Experimental and Clinical Endocrinology & Diabetes, Table of Contents

Neuroendocrine carcinoma arising in the sella: Diagnosis and treatment

CHM Jaspers ¹, R Salbeck ², C Kahl ³, V Hans ⁴, G Berding ⁵, UJ Knappe ⁶

¹Johannes Wesling Klinikum Minden, Klinik f. Rheumatologie und Endokrinologie, Minden, Germany
²Johannes Wesling Klinikum Minden, Institut für Radiologie, Minden, Germany
³Johannes Wesling Klinikum Minden, Institute of Radiotherapy, Minden, Germany
⁴Evangelisches Krankenhaus Bielefeld, Institute of Neuropathology, Bielefeld, Germany
⁵Hannover Medical School, Department of Nuclear Medicine, Hannover, Germany
⁶Johannes Wesling Klinikum Minden, Department of Neurosurgery, Minden, Germany

Abstract

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.