Apoplexy of a giant clival ectopic prolactinoma: A very rare case report

Abstract

Introduction and importance: Ectopic pituitary neuroendocrine tumor (EPNET) is a very rare entity, seldom with apoplexy evolution. Only three cases of intracranial ectropic pituitary neuroendocrine tumor apoplexy were reported in the literature.

Case presentation: We report the case of a 45-year-old woman with a history of amenorrhea, and headaches. Neuroimaging showed a very aggressive giant mass within the clivus with the invasion of the sphenoidal sinus and encasement of internal carotid arteries with an empty sella. Endocrinology work-up revealed an exceedingly high level of prolactin surprisingly without galactorrhea. Immunohistochemical analysis after an endonasal biopsy confirmed the diagnosis of prolactinoma. One month after Cabergoline initiation, an apoplexy of the ectopic pituitary neuroendocrine tumor occurred. Conservational management with a decrease in cabergoline dose was performed.

Discussion: This article highlights data from various cases reported in the literature in addition to our case to confirm the extreme rarity of apoplexy as a complication of EPNET.

Conclusion: Pituitary apoplexy in ectopic pituitary neuroendocrine tumor is extremely rare. Therefore, in case of unusual localization of pituitary neuroendocrine tumor, a thorough follow-up is necessary to detect complications and ensure early management.

Keywords: Apoplexy; Cabergoline; Case report; Clivus; Ectopic pituitary neuroendocrine tumor; Giant prolactinoma.

Fig. 1

a: Sagittal MRI T1WI showing the mass within the clivus, isointense, and arachnoidocele (white arrow) b: Sagittal T1 weighted contrast-enhanced image showing the mass within the clivus (yellow arrow), with homogenous enhancement c: Coronal MRI T1W1 showing the clival mass encased adjacent carotid artery (blue arrow) d: Sagittal MRI T1WI showing the isointense mass within the clivus with multiples hypointense necrotic lesions (green arrow) 1 month after Cabergoline initiation e: Sagittal MRI T1-weighted contrast-enhanced image showing the heterogeneous enhancement of the tumor with multiple necrotic lesions suggesting EPNET apoplexy (orange arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 2

(a) Histologic section showing epithelial neoplasm with monomorphic cells composition (x10) (b) Label of neoplasm cells by prolactin (x20) (c) The proliferation index is <1 % on Ki-67 staining (x40) (d) Immunohistochemical staining positive for Chromogranin (x10).