Ectopic Pituitary Neuroendocrine Tumors: A Case Report and Literature Review

Background: Ectopic pituitary neuroendocrine tumors (EPNT) are extremely rare pituitary neuroendocrine tumors located outside the sella turcica without any direct connection to the intra-sellar pituitary gland. We present a case and provide a literature review on this rare condition.

Case Presentation: A 21-year-old male presented with complaints of headaches. MRI revealed a heterogeneously enhancing mass in the suprasellar region posterior to the pituitary gland extending along the prepontine cistern with calcifications and blood products with mild ventriculomegaly ([Fig. 1 A] and [B]). There were no endocrine or visual abnormalities. The tumor was favored to be a craniopharyngioma. We performed an endoscopic endonasal transsphenoidal, transtuberculum, and transclival approach for the resection of the tumor ([Fig. 1C, D]). The patient recovered uneventfully from surgery with improvement in headaches. The final pathology was null cell.

Literature Review and Discussion: EPNTs, more commonly referred to as ectopic pituitary adenoma in the literature, are believed to arise from a disorder of the migration of the anterior lobe cells of the pituitary resulting in the final location outside the sella turcica, but the exact pathology is unknown. Our literature review yielded a total of 180 cases described in the literature. Such tumors are more commonly located in the sphenoid sinus, followed by suprasellar region, clivus, and the cavernous sinus. Most EPNTs are hormonally active, with ACTH being the most common secreted hormone. EPNTs are also more likely to be greater than 10 mm in size. The most commonly reported presenting symptoms are from hormonal oversecretion, particularly ACTH oversecretion. Headaches are also commonly reported. Based on tumor location, visual disturbances, oculomotor dysfunction and hearing loss are also possible. Nasal congestion, epistaxis, and rhinorrhea, especially from a sphenoid location, have also been reported.

Conclusion: EPNT is a rare but important differential for a mass along the migration path of anterior pituitary lobe cells and warrants high clinical suspicion for effective diagnosis and treatment.

Publication History

Article published online:
07 February 2025