'Ectopic' suprasellar type IIa PRL-secreting pituitary adenoma

Pituitary. 2017 Aug;20(4):477-484. doi: 10.1007/s11102-017-0807-9.

Abstract

Background: Ectopic pituitary adenomas (EPAs) are rare, and the suprasellar cistern seems to be the most common location. At this time, no detailed original classification, diagnosis, or treatment protocols for suprasellar pituitary adenomas (SPAs) have been described.

Case description: A 19-year-old man showed visual disturbances and lack of libido for 3 years, he suffered a sharp decline in vision with only light perception in the last week. Magnetic resonance imaging scans revealed a large suprasellar cystic lesion with a normal pituitary in the sella turcica. Endocrinological findings showed an extremely high prolactin level of 1250 ng/mL. Because of the sharp decline in vision, the patient underwent total removal of the suprasellar lesion using a transfrontal interhemispheric approach. The tumor pedicle originated in the lower pituitary stalk without any connection to the anterior pituitary gland in the sella turcica, while the diaphragma sellae was incomplete. Clinical and endocrinological cure criteria were fulfilled and postoperative pathology confirmed a prolactin-secreting pituitary adenoma.

Conclusion: Ectopic suprasellar pituitary adenomas (ESPAs) are extremely rare intracranial extracerebral tumors. SPAs can be classified into three types according to their origin and their relationship with surrounding tissue. Only type III is theoretically a true ectopic, based on previous reports. Thus, ESPAs are uncommon compared to other EPAs. Our case is the first reported case of a type IIa 'E'SPA and the first description of this subtype classification until now. The pars tuberalis may be different from the pars distalis, and each subtype of adenohypophyseal cells may have different migration characteristics, which leads to different proportions of each hormone-secreting subtype in SPAs and EPAs. Transsphenoidal surgery is minimally invasive, but transcranial surgery may remain a universal option for the treatment of suprasellar lesions.

Keywords: Diaphragma sellae; Ectopic suprasellar pituitary adenoma; Pituitary adenoma; Subdiaphragmatic; Supradiaphragmatic; Suprasellar.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Animals
  • Humans
  • Magnetic Resonance Imaging
  • Pituitary Neoplasms / diagnostic imaging*
  • Pituitary Neoplasms / metabolism*
  • Prolactinoma / diagnostic imaging
  • Prolactinoma / metabolism
  • Sella Turcica / metabolism*