Primary sellar melanocytoma: pathological, clinical and treatment review

J Endocrinol Invest. 2020 May;43(5):575-585. doi: 10.1007/s40618-019-01158-8. Epub 2019 Dec 3.

Abstract

Background: Sellar melanocytomas represent a small subgroup of primary melanocytic tumors. They arise from melanocytes located in the meningeal lining of the sellar floor or in the diaphragma sellae and this location is very uncommon. Usually, sellar melanocytomas are benign and slow-growing tumors with a high likelihood of recurrence.

Purpose: To our knowledge, due to the rarity of this condition, there are no guidelines regarding their diagnosis and treatment in the medical literature to date. We have developed a narrative review, analyzing the available studies regarding primary sellar melanocytomas reported in the medical literature. We have found ten papers on this topic and all of them are case reports. In all patients, tumor diagnosis was performed after the occurrence of neurological symptoms, in particular progressive visual loss or endocrinological disorders. The diagnosis is difficult, and it requires several preoperative and postoperative investigations, but histological examination is crucial.

Conclusions: Transsphenoidal surgery is the first-choice treatment. In case of tumor's recurrence or regrowth, the role of radiation therapy and chemotherapy is not entirely clear.

Keywords: Leptomeningeal melanocytes; Pituitary tumor; Primary melanocytic brain tumors; Sellar melanocytoma; Transsphenoidal surgery.

Publication types

  • Review

MeSH terms

  • Humans
  • Melanocytes / pathology*
  • Pituitary Gland / pathology*
  • Pituitary Gland / surgery
  • Pituitary Neoplasms / pathology*
  • Pituitary Neoplasms / surgery