Silent familial isolated pituitary adenomas: Histopathological and clinical case report

C. Villa, F. Magri, P. Morbini, A. Falchetti, P. Scagnelli, E. Lovati, D. Locatelli, F. R. Canevari, V. Necchi, E. Gabellieri, G. Guabello, L. Chiovato, E. Solcia

Research output: Contribution to journalArticlepeer-review

Abstract

Familial isolated pituitary adenoma (FIPA) is a rare condition independent of Carney Complex or MEN1. An international multicenter study recently described 28 nonfunctioning pituitary adenomas in 26 families with only two homogeneous nonsecreting phenotype families consistent of silent GH and silent gonadotroph adenomas, respectively. We present the clinical, genetic, and morphological analysis of two silent pituitary adenomas occurring in a man and his daughter, and discuss the differential diagnosis associated with their histological, immunohistochemical, and ultrastructural features. The patients developed invasive nonsecreting macroadenomas manifesting only with compressive symptoms. Genetic analysis in the father showed no MEN-1 germ-line mutation. Tissue samples obtained after paraseptal trans-sphenoidal surgery were studied by immunohistochemistry for adenohypophyseal hormones, low molecular weight cytokeratins (CAM 5.2), proliferation markers, and anterior pituitary transcription factors (Pit-1 and SF-1) and by electron microscopy for secretory granules. The clinical, histological, and immunohistochemical features of the lesions posed a differential diagnosis between a null cell adenoma and a silent corticotroph adenoma (Type II); on the basis of immunohistochemical stains for cytokeratin and adenohypophysis cell lineage markers, tumor behavior and ultrastructural studies we concluded for the second. The reported cases represent an as yet undescribed example of homogeneous family with silent corticotroph adenomas (Type II). Our observations support the trend for more aggressive behavior in nonsecreting FIPAs as compared with sporadic adenomas.

Original languageEnglish
Pages (from-to)40-46
Number of pages7
JournalEndocrine Pathology
Volume19
Issue number1
DOIs
Publication statusPublished - Apr 2008

Keywords

  • Familial isolated pituitary adenoma
  • Pituitary
  • Silent corticotroph pituitary adenomas
  • Transcription factors

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

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