A comprehensive long-term retrospective analysis of silent corticotrophic adenomas vs hormone-negative adenomas

Neurosurgery. 2013 Jul;73(1):8-17; discussion 17-8. doi: 10.1227/01.neu.0000429858.96652.1e.

Abstract

Background: Silent corticotrophic adenomas (SCAs) stain adrenocorticotropic hormone (ACTH)+ without causing Cushing disease. SCAs are reportedly more aggressive, but information comes from small series.

Objective: To determine whether SCAs behave more aggressively than hormone-negative adenomas (HNAs), and characterize SCA ACTH production alterations.

Methods: SCAs (n = 75) and HNAs (n = 1726) diagnosed at our institution from 1990 to 2011 were retrospectively reviewed. RT-PCR was used to compare expression of ACTH-producing factors.

Results: SCA patients exhibited comparable sex and age as HNA patients (P = .7-.9). SCAs exhibited comparable size as HNAs (2.2 vs 2.0 cm, P = .2), with cavernous sinus invasion in 30% of SCAs vs 18% of HNAs (P = .03). SCA patients had higher mean preoperative serum ACTH (46 vs 19 ng/L; P = .005; normal = 5-27 ng/L), but comparable serum cortisol (13 vs 12 μg/dL; normal = 4-22 μg/dL; P < .05) as HNA patients. SCAs were gross totally resected 59% of the time, vs 53% for HNAs (P = .8). Kaplan-Meier 3-year progression/recurrence rates were 34% for strongly ACTH-positive Type I SCAs, 10% for weakly ACTH-positive Type II SCAs, and 6% for HNAs (P < .001 SCA vs HNA; P < .001 Type I vs HNA; and P = .08 Type II vs HNA). Expression of ACTH precursor pro-opiomelanocortin was 900-fold elevated in SCAs and 1300-fold elevated in Cushing disease-causing adenomas (CDCAs) vs HNAs (P < .001). Transcription of PC1/3, which cleaves pro-opiomelanocortin into ACTH, was 30-fold higher in CDCAs than SCAs (P = .02).

Conclusion: In the largest series to date, SCAs exhibited comparable size, but increased cavernous sinus invasion and progression/recurrence vs HNAs. SCAs exhibit deficient pro-opiomelanocortin to ACTH conversion. Close follow-up is warranted for SCAs.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • ACTH-Secreting Pituitary Adenoma / blood*
  • ACTH-Secreting Pituitary Adenoma / epidemiology*
  • ACTH-Secreting Pituitary Adenoma / therapy
  • Adenoma / blood*
  • Adenoma / epidemiology*
  • Adenoma / therapy
  • Adolescent
  • Adrenocorticotropic Hormone / blood*
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Biomarkers / blood
  • Child
  • Female
  • Humans
  • Hydrocortisone / blood*
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local / blood*
  • Neoplasm Recurrence, Local / epidemiology*
  • Neoplasm Recurrence, Local / prevention & control
  • Prevalence
  • Reproducibility of Results
  • Retrospective Studies
  • Risk Factors
  • San Francisco / epidemiology
  • Sensitivity and Specificity
  • Sex Distribution
  • Treatment Outcome
  • Young Adult

Substances

  • Biomarkers
  • Adrenocorticotropic Hormone
  • Hydrocortisone