Two-step development of a pituitary adenoma: from hyperprolactinemic syndrome to Cushing's disease

Abstract

In this report we describe the case of a young female patient with amenorrhea-galactorrhea syndrome apparently due to pituitary PRL-secreting adenoma who, after three years of dopaminergic therapy without any shrinkage of the tumor, developed true Cushing's disease. Progression from hyperprolactinemia to hypersecretion of ACTH has been rarely described and it may be due to different possibilities. However, histopathological and immunohistochemical studies of the adenoma showed a pattern of PRL negative and ACTH positive cells, excluding mixed pituitary tumor. In order to explain the progression from hyperprolactinemia with amenorrhea-galactorrhea to an ACTH hypersecretion syndrome, it must be hypothesized either pituitary stalk compression or the influence of paracrine regulation factor(s) (such as Galanine) due to an "initially silent" corticotropinoma. This case confirms that the presence of hyperprolactinemia in a patient with pituitary tumor and amenorrhea-galactorrhea syndrome is insufficient to confidently conclude for prolactinoma. Furthermore, it underlines the importance both of clinically monitoring the patient with prolactin pituitary adenoma if dopaminergic therapy does not reduce tumor volume, and of accurately and repeatedly measuring the other pituitary hormonal secretions.