Pituitary carcinoma (PC) is defined by the presence of pituitary tumor that is either not contiguous with the primary sellar tumor and/or has metastasized to distant sites. It is very rare and associated with poor prognosis, with a median survival of 12 months when systemic metastases are present, and 30 months when metastases are confined to the central nervous system. A 20 years-old man was first diagnosed with a nonfunctioning pituitary macroadenoma in 1981 and submitted to transsphenoidal surgery (TSS). Histology showed ACTH positive tumor, p53 negative and Ki-67 1%. Three years later a recurrence was detected and a new TSS followed by conventional radiotherapy (Rxt) was performed. Hormone replacement therapy (HRT) was initiated for TSH, ACTH and FSH/LH deficiencies. In 2000, at age 39, he complained of prolonged nasal obstruction. Imaging showed a giant invasive sellar tumor with extension to suprasellar region, cavernous sinus, and nasopharynx; multiple extra-axial lesions in the posterior fossa suggesting meningeal secondary implants and a large mass in the cervical-thoraco-lumbar spine. Histology of the resected giant adenoma and the spinal lesion confirmed an ACTH-expressing PC with a high proliferative index. Fractionated stereotactic Rxt was performed. Cabergoline and pasireotide were prescribed, but discontinued after some months due to side effects. Tumor regrowth occurred in 2009 when surgery was repeated for removal of the main tumor followed by Rxt directed to cerebellar lesions, and again in 2015, when a microsurgery guided by localization systems and electrophysiological monitoring was carried out. Temozolomide was prescribed but patient refused the use. Currently, at age 55, he is doing well, working, and maintaining a stable clinical condition under HRT. Latency time from the diagnosis of pituitary adenoma to PC was 19 years, and his survival of 16 years after diagnosis of PC has no parallel in the literature to date.
20 - 23 May 2017
European Society of Endocrinology