title: Progression of an Invasive ACTH Pituitary Macroadenoma with Cushing's Disease to Pituitary Carcinoma.
abstract: Pituitary carcinomas are very rare tumors that in most cases produce prolactin and adrenocorticotropic hormone (ACTH). It is a challenge to diagnosis of a pituitary carcinoma before disclosed symptomatic metastasis. We report the case of a female patient with Cushing's disease who underwent three transsphenoidal surgeries, with pathological findings of common ACTH pituitary adenoma including Ki-67 expression <3%. She achieved hypocortisolism after the 3rd surgery although ACTH levels remained slightly elevated. The patient returned some time later with fast worsening of hypercortisolism. Magnetic resonance imaging showed clivus invasion, which led to a fourth surgery and radiation. This time, immunohistochemistry revealed strong Ki-67 (10% to 15%) and p53 expression. Liver and lumbar spine metastases were found on workup. The patient died after few months due to lung infection. Pituitary carcinomas are rare, and the transformation of an ACTH-secreting pituitary adenoma into a carcinoma is exceptional. The difficulty of defining markers for the diagnosis of carcinoma, before metastasis diagnosis, in order to change the management of the disease, is a challenge.
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Clarissa Groberio
authors.initials: CG
authors.lastname: Borba
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Rafael Loch
authors.initials: RL
authors.lastname: Batista
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Nina Rosa de Castro
authors.initials: NR
authors.lastname: Musolino
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Vanielle Carvalho
authors.initials: VC
authors.lastname: Machado
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Ana Elisa Evangelista
authors.initials: AE
authors.lastname: Alcantara
authors.affiliation: Pituitary Neurosurgery Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Gilberto Ochman
authors.initials: GO
authors.lastname: da Silva
authors.affiliation: Pituitary Neurosurgery Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Valter Angelo
authors.initials: VA
authors.lastname: Sperling Cescato
authors.affiliation: Neuroendocrinology Unit, Functional Neurosurgery Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.
authors.forename: Malebranche Berardo Carneiro
authors.initials: MB
authors.lastname: da Cunha Neto