Progression of an Invasive ACTH Pituitary Macroadenoma with Cushing's Disease to Pituitary Carcinoma.

Clarissa Groberio Borba ,Malebranche Berardo Carneiro Da Cunha Neto,Nina Rosa De Castro Musolino ,Valter Ângelo Sperling Cescato ,Rafael Loch Batista ,Gilberto Ochman Da Silva ,Vanielle Carvalho Machado ,Ana Elisa Evangelista Alcantara

doi:10.1155/2015/810367

Clarissa Groberio Borba , Malebranche Berardo Carneiro Da Cunha Neto + Show 6 more

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https://doi.org/10.1155/2015/810367

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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.

Highlights

Pituitary adenomas are a common type of intracranial tumor
The World Health Organization (WHO) classification categorizes pituitary adenomas as typical and atypical and the pathological features of atypical adenoma are defined as a Ki-67 index greater than 3% and/or extensive p53 immunoreactivity [1]
A 55-year-old female patient diagnosed with Cushing’s disease (CD) due to a macroadenoma (2,39 × 2,54 × 3,36 cm) underwent two transsphenoidal surgeries (TS) at another medical center (2008 and 2009, and we do not have data about the anatomopathological exam of these surgeries because these procedures were done in another region of country), without clinical improvement

Summary

Introduction

Pituitary adenomas are a common type of intracranial tumor. The more effective approach is transsphenoidal surgery. The World Health Organization (WHO) classification categorizes pituitary adenomas as typical and atypical and the pathological features of atypical adenoma are defined as a Ki-67 index greater than 3% and/or extensive p53 immunoreactivity [1]. Pituitary carcinomas are very rare (0.1%-0.2% of pituitary tumors) [2], with diagnosis requiring evidence of metastasis and they, frequently, show a higher index of Ki-67 and p53 protein than aggressive adenomas. Most of these tumors secrete prolactin and/or ACTH and they are usually resistant to radiotheraphy [1, 3, 4]

Case Description

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