Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case.

ABSTRACT:

Introduction

Primitive neuroectodermal tumors (PNET) form a group of tumors defined by their appearance that are thought to develop from primitive (undifferentiated) nerve cells in the brain. They are rare tumors and their incidence is not well defined.

Case presentation

An 18-month-old male presenting with typical Cushingoid appearance (moon face, central obesity, hirsutism and growth arrest) was admitted for evaluation of endocrine problems. Subsequent laboratory studies revealed markedly elevated adrenocorticotropic (ACTH) and cortisol levels, as well as a hypokalemic metabolic alkalosis, these data are consistent with the diagnosis of Cushing's disease. He was treated with metyrapone to control hypercortisolemia. One month and a half later, a mass was detected in the abd

SUBMITTER: Rahbar M 

PROVIDER: S-EPMC6287080 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature