Neuroendocrine ACTH-producing tumor of the thymus--experience with 12 patients over 25 years.

ABSTRACT:

Context

ACTH-producing neuroendocrine tumor (NET) of the thymus is a rare cause of Cushing's syndrome (CS). The literature consists mainly of isolated case reports.

Patients

We studied 12 cases (eight males and four females) diagnosed between 1986 and 2010 with CS and thymic NET who underwent surgical resection.

Main outcome measures

We measured time from onset of CS to diagnosis of thymic NET, tumor size, histological grade, time to recurrence, and survival and performed a meta-analysis of other published cases of CS associated with thymic NET.

Results

Eleven of 12 patients presented with classic features of CS at a median age of 21 yr (range, 7-51). Four were children. The 24-h urine free cortisol was greater than 16-fold of normal, and biochemical testing wa

SUBMITTER: Neary NM 

PROVIDER: S-EPMC3387392 | biostudies-literature | 2012 Jul

REPOSITORIES: biostudies-literature