Your institution is subscribed to Read Institutional Edition. Log in or Sign Up to read full text articles.


Prognostic factors in ectopic Cushing's syndrome due to neuroendocrine tumors: a multicenter study

Maria Vittoria Davi', Elisa Cosaro, Serena Piacentini, Giuseppe Reimondo, Nora Albiger, Giorgio Arnaldi, Antongiulio Faggiano, Giovanna Mantovani, Nicola Fazio, Alessandro Piovesan, Emanuela Arvat, Franco Grimaldi, Letizia Canu, Massimo Mannelli, Alberto Giacinto Ambrogio, Francesca Pecori Giraldi, Chiara Martini, Andrea Lania, Manuela Albertelli, Diego Ferone, Maria Chiara Zatelli, Davide Campana, Annamaria Colao, Carla Scaroni, Massimo Terzolo, Laura De Marinis, Sara Cingarlini, Rocco Micciolo, Giuseppe Francia
European Journal of Endocrinology 2017, 176 (4): 453-461

OBJECTIVE: Evidence is limited regarding outcome of patients with ectopic Cushing's syndrome (ECS) due to neuroendocrine tumors (NETs).

DESIGN: We assessed the prognostic factors affecting the survival of patients with NETs and ECS.

METHODS: Retrospective analysis of clinicopathological features, severity of hormonal syndrome, treatments from a large cohort of patients with NETs and ECS collected from 17 Italian centers.

RESULTS: Our series included 110 patients, 58.2% female, with mean (±s.d.) age at diagnosis of 49.5 ± 15.9 years. The main sources of ectopic ACTH were bronchial carcinoids (BC) (40.9%), occult tumors (22.7%) and pancreatic (p)NETs (15.5%). Curative surgery was performed in 56.7% (70.2% of BC, 11% of pNETs). Overall survival was significantly higher in BC compared with pNETs and occult tumors ( P  = 0.033) and in G1-NETs compared with G2 and G3 ( P  = 0.007). Negative predictive factors for survival were severity of hypercortisolism ( P  < 0.02), hypokalemia ( P  = 0.001), diabetes mellitus ( P  = 0.0146) and distant metastases ( P  < 0.001). Improved survival was observed in patients who underwent NET removal ( P  < 0.001). Adrenalectomy improved short-term survival.

CONCLUSIONS: Multiple factors affect prognosis of ECS patients: type of NET, grading, distant metastases, severity of hypercortisolism, hypokalemia and diabetes mellitus. BCs have the highest curative surgical rate and better survival compared with occult tumors and pNETs. Hypercortisolism plays a primary role in affecting outcome and quality of life; therefore, prompt and vigorous treatment of hormonal excess by NET surgery and medical therapy should be a key therapeutic goal. In refractory cases, adrenalectomy should be considered as it affects outcome positively at least in the first 2 years.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.