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.

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

ZATELLI, Maria Chiara;
2017

Abstract

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.
Davi', Maria Vittoria; Cosaro, Elisa; Piacentini, Serena; Reimondo, Giuseppe; Albiger, Nora; Arnaldi, Giorgio; Faggiano, Antongiulio; Mantovani, Giovanna; Fazio, Nicola; Piovesan, Alessandro; Arvat, Emanuela; Grimaldi, Franco; Canu, Letizia; Mannelli, Massimo; Ambrogio, Alberto Giacinto; Pecori Giraldi, Francesca; Martini, Chiara; Lania, Andrea; Albertelli, Manuela; Ferone, Diego; Zatelli, Maria Chiara; Campana, Davide; Colao, Annamaria; Scaroni, Carla; Terzolo, Massimo; De Marinis, Laura; Cingarlini, Sara; Micciolo, Rocco; Francia, Giuseppe
File in questo prodotto:
File Dimensione Formato  
EJE160809.pdf

solo gestori archivio

Descrizione: Full text author's copy
Tipologia: Full text (versione editoriale)
Licenza: NON PUBBLICO - Accesso privato/ristretto
Dimensione 3.24 MB
Formato Adobe PDF
3.24 MB Adobe PDF   Visualizza/Apri   Richiedi una copia
[1479683X - European Journal of Endocrinology] Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors_ a multicenter study.pdf

solo gestori archivio

Descrizione: Full text editoriale
Tipologia: Full text (versione editoriale)
Licenza: NON PUBBLICO - Accesso privato/ristretto
Dimensione 811.87 kB
Formato Adobe PDF
811.87 kB Adobe PDF   Visualizza/Apri   Richiedi una copia
Prognostic factors .... con copertina P copia.pdf

accesso aperto

Descrizione: Post print - accepted manuscript
Tipologia: Post-print
Licenza: PUBBLICO - Pubblico con Copyright
Dimensione 470.65 kB
Formato Adobe PDF
470.65 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11392/2364789
Citazioni
  • ???jsp.display-item.citation.pmc??? 17
  • Scopus 43
  • ???jsp.display-item.citation.isi??? 42
social impact