Thorac Cardiovasc Surg 2016; 64(02): 172-181
DOI: 10.1055/s-0035-1555125
Original Thoracic
Georg Thieme Verlag KG Stuttgart · New York

Bronchopulmonary Carcinoids causing Cushing Syndrome: Results from a Multicentric Study Suggesting a More Aggressive Behavior

Filippo Lococo
1   Department of Thoracic Surgery, IRCCS Arcispedale Santa Maria Nuova Reggio Emilia, Reggio Emilia, Italy
,
Stefano Margaritora
2   Department of General Thoracic Surgery, Catholic University, Rome, Italy
,
Giuseppe Cardillo
3   Unit of Thoracic Surgery, San Camillo-Forlanini Hospital, Rome, Italy
,
Perluigi Filosso
4   Unit of Thoracic Surgery, University of Turin, Turin, Italy
,
Pierluigi Novellis
2   Department of General Thoracic Surgery, Catholic University, Rome, Italy
,
Cristian Rapicetta
1   Department of Thoracic Surgery, IRCCS Arcispedale Santa Maria Nuova Reggio Emilia, Reggio Emilia, Italy
,
Francesco Carleo
3   Unit of Thoracic Surgery, San Camillo-Forlanini Hospital, Rome, Italy
,
Giulia Bora
4   Unit of Thoracic Surgery, University of Turin, Turin, Italy
,
Alfredo Cesario
5   International Research Activities Office and System Medicine, IRCCS-Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
,
Alessandro Stefani
6   Unit of Thoracic Surgery, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy
,
Giulio Rossi
7   Unit of Pathology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy
,
Massimiliano Paci
1   Department of Thoracic Surgery, IRCCS Arcispedale Santa Maria Nuova Reggio Emilia, Reggio Emilia, Italy
› Author Affiliations
Further Information

Publication History

24 February 2015

21 April 2015

Publication Date:
28 July 2015 (online)

Abstract

Objective Cushing syndrome (CS) caused by bronchopulmonary carcinoids (BCs) is a very rare entity. The aim of this study was to revisit the features of a multicenter clinical series to identify significant prognostic factors.

Methods From January 2002 to December 2013, the clinical and pathological data of 23 patients (treated in five different institutions) were retrospectively reviewed. Survival analysis was performed to explore the relative weight of potential prognostic factors.

Results Median age and male/female ratio were 48 years and 14/9, respectively. Most (> 80%) of the patients presented with CS-related symptoms at diagnosis. Tumor location was peripheral in 13 patients (57%) and central in 10 (43%). All patients but two (treated with chemotherapy) underwent surgical resection with curative intent. Definitive cyto/histology was indicative of typical carcinoid (TC) in 16 cases (70%) and atypical carcinoid (AC) in 7 cases (30%). A complete remission of CS was obtained in 16 cases (70%). Lymph nodal involvement was detected in 11 cases (48%), with N2 disease occurring in 7 (∼ 30% of all cases). Four patients (22%) experienced a relapse of the disease after radical surgery. Overall 5-year survival (long-term survival, LTS) was 60%, better in TCs when compared with AC (LTS: 66 v s. 48%, p = 0.28). Log-rank analysis identified ECOG performance status, cTNM and cN staging, pTNM and pN staging, persistence of CS and relapses (local p = 0.006; distant p = 0.001) as significant prognostic factors in this cohort of patients.

Conclusion BCs causing CS are characterized by a high rate of lymph–nodal involvement, a suboptimal prognosis (5-year survival = 60%, 66% in TCs) and a remarkable risk of relapse even after radical resection. Advanced stage, lymph–nodal involvement and the persisting of the CS after treatment correlate with a poor prognosis.

Note

Presented at “22nd European Conference on General Thoracic Surgery,” Copenhagen, 15–18 June 2014.


 
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