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

Filippo Lococo, Stefano Margaritora, Giuseppe Cardillo, Perluigi Filosso, Pierluigi Novellis, Cristian Rapicetta, Francesco Carleo, Giulia Bora, Alfredo Cesario, Alessandro Stefani, Giulio Rossi, Massimiliano Paci

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

ObjectiveCushing 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. MethodsFrom 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. ResultsMedian 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. ConclusionBCs 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.

Original languageEnglish
Pages (from-to)172-181
Number of pages10
JournalThoracic and Cardiovascular Surgeon
Volume64
Issue number2
DOIs
Publication statusPublished - Mar 1 2016

Fingerprint

Cushing Syndrome
Carcinoid Tumor
Lymph
Survival
Recurrence
Survival Analysis
Histology
Weights and Measures
Drug Therapy
Neoplasms

Keywords

  • diagnosis
  • lung cancer
  • pathology
  • tumor

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Bronchopulmonary Carcinoids causing Cushing Syndrome : Results from a Multicentric Study Suggesting a More Aggressive Behavior. / Lococo, Filippo; Margaritora, Stefano; Cardillo, Giuseppe; Filosso, Perluigi; Novellis, Pierluigi; Rapicetta, Cristian; Carleo, Francesco; Bora, Giulia; Cesario, Alfredo; Stefani, Alessandro; Rossi, Giulio; Paci, Massimiliano.

In: Thoracic and Cardiovascular Surgeon, Vol. 64, No. 2, 01.03.2016, p. 172-181.

Research output: Contribution to journalArticle

Lococo, Filippo ; Margaritora, Stefano ; Cardillo, Giuseppe ; Filosso, Perluigi ; Novellis, Pierluigi ; Rapicetta, Cristian ; Carleo, Francesco ; Bora, Giulia ; Cesario, Alfredo ; Stefani, Alessandro ; Rossi, Giulio ; Paci, Massimiliano. / Bronchopulmonary Carcinoids causing Cushing Syndrome : Results from a Multicentric Study Suggesting a More Aggressive Behavior. In: Thoracic and Cardiovascular Surgeon. 2016 ; Vol. 64, No. 2. pp. 172-181.
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title = "Bronchopulmonary Carcinoids causing Cushing Syndrome: Results from a Multicentric Study Suggesting a More Aggressive Behavior",
abstract = "ObjectiveCushing 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. MethodsFrom 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. ResultsMedian 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. ConclusionBCs 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.",
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T1 - Bronchopulmonary Carcinoids causing Cushing Syndrome

T2 - Results from a Multicentric Study Suggesting a More Aggressive Behavior

AU - Lococo, Filippo

AU - Margaritora, Stefano

AU - Cardillo, Giuseppe

AU - Filosso, Perluigi

AU - Novellis, Pierluigi

AU - Rapicetta, Cristian

AU - Carleo, Francesco

AU - Bora, Giulia

AU - Cesario, Alfredo

AU - Stefani, Alessandro

AU - Rossi, Giulio

AU - Paci, Massimiliano

PY - 2016/3/1

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N2 - ObjectiveCushing 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. MethodsFrom 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. ResultsMedian 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. ConclusionBCs 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.

AB - ObjectiveCushing 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. MethodsFrom 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. ResultsMedian 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. ConclusionBCs 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.

KW - diagnosis

KW - lung cancer

KW - pathology

KW - tumor

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