P1.12-06 Pulmonary Neuroendocrine (Carcinoid) Tumors: CommNETs/NANETS Endorsement and Update of the ENETs Best Practice Consensus

Singh, Simron; Bergsland, Emily K.; Card, Cynthia; Hope, Thomas A.; Kunz, Pamela L.; Laidley, David; Lawrence, Ben; Leyden, Simone; Michael, Michael; Modahl, Lucy; Myrehaug, Sten; Padda, Sukhmani K.; Pommier, Rodney F.; Ramirez, R.; Soulen, Michael C.; Strosberg, Jonathan R.; Thawer, Alia; Wei, Benjamin; Xu, Bin; Segelov, Eva

doi:10.1016/j.jtho.2018.08.841

Cited by 3 publications

(6 citation statements)

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“…In accordance with current guidelines [10], all our patients received a radical resection and the vast majority received SND. In contrast to other studies reporting on long-term outcomes after resection for BC, the vast majority of our patients were operated with VATS.…”

Section: Discussionmentioning

confidence: 96%

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Long-term survival and recurrence after resection of bronchopulmonary carcinoids: A single-center cohort study of 236 patients

et al. 2021

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The aim of this study was to determine overall survival and recurrence-free survival after resection of bronchopulmonary carcinoids by means of predominantly minimally invasive surgery and lung-sparing resections. In addition, we aimed to identify prognostic factors for overall survival. Materials and methods: Retrospective review of consecutive patients operated for bronchopulmonary carcinoids between January 2009 and October 2020 identified from a prospectively collected database. Results: A total of 236 patients representing 240 cases of bronchopulmonary carcinoids were included. Of these, 212 (88.3 %) were typical carcinoids, while 28 (11.7 %) were atypical carcinoids. A Video-Assisted Thoracoscopic Surgery (VATS) approach was used in 75 % of cases. There was no 30-day mortality. The median followup was 5.6 years for overall survival and 4.7 years for recurrence-free survival. 5-and 10-year overall survival rates were 89 % and 71 %, while 5-and 10-year recurrence-free survival rates were 84 % and 71 %. Patients with atypical carcinoids had significantly reduced overall survival and recurrence-free survival rates (HR 3.4; 95 % CI 1.5-7.6; p = 0.003 and HR 5.4; 95 % CI 2.6-11.4; p < 0.001). Independent predictors of overall survival included atypical carcinoid (HR 2.7; 95 % CI 1.2-6.0; p = 0.018) and age > 60 years (HR 2.9; 95 % CI 1.2-7.3; p = 0.021). Conclusion: Surgery for bronchopulmonary carcinoids by means of predominantly VATS and lung-sparing resections provides favorable long-term survival. Atypical carcinoids and age > 60 years are independent predictors of poor overall survival.

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Section: Discussionmentioning

confidence: 96%

“…On the contrary, AC have a more aggressive behavior with a higher rate of metastases and lower overall survival (OS) and recurrence-free survival (RFS) [7][8][9]. Surgery remains the mainstay of treatment for patients with BC and choice of resection may vary according to tumor histology and stage [10].…”

Section: Introductionmentioning

confidence: 99%

Long-term survival and recurrence after resection of bronchopulmonary carcinoids: A single-center cohort study of 236 patients

et al. 2021

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“…There are substantial regional differences in the approach to patients with NETs (Singh et al 2020), and, likely, DIPNECH. In the United States, patients with DIPNECH may receive care from either medical oncologists or pulmonologists.…”

Section: General Principlesmentioning

confidence: 99%

“…Given their substantial toxicity, including pulmonary toxicity, it is our opinion that mTOR inhibitors should not be instituted for the sole purpose of ameliorating respiratory symptoms, and their use is preferably reserved for DIPNECH patients with progressive respiratory failure (Mourad & Hamblin 2015, Russier et al 2018, and/or metastatic carcinoids (per carcinoid tumor management guidelines) (Yao et al 2016, Singh et al 2020.…”

Section: :10mentioning

confidence: 99%

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition

Samhouri

Halfdanarson

Koo

et al. 2023

Endocrine-Related Cancer

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DIPNECH is an increasingly recognized entity that primarily affects middle-aged and elderly women. It is characterized by abnormal proliferation of pulmonary neuroendocrine cells (PNECs) and considered a preinvasive lesion for carcinoid tumorlets/tumors. Sometimes, DIPNECH is accompanied by constrictive bronchiolitis which usually manifests as chronic cough and/or dyspnea, along with airflow limitation on spirometry. The telltale imaging sign of DIPNECH is the presence of multiple noncalcified pulmonary nodules and mosaic attenuation on CT. However, these clinico-radiologic features of DIPNECH are characteristic but nonspecific; thus, histopathologic confirmation is usually necessary. DIPNECH has an indolent course, and only rarely leads to respiratory failure or death; progression to overt neuroendocrine tumor (carcinoid) of the lung occurs in a minority of patients. Of available therapies, somatostatin analogs and mTOR inhibitors are most promising. In this review, we provide an update regarding the diagnosis and management of DIPNECH and describe critical gaps in our understanding of this entity, including the central terms “diffuse” and “idiopathic”. We also summarize the inconsistencies in definitions employed by recent studies and discuss the pitfalls of the DIPNECH definitions proposed by the World Health Organization in 2021. In this context, we propose an objective and reproducible radio-pathologic case definition intended for implementation in the research realm and seeks to enhance homogeneity across cohorts. Furthermore, we discuss aspects of PNECs biology which suggest that PNEC hyperplasia may contribute to the pathogenesis of phenotypes of lung disease aside from constrictive bronchiolitis and carcinoid tumorlets/tumors. Finally, we steer attention to some of the most pressing and impactful research questions awaiting to be unraveled.

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“…Current National Comprehensive Cancer Network (NCCN) guidelines propose that PRRT should be considered for patients with lung NETs following progression on SSA, if SSTR positive on imaging [28,29]. The Commonwealth Neuroendocrine Tumour Collaboration (CommNETs) and North American Neuroendocrine Tumor Society (NANETS) Consensus for Lung Neuroendocrine Tumors (LNET) also recommend that PRRT may be an option in patients with SSTR positive tumors, based on international expert opinion [30].…”

Section: Introductionmentioning

confidence: 99%

Australian experience of peptide receptor radionuclide therapy in lung neuroendocrine tumours

et al. 2020

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Background: Peptide receptor radionuclide therapy (PRRT) is an approved treatment modality for gastroenteropancreatic neuroendocrine tumours (GEP NETs), Although Phase III randomised clinical trial data is not available for NETs of other site of origin, in practice, PRRT is used more widely in clinical practice, based on its mechanism of targeting the somatostatin receptor. Use of PRRT for lung (bronchial) NET, specifically typical and atypical carcinoid (TC, AC), has been reported only in small retrospective case series. This multicentre study adds to the evidence regarding utility of PRRT for lung NETs. Materials and Methods: A retrospective chart review of patients with TC and AC who received 177 Lu-dotatate PRRT between January 2002 and June 2019 in six hospitals across Australia was undertaken. Data regarding demographics, efficacy and toxicity was evaluated at each site by the treating clinician. Results: Forty-eight patients (32 AC, 15 TC, 1 unclassified) received a median of four 177 Lu-dotatate treatments. There was a median of one prior line of systemic treatment (range: 0-3). The response rate to 177 Lu-dotatate was 33%, with a median overall survival of 49 months (range of 3-91), at a median follow up of 33 months. This compares favourably with GEP NET. Overall toxicity was recorded as modest. Conclusions: 177 Lu-dotatate PRRT in patients with lung NETs is used in real world practice, where it appears well-tolerated with some efficacy. Further evidence could be obtained through a global prospective clinical or registry trial.

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P1.12-06 Pulmonary Neuroendocrine (Carcinoid) Tumors: CommNETs/NANETS Endorsement and Update of the ENETs Best Practice Consensus

Abstract: HGNEC. Sublobar resection might increase the risk of death in HGNEC patients. Therefore if the general condition of the patient permits, perioperative chemotherapy should be performed, and the extent of resection in the treatment of HGNEC should be lobectomy or more.

Cited by 3 publications

References 0 publications

Long-term survival and recurrence after resection of bronchopulmonary carcinoids: A single-center cohort study of 236 patients

Long-term survival and recurrence after resection of bronchopulmonary carcinoids: A single-center cohort study of 236 patients

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition

Australian experience of peptide receptor radionuclide therapy in lung neuroendocrine tumours

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