Diagnostic and Management Pathways for Pulmonary Carcinoid Tumours in the United Kingdom: Results from the National Lung Neuroendocrine Tumour Pathway Project.

Wasat Mansoor,Victoria Ross,Stuart Ferguson,Denis Talbot

doi:10.1155/2020/9287536

Abstract

There is inconsistency among published guidelines for the optimal diagnostic and management pathways for patients with typical (TC) or atypical (AC) pulmonary carcinoid tumours. We conducted a UK-wide clinician survey to assess current practice for the diagnosis, management, and follow-up of patients with TC/AC and descriptively compared management between European Neuroendocrine Tumor Society (ENETS) accredited centres of excellence (CoE) and nonaccredited centres (non-CoE). Twenty-seven clinicians (10 CoE; 17 non-CoE) participated. Computed tomography of thorax, abdomen, and pelvis was the most commonly reported diagnostic tool (96% of respondents), and bone scans and gallium somatostatin receptor scintigraphy positron emission tomography (SRS PET) were the least commonly reported (30% and 37% of respondents, respectively). Adjuvant therapy is considered for resected TC/AC by <5% of respondents for patients with stage N0 M0 AC or TC, up to 48% of respondents for patients with AC with R1 disease. Somatostatin analogues were the most commonly reported first-line treatment (63% of respondents), and chemotherapy was the most commonly reported second-line therapy and third-line therapy (33% and 41%, respectively) for unresectable and metastatic disease. Reported frequency of initial follow-up after primary surgery ranged from every 2 months to annual, and total follow-up duration ranged from 2 years to indefinite depending on disease type (TC/AC) and stage. For most diagnostic investigations, the highest reported frequency of use was in CoE, most notably gallium SRS PET (70% CoE vs. 18% non-CoE respondents). 93% of respondents (100% CoE; 88% non-CoE) reported having neuroendocrine tumour- (NET-) specialist multidisciplinary team meetings at their centre; 59% (90% CoE; 41% non-CoE) had a NET Clinical Nurse Specialist (CNS) and 48% (80% CoE; 29% non-CoE) had a lung NET patient database. The survey results suggest variability between UK centres in diagnostic pathways and management of patients with TC/AC and suggest that CoE may be able to offer an improved service to patients.

Highlights

Pulmonary carcinoid tumours (PC), a subset of pulmonary neuroendocrine tumours (NETs), are rare neuroendocrine epithelial malignancies [1, 2]
A bespoke survey was designed and developed by the investigators based on perceived knowledge gaps in the current TC and atypical carcinoid tumours (AC) management pathway. e survey questions were developed by their medical team and validated by a neuroendocrine tumour- (NET-)specialist clinician. e survey consisted of 22 questions, which were based on topics including the epidemiology, diagnostics, and treatment pathways for lung NETs, focusing on TC and AC. e results of the survey were intended to describe centre-level management rather than the practices of the individual respondents
Of the 27 respondents, 18 provided estimates of the number of new patients presenting to their centres with TC or AC per year, with estimates ranging between 2 and 80 overall (6–80 patients per year for centres of excellence (CoE) respondents and 2–29 patients per year for non-CoE respondents). e estimated proportion of new patients presenting with advanced disease was 10% for TC and 30% for AC

Summary

Introduction

Pulmonary carcinoid tumours (PC), a subset of pulmonary neuroendocrine tumours (NETs), are rare neuroendocrine epithelial malignancies [1, 2]. PC are classified as well-differentiated low-grade (or grade 1) typical carcinoid tumours (TC) and poorly differentiated intermediate-grade (or grade 2) atypical carcinoid tumours (AC) [1,2,3,4,5]. Accurate and timely diagnosis of PC can determine the success of treatment as each subtype requires a specific treatment approach [2, 9]. TC and AC have subtle histopathological differences [11, 12] that make accurate diagnosis more challenging. Patients with AC and TC require different treatment approaches [15], emphasising the importance of employing a clear diagnostic strategy involving the use of appropriate diagnostic tools [16]

Methods

Results

Conclusion