The treatment of pulmonary carcinoid has changed over the last decades. Although surgical resection is still the gold standard, minimally invasive endobronchial procedures have emerged as a parenchyma sparing alternative for tumors located in the central airways. This review was performed to identify the optimal treatment strategy for pulmonary carcinoid, with a particular focus on the feasibility and outcome of parenchyma sparing techniques versus surgical resection. A systematic review of the literature was carried out using MEDLINE, Embase and the Cochrane databases, based on the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement. Two separate searches of publications in endobronchial and surgical treatment in patients with pulmonary carcinoid, were performed. Outcomes were overall survival, disease free survival, recurrence rate, complications, quality of life, and healthcare costs. Combining the two main searches for endobronchial therapy and surgical therapy yielded 3111 records. Finally, 43 studies concerning surgical treatment and 9 studies related to endobronchial treatment for pulmonary carcinoid were included. Assessment of included studies showed that lymph node involvement, histological grade, tumor location and tumor diameter were identified as poor prognostic factors and seem to be important for patients with pulmonary carcinoid. For patients with a more favorable prognosis, tumor location and tumor diameter are important factors that can help decide on the optimal treatment strategy. Centrally located small intraluminal pulmonary carcinoids, without signs of metastasis can be treated with minimally invasive alternatives such as endobronchial treatment or parenchyma sparing surgical resection. Patients with parenchyma sparing resections should be followed with long term follow up to exclude recurrence of disease. In a multidisciplinary setting, it should be determined whether individual patients are eligible for parenchyma sparing procedures or anatomical resection. Overall evidence is of low quality and future studies should focus on prospective trials in the treatment of pulmonary carcinoid.

Background: Traditionally, surgical resection is the preferred treatment for typical carcinoids and atypical carcinoids located in the lungs. Recently however, several studies have shown excellent long-term outcome after endobronchial treatment of carcinoid tumors located in the central airways. This study investigates clinical and radiological features as predictors of successful endobronchial treatment in patients with a bronchial carcinoid tumor. Objectives: To identify clinical and radiological features predictive of successful endobronchial treatment in patients with bronchial carcinoid. Methods: This analysis was performed in a cohort of patients with typical and atypical bronchial carcinoid referred for endobronchial treatment. Several patient characteristics, radiological features, and histological grade (typical or atypical carcinoid) were tested as predictors of successful endobronchial treatment. Results: One hundred and twenty-five patients with a diagnosis of bronchial carcinoid underwent endobronchial treatment. On multivariate analysis, a tumor diameter <15 mm (odds ratio 0.09; 95% confidence interval 0.02–0.5; p = <0.01) and purely intraluminal growth on computer tomography (CT scan) (odds ratio, 9.1; 95% confidence interval 1.8–45.8; p = <0.01) were predictive of radical endobronchial treatment. The success rate for intraluminal tumors with a diameter <20 mm was 72%. Conclusions: Purely intraluminal disease and tumor diameter on CT scan seem to be independent predictors for successful endobronchial treatment in patients with bronchial carcinoid. Based on these data, patients with purely intraluminal carcinoid tumors with a diameter <20 mm on CT scan are good candidates for endobronchial treatment, regardless of histological grade. In contrast, all patients with a tumor diameter ≥20 mm should be directly referred for surgery.

In the 2021 WHO thoracic tumors, gradation of lung carcinoids in biopsies is discouraged. We hypothesized that atypical carcinoid (AC) could be reliably diagnosed in larger preoperative biopsies. Biopsy-resection paired specimens of carcinoid patients were included, and definitive diagnosis was based on the resection specimen according to the WHO 2021 classification. A total of 64 biopsy-resection pairs (26 typical carcinoid (TC) (41%) and 38 AC (59%)) were analyzed. In 35 patients (55%), tumor classification between the biopsy and resection specimen was concordant (26 TC, 9 AC). The discordance in the remaining 29 biopsies (45%, 29 TC, 0 AC) was caused by misclassification of AC as TC. In biopsies measuring < 4 mm², 15/15 AC (100%) were misclassified compared to 14/23 AC (61%) of biopsies ≥ 4 mm². Categorical concordance of Ki-67 in biopsy-resection pairs at threshold of 5% was 68%. Ki-67 in the biopsy was not of additional value to discriminate between TC and AC, irrespective of the biopsy size. Atypical carcinoid is frequently missed in small bronchial biopsies (< 4 mm²). If the carcinoid classification is clinically relevant, a cumulative biopsy size of at least 4 mm² should be considered. Our study provides strong arguments to make the diagnosis of AC in case of sufficient mitosis for AC on a biopsy and keep the diagnosis “carcinoid NOS” for carcinoids with ≤ 1 mitosis per 2 mm². Ki-67 has a good concordance but was not discriminative for definitive diagnosis.

Background: Curatively treated bronchial carcinoid tumors have a relatively low metastatic potential. Gradation into typical (TC) and atypical carcinoid (AC) is limited in terms of prognostic value, resulting in yearly follow-up of all patients. We examined the additional prognostic value of novel immunohistochemical (IHC) markers to current gradation of carcinoids. Methods: A retrospective single-institution cohort study was performed on 171 patients with pathologically diagnosed bronchial carcinoid (median follow-up: 66 months). The risk of developing distant metastases based on histopathological characteristics (Ki-67, p16, Rb, OTP, CD44, and tumor diameter) was evaluated using multivariate regression analysis and the Kaplan–Meier method. Results: Of 171 patients, seven (4%) had disseminated disease at presentation, and 164 (96%) received curative-intent treatment with either endobronchial treatment (EBT) (n = 61, 36%) or surgery (n = 103, 60%). Among the 164 patients, 13 developed metastases at follow-up of 81 months (IQR 45–162). Univariate analysis showed that Ki-67, mitotic index, OTP, CD44, and tumor diameter were associated with development of distant metastases. Multivariate analysis showed that mitotic count, Ki-67, and OTP were independent risk factors for development of distant metastases. Using a 5% cutoff for Ki-67, Kaplan–Meier analysis showed that the risk of distant metastasis development was significantly associated with the number of risk predictors (AC, Ki-67 ≥ 5%, and loss of OTP or CD44) (p < 0.0001). Six out of seven patients (86%) with all three positive risk factors developed distant metastasis. Conclusions: Mitotic count, proliferation index, and OTP IHC were independent predictors of dissemination at follow-up. In addition to the widely used carcinoid classification, a comprehensive analysis of histopathological variables including Ki-67, OTP, and CD44 could assist in the determination of distant metastasis risks of bronchial carcinoids.

Recurrent Tracheoesophageal Fistula (rTEF) is a complication of Esophageal Atresia (EA) that can lead to severe respiratory symptoms. RTEF can be corrected via endotracheal treatment (ET) or surgical treatment (ST). The efficacy of these techniques varies in literature. This study presents the outcome of rTEF treatment after EA correction at the Wilhelmina Children's Hospital in Utrecht. From 2000 until 2023, patients with EA (n = 251) underwent thoracoscopic EA correction by using the native esophagus at the Wilhelmina Children's Hospital in Utrecht. All EA patients who had developed rTEF (n = 19) were evaluated. Success rates, number of treatment attempts, and complication rates of ET and ST were collected in our EA database. Data was expressed as medians with interquartile ranges (IQR). In total, 19/251 (8 %) patients had developed an rTEF after EA correction. For 10/19 (53 %) patients, primary ET was performed, with a median number of 2 procedures per patient [range 1-3]. In 8/10 patients, ET failed, therefore, these eight patients underwent secondary ST. For 9/19 (47 %) patients, primary ST was performed, with a median number of 1 procedure per patient [range 1-3]. In all patients, primary ST was performed via thoracoscopy with a success rate (absence of recurrence after 1 procedure) of 78 % (7/9) and no conversions. The complication rate was 10 % for primary ET and 44 % for primary ST, and 75 % for secondary ST. All complications were classified as minor. Thoracoscopic correction of rTEF has a high success rate. Similar to previous studies, the Utrecht success rate of ET for rTEF treatment is poor. Retrospective cohort study. IV.