BackgroundCarcinoid syndrome from pulmonary carcinoids without hepatic metastases is rare and diagnostically challenging when biochemical markers are normal.Case PresentationA woman in her mid‐60 s presented with an incidental right adrenal mass during evaluation for dyspnea and elevated D‐dimer. She reported a 20‐year history of paroxysmal tachycardia and 5 years of episodic flushing, profuse sweating, exertional dyspnea, and fine tremors triggered by minimal physical activity. Imaging revealed a 3.6 cm lipid‐rich adrenal adenoma and an 8 × 10 mm left upper lobe pulmonary nodule. Comprehensive biochemical evaluation was normal: 24 h urinary 5‐hydroxyindoleacetic acid 6 mg/d (normal 0–15), chromogranin A 50 ng/mL (normal 0–187), plasma metanephrine 0.12 nmol/L (normal 0.00–0.49), and normetanephrine 0.68 nmol/L (normal 0.00–0.89), excluding pheochromocytoma. Fine needle aspiration of the left pulmonary nodule confirmed a typical carcinoid tumor. Left upper lobe trisegmentectomy revealed low‐grade neuroendocrine neoplasm (pT1b pN0) with minimal mitotic activity (<1 per 10 HPF) and no necrosis. Postoperative 68Ga‐DOTATATE PET/CT demonstrated a radiotracer‐avid suspicious right lower lobe nodule (SUVmax 2.8) without hepatic metastases. Surgery markedly improved carcinoid syndrome symptoms, with residual episodes responding to long‐acting octreotide 20–30 mg every 4 weeks.DiscussionThis case illustrates that pulmonary carcinoid tumors can present with carcinoid syndrome despite normal biochemical markers and the absence of hepatic metastases. The temporal improvement following resection and response to octreotide established the diagnosis when biochemical testing was uninformative.ConclusionClinicians should maintain high suspicion for pulmonary carcinoids in patients with unexplained paroxysmal symptoms, even with negative biochemical testing and absent metastatic disease.

A Typical bronchial carcinoid tumor is a rare malignant neoplasm of neuroendocrine origin. The clinical presentation is usually nonspecific, and the diagnosis is made by fibrobronchoscopy, histopathologic confirmation, and immunohistochemistry. The treatment of choice is complete surgical resection with preservation of lung tissue, with a 20-year survival rate of 94%. This clinical case stands out due to the infrequency and nonspecific presentation of a bronchial carcinoid tumor in adolescence, highlighting the importance of recognizing it as an alternative diagnosis in the face of persistent respiratory symptoms without improvement—a male adolescent with a history of recurrent Pneumonia and persistent atelectasis in the right lung base. Bronchoscopy showed a 100% occlusive endobronchial mass, with pathology and immunohistochemistry confirming the diagnosis of a typical carcinoid tumor. After staging studies and regular biochemical markers, it was decided to perform surgery for curative purposes. Resection of the right middle and lower lobes and of the tumor lesion, bronchoplasty, and mediastinal lymph node drainage were successfully performed. However, the patient presented a stationary evolution due to Pneumonia complicated with pleural effusion and pneumothorax that required antibiotic management for 21 days and closed thoracostomy plus thoracoscopy with decortication. Metastatic tumor involvement was ruled out with octreotide scintigraphy and infectious profile studies were extended, which confirmed infection by Mycobacterium tuberculosis. The importance of the diagnostic suspicion of a neoplasm in the management of an adolescent with recurrent Pneumonia or atelectasis (in the exact location), which is uncommon, where bronchoscopy and computed tomography (CT) could guide the diagnosis.

Background . Neuroendocrine tumors associated with ACTH-ectopic secretion are rare, and pose a diagnostic challenge due to varied symptoms, leading to delayed treatment. In addition, the presence of ectopic Cushing syndrome significantly increases the risk of metabolic, infectious and surgical complications, but surgery remains the primary and curative treatment for these patients. aim of study: to improve the treatment outcomes of patients with neuroendocrine lung tumors associated with ACTH-paraneoplastic syndrome. Case presentation . A 65-year-old female patient presented to the Kommunarka Medical and Clinical Center with neuroendocrine lung tumor associated with ACTH-ectopic syndrome. The patient underwent a comprehensive laboratory and instrumental examinations to assess the extent of the tumor, functional status, hormonal profile, and to differentiate between ACTH-dependent and ACTH-independent hypercorticism. The patient underwent thoracoscopic lobectomy. In the postoperative period, there was a regression of the clinical and serological manifestations of the ACTH-ectopic syndrome. Histological and immunohistochemical examinations of surgical specimen confirmed the diagnosis of typical lung carcinoid. Conclusion . This case report and literature analysis demonstrate challenges in diagnosing lung carcinoid tumors, the need for a polyvalent approach, preoperative correction of endocrine disorders, and radical surgery, which provides the best prognosis.

BackgroundNeuroendocrine tumours (NETs) represent a heterogeneous group of neoplasms arising from neuroendocrine cells, which are diffusely distributed throughout the body. The lungs and gastrointestinal tract are the most frequent primary sites. This study aimed to evaluate 5-year overall survival (OS) rates across various subtypes of pulmonary NETs (PNETs) following anatomical resection. Furthermore, it sought to identify potential prognostic factors influencing survival outcomes.MethodsA retrospective analysis was performed on patients diagnosed with PNETs who underwent anatomical resection at our institution between 2008 and 2023. Tumour staging was conducted in accordance with the 8th edition of the International Association for the Study of Lung Cancer (IASLC) classification. Survival outcomes were estimated using the Kaplan-Meier method, and prognostic factors were analysed via Cox regression analysis.ResultsBetween 2008 and 2023, a total of 3,345 patients underwent anatomical resections for lung cancer, of whom 145 were diagnosed with NETs. The overall 5-year survival rate for this cohort was 77%. Subgroup analysis revealed survival rates of 87% for typical carcinoid (TC) tumours, 83% for atypical carcinoid (AC), and 86% for large cell neuroendocrine carcinoma (LCNEC). In contrast, the 5-year survival rate for patients with small cell lung carcinoma (SCLC) was 59%.ConclusionsSurgical resection remains an effective therapeutic strategy for all histological subtypes of PNETs. Our 5-year survival analysis indicates that tumour histology, postoperative tumour category (T category), and N2 nodal status are the most significant prognostic factors influencing survival.

Disclosure: F.C. Yap: None. J.Z. Lacson: None. J.P. de Leon: None. M.G. Famorcan: None. A. Hernandez: None. L.B. Mercado-Asis: None.Introduction:Bronchopulmonary carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that account for 0.5-5% of pulmonary malignancies. These tumors often challenging to diagnose due to their diverse presentations and the absence of distinct biochemical markers. Compared to other lung cancers, these types of tumor generally have more favorable prognosis, however their potential complications such as carcinoid crisis post significant risk.Clinical Case:We present a case of a 26-year-old male diagnosed with a typical bronchopulmonary carcinoid tumor in the left main bronchus. This case highlights the importance of a comprehensive, multidisciplinary approach to the diagnosis and management of this rare condition. Critical to the successful outcome was meticulous preoperative preparation, including the administration of octreotide to prevent a potentially life-threatening carcinoid crisis.Preoperative planning also involved an extensive pulmonary function assessment to evaluate the patient’s respiratory capacity and the risks associated with surgical resection. Despite negative biochemical markers for carcinoid syndrome, the patient presented with symptoms typical of this condition. This highlights the importance of clinical assessment in diagnosing rare tumors, especially when laboratory findings are inconclusive.Following a thorough evaluation, the decision was made to proceed with surgery. The patient then underwent a post-operative pulmonary rehabilitation program aimed to optimize recovery and return patient to baseline conditions.Conclusion:This case highlights the effectiveness and essentiality of multidisciplinary collaboration that begins from diagnosis and preoperative planning, as well as individualized postoperative care to achieve the best outcome for patients presenting with bronchopulmonary carcinoid tumors. A tailored, multi-disciplinary approach combining surgical expertise, early diagnosis, genetic evaluation, and long-term surveillance is crucial for the successful management of bronchopulmonary NETs.Presentation: Sunday, July 13, 2025

Bronchial sleeve resection is the preferred technique over pneumonectomy for centrally located tumours, preserving lung function and improving survival. We present a uniportal robotic-assisted thoracic surgery (RATS) technique for left main bronchus (LMB) sleeve resection with secondary carinal reconstruction in a patient with a typical carcinoid tumour. Through a single 4 cm incision, the LMB and its bifurcation were resected and reconstructed using a continuous absorbable barbed suture. Postoperative recovery was uneventful, with chest drain removal and discharge on postoperative day 2. Final pathology confirmed tumour-free margins without lymph node involvement. Uniportal RATS offers a feasible and minimally invasive option for complex airway reconstruction, potentially improving postoperative recovery while maintaining oncological standards in selected patients with appropriate surgical expertise.

Lung carcinoids are rare neuroendocrine tumors for which surgical resection is often curative. Proximal location can increase the complexity of removal. We report a case of a 27-year-old female with a proximal left mainstem bronchial carcinoid tumor. Chronic obstruction resulted in irreversible damage to the left lung. The patient underwent a successful left carinal pneumonectomy with extracorporeal membrane oxygenation (ECMO) support using a modified bypass circuit. Postoperative recovery was uneventful, with discharge on day 7. Histopathology confirmed complete resection of a typical carcinoid tumor with no malignant adenopathy. This case highlights management of proximal bronchial carcinoids that can require airway reconstruction and the importance of a multidisciplinary approach, including mechanical support for oxygenation. To our knowledge, this is the first report of carinal pneumonectomy on ECMO support in the COSECSA region. Further work is needed to identify patients and optimize diagnostic and therapeutic strategies that expedite care.

Pulmonary neuroendocrine proliferations and neoplasms represent a broad spectrum of diseases, ranging from neuroendocrine cell hyperplasia and tumorlets to carcinoid tumors. Carcinoid tumorlets are most commonly located in the peripheral airways and are often incidentally detected as pulmonary micronodules on chest CT. We report the radiological, bronchoscopic, and pathological findings of a case of carcinoid tumorlets presenting as endobronchial nodules in the left main bronchus. The patient had previously undergone a left lower lobectomy five years earlier for a typical carcinoid tumor. Follow-up imaging revealed new endobronchial nodules, which were subsequently confirmed as carcinoid tumorlets through histopathologic analysis. This case highlights the rare presentation of carcinoid tumorlets in the central airways, emphasizing the importance of recognizing their potential for late recurrence and atypical localization. It underscores the necessity for physicians to be aware that pulmonary neuroendocrine tumors can recur over the long term and may present in a multicentric fashion within the disease spectrum.

Bronchial carcinoids are rare childhood malignancies, comprising only a small proportion of pediatric lung tumors. This report details the case of a 9-year-old girl with persistent pneumonia that lasted one month and remained unresponsive to antibiotic therapy. Radiological imaging revealed atelectasis of the superior lobe of the left lung, while computed tomography showed an endobronchial lesion in the left main bronchus. An endobronchial biopsy confirmed the diagnosis of a typical bronchial carcinoid tumor. The tumor was completely resected using bronchoscopy, and cryotherapy was used to prevent the tumor's recurrence. No evidence of tumor recurrence was observed in subsequent follow-up bronchoscopy. This case underscores the importance of considering bronchial carcinoid tumors in the differential diagnosis of pediatric patients with recurrent pneumonia or wheezing that is resistant to standard treatments. Through early identification and minimally invasive management, such as bronchoscopy and cryotherapy, it is possible to achieve successful outcomes and avoid more extensive surgery. Our findings add to the scarce literature on pediatric bronchial carcinoid tumors and highlight the instrumental role of prompt and accurate diagnosis in improving prognosis.

Carcinoid neoplasms, even rare, are the most common pulmonary tumors during childhood. They are classically divided into typical and atypical tumors according to pathology pattern. Cornerstone therapy is to obtain a complete tumor resection. The aim was to describe clinical characteristics and treatment of young patients diagnosed with bronchial carcinoid tumors (BCT) in France. Using the National Registry of Childhood Cancers and the pediatric very rare tumors FRACTURE (French Group for Rare Childhood Tumors) database, we conducted a nationwide retrospective multicenter study including patients aged under 18years diagnosed with a BCT between 2011 and 2022. Thirty-eight patients were included (median age: 15.6years). Diagnosis was performed during bronchoscopy (n = 23) or by immediate resection (n = 14) (missing data [MD]: 1). Twenty-six patients (68%) had typical carcinoid tumors. Tumors were mainly localized (T1/T2-N0-M0; 28 cases); five had regional lymph node involvement (LN+) and none had metastatic disease. All patients underwent tumor resection, mainly with lobectomy (n = 29) associated to lymph node dissection (35 cases, including five LN+; MD: three). Pneumonectomy was required in three cases. Margin status was complete in 36 cases (microscopic residue: two cases). No patient received medical treatment as first-line therapy. With a median follow-up of 33months (range: 0-120), one patient with N0 R0 typical BCT developed a distant relapse. Five-year progression-free and overall survivals were, respectively, 87.5% (95% confidence interval: 38.7-98.1) and 100%. BCTs are rare and frequently "typical," with a very favorable prognosis in children after an exclusively surgical strategy, even in LN+ cases.

ABSTRACT Pulmonary neuroendocrine neoplasms are rare malignancies, with typical carcinoid (TC) tumors accounting for 2% of lung cancers. Carcinoid heart disease is uncommon, and pericardial involvement without valvular heart disease is exceedingly rare. We present a 40-year-old male with TC tumor and extensive mediastinal involvement who developed retrosternal discomfort, ultimately diagnosed as carcinoid heart disease. Imaging and clinical evaluation confirmed acute pericarditis, which progressed to impending cardiac tamponade, necessitating emergency pericardiocentesis.

Pulmonary neuroendocrine cells: Spectrum of diseases and their radiological-pathological correlations.

Abstract Introduction: Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare pulmonary disease that predominantly affects middle-aged, non-smoking women, often presenting with symptoms like chronic cough and dyspnea. DIPNECH is considered a pre-invasive precursor to pulmonary carcinoid tumors, but due to symptom overlap with obstructive airway diseases, it is frequently misdiagnosed. This case highlights a 56-year-old female with a chronic cough and dyspnea, initially attributed to a post-COVID-19 respiratory syndrome, underscoring the diagnostic challenges associated with DIPNECH. Case: A 56-year-old non-smoking female accountant with a history of chronic allergic rhinitis and hypertension presented with an intermittent cough, dyspnea, and occasional wheezing, which had persisted since a COVID-19 infection in 2021. Nine months prior to her pulmonary consultation, imaging revealed multiple non-calcified nodules, including a dominant nodule in the right upper lobe measuring 19 x 21 mm. A PET scan showed mildly increased uptake (SUV 4.3) in the dominant nodule. Initial pulmonary workup included pulmonary function tests showing severe parenchymal obstruction with significant air trapping. She was started on inhaled corticosteroids with symptomatic improvement. A follow-up CT demonstrated diffuse bronchial wall thickening and mosaic attenuation with the dominant nodule now reduced to 15 x 13 mm. Due to her persistent respiratory symptoms and radiographic findings, robotic bronchoscopy was performed to biopsy the nodule. Pathology revealed a submillimeter neuroendocrine tumorlet, staining positive for neuroendocrine markers, consistent with DIPNECH. Although the PET findings raised concerns about an atypical carcinoid, subsequent DOTATATE PET/CT showed no significant uptake, ruling out metastasis. The patient underwent robotic wedge resection of the right upper lobe, which confirmed a typical carcinoid tumor with negative margins and no lymph node involvement (T1a, N0). She is scheduled for follow-up imaging in six months and continues to do well postoperatively. Discussion: DIPNECH often presents as a chronic, undiagnosed cough in middle-aged, non-smoking women. Frequently misdiagnosed as asthma or other obstructive airway conditions, the rarity of DIPNECH, combined with its overlapping symptoms, often leads to delayed diagnosis. The case emphasizes the importance of considering DIPNECH in patients with unexplained respiratory symptoms and persistent lung nodules. Current treatment focuses on symptom management using asthma therapies, with somatostatin analogs also showing potential benefit. Regular monitoring through imaging and pulmonary function testing is recommended for early detection and intervention to prevent progression to neuroendocrine tumors. Enhanced awareness and early diagnosis are crucial in improving patient outcomes for this uncommon pulmonary disease.

Carcinoid tumors, a subset of neuroendocrine tumors, are exceptionally rare in the nose and paranasal sinuses. Owing to their indolent nature, nonspecific clinical symptoms, and variable radiological features, treatment of carcinoid tumors remains a challenge. In this report, we present a case involving a 49 year-old female patient who presented with a carcinoid tumor originating in the sphenoid sinuses. The patient underwent a surgical resection of the tumor and has been followed up in the outpatient clinic, recurrence-free. In addition, we performed an English literature search of cases of carcinoid tumor of the nose and paranasal sinuses from January 2000 through January 2024 in the MEDLINE, EMBASE, and Scopus databases. Furthermore, the clinical characteristics and interventions of carcinoid tumor of the nose and paranasal sinuses were discussed, which might enhance the early diagnosis and provide appropriate management strategies.

Background: Primary lung tumors in pediatric patients are rare, predominantly malignant, and present diagnostic challenges due to symptom overlap with more common conditions such as inflammatory processes or asthma. Evidence-based approaches for managing these rare neoplasms in childhood are scarce. This retrospective study reports the experience of a pediatric referral center in diagnosing and treating these tumors. Methods: Pediatric primary lung tumors treated at Giannina Gaslini Children's Hospital between January 2016 and January 2024 were included. Data on clinical presentation, histopathology, imaging, treatment approaches, and outcomes were systematically collected and analyzed. Results: Nine patients (six males and three females) were identified, with a mean age (±SD) at diagnosis of 8.81 ± 5 years. The most common clinical manifestation was recurrent pneumonia (four patients), followed by persistent cough and wheezing (three patients). The average duration of symptoms before diagnosis was 12.8 months ± 12.2 months. Histopathological diagnoses were typical carcinoid tumors (n = 2), atypical carcinoid tumors (n = 2), inflammatory myofibroblastic tumors (n = 2), congenital peribronchial myofibroblastic tumor (n = 1), myoepithelial carcinoma (n = 1), and pleuropulmonary blastoma (n = 1). Radical surgery resulted in complete response for seven patients, with a median follow-up of 52 months (IQR 39 months). The myoepithelial carcinoma was treated with multimodal therapy, relapsed after 17 months, and adjuvant chemotherapy is currently ongoing. Neoadjuvant chemotherapy for the pleuropulmonary blastoma is currently ongoing. Conclusions: Primary lung tumors in children, though rare, may have favorable outcomes when appropriately managed. Nonspecific clinical presentations often contribute to diagnostic delays. This study highlights the critical need of thorough evaluation in cases of persistent, therapy-resistant aspecific respiratory symptoms. Early diagnosis, coupled with complete surgical resection, significantly improves prognosis.

Pulmonary hamartoma, despite its benign nature, can pose diagnostic challenges due to imaging findings that mimic malignancy. This case series discusses the diagnostic and therapeutic difficulties in pulmonary hamartomas. These three cases that are presented here were surgically managed under suspicion of malignancy and one case was diagnosed as a typical carcinoid tumor postoperatively. The study retrospectively evaluated four cases referred for surgery due to suspected malignancy. The cases were analyzed in terms of radiological, surgical, and pathological findings. Three cases showed benign features but were operated on due to suspected malignancy and former history of malignancy, and were confirmed as hamartomas on pathology. One case, despite radiological features suggesting benignity, was identified as a typical carcinoid tumor postoperatively. Pulmonary hamartomas, despite their benign nature, can mimic malignancy and rarely undergo malignant transformation. Therefore, a multidisciplinary approach, surgical resection, and histopathological examination are essential for accurate diagnosis. Incorporating genetic analyses could enhance the diagnostic process further.

Background Typical carcinoid is a subtype of neuroendocrine neoplasia of the lung making up <1% of all lung cancers and 2% of resected lung tumors. This study assessed the impact of demographic features, racial and gender disparities, and tumor characteristics on survival in patients with typical carcinoid tumors. Methods Data were extracted from the SEER database from 2000 to 2018. Multivariate Cox regression analyses were used to analyze the survival based on demographic and clinical factors. Results We identified 11,713 cases of typical carcinoid tumors of the lungs. The mean age was 61.8 years (standard deviation, 14.7), and the tumors affected predominantly women (70.1%) and White (89.4%) individuals. The overall 5-year survival rate was 84.3% (95% confidence interval [CI] 83.5–85.2). The 5-year survival rate for those treated with systemic therapy was 49.8% (95% CI 43.5–55.9). Those treated with surgery had a 5-year survival rate of 97.2% (95% CI 96.7–97.6), and those treated with radiation had a 5-year survival rate of 71.9% (95% CI 60.3–80.6). Combination therapy provided a survival rate of 86.9% (95% CI 64.6–95.6). Conclusion Surgery was the most common treatment modality and offered the best prognosis. On multivariate analysis, age >60, male gender, distant spread, and liver metastases were associated with worse prognosis.

Background: Bronchial sleeve resection with complex reconstruction is a rare and intricate surgical procedure, particularly when addressing metastatic carcinoid tumors. This case report details the surgical management of a young male with a typical carcinoid tumor metastasized to the hilar and subcarinal lymph nodes. Case Presentation: A 28-year-old medically fit male presented with cough and occasional blood-tinged sputum for 2 months that was diagnosed to be due to a typical carcinoid tumor involving the left main bronchus, with metastasis to the hilar and subcarinal lymph nodes. The patient underwent a left bronchial sleeve resection with complex reconstruction of the left lower lobe bronchus. The reconstructed bronchus was then anastomosed to the main bronchus followed by hilar and subcarinal lymph nodes dissection. The surgical approach aimed to preserve lung parenchyma while ensuring complete tumor resection. Postoperative recovery was uneventful, with the patient demonstrating satisfactory respiratory function. Histopathological examination confirmed the complete resection of the carcinoid tumor and metastatic lymph nodes (hilar and inter-lobar LN (positive 2/5) and subcarinal LN (positive 1/6)). The patient had no signs of recurrence at the 3-month follow-up. Conclusions: This case highlights the feasibility and effectiveness of bronchial sleeve resection with bronchial reconstruction in managing metastatic carcinoid tumors. The successful outcome underscores the importance of meticulous surgical planning and execution in achieving favorable results in complex thoracic surgeries.

Advanced age chest wall hamartoma and typical carcinoid tumor: a very rare coexistence

Aims: This study aims to compare the efficacy of FDG-18 PET/CT and 68Ga-DOTATATE PET/CT imaging techniques in patients with lung carcinoid tumors, identifying the most appropriate preoperative nuclear medicine technique for diagnosis and staging. Methods: We retrospectively analyzed data from 123 patients who underwent surgery for lung carcinoid tumors at our center between 2009 and 2021. All of the patients were scanned with FDG-18 PET/CT before surgery. In addition to FDG-18 PET/CT, 68Ga-DOTATATE PET/CT scanning was performed in 28 patients: 17 in the preoperative and 11 in the postoperative period. Demographic data, mean higher maximal standard uptake (SUVmax) values of primary mass, lymph nodes and extrathoracic foci, pathologic subtype, and type of surgery were recorded. Compliance with normal distribution for numerical data was assessed using the Shapiro-Wilk test. The Wilcoxon signed-rank test was employed for groups meeting normal distribution to compare continuous numerical variables. The Mann-Whitney U and Kruskal-Wallis tests were used when normal distribution assumptions were unsatisfied. Results: The mean SUVmax value in 68Ga-DOTATATE PET/CT was significantly higher than FDG-18 PET/CT in patients with a lung carcinoid tumor (20 vs 4.4). For 68Ga-DOTATATE PET/CT scan, typical carcinoids had higher mean SUVmax value than atypical carcinoids (26 and 5.6 respectively), and the difference was statistically significant (p=0.002). For that FDG18 PET/CT, on the contrary, the mean SUVmax value was higher in atypical carcinoids than typical carcinoids (5.4 and 3.8, respectively), and the difference was not significant (p=0.126) Conclusion: The SUVmax values from 68Ga-DOTATATE PET/CT and FDG-18 PET/CT in lung carcinoid tumors vary by tumor subtype. 68Ga-DOTATATE PET/CT demonstrates higher SUVmax values in typical carcinoid tumors, indicating its superiority over FDG-18 PET/CT for this subtype. Although 68Ga-DOTATATE PET/CT also shows elevated SUVmax in atypical carcinoid tumors, the difference compared to FDG-18 PET/CT does not reach statistical significance.