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Cowden syndrome (CS) is a rare genetic condition due to the various germline mutations in the phosphatase and tensin homologue on chromosome ten (PTEN) tumour suppressor gene. As a result, CS is characterised by an increased risk of developing various benign and malignant tumours, such as thyroid, breast, endometrial and urogenital neoplasms, as well as gastrointestinal tract tumours. However, the neuroendocrine tumour association with CS is not elucidated yet. We present a case of a 46-year-old male patient diagnosed with testicular seminoma and follicular thyroid cancer in his medical history. Our patient met the clinical diagnostic criteria of Cowden syndrome. Genetic analysis established the clinical diagnosis; a known heterozygous PTEN mutation was detected [PTEN (LRG_311t1)c.388 C > T (p.Arg130Ter)]. Incidentally, he was also seen with multiple pulmonary lesions during his oncological follow-up. A video-assisted thoracoscopic left lingula wedge resection and later resections from the right lung were performed. Histological findings revealed typical pulmonary carcinoid tumours and smaller tumorlets. Somatostatin receptor SPECT-CT, 18 F-FDG-PET-CT and 18 F-FDOPA-PET-CT scans and endoscopy procedures could not identify any primary tumours in other locations. Our patient is the first published case of Cowden syndrome, associated with multifocal pulmonary carcinoids. Besides multiple endocrine neoplasia type 1, we propose Cowden syndrome as another hereditary condition predisposing to multiple pulmonary tumorlets and carcinoid tumours., (© 2024. The Author(s).)

Introduction: Ectopic Cushing's syndrome (CS) is a rare condition nevertheless well-known to endocrinologists. The pneumologist may be called upon to treat CS not only because bronchial carcinoid tumors are the most frequent source of ectopic ACTH secretion, but also due to the fact that the immunosuppression induced by hypercorticism favors lower respiratory tract infections., Case Report: We report the case of a female patient presenting with acute respiratory failure secondary to Enterobacter cloacae pneumonia exacerbated by SC. Further investigations confirmed ectopic ACTH secretion and revealed a right upper lobe pulmonary nodule. After appropriate antibiotic therapy, the patient received preoperative adrenolytic treatment. Management by right upper lobectomy resulted in the extraction of a 12mm tumor. Pathological analysis was consistent with the diagnosis of a typical carcinoid tumor. Immunohistochemistry confirmed ACTH secretion by the tumor. Even though the postoperative course showed CS regression, the patient developed adrenal insufficiency., Conclusion: Ectopic CS induces immunosuppression, which aggravates lower respiratory tract infections. Search for a pulmonary neuroendocrine tumor should be systematic. Following control of the secretory syndrome by adrenolytic treatment, and if the diagnosis of carcinoid tumor is confirmed, surgical treatment is the preferred option., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: We aimed to evaluate and compare the clinical, biochemical and radiological profile and outcomes of patients with ectopic ACTH syndrome (EAS) and Cushing disease (CD) treated over a period of 10 years (2013-2022)., Methods: In this ambispective observational study, we collected data for 146 patients with ACTH-dependent CS (EAS, n = 23; CD, n = 94; occult ACTH source, n = 29). Relevant details were filled in a predesigned proforma and outcomes were ascertained at the most recent visit., Results: EAS was more common in males (65.2 vs. 27.6%, p < 0.001). Patients with EAS had a shorter duration of symptoms [12 (6-12) vs. 31.5 (15-48) months, p < 0.001] and were more likely to have hypokalemia (82.6 vs. 21.0%, p = 0.001), pedal edema (65.2 vs. 34.2%, p = 0.015), weight loss (34.8 vs. 4.0%, p < 0.001) and systemic infection (30.4 vs. 6.5%, p = 0.006). They also had significantly higher 8 a.m. serum cortisol, midnight serum and salivary cortisol and 8 a.m. plasma ACTH levels. Bronchial carcinoid (n = 10, 43.5%) was the most common etiology of EAS. Bilateral adrenalectomy was performed in 11 (47.8%) patients with EAS. Eight patients (34.8%) with EAS died at the last follow-up, of whom 7 (87.5%) had metastatic disease. In CD group, overall remission rate was 69.4% (56.1%, early and 13.3%, delayed) and 26.3% of patients with an initial remission had recurrence., Conclusions: Bronchial carcinoid was the most common cause of EAS in our cohort. Bilateral adrenalectomy was performed in approximately every 1 in 2 patients with EAS and approximately every 1 in 3 patients expired till the last follow-up., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Although Cushing's Syndrome (CS) is usually caused by pituitary/adrenal adenomas, in the remaining few cases, Adrenocorticotrophic hormone (ACTH) is secreted by extrapituitary neuroendocrine tumors (NET). In typical pulmonary / bronchial carcinoid tumors leading to ectopic ACTH syndrome (EAS). The main principle of treatment is the localization of the ACTH-secreting tumor and its surgical removal. In this case report, we aimed to present two typical carcinoid cases, whose ACTH and cortisol levels returned to normal after lung surgery for ectopic ACTH. One of the cases, a 32-year-old female patient with CS symptoms and signs, was referred to our department after detecting a lesion in the left lower lobe in the thorax CT, which did not show an obvious focus on cranial MRI. The other patient, a 36-year-old male, had previously undergone adrenalectomy for Cushing's syndrome and was admitted to the emergency department due to adrenal insufficiency. The lesion seen in the right lower lobe on thorax CT was evaluated as an ectopic ACTH focus. After the tumors of the patients were resected according to surgical principles, CS clinic regressed, and ACTH and cortisol values returned to normal. Pathological examinations of the surgically resected tumors were reported as typical carcinoid. With surgically removed typical carcinoids tumors, excellent longterm survival results can be achieved and hypercortisolism can be controlled.

Objective: To determine the main forms of primary tracheal cancer (PTC), to specify the indications for various surgeries in these patients depending on extent and localization of lesion., Material and Methods: There were 263 PTC patients. Benign tumors were diagnosed in 68 (25.9%) patients, malignancies - in 195 (74.1%) cases. Tracheal cancer includes 3 basic morphological variants - adenocystic cancer (49.7%), carcinoid (18.7%) and squamous cell carcinoma (19.0%). Other forms of malignancies were much less common. We applied endoscopic intraluminal and open surgeries. In malignant PTC, open surgeries were performed in 165 (84.6%) out of 195 patients. Baseline palliative endoscopic treatment was performed in 30 patients. They underwent airway recanalization (with subsequent tracheal stenting in 19 patients). Endoscopic resection was preferred for benign tumors., Results: Twenty (12.1%) patients died after open surgery, and 1 (3.3%) patient died after endoscopic procedure. Most lethal outcomes occurred in early years of development of tracheal surgery. The causes of mortality were tracheal anastomotic failure in 12 patients, pneumonia in 6 patients, and arterial bleeding in 2 patients. Severe postoperative period was observed in all 3 patients after tracheal replacement with a silicone prosthesis. Long-term treatment outcomes depended on morphological structure of PTC. Favorable results were observed in patients with neuroendocrine tumor (carcinoid), worse outcomes in adenocystic cancer and unfavorable results in squamous cell carcinoma ( p <0.0013). Five-year survival rates were 75%, 65.6%, and 13.3%; 10-year survival rates were 75%, 56.2%, and 13.3%, respectively. These outcomes after combined treatment of primary tracheal cancer were significantly better compared to lung cancer ( p <0.05 when compared to global data)., Conclusion: Treatment of primary tracheal cancer should be based on classical principles of modern oncology (combined therapy, tumor resection with lymphadenectomy). Open and endoscopic interventions are justified. PTC is characterized by more favorable outcomes compared to lung cancer. It is difficult to analyze long-term results in tracheal cancer depending on various features of tumor process due to small number of observations. Accurate conclusions require multiple-center studies, preferably with international participation, which can convincingly prove certain concept.

Carcinoid tumors are relative rare neuroendocrine tumors of the lung. Bronchial obstruction's symptoms as cough, hemoptysis and pneumonia may be present when they are centrally located and lung-sparing surgery is usually preferred. We describe the case of an adult patient with a central right upper lobe carcinoid tumor and a concurrent tracheal bronchus malformation. This peculiar bronchial malformation allowed a sleeve S2 segmentectomy sparing the S1-S3 segments and the middle and lower lobes. The patient was discharged on day 4 without complications.

Cushing’s syndrome (CS) is rare in childhood and adolescence. The most common paediatric cause of CS is exogenous administration of glucocorticoids; either topical, inhaled or oral corticosteroids. Endogenous causes can be classified into adrenocorticotropic hormone (ACTH) independent and ACTH dependent causes. Herein, we report our experience of managing a 12 year old girl who presented with features of CS and was found to have an ectopic, ACTH-secreting bronchial carcinoid tumour, which was resected surgically. Our patient was managed successfully by multidisciplinary approach and has recovered from hypertension and Cushing’s habitus. The English language literature was searched from 2019 back, using PubMed, Google and Google Scholar. Keywords used for the search were; “Ectopic ACTH syndrome (EAS) in children”, “bronchial carcinoid in children” and “Cushing’s Syndrome in children”. Children with bronchial carcinoid tumours causing EAS were identified. Case variables such as age, sex, type of carcinoid, investigations, surgery, recurrences and outcome were reviewed. Fourteen cases of paediatric bronchial carcinoid producing ACTH were found with a mean age of 15.8 years and female preponderance. Most of the patients had a right lung lesion and histological appearance was typical of carcinoid tumour. Bronchial carcinoid is extremely rare in children and only 4% are associated with CS. The postoperative treatment of CS is challenging with a high prevalence of hypertension, increased body mass index and visceral fat mass, impaired cognitive function and decreased quality of life. A careful follow up is indispensable for monitoring recurrence of carcinoid and complete remission of CS.

Treatment of Cushing syndrome depends on diagnosis of etiology responsible for hypercortisolism in the body, which sometimes presents with a challenge. Inferior petrosal sinus sampling for ACTH levels, followed by peripheral venous sampling is a proven tool to be a gold standard for differentiating between peripheral and central cause of ACTH dependent Cushing syndrome. This case report is of an elderly female who presented as an outpatient in the endocrinology clinic of Aga Khan university hospital on 22/6/2017 with clinical features of hypercortisolism. After workup she was found to have cushing syndrome secondary to ACTH secreting bronchial carcinoid tumour.

Background: Postpneumonectomy-like syndrome is a rare condition resulting from unilateral lung disease with severe lung volume loss leading to excessive mediastinal shift and herniation of the healthy lung into the contralateral hemithorax, mimicking the mediastinal shift observed in postpneumonectomy syndrome after pneumonectomy. We report a unique case of postpneumonectomy-like syndrome caused by an atypical bronchial carcinoid completely occluding the left main bronchus., Case Presentation: A 25-year-old woman presented with symptoms of chronic exertional dyspnea and productive cough. Imaging studies showed complete left lung atelectasis due to a mass occluding the left main bronchus, as well as extreme mediastinal deviation and substantial herniation of the right lung into the left hemithorax. Bronchoscopic biopsy of the tumor and subsequent left pneumonectomy with concurrent lymph node dissection revealed an atypical carcinoid. Sixteen months after surgery the patient has been asymptomatic with repeat imaging studies showing no change in mediastinal shifting., Conclusion: Bronchial carcinoids are notorious for causing bronchial obstruction. The present case represents an extreme complication of centrally located bronchial carcinoid, resulting in postpneumonectomy-like syndrome with severe mediastinal shift and herniation of the healthy lung into the diseased hemithorax.

Background: Pulmonary carcinoids are included in the group of neuroendocrine tumors (NET) and derive from pulmonary neuroendocrine cells. The incidence of these tumors is increasing, but disease awareness remains low among clinicians. The synchronous presentation of lung cancer and mycobacterial infection is well known but the combination of pulmonary carcinoid and mycobacterial infection is rare., Case Presentation: We treated a 45-year-old female who presented with recurrent pneumonia. Chest X-ray showed a consolidation in the left upper lobe. The patient was treated with various courses of antibiotics without full recovery after six months. Computed tomography (CT) scan demonstrated a central mass in the left upper lobe. Bronchoscopy revealed an endobronchial, well-defined lesion that totally obstructed the left upper lobe bronchus. Bronchial biopsy showed typical carcinoid tumor. Rigid bronchoscopy with electrocautery was attempted, but we were unable to radically remove the tumor. Therefore lobectomy was performed. The surgical pathology specimen showed atypical bronchial carcinoid and consolidations in the lung parenchyma with granulomatous inflammation distally of the bronchial obstruction. Ziehl-Neelsen staining demonstrated acid fast bacilli indicative of mycobacterial infection., Conclusions: This case history illustrates the importance of careful surgical pathologic examination, not only of the resected tumor, but also of the postobstructive lung parenchyma. Specific postobstructive infections such as tuberculosis or nontuberculous mycobacteria (NTM) can have clinical implications.

Ectopic ACTH syndrome (EAS) is a potentially fatal endocrine disease that results from a variety of neuroendocrine tumors (NETs), such as small cell lung cancer (SCLC) and bronchial typical carcinoid. Typical carcinoid is usually slow growing, not associated with plasma progastrin releasing peptide (ProGRP) elevation. Here, we report a 47-year-old female smoker with progressive typical carcinoid and plasma ProGRP elevation. Several types of Cushingoid features were found on physical examination. In addition, laboratory examination showed elevated plasma ACTH and serum cortisol levels. These findings indicated ACTH-dependent Cushing's syndrome. Moreover, the serum cortisol level was not suppressed by overnight high-dose dexamethasone treatment, suggesting the presence of an extra-pituitary tumor. Contrast-enhanced brain MRI revealed no pituitary adenoma, which also supported the idea that EAS occurred in the present case. Strikingly, chest computed tomographic (CT) scan showed a single 18-mm peripheral nodule in the right middle lobe of the lung. Tumor marker analysis revealed an elevation in plasma ProGRP. These data suggested a possibility that SCLC secreted ACTH and caused EAS in this patient. Of note, the plasma ACTH level was increased (1.7 fold) in l-desamino-8-D-arginine vasopressin (DDAVP) test, also suggesting the specific clinical feature in this case. After additional imaging examinations, we performed surgical resection with the suspicion of limited SCLC. As a result, pathological examination revealed a vasopressin receptor Ib (V1b) receptor-negative bronchial typical carcinoid with ACTH production and mediastinal lymphatic metastasis. In summary, we present a case of EAS caused by progressive bronchial typical carcinoid with plasma ProGRP elevation. We propose a novel subtype of lung typical carcinoid.

Pneumothorax is a rare clinical manifestation of lung cancer. It can exceptionally reveal a bronchial carcinoid tumor. We present the case of a 27-year-old woman in whom recurrent pneumothoraxes were the clinical manifestation of a bronchial carcinoid tumor. The interest for chest computed tomography and bronchoscopy to identify etiology of secondary pneumothoraxes will be discussed., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: Bronchial carcinoid tumors (BCTs) are rare neuroendocrine neoplasms of the lung that mainly have a central distribution. They are classified as typical and atypical, with the former variant generally conferring a more favorable survival. Central tumors are usually symptomatic with features of bronchial obstruction, whereas peripheral tumors may remain silent., Case Report: A 36-year-old woman presented to our hospital due to an episode of massive hemoptysis 5 days prior to admission. She had experienced another episode of hemoptysis 4 years before, for which a chest x-ray had shown no pathological findings. A new chest x-ray showed complete collapse of the left lung, with remarkable tracheal deviation. Computed tomography revealed a large endobronchial lesion causing occlusion of the left main bronchus and significant mediastinal shift to the left. Despite the collapse of the left lung, no symptoms of bronchial obstruction were evident. The patient underwent a successful left pneumonectomy and pathology of the resected specimen revealed a typical stage pT2b N1 Mx endobronchial carcinoid tumor., Conclusion: Central tumors are usually symptomatic with features of bronchial obstruction, whereas peripheral tumors may remain silent. Although a significant amount of bronchial occlusion may be present, symptoms of obstruction might not be apparent. Hemoptysis should always be thoroughly investigated, as it may be the only sign of a severe underlying disease., (Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Objective: Cushing 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., Methods: From 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., Results: Median 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., Conclusion: BCs 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., (Georg Thieme Verlag KG Stuttgart · New York.)

Patients with multiple endocrine neoplasia type 1 (MEN1) are commonly evaluated for clinical manifestations of this syndrome with the rationale that early diagnosis and adequate treatment will result in improved survival and quality of life. Thymic and bronchial carcinoid tumours are uncommon but important manifestations of MEN1. Current practice guidelines recommend evaluation with computed tomography or magnetic resonance imaging scan of the chest every 1-2 years to detect these neoplasms. However, the certainty that patients will be better off (increased survival or quality of life) as a result of this case detection strategy is based on evidence at moderate-high risk of bias that yields only imprecise results of indirect relevance to these patients. In order to improve the care that patients with MEN1 receive, co-ordinated efforts from different stakeholders are required so that large, prospective, multicentre studies evaluating patient important outcomes are carried out., (© 2015 John Wiley & Sons Ltd.)

Endobronchial atypical carcinoid tumors are rare in young children and can have a varied presentation. Early diagnosis and surgical resection provide an excellent outcome and good long-term survival. However, due to the risk of recurrence and later metastasis, long-term follow-up is necessary.

Bronchial carcinoid tumours are an uncommon cause of recurrent pneumonia in young patients. Diagnosis is determined from imaging studies, bronchoscopy, and histological confirmation, and treatment is generally surgical. Two cases are reviewed in order to examine the value of (111)In-DTPA-Phe-octreotide ((111)In-pentetreotide) scintigraphy in the pre-surgical evaluation of these patients. After a suspicious area was observed in other tests (standard X-ray, CT), a neuroendocrine tumour was diagnosed using this technique and the presence of regional or distant disease was ruled out. Comparison with the less valuable (18)F-FDG PET (carried out in one of the cases) highlights the usefulness of SPECT-CT, which performs notably better in terms of the localization and characterisation of findings., (Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.)

Background: Carcinoid tumors (CTs) are rare in the pediatric population and are generally noted as an incidental finding on histopathologic examination. Cure is usually achieved with wide surgical excision. Second primary malignancies (SPM) of the gastrointestinal tract after CTs have been reported in 13% to 33% of affected adults. The risk of SPM appears highest after small bowel or appendiceal CTs and usually presents within 7 years from diagnosis., Purpose: The purpose of this study was to investigate the natural history of CTs in pediatric patients treated at a major children's hospital and to determine whether children and adolescents with primary CTs developed SPM during routine long-term follow-up., Methods: We conducted a retrospective review of the medical records of children and adolescents with CTs diagnosed at Nationwide Children's Hospital, Columbus, Ohio between 1945 and 2012., Results: Thirty-two patients with CT were identified, representing 0.48% of all malignancies diagnosed at Nationwide Children's Hospital. Mean age at presentation was 13 years (range, 8 to 20 y). The majority were appendiceal (87.5%) followed by bronchial (9.4%). Most of the appendiceal tumors presented with clinical appendicitis (25/28). Three had incidental appendectomies at the time of a planned abdominal surgery for other reasons. Four patients with appendiceal CTs had invasive features. All patients with appendiceal and bronchial CTs were successfully treated by complete surgical excision and were free of disease at an average of 7 years from diagnosis. None of our patients developed SPM during the period of observation (median 84 mo; range, 12 to 156 mo)., Conclusions: In this single-institution retrospective review, survival of children with CT was excellent. No SPMs were observed over the period of follow-up differing from previously reported adult CT series.

We report a rare case of acromegaly due to a growth hormone releasing hormone-secreting bronchial carcinoid tumour. A 40-year-old man initially presented with acromegalic features, and was subsequently found to have a large lung mass in the right lower zone on chest X-ray. Right lower lobectomy was performed, and the tumour was confirmed to be a bronchial carcinoid tumour on histology. Resection of the tumour led to normalisation of serum insulin-like growth factor 1 level and growth hormone responses to an oral glucose tolerance test.

Context: The ectopic production of ACTH is responsible for approximately 10% of cases of Cushing's syndrome. Whenever possible, once hypercortisolemia is under control with medical therapy, the final treatment consists of surgical excision of the tumor. We report a case of a patient with high surgical risk and poor response to medical therapy in which hypercortisolemia has been successfully treated with radiofrequency ablation of the bronchial carcinoid tumor., Case Presentation: A 43-year-old woman came to our hospital because of severe and rapidly worsening signs and symptoms of hypercortisolism over the previous 3 months. Hormonal tests suggested the presence of Cushing's syndrome due to ectopic ACTH production. Imaging studies detected an 8-mm pulmonary nodule with fluorine-18-fluorodeoxyglucose uptake localized in the middle right lobe. The patient started therapy with ketoconazole with poor response. Middle right lobectomy was indicated but, due to the patient's very high surgical risk, a thermal ablation with radiofrequency of the bronchial nodule was performed., Outcomes and Results: After the procedure, ACTH and cortisol levels dropped and fluorine-18-fluorodeoxyglucose positron emission tomography showed complete response to treatment. Clinical conditions progressively improved, and 6 weeks later, the patient underwent middle lobectomy without complications. Histology showed a 0.7-cm ACTH-producing typical bronchial carcinoid tumor., Conclusions: Thermal ablation with radiofrequency allows achieving a rapid control of hypercortisolism with subsequent improvement of symptoms. This procedure should therefore be considered as a viable therapeutic option in those cases of bronchial ACTH-secreting tumors in which the surgical approach is initially contraindicated.

We present a case of pneumothorax associated with an endobronchial carcinoid tumor in a 18-year-old man with dyspnea and chest pain. Additional tests were done, identifying in the chest roentgenogram a complete left pneumothorax with persistent leak, which was confirmed by computed tomography of the chest, and also finding an endobronchial lesion that limited the complete reexpansion of the left lung. Surgical excision was performed, and the lesion was identified as a typical bronchial carcinoid, with satisfactory outcome after the intervention., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: To bring attention to a rare diagnosis in the pediatric population that is in the differential diagnosis for not well-controlled asthma., Data Sources: Case presentation., Conclusions: Pulmonary carcinoid tumors are rare and usually present late in adolescence. Most of these tumors are located in the proximal airways and symptoms may be similar to those of asthma including cough, wheeze, chest pain, or recurrent pneumonia., Implications for Practice: Bronchial carcinoid should be in the differential diagnosis for adolescents with difficult to control asthma, who have symptoms including chronic cough and focal wheeze. Referral to a pulmonary specialist should be considered to help work up the differential diagnoses., (©2013 The Author(s) ©2013 American Association of Nurse Practitioners.)

We present a case of a middle-aged man with a 3-month history of progressive shortness of breath and peripheral oedema. Ten years prior to this, he had undergone a left pneumonectomy for metastatic bronchial carcinoid. Clinical examination revealed significant right heart failure, supported by transthoracic echocardiography. CT pulmonary angiogram revealed the cause to be marked progression of the bronchial carcinoid causing severe external compression of right pulmonary artery (RPA). In view of the distressing symptoms, a palliative endovascular intervention to the RPA was attempted to relieve obstruction, improve blood flow through the right lung and offload the right ventricle. This was performed under general anaesthesia involving interventional cardiology and radiology specialists together with a specialist anaesthetic team with extensive experience of managing carcinoid patients. The result was a marked improvement in symptoms and right heart function and the patient was discharged 2 days later.

Tumours that cause ectopic acromegaly can do so through the secretion of GH or GHRH. One hundred cases of ectopic acromegaly due to secretion of GHRH have been described. Given the rarity of this pathology, we present a clinical case with the aim of contributing our diagnostic-therapeutic experience and the subsequent follow-up. We present the case of a patient with acromegaloid physical features that had evolved over several years. Concomitantly, he also presented other accompanying symptoms that were suggestive of a possible bronchial origin. Facing the clinical suspicion of acromegaly, we opted to confirm it biochemically and subsequently through image study. A hypophysary origin was ruled out, so we carried out screening for a bronchial neuroendocrine and/or gastrointestinal tumor as they are the most frequent localizations. The treatment of choice was surgical resection.

Background and Objective: To describe the casuistry of bronchial carcinoid tumor in the last 20 years in our hospital and determine survival after surgical treatment., Patients and Method: We retrospectively reviewed the medical records from January 1992 to June 2012 of patients diagnosed with carcinoid tumor by the pulmonary service., Results: Fifty-two patients (87%) had typical carcinoid and 8 (13%) atypical carcinoid. The mean age at diagnosis was 60 years (SD: 14.4). There was no relationship between consumption of tobacco and carcinoid tumor. Twenty-two per cent were asymptomatic radiographic finding (incidental finding) Three patients showed carcinoid syndrome and one patient had Cushing syndrome. There was a right dominance and the mean lesion size was between 2.1 and 5 cm. Nine per cent had lymph node involvement, predominantly in atypical carcinoid. Overall survival at 3.5 and 10 years was 94%, 86% and 82%. Survival at 5 years was 90% for typical and 86% for atypical and survival at 10 years was 85% for typical and 57% for atypical carcinoids., Conclusion: Carcinoid tumors are malignant tumors by their ability to metastasize. In our study, both histological type and staging were predictors of survival., (Copyright © 2012 Elsevier España, S.L. All rights reserved.)

Objective: We report a 44-year old man with ectopic adrenocorticotropic hormone (ACTH) syndrome caused by bronchial carcinoid that developed Cushing syndrome., Methods: We performed several imaging studies, including chest and abdominal CT, for exploration of nodules and selective pulmonary arterial sampling for localizing a source of ectopic ACTH production., Results: The patient was diagnosed as Cushing syndrome due to ectopic production of ACTH without identification of its source(s). After 2 years' follow-up with repeated CT scans every 6-12 months and treatment with metyrapone, chest CT revealed two small nodules respectively in the segment (S) 4 and 10 of the right lung. We performed selective pulmonary arterial sampling from branches of the right pulmonary artery to obtain blood from the nodules in a reverse flow fashion: wedged sampling from the basal branch (A8, 9 and 10) revealed significant elevation of ACTH, whereas sampling from the lateral branch (A4) did not, indicating that the S10 nodule produced ACTH ectopically. The video-assisted thoracoscopic surgery removing the right inferior lobe normalized plasma ACTH, serum cortisol and 24-hour urinary free cortisol. The S10 nodule was histologically diagnosed as atypical bronchial carcinoid containing immunoreactive ACTH., Conclusions: Selective pulmonary arterial sampling was useful for localizing the lesion of ectopic ACTH production and helped make the decision for its surgical removal. This procedure should be considered once lung nodules suspicious for ectopic ACTH production are identified in patients with EAS.

Multiple Endocrine Neoplasia type 1 syndrome (MEN1) is characterized by the presence of tumors in parathyroid glands, anterior pituitary gland, endocrine pancreas and duodenum. However, other tumors may also occur. One of them is the carcinoid tumor, which in this context, is more common in the gastrointestinal tract. Less common is the presence of carcinoid tumors of bronchial origin, which with histologic confirmation, may occur in 5-8% of cases and that appears more frequently in patients with hypergastrinemia. We report a patient with MEN1 syndrome, hypergastrinemia and an incidental finding in a somatostatin receptor scintigraphy of an unsuspected bronchial carcinoid tumor that was confirmed histologically., (Copyright © 2012 Elsevier España, S.L. y SEMNIM. All rights reserved.)

Introduction: Cushing's Syndrome is an endocrine condition with complex diagnostic pathways. Cortisol suppression from high dose dexamethasone usually points to the pituitary as the cause. We present the case of a patient with dexamethasone suppressible Cushing's Syndrome from a bronchopulmonary carcinoid tumor. The tumor was only able to be localized with bronchoscopy. Our objective is to inform other physicians of dexamethasone suppressible carcinoid tumors which may require bronchoscopy to localize., Case Report: A 52-year-old female presented with signs and symptoms of Cushing's Syndrome. Cortisol and ACTH levels were significantly elevated. High dose dexamethasone suppressed cortisol production. However, no pituitary source was found. Standard imaging did not localize an ectopic source. The patient continued to have significant morbidity from the hypercortisolism. In order to avoid adrenalectomy, a bronchoscopy was empirically performed which revealed a bronchopulmonary carcinoid tumor., Discussion: Bronchopulmonary carcinoid tumor should be in the differential diagnosis of dexamethasone suppressible Cushing's Syndrome if a pituitary source is not localized. Also, we suggest that bronchoscopy be added to the diagnostic algorithm when conventional imaging studies fail to reveal the ectopic source. This may result in cure of the carcinoid malignancy as well as the Cushing's Syndrome.

Dedicated linear endobronchial ultrasound (EBUS) is an established modality in sampling central pulmonary lesions, mediastinal and hilar lymph nodes to diagnose and stage lung malignancies as well as evaluate a number of inflammatory conditions. In addition, power Doppler modality of EBUS allows for vascular evaluation. We describe a case where EBUS visualized a submucosal lesion within a vascular malformation, which was eventually proven to be a carcinoid tumor. Computed tomographic scan of the chest failed to differentiate the 2 structures and bronchoscopy showed no endobronchial component. This case underscores the added diagnostic capabilities of endobronchial Doppler ultrasonography. We suggest considering EBUS in investigating central opacities associated with vascular features within the lung parenchyma when technically feasible.

Neuroendocrine tumours require dedicated interventions to control their capacity to secrete hormones but also, antitumour growth strategies. Recommendations for early interventions in NET include the management of hormone-related symptoms and poorly differentiated neuroendocrine carcinomas. In contrast, prognostic heterogeneity is a key feature of well differentiated NET that complexified the antitumour strategy whatever the stage in this subgroup of tumour. In this review, timely therapeutic interventions to control hormone-related symptoms and tumour growth in GEP NET patients are discussed. The necessity of controlling hormone-related symptoms as the first step of any strategy affects also the tumour growth control strategy. In the absence of cure at the metastatic stage, progresses are expected in the recognition of well differentiated NET subgroups that display either excellent or poor prognosis., (Copyright © 2013. Published by Elsevier Ltd.)

Background: The aim of this study was to revisit the characteristics and outcomes of adrenocorticotropin-secreting bronchial carcinoid tumor (BCT) responsible for Cushing's syndrome (CS)., Methods: We conducted a single-institution retrospective review of 14 patients who underwent pulmonary resection for BCT that presented as CS from October 1993 to November 2011., Results: The group consisted of 8 male patients (57%) and 6 female patients. The mean age was 40 years (range, 16-63 years). Three patients (21%) underwent unnecessary adrenalectomy or hypophysectomy, or both, before diagnosis of the main cause. The mean interval between clinical presentation and the chest operation was 33 months (range, 3-136 months). Operations included 12 lobectomies (86%), 1 segmentectomy, and 1 wedge excision. All patients underwent radical lymph node dissection. Histologic examination showed 11 typical carcinoids (79%) and 3 atypical carcinoids. Twelve patients were classified pT1 (86%) and 2 patients were classified pT3 because of the presence of 2 tumors in the same lobe. Lymph node metastases were found in 7 patients (50%) (3 pN1 and 4 pN2). The mean follow-up was 59 months (range, 3-174 months). No recurrence was observed., Conclusions: Early detection of adrenocorticotropin-secreting BCTs is challenging. However, it avoids adrenalectomy and unnecessary hypophysectomy, limits the deleterious effects of chronic hypercortisolism, and reduces the risk of metastasis. The high prevalence of lymph node involvement confirms the aggressiveness of these tumors and justifies anatomic resection and radical lymph node dissection. Under these circumstances, the prognosis remains favorable, even in cases of N2 disease., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We review here the literature on neuroendocrine neoplasms metastatic to the pituitary and present an example of the disease. Metastasis of bronchial carcinoid tumors to the sellar region are rare. Herein, we describe the case of a 63-year-old woman who presented with constant cough and headaches. She had previously been operated for carcinoid tumor of the lung. During the preoperative investigation, a CT scan of the head revealed a sellar mass. Six months after a left lower lobectomy, the sellar lesion was removed by transsphenoidal surgery. The two tumors were evaluated by histology, immunohistochemistry and electron microscopy. Both showed identical morphologic features, those of carcinoid tumor. Immunohistochemistry revealed immunoreactivity for the endocrine markers, synaptophysin and chromogranin, as well as CD-56, serotonin, bombesin and vascular endothelial growth factor. The sellar neoplasm showed nuclear immunopositivity for thyroid transcription factor-1, supporting the diagnosis of a metastatic bronchial carcinoid tumor. In conclusion, this is the first report of a serotonin- and bombesin-immunopositive atypical bronchial carcinoid tumor metastatic to the sella.

Context: Ectopic GHRH secretion is a rare cause of acromegaly, and case reports are mainly isolated., Setting: From the registry of the sole laboratory performing plasma GHRH assays in France, we identified cases of ectopic GHRH secretion presenting with acromegaly between 1983 and 2008., Patients: Twenty-one patients aged 14-77 yr were identified from 12 French hospitals. Median GHRH was 548 (270-9779) ng/liter., Main Outcome Measures: Outcome measures included description of tumor features and outcome and the relation between plasma GHRH values and tumor site, size, and spread., Results: The primary neuroendocrine tumor was identified for 20 of 21 patients (12 pancreatic, seven bronchial, one appendicular). Tumors were large (10-80 mm), identified on computed tomography scan in 18 cases and by endoscopic ultrasound and somatostatin receptor scintigraphy in two. Somatostatin receptor scintigraphy had a similar sensitivity to computed tomography scan (81 vs. 86%). Tumors were all well differentiated; 47.6% had metastasized at the time of diagnosis of acromegaly. After a median follow-up of 5 yr, 85% of patients were alive. Ninety-one percent of patients whose tumor was completely removed were considered in remission, and most had normalized plasma GHRH. The remaining patients were treated with somatostatin analogs: IGF-I normalized except for one patient who required pegvisomant, but GHRH levels remained elevated. No correlations were found between GHRH levels and tumor site or size or the existence of metastases. Identification of increased plasma GHRH during follow-up was an accurate indicator of recurrence., Conclusions: The prognosis of endocrine tumors responsible for GHRH secretion appears relatively good. Plasma GHRH assay is an accurate tool for diagnosis and follow-up.

Objective: Carcinoids are low-grade, slow growing malignant tumors in the bronchi usually producing symptoms secondary to bronchial obstruction. We describe a case of 25-year-old woman who was initially diagnosed with asthma., Method: Case report., Results: Because of exacerbation and unresponsiveness to proper asthma treatment she was referred to spirometry, which showed low values of forced expiratory volume in 1 second. The shape of inspiratory limb of flow volume curve suggested an obstruction in the main bronchus or in the trachea. Further bronchoscopy revealed a tumor of the right main bronchus with characteristic histological features for carcinoid., Conclusions: Both the inspiratory curve and the expiratory part of the flow-volume loop should be evaluated in patients being evaluated for asthma. If there are changes in the shape of the inspiratory limb suggesting an obstruction, CT and/or bronchoscopy should be considered.

The authors present a case of a 44-year-old man who presented with acute onset shortness of breath. He had severe subcutaneous emphysema and his chest x-ray and CT scan confirmed presence of air in mediastinum. Rigid bronchoscopy revealed a bronchial tumour which was proven to be a carcinoid on histology. Patient recovered following the surgical excision of the tumour.

Children with neurological disorders may suffer from gastroesophageal reflux disease (GERD). Typical symptoms are vomiting, regurgitation and hematemesis. Patients present with respiratory symptoms only in cases with swallowing disorders causing chronic airway aspiration. We report the case of a patient affected by chromosome 8 p deletion syndrome with mental retardation, referred to our unit for suspected GERD. Chest X-ray, performed at admission for coexisting respiratory complaints, showed left lower lobe pneumonia; esophageal pH monitoring and upper endoscopy were normal for GERD. To rule out chronic airway aspiration, gastroesophageal 99 mTc scintigraphy with lung scan 18 to 24 h after a test meal and video fluoroscopy swallowing study were performed, both negative. Two months later, a second episode of left lower lobe pneumonia occurred. A chest CT scan was performed and showed an endobronchial mass; the biopsy taken during the broncoscopy was not conclusive. Surgical excision resulted in a diagnosis of pulmonary carcinoid. Bronchial carcinoids, although rare, should be taken into consideration as a potential cause of recurrent pneumonia even in the presence of demonstrated GERD where severe respiratory infections only occur with coexisting chronic pulmonary aspiration, even in neurologically impaired people., (© Georg Thieme Verlag KG Stuttgart · New York.)

A 33-year-old man with wheezing and haemoptysis was evaluated at the emergency department. At physical examination, diminished breathing was heard over the left lung. Two X-rays were made, an inspiratory and an expiratory one. The expiratory X-ray showed signs of an obstruction of the left main bronchus. The cause was a carcinoïd.

Introduction: Carcinoid tumors are a type of neuroendocrine tumors which usually involve the upper airways and the patients most often complain of cough and hemoptysis., Materials and Methods: This study was performed from 1990 through 2007 on 40 patients with carcinoid tumor who had been followed up for at least 3 years. The statistical analysis were based on tumor epidemiology, treatment and the 3-year survival. The factors influencing the survival were analyzed using SPSS and exact Fisher test., Results: M/F was 16/24 with mean age 34 years. Their most common symptoms were coughing (90%) . The left main bronchus was the most common site of involvement (25%). 95% of all the cases underwent surgery and 5% of the patients underwent chemoradiotherapy due to distant metastasis . The most common surgical procedure was lobectomy or biloectomy (57.8%). Bronchial sleeve resection was performed on 10.4% of the patients. The most common pathology was the typical form (90%) and 5% of the madiastinal lymph nodes were involvd all of the atypical type. Carcinoid syndrome was seen in one patient (2.5%) and post operative adjuvant treatment was done in 5% of the patients after surgery because of mediastinal lymph node involvement. Post operative recurrence occurred in one patient (2.6%) of the atypical form with mediastinal lymph nodes involvement. The most common complication of surgery was a long- term air leakage (10.4%) and the surgical death rate was 0%. 3-year survival was 92.5%. The factors influencing the survival included the pathological type, distant metastasis and mediastinal lymph node involvement., Conclusion: Carcinoid tumors have mostly been responsive to surgical intervention, resulting in a long term survival.

We present a patient with a typical bronchopulmonary carcinoid tumor who presented with proximal muscle weakness. Laboratory analysis and a muscle biopsy specimen led to the diagnosis of polymyositis. Chest imaging revealed a bronchopulmonary carcinoid tumor. Symptoms and laboratory derangements remitted after removal of the tumor. This case represents a rare report of a typical carcinoid tumor presenting with the paraneoplastic syndrome of polymyositis., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Centrally located endobronchial tumors present diagnostic and therapeutic challenges. We report the case of a 30-year-old woman presenting with nonspecific respiratory symptoms and wheezing, who was initially diagnosed with asthma, but eventually was found to have a non-secreting typical carcinoid tumor of the right main bronchus. Surgical management with isolated resection of the right main bronchus allowed us to avoid any parenchymal loss. This case is an instructive example showing that not every wheeze is asthma, especially if the wheezing is unilateral. The excellent long-term outcome of our patient highlights the fact that for central carcinoids, parenchyma-saving resection together with systematic lymphadenectomy should be considered the standard surgical procedure.

Sarcoidosis is an inflammatory disease of unknown cause which is characterized by the presence of noncaseating granulomas. The association of sarcoidosis with malignancy has been an issue and remains controversial. We herein report endobronchial carcinoid tumor in a patient with sarcoidosis diagnosed by nasal mucosal biopsy, excisional biopsy of cervical lymph nodes and mediastinal lymph node dissection.