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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: Patients with central neoplasms and haemoptysis show low survival rates. Symptom control without recurrence 48 h after bronchoscopic interventions may improve the prognosis of these patients. Bronchoscopic argon plasma coagulation (APC) is a useful technique for endobronchial management of haemoptysis in patients with central malignancies. Nevertheless, limited data are available in the literature on its efficacy and safety and the main predictors of success are still unclear., Methods: An observational, prospective, single-centre cohort study was carried out to assess the efficacy (i.e., immediate bleeding cessation without recurrence during the following 48 h) of bronchoscopic APC in the treatment of patients with haemoptysis caused by endobronchial malignancies and the main predictors of success., Results: A total of 76 patients with median age 75 years (interquartile range: 65-79) were enrolled. 67 (88.2%) patients had bleeding cessation without recurrence 48 h after bronchoscopic APC. A low rate of non-serious adverse events (5.3%) was recorded and a low (7.6%) recurrence rate of haemoptysis at 3.5 months after the procedure was also shown. No clinical, demographic and endoscopic variables related to a successful procedure at 48 h were found., Conclusion: This study demonstrates that bronchoscopic APC is an effective procedure in the treatment of patients with haemoptysis caused by endobronchial malignancies, regardless of the clinical characteristics of the patients, the endoscopic and histological features of the neoplasm and the severity of the symptom. Furthermore, it shows a low rate of complications and long-term efficacy in bleeding control., (© 2024 S. Karger AG, Basel.)

Endobronchial lipoma (EL) is a rare benign tumor characterized by tracheobronchial smooth-surfaced mass, often resulting in bronchial obstruction without standard guidelines for management. This study seeks to clarify the clinical features and interventions of EL, aiming to improve its diagnosis and outcomes. A retrospective review was conducted on 28516 outpatients treated between January 2015 and December 2019 at the Department of Respiratory and Critical Care Medicine of the Second Affiliated Hospital of Air Force Medical University to collect patients diagnosed with EL. Their clinical, bronchoscopic, chest imaging, and histopathological features along with management were analyzed. Among the patients reviewed, nine were histopathologically diagnosed with EL, comprising seven males and two females. All EL patients exhibited noticeable symptoms, including cough (in eight patients), dyspnea (in six patients), fever (in three patients), expectoration (in two patients), chest pain (in two patients), hemoptysis (in one patient), and fatigue (in one patient). Chest CT abnormalities included endobronchial mass (in four patients), inflammatory exudation (in three patients), atelectasis (in three patients), and infiltration or consolidation (in two patients). In three patients, imaging showed fat density, directly leading to the diagnosis of EL. The EL lesions were distributed with six in the right lung and three in the left lung, all located within the first three subdivisions of the tracheobronchial tree. Treatment approaches varied, with one patient undergoing combined bronchoscopic resection and surgery. The remaining patients received bronchoscopic intervention such as electrosurgical snare resection, argon plasma coagulation (APC), cryotherapy, and holmium laser. Histopathological analysis confirmed the EL diagnosis. Finally, the mass removal restored bronchus patency. Taken together, EL symptoms lack specificity, necessitating reliance on histopathology for EL accurate diagnosis. Bronchoscopic interventions emerge as the preferred option for EL management, surpassing surgical approaches., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2023 Jian Chen et al.)

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.

Background: The co-incidence of systemic lupus erythematosus (SLE) and tuberous sclerosis with pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML) is rare. In such patients, the rupture of renal AML may result in fatal circumstances, but this may be preventable., Methods: A 22-year-old Asian woman with SLE was admitted to our hospital with severe left-flank pain. Imaging studies showed the bilateral rupture of multiple renal AMLs., Results: The patient underwent emergency selective transcatheter embolization (TE) of the left renal artery. After TE and massive hydration, the patient complained of dyspnea and postembolization syndrome with fever. The chest computed tomography (CT) revealed pulmonary LAM, pulmonary edema with bilateral pleural effusions, and pneumonic consolidation. After the emergency procedure, the patient was treated with intravenous administration of antibiotics, diuretics, and nonsteroidal anti-inflammatory drugs for 10 days. The patient recovered favorably and was discharged 20 days after the treatment. She was diagnosed with renal AML and pulmonary LAM along with facial angiofibromas as well as tuberous sclerosis complex (TSC), although she had no TSC1 or TSC2 gene mutations., Conclusion: Although rare, SLE may coexist with TSC, along with LAM and AML, with a risk of AML rupture. The activation of the mTOR signaling pathway is shared between SLE and TSC. Thus, in patients with SLE, clinicians should consider imaging studies, such as kidney sonography and chest CT, to screen for possible manifestation of AML and LAM., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

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.

Case Presentation: A 55-year-old woman with COPD, heart failure with preserved ejection fraction (congestive heart failure), diabetes mellitus, and hypertension presented with baseline dyspnea at rest that had worsened over the last week. She reported associated runny nose, congestion, and cough productive of green sputum. She smoked six cigarettes per day and denied alcohol, drugs, or occupational exposure. She was admitted and initiated on treatment for acute exacerbation of COPD; however, her condition did not improve with steroid, ceftriaxone, and nebulized albuterol and budesonide treatments. She had been diagnosed with asthma and COPD without ever undergoing pulmonary function testing. She presented 11 times to the ED with six hospital admissions in the last 1.5 years for worsening dyspnea at rest, wheezing, and lower extremity edema deemed secondary to exacerbation of her COPD or congestive heart failure. She reported medication compliance, which included fluticasone-vilanterol, tiotropium bromide, and furosemide. She repeatedly demonstrated mild vascular congestion on imaging without hyperinflation, a normal to mildly elevated brain natriuretic peptide (<10 to 200 pg/mL), and dyspnea without hypoxia. She was treated normally for both COPD and congestive heart failure exacerbations simultaneously with methylprednisolone, albuterol, and furosemide with rapid improvement over the course of 1 to 2 days. No significant improvement was noted with steroid therapy, despite receiving them as an inpatient and outpatient. At the time of discharge, her symptoms would be at her baseline., (Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

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.

In recent years, high flow nasal cannula is being increasingly used. Most studies showed positive results when used in hypoxaemic respiratory failure. Its use in a patient with a large endobronchial mass has not yet being described. We report a patient who presented with hemoptysis and hypoxaemic respiratory failure second to a large mass obstructing the right main bronchus. High flow oxygen via nasal cannula was initiated with a quick improvement of the hemoptysis and the oxygen saturation. Thus, allowing the patient to be rapidly stabilized.

Endobronchial hamartochondroma is a rare benign tumor which differs from the parenchymal form in its symptomatology and also by its treatment which should be as conservative as possible. The endobronchial location is exceptional. Here we present the cases of two patients with endobronchial hamartochondroma associated with clinical manifestation, chest pain and repeated pulmonary infections, respectively. The diagnosis was made after performing a CT-scan, a PET-SCAN and histological analysis. After discussion in a multidisciplinary staff meeting, conservative treatment was chosen in both cases., (Copyright © 2020 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Cunninghamella is a member of the class Zygomycetes. Cunninghamella species include ubiquitous filamentous fungi; infections caused by Cunninghamella species are less frequent but have higher mortality rates than infections caused by Mucorales group members such as Rhizopus and Mucor. Herein, we reported a rare fatal case of endobronchial metastasis from breast cancer accompanied with Cunninghamella bertholletiae tracheobronchial mycetoma. A 73-year-old female with a history of right-sided breast cancer who had undergone mastectomy 11 years previously and had no recurrence presented to our emergency department with a 1-week history of left-sided back pain. Chest X-ray revealed left lung atelectasis; bronchoscopy revealed an endobronchial mass lesion in the left main bronchus. Pathological examination revealed fungal mycetoma but malignant lesions were not detected. Endobronchial and lung mycetoma caused by Cunninghamella bertholletiae were initially diagnosed; liposomal amphotericin B was administered, but her condition deteriorated. Rigid endoscopy showed growth of hemorrhagic tissue occupying the left main bronchus just under the carina. Pathological examination of the shaved lesion revealed metastasis from breast cancer covered with abundant necrotic tissue. No mold was observed in the necrotic tissue; this was probably due to liposomal amphotericin B treatment. To our knowledge, this is the first case of endobronchial metastasis from breast cancer accompanied with Cunninghamella bertholletiae mycetoma. Distinguishing endobronchial metastases from breast cancer and atypical presentations of Cunninghamella endobronchial mycetomas can be very difficult. Repeated bronchoscopies maybe helpful in establishing an accurate diagnosis when clinical prognosis does not match the initial diagnosis., (Copyright © 2019 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

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.

A 65-year-old man, diagnosed with bronchus cancer metastasized to the brain and lymph nodes, was seen at the emergency department. He complained of 'an airy feeling' within the left side of his thorax. Imaging revealed a pneumopericardium, probably caused by a fistula between the primary tumour and the pericardium.

Endobronchial metastasis from extrapulmonary solid tumors is rare, and endobronchial metastasis from the prostate is even more unusual. An 80-year-old patient presented with significant dyspnea secondary to metastatic stromal cell sarcoma of the prostate, which occluded the right main bronchus. The tumor, causing complete collapse of the right lung, was found on computed tomography and confirmed by bronchoscopy. We successfully excised the lesion using cryotherapy, with immediate resolution of symptoms and radiological lung reexpansion.

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.

Background: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5 mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease., Methods: A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology., Results: Our patient cohort consisted of twelve females and one male with a mean age of 63 years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of long-acting somatostatin analog and everolimus. Five patients had concomitant bronchial carcinoids., Conclusions: DIPNECH is a rare pathology that can profoundly affect a patient's quality of life. Paroxysmal coughing episodes can be difficult to treat. Our limited single center experience shows encouraging response to use of somatostatin analogs, azithromycin, and everolimus in the management of debilitating DIPNECH associated symptoms.

Pneumonectomy is known to be associated with a high rate of morbidity and mortality and may be contraindicated in patients with altered lung function. Sleeve lobectomy is a treatment option, but it may be technically impossible in cases of large bronchial involvement. Here, we describe a patient with impaired lung function and right upper lobe lung cancer that involved the intermediate bronchial trunk. The patient was treated successfully with a right upper sleeve lobectomy and bronchial replacement with the use of a thoracodorsal artery perforator flap and a temporary endostent. Clinical outcomes were favorable, and no recurrence has been observed in the 4 years since the operation., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 75-year-old woman presented with minor hemoptysis. Past history included atrial fibrillation and rheumatic mitral stenosis. Bronchoscopy revealed a striking abnormality of widespread dilated mucosal and submucosal vessels. Initially the concern was whether there was widespread endobronchial malignancy; however, narrow band imaging (NBI) demonstrated the symmetrical uniform nature of the vessels. In addition, there was a focal area of in situ carcinoma with classic NBI features of malignancy. Following external-beam radiation therapy, NBI features had dramatically changed in the treated area which was now biopsy negative. The case highlights the utility of NBI in identifying endobronchial microvessel appearance even where there are widespread nonmalignant vessel changes in the bronchial mucosa.

A middle-aged woman with recurrent malignant melanoma presented initially with massive left pleural effusion. There was a complete obliteration of the left main bronchus on flexible bronchoscopy caused by a mass. Serial cryo-debulking of the tumour was done under rigid bronchoscopy; however, the outcome was not favourable due to the aggressive tumour growth. Vemurafenib was planned after thoracic radiation. She was not keen for the biologics treatment due to financial constraints. We report a case of central airway obstruction due to recurrent aggressive melanoma. More evaluations are needed on the role of interventional pulmonologist for bronchoscopic debulking of this rapidly growing tumour as well as the role of biological agents in treating such cases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

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.)

In recent years, there have been many reports about the efficacy of stenting for central bronchial stenosis. When central bronchial stenosis is due to metastasis of a malignant tumor to the trachea and/or bronchi (endobronchial metastasis: EM), it is classified as "narrow EM" and "broad EM." [1] We managed two patients in whom bilateral stent placement was required for narrow and broad EM arising from colorectal cancer. Case 1: In September 2011, a 66-year-old man underwent low anterior resection for advanced colorectal cancer associated with unresectable liver metastasis. The liver metastasis became resectable after chemotherapy, with two resection procedures and radiofrequency ablation (RFA) being performed. Thereafter, lung metastasis occurred and a tumor in the left lung was resected. In May 2015, he developed respiratory distress. CT identified multiple lesions protruding into the lumen of the trachea and the left and right main bronchi. There was no evidence of mediastinal relapse or local relapse at the resection margin, and tumors were only detected in the tracheobronchial walls. Accordingly, narrow EM was diagnosed. An expandable metallic stent (EMS) was placed on the right side where stenosis was more severe, and radiation therapy was conducted for the non-stented tumors. The patient died 8 months later. Case 2: A 69-year-old woman had undergone laparoscopic right hemicolectomy and adjuvant chemotherapy for Stage lllb cancer of the ascending colon. Due to subsequent elevation of tumor markers, PET-CT was conducted and abnormal uptake was seen in the apex of the right lung and right upper abdomen. Both lesions were resected, and omental and lung metastases were diagnosed. She received treatment with UFT / calcium folinate, but relapse occurred at the resection margin in the right lung. At 7 years and 5 months after initial surgery, she complained of respiratory distress at an outpatient visit. CT demonstrated displacement of the trachea and right main bronchus due to enlargement of upper mediastinal lymph nodes. There was also severe stenosis of the right main bronchus due to tumor infiltration. Because there was both infiltration from local recurrence after resection and upper mediastinal lymph node enlargement, broad EM was diagnosed. An EMS was placed at the site of severe stenosis in the right main bronchus. Similar to Case 1, radiation therapy was also conducted, but respiratory distress occurred after 3 months due to tumor re-growth at the stent margin. Accordingly, stent-in-stent placement was performed and her respiratory symptoms improved. However, superior vena cava syndrome occurred 1 month later and the patient died. We consider that placing an EMS is effective in patients with tracheal stenosis due to EM that is judged to be an oncological emergency.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Hemoptysis is rare in children and adolescents. We describe an 11-year-old girl who presented with hemoptysis, cough, and exertional dyspnea. Radiologic and bronchoscopic assessment revealed a pedunculated mass in the right main stem bronchus with a ball valve effect. Carcinoid or hemangioma was suspected as cause of the mass. The tumor could be excised bronchoscopically, and histologic examination showed a capillary hemangioma. In the literature, bronchial hemangiomas are described in infants and adults. This case demonstrates that bronchial hemangioma should also be taken into consideration as a cause of hemoptysis in adolescents., (© 2017 Wiley Periodicals, Inc.)

Purpose: Endobronchial metastasis (EBM) originating from primary cancers outside the lung is rare. External beam radiotherapy is often attempted for control of symptoms with variable effectiveness and retreatment is challenging if symptoms recur. There is limited documentation of high-dose-rate brachytherapy for EBM in the literature., Methods and Materials: A prospective database was created from 2006 to 2015. Patients with EBM who received high-dose-rate brachytherapy were included. Cough, dyspnea, chest pain, and hemoptysis were assessed and graded (0-4) at the time of initial consult and in followup. Symptom-free survival and re-expansion were assessed., Results: Thirty-five patients with EBM were identified. Most patients received three fractions of 700 cGy, and 17 patients had prior external beam radiotherapy. Median symptom-free and overall survival were 67 and 117 days. After brachytherapy, improvement in cough was documented in 75.0%, hemoptysis in 76.4%, dyspnea in 60.0% for a median of 3-6 months. Of the 22 patients who had subsequent chest imaging, re-expansion was documented in 32%. There were no significant toxicities reported., Conclusions: Brachytherapy appears effective in achieving durable symptom control of cough hemoptysis, and dyspnea in patients with EBM and should be considered routinely for palliation where available. Further studies are required to better characterize expected symptom improvement, lung re-expansion rates, and efficacy in comparison with other local treatments., (Copyright © 2017 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

Only nine cases of bronchial granular cell tumor have previously been reported in pediatric patients. We present a 15-year-old girl with acute-onset right shoulder pain, discovered to have a granular cell tumor causing bronchial stenosis and a cavitating post-obstructive right upper lobe pneumonia. The patient was treated with lobectomy. Bronchial granular cell tumors are benign neoplasms that typically present with recurrent pneumonia. Imaging may demonstrate an endobronchial lesion or an associated post-obstructive opacity, but diagnosis requires tissue sampling. To our knowledge, this is the tenth case of bronchial granular cell tumor in a pediatric patient to be reported since 1926., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Tracheoesophageal fistulas (TEFs) often occur with esophageal or bronchial carcinoma. Currently, we rely on implantation of delicate devices, such as self-expanding and silicone stents, in the esophagus or trachea to cover the fistula and expand the stenosis in order to relieve patient pain. However, because each case is different, our approach may not be effective for every patient. Consequently, new devices and technology have emerged to address these situations, such as degradable stents, Amplatzer ® devices, endobronchial one-way umbrella-shaped valves, and transplantation of mesenchymal stem cells. Although some studies have shown such alternatives can be reasonable solutions in special cases, further development of other new and effectual techniques is of utmost importance.

Objective: To report the surgical treatment of a pulmonary emphysematous cyst concurrent with primary pulmonary bronchoalveolar papillary carcinoma in a dog., Study Design: Clinical case report., Animals: 12-year-old 6.4 kg spayed female Shih Tzu dog., Methods: The dog presented for surgical treatment of pulmonary emphysema. Radiography revealed that more than half of the left caudal lung lobe was enlarged and hyperlucent and computed tomography (CT) confirmed the presence of an emphysematous space. Thoracoscopic lung lobectomy was attempted but was converted to an intercostal thoracotomy due to poor visualization and pleural adhesions. A left caudal total lung lobectomy was performed using a self-cutting endoscopic stapler., Results: The dog recovered uneventfully and a postoperative histopathologic diagnosis of pulmonary cystic bronchoalveolar papillary carcinoma was made. Re-evaluation using a CT scan with contrast study on postoperative days 27 and 177 revealed no evidence of residual, metastatic, or recurrent lesions. The dog has been doing well since surgery during the 11 month follow-up period., Conclusion: This case report suggests a potential relationship between pulmonary emphysematous diseases and primary lung tumors in dogs., (© 2016 The American College of Veterinary Surgeons.)

Palliation of malignant tracheobronchial stenosis is challenging. Published experience with self-expanding Y-shaped stents is limited and it seems necessary to evaluate whether they improve clinical results with respect to alternative prostheses. We present a retrospective case series of 20 consecutive patients with malignant tracheobronchial stenosis that underwent placement of a single-unit, Y-shaped covered metallic stent. Outcomes were: safety of the procedure, palliation of dyspnea, complications, and survival. All stents were safely and easily placed using a rigid tracheoscope within 24hours of admission. Dyspnea was effectively palliated in all patients, and no early or late adverse stent-related events were observed. Thirty-day mortality was 40%. Median survival was 12.2 weeks. Placement of Y-shaped self-expanding stents is a safe and effective procedure for the palliation of malignant tracheobronchial stenosis, and is currently our stent of choice for this subgroup of patients., (Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)