[Surgical Case of Bronchial Mucoepidermoid Carcinoma Safely Diagnosed by Enucleation Under the Bronchoscopy After Bronchial Arterial Embolization].

A 28-year-old male presented to our hospital with hemoptysis and his chest computerized tomography (CT) showed the right middle and lower lobe atelectasis due to the tumor of right intermediate bronchial trunk. To reduce the blood flow to the tumor, bronchial arterial embolization was performed and the tumor was resected using Cryoprobe with a flexible endobronchial scope. Thus, we could observe the tumor localization and diagnose before the surgical procedure. We performed the right sleeve middle lobectomy and the right lower lobe was safely preserved.

Surgical Strategy and Clinical Outcome in Patients with Bronchial Carcinoids.

Carcinoids are malignant neuroendocrine neoplasms showing good long-term survival after oncologic therapy. The study evaluated the influence of operative strategies and individual decision-making on the outcome and long-term survival in 222 patients with bronchial carcinoids. The patients underwent preoperative pulmonary function tests and bronchoscopy to facilitate surgical decision-making. A hundred and twelve tumors were detected endoscopically, including 32 in the main and lobar bronchi. We performed 5 isolated bronchus resections, 4 segmentectomies, 15 wedge resections, 10 pneumonectomies, 19 sleeve resections, 26 bilobectomies, 138 lobectomies, and 2 chest wall resections. Three patients were technically inoperable. Systematic mediastinal lymphadenectomy was routinely performed although most patients' computer tomography scans showed N0. A hundred and sixty-two patients had typical (155 N0, 7 N+) and 60 patients had atypical carcinoids (39 N0, 21 N+). There was no intraoperative mortality. The hospital mortality was below 2%. Overall, 1-, 5-, and 10-year survival rates were 99%, 94%, and 89%, respectively, in typical carcinoids. Atypical carcinoids show similar 1- and 5-year survival rates, but the 10-year survival rate was below 70%, decreasing in higher N-stages. The N-stage was the most important survival factor. In conclusion, bronchial carcinoids should be surgically treated the way lung cancer is. Anatomic resection and systematic lymphadenectomy are the treatments of choice. The availability of bronchoplastic techniques and preoperative assessment is essential for individual decision-making, focusing predominantly on postoperative quality of life.

[Endobronchial Lipomatous Hamartoma Extended beyond a Bronchial Wall:Report of a Case].

Pulmonary hamartomas are common benign lung tumors. Most cases are parenchymal chondromatous hamartomas, whereas endobronchial lipomatous hamartomas are rarely encountered. A 78-year-old man was referred for left superior subsegmental atelectasis due to obstruction by an endobronchial tumor that was found incidentally on chest computed tomography for the postoperative follow-up of colon cancer. Bronchoscopy showed a polypoid tumor in the orifice of the left upper segmental bronchus. Lipoma was diagnosed by a bronchoscopic biopsy. We performed a segmentectomy of the upper division of the left lung because the tumor was presumed to be located peripherally to the left B3 segmental bronchus. A histopathological examination revealed that the tumor was an endobronchial lipomatous hamartoma that extended to the pulmonary parenchyma beyond the bronchial wall.

Multiple endo bronchial lipoma: a rare case report.

BACKGROUND: Endobronchial lipoma is an extremely rare benign tumor, which is generally located in the first three subdivisions of the tracheobronchial tree. According to the existing literature, all endobronchial lipomas are single (one per patient). Here, we report a rare case in which the patient presented with two endobronchial lipomas in the same patient, and underwent a bronchoscopic tumor resection in the left main bronchus and the left lower bronchus. Both tumors were pathologically confirmed as endobronchial lipoma. CASE PRESENTATION: A 52-year-old Chinese man presented at the clinic reporting a mild cough with yellow color sputum and exertional dyspnea for 2 weeks. He was a heavy smoker (45 pack-years). Chest auscultation demonstrated faint wheezing in left lower lobe. Computed tomography (CT) revealed two low-density endobronchial masses located in the middle segment of the left main bronchus and the posterior basilar segmental bronchus of the left lower lobe. The neoplasms measured a CT-attenuation value of -70HU, -98HU in density with air trapping and atelectasis in the segmental bronchus of the left lower lobe. The patient underwent interventional bronchoscopic management to remove the neoplasms by using an electrosurgical snare, cryotherapy, and electrocautery. The locations of the neoplasms were confirmed at the left main bronchus and the superior segment of the left lower lobe during bronchoscopic intervention. Histopathological examination confirmed that both tissues were consistent with lipomas. After 18 months of follow-up, the patient was free of symptoms and CT revealed that bronchiectasia remained in the superior segment of the left lower lobe; however, no mass lesion was present in the left bronchus. CONCLUSIONS: This case suggests that an endobronchial lipoma can present as multiple lesions, and both proximal and distal types can simultaneously occur in the same patient. Thus, these findings help us further understand the biology of endobronchial lipomas.

Intrapulmonary mature cystic teratoma of the lung: case report of a rare entity.

BACKGROUND: Intrapulmonary teratoma (IPT) is a rare type of extra gonadal teratoma which often presents with non-specific symptoms and can be misdiagnosed as other diseases. Here we report a patient with IPT which was initially misdiagnosed as lung hydatid cyst versus abscess. CASE PRESENTATION: We report an intrapulmonary teratoma in a 27-year-old female presenting with persistent chest pain and dyspnea since a few years prior to her admission with associated symptoms of cough and fever. Chest x-ray only showed left side massive pleural effusion and computed tomography scan of the lungs was suggestive of hydatid cyst or a lung abscess. She underwent lobectomy and postoperative histopathological study revealed IPT as the final diagnosis. CONCLUSION: Due to the non-specific symptoms and rarity, IPT can be easily misdiagnosed at first. It is essential that physicians take into account the possibility of IPT when approaching a new case of lung mass.

DOTA-ZOL: A Promising Tool in Diagnosis and Palliative Therapy of Bone Metastasis-Challenges and Critical Points in Implementation into Clinical Routine.

The novel compound 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA)-ZOL (DOTA-conjugated zoledronic acid) is a promising candidate for the diagnosis and therapy of bone metastasis. The combination of the published methodology for this bisphosphonate with pharmaceutical and regulatory requirements turned out to be unexpectedly challenging. The scope of this work is the presentation and discussion of problems encountered during this process. Briefly, the radiolabelling process and purification, as well as the quality control published, did not meet the expectations. The constant effort setting up an automated radiolabelling procedure resulted in (a) an enhanced manual method using coated glass reactors, (b) a combination of three different reliable radio thin-layer chromatography (TLC) methods instead of the published and (c) a preliminary radio high-pressure liquid chromatography (HPLC) method for identification of the compound. Additionally, an automated radiolabelling process was developed, but it requires further improvement, e.g., in terms of a reactor vessel or purification of the crude product. The published purification method was found to be unsuitable for clinical routine, and an intense screening did not lead to a satisfactory result; here, more research is necessary. To sum up, implementation of DOTA-ZOL was possible but revealed a lot of critical points, of which not all could be resolved completely yet.

Initial Experience with Hybrid Argon Plasma Coagulation as a Novel Local Treatment Method for Tracheobronchial Mucoepidermoid Carcinoma.

Tracheobronchial mucoepidermoid carcinoma (MEC) is a type of salivary gland tumor. Surgical resection is the main treatment for MEC, but it is associated with risks. Hybrid argon plasma coagulation (HybridAPC®) is an innovative technique combining APC with a water cushion function which can be used for the treatment of MEC. We aimed to evaluate the efficacy and safety of HybridAPC for MEC in 2 patients diagnosed with MEC based on histological examination of biopsies. Full preoperative assessments were done by white-light bronchoscopy, autofluorescence imaging, narrow-band imaging, and radial probe endobronchial ultrasound. HybridAPC was administered after these evaluations. Both patients were followed up for more than 3 months. HybridAPC ablation was completed successfully, with no complications. HybridAPC thus appears to be a safe and efficient treatment for MEC.

Atypical bronchial carcinoid with postobstructive mycobacterial infection: case report and review of literature.

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.

Postpneumonectomy-like syndrome due to bronchial carcinoid: a unique case report.

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.

Challenges in diagnosis of disease reported 100 years back: Cushing syndrome; recent advances.

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.

[Clinical and Imaging Manifestations of Primary Benign Tracheobronchial Tumors].

Objective To improve our knowledge of primary benign tracheobronchial tumors and increase the early diagnosis rate. Method The clinical and imaging features of 22 patients with benign tracheobronchial tumors were retrospectively analyzed. The lesions were surgically or pathologically confirmed as schwannomas(n=2),lipomas(n=3),hamartomas(n=3),leiomyomas(n=9),inflammatory myofibroblastoma(n=1),and pleomorphic adenomas(n=2).The early symptoms were concealed and atypical,accompanied by misdiagnoses at different time points.The tumors were located at trachea in 5 patients and at bronchus in 17 patients.All lesions manifested as intraluminal growth with mild to moderate enhancement,without thickening of the tracheobronchial wall.They had smooth margins and wide basements.The lesions were cast-shaped and occluded the lumen in 3 cases;in the remaining 19 cases,the lesions appeared as round or oval nodules. Conclusions Primary benign tracheobronchial tumors are rare.Patients with repeated cough and expectoration that respond poorly to treatment should be screened for benign tracheobronchial tumors.On CT,the benign tracheobronchial tumors are small intraluminal nodules with the smooth surface and wide basement,without thickening of the wall.

The role of interventional pulmonology in endobronchial metastasis of renal cell carcinoma.

INTRODUCTION: Although metastasis of extrapulmonary solid organ malignant tumors to the lungs is very common, endobronchial metastases are very rare. The most common extrapulmonary tumors that make endobronchial metastases are breast, kidney and colorectal carcinomas. MATERIALS AND METHODS: In this study, we retrospectively examined the data of eleven renal cell carcinoma patients who underwent endobronchial metastases in an eight-year period. RESULT: Ten of the patients were male and the mean age was 55 ± 1 (41-71) years. The most common symptom was cough (45.5%, n= 5), on the other hand 18.2% (n= 2) of the patients had no complaints. Right bronchial system, left bronchial system and tracheal distribution rates of endobronchial lesions were 45.5% (n= 5), 63.6% (n= 7) and 27.3% (n= 3) respectively. The mean time from diagnosis of primary renal cell carcinoma to endobronchial metastases was 47.5 ± 32 (5.2-100.5) months. A total of twenty two interventional procedures were performed. All except one patient underwent endobronchial treatment. Argon plasma coagulation was most commonly used as the endobronchial metastases option (n= 10, 100%). The mechanical resection (n= 6, 60%), laser (n= 5, 50%), cryoextraction (n= 5, 50%) and cryotherapy (n= 4, 40%) methods were used other than argon plasma coagulation. The mean survival time was 19.4 ± 15.7 (3.2-40.5) months after endobronchial metastases and 54.0 ± 40.4 (8.7-107.6) months after renal cell carcinoma diagnosis. CONCLUSIONS: Extrathoracic malignancy that most metastasis to the airways is renal cell carcinoma. Endobronchial treatment procedures for endobronchial metastasis of renal cell carcinoma can be performed with low complication rates.

Endobronchial Paraganglioma: AIRP Best Cases in Radiologic-Pathologic Correlation.

Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).

Proximal Left Main Bronchus Resection-Reconstruction through Left Thoracotomy with Tracheal Traction.

Surgical approach for resection-reconstruction of the proximal left main bronchus has been a matter of discussion. Through standard left thoracotomy, the proximal main bronchus close to the carina is not easily exposed. Accordingly, median sternotomy or right thoracotomy may be commonly employed, but the exposure and management of the distal left main bronchus may be difficult through the approach. Here, we present a left thoracotomy approach with traction of the trachea, which may easily allow excellent exposure of the proximal end of the left main bronchus and the carina.

Colon carcinoma with endobronchial metastasis masquerading as bronchial asthma causing ball valve effect.

Malignant central airway obstruction (CAO) with ball valve effect (BVE) in the lung is rare. We discuss a case of metastatic colon cancer who presented with asthma like symptoms which thoracic computed tomography and bronchoscopy revealed an intraluminal tumour obstructing the left main bronchus in a ball valve manner. Airway patency was restored urgently with immediate alleviation of symptoms. This illustrates the importance of recognizing subtle features of central airway obstruction to allow expedition of appropriate investigations and therapy.

Autofluorescence and White Light Bronchoscopy in the Diagnosis of Endobronchial Malignant Lesions.

BACKGROUND: Autofluorescence bronchoscopy (AFB) allows a more sensitive approach to the diagnosis of premalignant and malignant endobronchial lesions than white light bronchoscopy (WLB) can do. AIM: To assess the autofluorescence bronchoscopy and white light bronchoscopy in diagnosing malignant endobronchial lesions. MATERIALS AND METHODS: The design of the study is a retrospective case-control study. Thirty-two parameters were entered into an Excel file and analysed with SPSS v. 21 for Mac book Pro. Endoscopy findings were graded in 4 options and morphological results - in 9 options according to WHO classification. The results are presented using McNemar's test and sensitivity, specificity and positive and negative predictive values as well. RESULTS: Three hundred and three patients were included in the study. Lung cancer was found in 38.3% of the patients using histology and in 35.6% - using cytology. McNemar's test for AFB finding for suspected and malignant lesions OR was 8.333 (95% CI 3.571-23.784) while for WLB OR was 0.128 (95% CI 0.045-0.299). For cytological results OR was 3.800 (95% CI 2.123-7.227) and 3.471 (95% CI 1.996-6.351), respectively. P value was <0.0001 for all tests. Sensitivity for AFB and WLB was 94.83% but specificity was 52.83% and 55.66% if histology was used. For cytology these numbers were respectively 86.11% and 84.26% for sensitivity, and 63.69% and 62.42% for specificity. CONCLUSION: AFB has an advantage over WLB in diagnosing endobronchial malignant lesions. Biopsying suspicious, not only visible malignant lesions, increased diagnostic sensitivity.

Observation of Zn-photoprotoporphyrin red Autofluorescence in human bronchial cancer using color-fluorescence endoscopy.

BACKGROUND: We observed red autofluorescence emanating from bronchial cancer lesions using a sensitive color-fluorescence endoscopy system. We investigated to clarify the origin of the red autofluorescence. METHODS: The wavelengths of the red autofluorescence emanating from lesions were measured in eight patients using a spectrum analyzer and compared based on pathologic findings. Red autofluorescence at 617.3, 617.4, 619.0, and 617.1 nm was emitted by normal bronchus, inflamed tissue, tissue exhibiting mild dysplasia, and malignant lesions, respectively. Protoporphyrin, uroporphyrin, and coproporphyrin, the major porphyrin derivatives in human blood, were purchased to determine which porphyrin derivative is the source of red fluorescence when acquired de novo. We synthesized photoporphyrin, Zn-protoporphyrin and Zn-photoprotoporphyrin from protoporphyrin. RESULTS: Coproporphyrin and uroporphyrin emitted only weak fluorescence. Fluorescence was emitted by our synthesized Zn-photoprotoporphyrin at 625.5 nm and by photoprotoporphyrin at 664.0 nm. CONCLUSIONS: From these results, we conclude that Zn-photoprotoporphyrin was the source of the red autofluorescence observed in bronchial lesions. Zn-protoporphyrin is converted to Zn-photoprotoporphyrin by radiation with excitation light. Our results suggest that red autofluorescence emanating from Zn-photoprotoporphyrin in human tissues could interfere with photodynamic diagnosis using porphyrin derivatives such as Photofrin® and Lazerphyrin® with a sensitive endoscopy system, because color cameras cannot differentiate Zn-photoprotoporphyrin red fluorescence from that of other porphyrin derivatives.

ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA.

OBJECTIVE: Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India. METHODS: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented. We compared the biochemical findings in these patients with 20 consecutive patients with Cushing disease (Cushing syndrome of pituitary origin). RESULTS: A total of 21 patients were studied. The median age at presentation was 34 years (range, 19 to 55 years). Seven patients had thymic carcinoid, 7 had bronchial carcinoid, 3 had lung malignancies, 2 had medullary carcinoma thyroid, 1 patient had a pancreatic neuroendocrine tumor, and 1 patient had an occult source of ACTH. The most common clinical features at presentation were muscle weakness (95%), hyperpigmentation (90%), facial puffiness (76%), easy bruising (61%), edema (57%), and striae (52%). Extensive acne was seen in a large number of patients (43%). Only 3 patients (14%) had central obesity. The median 8 am cortisol was 55.5 µg/dL (range, 3.8 to 131 µg/dL), median 8 am ACTH was 207 pg/mL (range, 31.1 to 703 pg/mL), and the median 24-hour urinary free cortisol was 2,484 µg (range, 248 to 25,438 µg). Basal cortisol and ACTH, as well as midnight cortisol and ACTH level, were markedly higher in patients with ectopic Cushing syndrome as compared to patients with Cushing disease. Twelve of 21 patients had developed life-threatening infections by follow-up. Nine patients had undergone surgical intervention to address the primary tumor. However, only 1 patient exhibited a complete cure on follow-up. CONCLUSION: In our series, ectopic Cushing syndrome was most commonly seen in association with intrathoracic tumors such as bronchial or thymic carcinoid. Hyperpigmentation and proximal myopathy were frequent, while central obesity was uncommon. Early and rapid control of hypercortisolemia was important in order to prevent life-threatening infections and metabolic complications. ABBREVIATIONS: ACTH = adrenocorticotropic hormone CT = computed tomography DOTATATE = 68Ga-DOTA-Tyr3-octreotate ECS = ectopic Cushing syndrome FDG = fluorodeoxyglucose MTC = medullary thyroid cancer NET = neuroendocrine tumor PET = positron emission tomography.