Typical Pulmonary Carcinoid Tumors Research Articles

Huge typical pulmonary carcinoid presented with gigantism syndrome

A 27-year-old male patient presented with cough and right-sided, light thoracic pain. His physical appearance showed typical features of gigantism. Subsequently, further diagnostic work-up showed elevated level of growth hormone and a huge tumor of the right lung, identifying a typical pulmonary carcinoid tumor (TPCT). Curative surgery was performed leading to normalization of the elevated growth hormone levels few days after surgery. Two- and five-year follow-up showed no signs of recurrence. Respected to tumor size, we determined the largest TPCT to be reported in medical literature history.

Loss of ATRX expression predicts worse prognosis in pulmonary carcinoid tumors

Loss of alpha thalassemia/mental retardation syndrome X-linked (ATRX), a chromatin regulator, is associated with worse prognosis in pancreatic neuroendocrine tumors. We investigated ATRX expression in pulmonary carcinoid tumors (PCT) and its diagnostic and prognostic role in these patients. Resected PCTs (1997-2017) were reviewed. Tumors were staged according to 8th UICC/AJCC system. ATRX nuclear expression was recorded independently by 2 reviewers. A cutoff of ≤5% of nuclear ATRX expression was statistically established as loss of expression. One-hundred-fifteen patients (72 women [63%]; median age of 60.5 years [interquartile range, 50.8-71.5]) harbored 69 (60%) typical and 46 (40%) atypical PCTs. Median tumor size was 2.3 cm (interquartile range, 1.6-3.8 cm). Loss of ATRX expression was associated with atypical PCTs (OR 7.4 [95% CI, 2.6-23, P < .001]), when adjusted for lymphovascular invasion and perineural invasion. ATRX expression predicted atypical PCT with sensitivity of 37% (95% CI, 24%-52%), specificity of 92% (95% CI, 86%-98%), AUC of 0.62 (95% CI, 0.52-0.72). Loss of ATRX expression was associated with shorter disease-specific survival (HR = 11, 95% CI, 1.8-68, P = .01), after adjusting for lymphovascular invasion and presence of metastatic disease at time of diagnosis. Interobserver agreement on ATRX expression by two reviewers was substantial (κ = 0.72 [95% CI, 0.60-0.80]). ATRX expression is more commonly lost in atypical than in typical PCT, and is associated with more aggressive tumor characteristics and shorter disease-specific survival.

PRIMARY TYPICAL CARCINOID OF THE PLEURA: A RARE INCIDENTAL FINDING

SESSION TITLE: Disorders of the Pleura 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Carcinoid tumors are a rare occurrence of the thoracic cavity, constituting roughly 0.5 to 2.5% of all pulmonary neoplasms. This case discusses an incidental finding of a primary pleural carcinoid in a patient presenting with spontaneous pneumothorax requiring operative management. CASE PRESENTATION: A 73 year old male with a history of COPD presented with a shortness of breath and was transferred to our facility for a persistent spontaneous right pneumothorax. A CT scan was obtained showing multiple bilateral emphysematous blebs, but no pulmonary or chest wall lesions (Figure 1). The patient was taken to the operating room for a right VATS, partial pleurectomy, and talc pleurodesis after a persistent pneumothorax despite multiple chest tubes. Intraoperatively, no abnormalities were noted in the pleura or chest wall at the time of pleurectomy. Two chest tubes were positioned intraoperatively and placed to waterseal on POD3. They were removed on POD5 and patient was discharged POD7. The parietal pleural specimen was described as an irregularly shaped yellow membrane measuring 14.5X0.5X0.1 cm. There were no gross lesions. Microscopy revealed a well differentiated neuroendocrine carcinoid tumor with a maximum diameter of 3mm. Nests of neuroendocrine cells were present and stained positive for CD-56, CK7, synaptophysin, and CEA. ERG, CK20, WT-1, cam 5.2, TTF-1, CDX-2, and calretinin were negative (Figure 2). Follow-up PET-CT at clinic revealed no evidence of residual disease (Figure 3). DISCUSSION: The most common primary location of carcinoid tumors is the small intestine (30.4%), followed by pulmonary (29.8%) and colon (9.2%). A majority of reported pleural cases are of metastatic disease. Therefore, primary pleural carcinoid disease is a rarity. CT imaging can identify thoracic or gastrointestinal primary carcinoid tumors. In a retrospective review of 50 patients with carcinoid syndrome, Moss et al. identified pleural thickening in nine patients, of which 4 had a primary tumor. Presentation of these patients with primary pleural carcinoid is usually due to mass effect or as an incidental finding on imaging. In this case report, the patient was found to have a 3 mm focus in a pleurectomy specimen. Given the rarity of primary pleural carcinoid, this finding warranted further investigation to ensure that this was not metastatic disease. CONCLUSIONS: A multidisciplinary approach to these rare cases with pathology and radiology cannot be stressed enough. The first step is to rule out other more common pleural malignancies though a panel of histologic stains. Further imaging, including a PET CT, is suggested to identify FDG avid lesions that can be the primary origin. Reference #1: Bertino EM, Confer PD, Colonna JE, Ross P, Otterson GA. Pulmonary neuroendocrine/carcinoid tumors. Cancer. 2009;115:4434-4441. Reference #2: Townshend AP, Lakshminarayanan B, Ucar AE, Chaudry ZR, Duffy JP. Rare pleural recurrence of typical pulmonary carcinoid tumor 30 years after lobectomy. The Annals of thoracic surgery. 2007;83:1523-1524. Reference #3: Moss SF, Lehner PJ, Gilbey SG, et al. Pleural involvement in the carcinoid syndrome. QJM: An International Journal of Medicine. 1993;86:49-53. DISCLOSURES: No relevant relationships by Ernest Chan, source=Web Response No relevant relationships by Lawrence Crist, source=Web Response No relevant relationships by Rajeev Dhupar, source=Web Response I own stock in this company relationship with Inntuitive SUrgical Please note: $20001 - $100000 Added 03/03/2018 by James Luketich, source=Web Response, value=None, I purchsed my self as an stock holder relationship with johnson and Johnson Please note: $20001 - $100000 Added 03/03/2018 by James Luketich, source=Web Response, value=none, I purchsed myself as an no disclosure on file for Anupama Sharma; no disclosure on file for Diane Strollo

Primary typical pulmonary carcinoid tumor: an incidental finding

Bronchopulmonary carcinoid tumors are rare tumors (only 0.5 to 2.5% of all pulmonary tumors) that arise from neuroendocrine cells known as Kulchitsky cells. They are characterized by slow growth and rarely metastasize beyond the lungs. However, they can occur in the central or peripheral parts of the lung. Secondary locations can be asymptomatic, but central carcinoids often present with obstructive signs including: recurrent pneumonias and hemoptysis. We report a rare, asymptomatic case of a primary bronchopulmonary carcinoid in a 62-year-old man. A 62-year-old man presented with history of intermittent right upper quadrant pain, especially after a high fat meal, for 6 months. Systemic examination was unremarkable. Ultrasonography of gall bladder showed cholelithiasis, and he was scheduled for cholecystectomy. On preoperative cardiology consult, the chest X-ray showed an incidental homogenous opacity in the right middle zone without any respiratory symptoms. Patient underwent fiber optic bronchoscopy and biopsy. Histopathologic examination showed monomorphic tumoral cells forming small nests, rosette-like arrangements, and cords with salt and pepper, stippled appearance nuclei. Patient underwent right middle lobectomy. The postoperative period was uneventful, and the patient was disease-free in the following 25 months. Carcinoid syndrome is very rare in patients with bronchopulmonary carcinoid tumors. Overall prognosis for patients with typical bronchopulmonary carcinoid tumors is favorable. If diagnosed in the early stages, the chances for radical treatment success are increased.

Giant cell arteritis and polymyalgia rheumatica as first manifestation of typical pulmonary carcinoid tumor

Giant cell arteritis (GCA), a systemic vasculitis of unknown origin, may appear rarely as a paraneoplastic syndrome. Cases secondary to pulmonary neuroendocrine tumors have not been reported. A 75-year-old female developed prednisone-responsive GCA/polymyalgia rheumatica (PMR) shortly followed by syndrome of inappropriate antidiuretic hormone secretion. An 8 mm carcinoid lung tumor with positron emission tomography normal uptake was found. After a thoracoscopic tumor resection the patient experienced complete clinical and laboratory remission. This is the first report of GCA with PMR in the context of carcinoid lung tumor. It emphasizes the role of paraneoplastic vasculitis as a possible cause of GCA.

Natural History of Typical Pulmonary Carcinoid Tumors: A Comparison of Non surgical and Surgical Treatment

BACKGROUND:The natural history of typical pulmonary carcinoid tumors has not been described and has important implications for counseling elderly patients or patients with high operative-risk about surgical resection.METHODS: Data from the Surveillance, Epidemiology, and End Results Program were used to identify 4,111 patients with biopsy specimen-proven lymph node-negative typical carcinoid tumor of the lung between 1988 and 2010; 306 had no resection, 929 underwent sublobar resection, and 2,876 underwent lobectomy. Overall survival and disease-specific survival (DSS) were analyzed using Kaplan-Meier plots. Multivariate analysis was used to determine predictors of survival.RESULTS: Five-year overall survival in patients who underwent lobectomy, sublobar resection, or no surgery was 93%, 92%, and 69%, respectively (P<.0001); 5-year DSS was 97%, 98%, and 88%, respectively (P<.0001). Among T1 tumors, DSS was 98% for patients who underwent lobectomy and sublobar resection and 92% for no surgery; among T2 tumors, DSS was 97%, 100%, and 87%, respectively, and among T3 and T4 tumors, it was 96%, 100%, and 75%, respectively. On multivariate analysis, nonoperative management was associated with an increased risk for disease-specific mortality compared with lobectomy (hazard ratio, 2.14; 95% CI, 1.35-3.40;P=.0013).CONCLUSIONS: In this population-based cohort, surgical resection of lymph node-negative carcinoid tumors is associated with a survival advantage over nonoperative treatment. However, the DSS at 5 years was still high without any treatment, suggesting that observation of asymptomatic peripheral typical carcinoid tumors or endoscopic management of symptomatic central carcinoid tumors may be considered in patients at high risk for surgical resection.

慢性腎臓病患者における血清ProGRP異常高値を伴った肺原発定型的カルチノイドの1切除例

慢性腎臓病患者における血清ProGRP異常高値を伴った肺原発定型的カルチノイドの1切除例

Medical management of pulmonary carcinoid tumors.

e18512 Background: The optimal medical management of pulmonary carcinoid tumors, which comprise 1-2% of all lung cancers, remains to be determined. Methods: We conducted a retrospective review of patients with typical and atypical pulmonary carcinoid tumors treated at our institution between 1990-present. Results: We identified 29 patients treated with chemotherapy, including 21 patients with metastatic disease (19 atypical, 2 typical), and 8 patients treated with adjuvant platinum-etoposide chemoradiation (6 atypical, 2 typical, 6 stage IIIA, 2 stage IIB). At an average follow-up of 12 months there were two recurrences in the 8 patients receiving adjuvant treatment, both of whom had atypical carcinoid (1 stage IIIA, 1 stage IIB). Of patients with metastatic disease, the principal sites of metastasis were the liver (70%), bone (40%), and brain (25%); median survival after diagnosis of metastatic disease was 3.8 y with a 5 y survival of 37%. Regimens showing efficacy in metastatic disease include octreotide (92% stable disease), etoposide-cisplatin (25% disease control, 2 partial response, 1 stable disease), and temozolomide (44% disease control, 2 partial responses, 2 stable disease). Conclusions: These results support our previous finding that pulmonary carcinoid tumors are responsive to chemotherapy and that adjuvant therapy should be offered to patients with stage II or IIIA resected disease.

Limited role of Ki-67 proliferative index in predicting overall short-term survival in patients with typical and atypical pulmonary carcinoid tumors

Limited role of Ki-67 proliferative index in predicting overall short-term survival in patients with typical and atypical pulmonary carcinoid tumors

Synchronous bilateral multiple typical pulmonary carcinoid tumors: a unique case with 10 typical carcinoids.

Bilateral multiple typical carcinoid tumors of the lung are uncommon malignancies. We discuss the case of a 64 year-old female with a nonproductive cough as the initial symptom. Thoracic computed tomography revealed multiple nodular lesions on both sides, which were initially misdiagnosed as multiple metastases of the lung with an unknown primary. After resection of nodules in the right hemithorax, pathologic examination revealed a typical carcinoid tumor. Bilateral sequential thoracotomy was performed and all ten nodules, (six on the right side and four on the left side), were treated by sublobar resection due to poor respiratory function. Pathological examination revealed all nodules to be typical carcinoid tumors. Following the resections, the patient has remained disease-free for ten months.

A case of typical pulmonary carcinoid tumor treated with bronchoscopic therapy followed by lobectomy

Carcinoid bronchopulmonary tumors represent approximately 25% of all carcinoid tumors and 1%–2% of all lung neoplasms. The most common symptoms are: persistent cough, asthma-like wheezing, chest pain, dyspnea, hemoptysis and obstructive pneumonitis. We present a case of a young adult diagnosed with a typical carcinoid tumor. The diagnosis was established on the basis of imaging examination and bronchoscopic biopsy. The patient was treated with bronchoscopic electrocautery therapy to relieve the obstructed airway, followed by surgical lobectomy in order to entirely remove the exophytic damage. This approach was not only a palliative management to bronchial obstruction but also avoided pneumonectomy. Recent studies support the use of such interventional resection methods, as they may result in a more conservative surgical resection.

Typical Pulmonary Carcinoid Tumor With Gangliocytic and Paraganglioma-Like Differentiation

Neuroendocrine neoplasms are routinely encountered in the lung, and carcinoid tumors represent the most common type. The diagnosis for these neoplasms is usually straightforward; however, the presence of divergent differentiation raises differential diagnostic possibilities not usually encountered. When a carcinoid tumor demonstrates gangliocytic and paraganglioma-like areas, the possibility of metastasis or a primary pulmonary paraganglioma is considered. The final diagnosis depends on extensive clinical correlation and immunohistochemical investigation.

Synchronous Bilateral Multiple Typical Pulmonary Carcinoid Tumors

Synchronous bilateral multiple carcinoid tumors of the lung are uncommon. We report an unusual case of synchronous, bilateral, and multiple typical pulmonary carcinoids. A 58-year-old woman with cough and chest pain was admitted to our clinic. A thoracic computed tomographic scan showed multiple bilateral nodular lesions. Bronchoscopic evaluation was normal. We performed sequential bilateral thoracotomies, frozen section examination, wedge resection, and lymphadenectomy. Histopathologic examinations revealed typical carcinoid tumor in both lungs. No pathologic lesions were observed in 18-month postoperative follow-up. We discuss treatment of synchronous bilateral multiple carcinoid tumors.

Rare Pleural Recurrence of Typical Pulmonary Carcinoid Tumor 30 Years After Lobectomy

A 68-year-old man, who underwent a left lower lobectomy for a typical carcinoid tumor 30 years previously, presented with a 2-year history of left-sided chest pain. A computed tomographic scan, an octreotide scan, and pleural biopsy all showed recurrence of carcinoid tumor in the pleura. He underwent left chest wall resection, and histopathologic examination confirmed the diagnosis of typical carcinoid tumor. A review of the literature indicates that this is only the second reported case of pleural recurrence after previous complete resection of typical pulmonary carcinoid tumor. This case is even more unusual in that the recurrence presented so long after surgery.

Long-term outcome of bronchoscopically resected endobronchial typical carcinoid tumors

Typical pulmonary carcinoid tumors represent less than 1% of lung tumors. In a subgroup of patients with this abnormality, the tumor is entirely endobronchial. We assessed the long-term outcome of such cases in which the patient was managed with endobronchial resection only. Patients who underwent bronchoscopic resection for a typical carcinoid tumor were identified through case records and histology reports. Data were collected retrospectively, but follow-up was on a prospective basis through the outpatient clinic. Between 1978 and 2004, 28 patients underwent bronchoscopic resection of endobronchial carcinoid tumors. The mean age was 49 years (standard deviation, 19 years; age range, 11-82 years), with 46% (13/28) of the patients being male. The tumor arose from the left bronchial tree in 61% (17/28), with the most common site being the left lower lobe bronchus (8/28). On average, patients required 5 bronchoscopic resections to achieve complete resection. The median follow-up was 8.8 years (interquartile range, 4.5-13.7 years). At 1 and 10 years, 100% and 94% of patients were disease free, respectively. The 1- and 10-year survivals were 89% (interquartile range, 84%-93%) and 84% (interquartile range, 77%-91%), respectively. In a selected group of patients, proximal polypoid typical bronchial carcinoid tumors can be treated endobronchially with good outcome.

Typical and Atypical Pulmonary Carcinoid Tumor Overdiagnosed as Small-Cell Carcinoma on Biopsy Specimens: A Major Pitfall in the Management of Lung Cancer Patients

Typical and Atypical Pulmonary Carcinoid Tumor Overdiagnosed as Small-Cell Carcinoma on Biopsy Specimens: A Major Pitfall in the Management of Lung Cancer Patients

Typical and Atypical Pulmonary Carcinoids: Outcome in Patients Presenting With Regional Lymph Node Involvement

Typical pulmonary carcinoid tumors are well-differentiated neuroendocrine tumors that are associated with good patient survival rates, while atypical carcinoid tumors are more aggressive and have worse patient survival rates. Because these tumors rarely involve the thoracic lymph nodes at presentation, it is currently unknown to what extent the presence of thoracic lymph node metastases at the time of diagnosis influences patient survival. A computerized search of the medical records for pulmonary carcinoid tumor at the Mayo Clinic from 1976 to 1997 revealed 517 patients, from which we identified 36 patients with pulmonary carcinoid tumors involving regional thoracic lymph nodes but without distant disease. For each patient, we reviewed the tumor histology, stage, and outcome. In addition, because the histologic criteria for the diagnosis of carcinoid tumors had changed significantly during the time of the study, we reexamined all of the histologic specimens using the current World Health Organization (WHO) criteria for classifying pulmonary neuroendocrine tumors. After reclassification with the WHO criteria for neuroendocrine tumors, 23 patients had typical carcinoid tumors with thoracic lymph node involvement. At the last follow-up, 19 patients had no evidence of disease (NED), 2 patients had developed systemic metastases (SM) and are still alive, and 2 patients had died. Eleven patients had atypical carcinoid tumors with thoracic lymph node involvement. At the last follow-up, four patients had NED, seven patients had developed SM within a median time of 17 months, and six patients with SM died shortly thereafter (median survival time, 25.5 months), while one is still alive. Two patients had been reclassified with large cell neuroendocrine carcinoma at the time of this review; both of these patients had developed SM (at 4 months and 21 months after diagnosis) and had died (at 15 months and 21 months after diagnosis, respectively). These data suggest that patients with atypical pulmonary carcinoid tumors with regional lymph node metastases have a high likelihood of developing recurrent disease if treated with surgical resection alone and have significantly worse outcome (p < 0.001) compared to those patients with typical carcinoid tumors with thoracic lymph node involvement.

Spontaneous Partial Expectoration of an Endobronchial Carcinoid

Typical pulmonary carcinoid tumors often present as proximal endobronchial masses discovered during the evaluation of cough and/or hemoptysis. We present a case of a carcinoid tumor that presented with spontaneous partial expectoration. A review of the literature revealed 16 cases of expectoration of fragments from various primary and metastatic tumors. Our case appears to be the first report of the expectoration of a carcinoid tumor.