Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases / Hiperplasia neuroendocrina difusa pulmonar idiopática: estudio de dos casos

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern. Patients and methods: We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea. Results and discussion: The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions

Introducción: La hiperplasia neuroendocrina difusa pulmonar idiopática (HNDPI) es una patología poco frecuente que se caracteriza por la proliferación de células neuroendocrinas en el parénquima pulmonar. Se describe clásicamente como una enfermedad que afecta a mujeres de mediana edad no fumadoras y que presentan tos persistente, disnea y sibilancias. La TC muestra una imagen de patrón en mosaico como resultado del atrapamiento aéreo. Pacientes y métodos: Presentamos dos casos de HNDPI que fueron enviados a nuestro servicio para realizar una biopsia pulmonar con la sospecha diagnóstica de enfermedad intersticial difusa. Ambos casos fueron mujeres con antecedentes de tos crónica y disnea de esfuerzo moderado. Resultados y discusión: El objetivo de este documento es que los facultativos tengan en cuenta este diagnóstico antes de tratar como asmática a una paciente con estas características, no olvidando además que son lesiones preneoplásicas

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases.

INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern. PATIENTS AND METHODS: We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea. RESULTS AND DISCUSSION: The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions.

Surgical treatment of low and intermediate grade lung net.

BACKGROUND: Carcinoids now constitute complex tumours which require a multidisciplinary approach and long-term follow-up. Surgical intervention is nowadays confirmed as the mainstay of treatment. METHODS: From 1980 to 2015, EMETNE-SEPAR collected 1,339 patients treated surgically for bronchial carcinoid (1,154 typical and 185 atypical carcinoids). Standard and conservative procedures were considered with regard to surgical approach. All the patients with carcinoid were pathologically coded following the standards of the 7th edition 2009 TNM lung cancer staging. Statistical analyses were performed in order to determine whether histology, nodal affectation and surgical technique were associated with significant differences in survival, presence of metastases and local recurrence. RESULTS: The influence of the surgical procedure on overall survival, the presence of metastases and local recurrence were demonstrated as no significant in our sample in central tumours (P>0.05). Sublobar resections in peripheral tumours are related to a decrease in survival in typical carcinoids (P=0.008) with nodal involvement and an increased number of recurrences in atypical carcinoids without nodal involvement (P=0.018). CONCLUSIONS: In central typical carcinoid, the use of lung-sparing bronchoplastic techniques could influence local recurrence in some cases. This observation demands the intraoperative pathologic verification of an adequate surgical margin by frozen section. Peripheral typical carcinoids have been surgically treated, occasionally, by sublobar resection. However, in peripheral atypical carcinoid after a limited sublobar resection the observed increase of the probability of local recurrence makes it, in our opinion, not advisable.

Cross-industry standard process for data mining is applicable to the lung cancer surgery domain, improving decision making as well as knowledge and quality management.

OBJECTIVES: The aim of this study was to assess the applicability of knowledge discovery in database methodology, based upon data mining techniques, to the investigation of lung cancer surgery. METHODS: According to CRISP 1.0 methodology, a data mining (DM) project was developed on a data warehouse containing records for 501 patients operated on for lung cancer with curative intention. The modelling technique was logistic regression. RESULTS: The finally selected model presented the following values: sensitivity 9.68%, specificity 100%, global precision 94.02%, positive predictive value 100% and negative predictive value 93.98% for a cut-off point set at 0.5. A receiver operating characteristic (ROC) curve was constructed. The area under the curve (CI 95%) was 0.817 (0.740- 0.893) (p < 0.05). Statistical association with perioperative mortality was found for the following variables [odds ratio (CI 95%)]: age over 70 [2.3822 (1.0338-5.4891)], heart disease [2.4875 (1.0089-6.1334)], peripheral arterial disease [5.7705 (1.9296-17.2570)], pneumonectomy [3.6199 (1.4939-8.7715)] and length of surgery (min) [1.0067 (1.0008-1.0126)]. CONCLUSIONS: The CRISP-DM process model is very suitable for lung cancer surgery analysis, improving decision making as well as knowledge and quality management.

Lung carcinoma with rhabdoid component. A series of seven cases associated with uncommon types of non-small cell lung carcinomas and alveolar entrapment.

Rhabdoid tumor, included in the WHO classification among large cell carcinomas of the lung, is an uncommon type of lung carcinoma with poor prognosis. We report a series of 7 cases of lung carcinomas with rhabdoid component in 10% and 80% of the tumor. The associated tumor was adenocarcinoma in 3 cases--one of them with focal micropapillary pattern--large cell carcinoma in 2 cases, squamous cell carcinoma in 1 case and pleomorphic carcinoma in 1 case. Two adenocarcinomas showed a focal spindle cell component. Micropapillary and pleomorphic types had not been reported before as a component associated with rhabdoid carcinomas. All cases were positive for vimentin, and AE1/AE3 cytokeratin and 5 cases for cytokeratin 7. All cases were negative for muscle and endothelial markers and for chromogranin A. Synaptophysin was focally positive only in one case. Alveolar trapping inside the tumor was present in 3 cases--a phenomenon not well studied in lung carcinomas and also not reported in tumors with rhabdoid component. Five patients died because of the tumor within 2 to 31 months after diagnosis, one of myocardial infarction and only one is alive and disease free 123 months after the diagnosis. In summary, we describe 7 new cases of this uncommon lung tumor with aggressive clinical course, associated with infrequent histological types in nonrhabdoid component and with alveolar trapping, a nondescribed finding.

Extraction of airway foreign bodies in adults: experience from 1987-2008.

To determine the incidence and the clinical, radiographic, and endoscopic characteristics of adult patients in our area diagnosed with tracheobronchial foreign bodies (FBs), we have performed a descriptive retrospective study analysing rigid and flexible bronchoscopies practised at our department between 1987 and 2008 in patients older than 14 years. Of the 9781 bronchoscopies performed, 32 involved cases of bronchoaspiration of FBs. The mean age of the patients was 43.81 years (S.D. 21.43); 65.6% were male and 34.4% were female. Acute or recurrent infection was the most frequent clinical presentation. Chest radiographs provided data for diagnosis in 68% of the cases. The most common FB aspirated were inorganic (pins and plastic devices 21.4%, respectively). In conclusion, we can state that in our area tracheobronchial aspiration of FBs by adults is not common. The clinical symptoms are highly variable and the FBs are usually lodged in the right bronchial tree.

Typical and atypical carcinoid tumours: analysis of the experience of the Spanish Multi-centric Study of Neuroendocrine Tumours of the Lung.

BACKGROUND: This study examines the experience of the Spanish Multi-centric Study of Neuroendocrine Tumours of the Lung with patients treated surgically for typical and atypical carcinoid tumours. METHODS: From 1980 to 2002, 661 patients were treated surgically for 569 typical carcinoid tumours and 92 atypical carcinoid tumours. Three hundred and four cases were studied retrospectively from 1980 to 1997 (261 typical carcinoid and 43 atypical carcinoid tumours); the other 357 new cases (308 typical carcinoid and 49 atypical carcinoid tumours) were collected prospectively from 1998 to 2002. Tumours were classified according the 1999 classification from the WHO and the International Association for the Study of Lung Cancer (IASLC). Several variables were reviewed in all patients. Univariate and multivariate statistical analyses were performed in order to determine whether clinical characteristics were associated with significant differences in survival. RESULTS: In the total of the patients, 5-year survival for different tumours was as follows: typical carcinoid: overall survival 97%; with nodal involvement 100%; atypical carcinoid: overall 78%; with nodal involvement 60%. A significant difference in survival was found between patients in the retrospective and prospective groups with atypical carcinoid and nodal involvement. The comparative analysis of several factors in typical and atypical carcinoid tumours showed a significant difference for mean age, tumour size, nodal involvement and distant metastases. CONCLUSION: Nodal involvement and histological sub-type appear as the most important factors influencing the prognosis. Adequate lung resection and systematic radical mediastinal lymphadenectomy should always be performed. Sleeve resection could be performed in central typical and atypical carcinoid tumours, avoiding pneumonectomy.

Local recurrence and metastatic disease in a typical N1 carcinoid bronchial tumour.

Typical carcinoid bronchial tumour is a well-known disease that, for years, was considered benign. Currently, it is classified within the group of neuro-endocrine lung tumours. It is a low-grade malignancy tumour with a capability of local and distant recurrence. Complete resection with mediastinal lymphadenectomy is the treatment-of-choice. There are, usually, long-term survivors, even in cases of recurrence or mediastinal node invasion. These patients could benefit from removal of recurrent or metastatic disease. We present, here, a case of a 19-years-old female diagnosed as having N1-bronchial typical carcinoid tumour. She underwent radical surgery, but with mediastinal recurrence and hepatic metastases. A new radical lung resection was performed, and a liver transplant was the therapy-of-choice for the metastatic lesion.

Local recurrence and metastatic disease in a typical N1 carcinoid bronchial tumour

No disponible

Typical carcinoid bronchial tumour is a wellknown disease that, for years, was considered benign. Currently, it is classified within the group of neuro-endocrine lung tumours. It is a low-grade malignancy tumour with a capability of local and distant recurrence. Complete resection with mediastinal lymphadenectomy is the treatment-ofchoice. There are, usually, long-term survivors, even in cases of recurrence or mediastinal node invasion. These patients could benefit from removal of recurrent or metastatic disease. We present, here, a case of a 19-years-old female diagnosed as having N1-bronchial typical carcinoid tumour. She underwent radical surgery, but with mediastinal recurrence and hepatic metastases. A new radical lung resection was performed, end a liver transplant was the therapy-of-choice for the metastatic lesion

Liposarcomas mediastínicos de localización atípica. Aportación de 2 casos / Liposarcomas of the mediastinum in atypical locations: reports of two cases

El liposarcoma mediastínico es un tumor raro que se caracteriza clínicamente por la compresión de las estructuras vecinas y la tendencia a la recidiva. La tomografía computarizada y la resonancia magnética aportan información relevante para su diagnóstico. La resección quirúrgica es la mejor opción terapéutica en estos tumores, según la bibliografía revisada. A continuación presentamos 2 casos de liposarcoma mediastínico: 1 liposarcoma recidivado de un lipoma atípico cervical y 1 liposarcoma gigante de mediastino posterior con extensión a ambos hemitórax (AU)

Liposarcomas of the mediastinum are unusual tumors characterized by compression of neighboring structures and frequent recurrence. Computed tomography and magnetic resonance imaging provide useful data for diagnosis. According to a review of the literature, surgical resection is the most effective treatment. We report two cases of mediastinal liposarcoma: the first was a liposarcoma that was a recurrence of an atypical lipoma of the neck, and the second was a giant mediastinal liposarcoma involving both hemithoraces (AU)

[Liposarcomas of the mediastinum in atypical locations: report of two cases]. / Liposarcomas mediastínicos de localización atípica. Aportación de 2 casos.

Liposarcomas of the mediastinum are unusual tumors characterized by compression of neighboring structures and frequent recurrence. Computed tomography and magnetic resonance imaging provide useful data for diagnosis. According to a review of the literature, surgical resection is the most effective treatment. We report two cases of mediastinal liposarcoma: the first was a liposarcoma that was a recurrence of an atypical lipoma of the neck, and the second was a giant mediastinal liposarcoma involving both hemithoraces.