RESUMO
Neuroendocrine neoplasms (NENs) are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise mainly due to an improvement on diagnostic techniques and awareness. Earlier detection, along with steadfast improvements in therapy, has led to better prognosis over time for advanced gastrointestinal and pancreatic neuroendocrine tumors. The aim of this guideline is to update evidence-based recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification, and therapeutic options, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are reviewed and discussed, and treatment algorithms to guide therapeutic decisions are provided.
Assuntos
Neoplasias Brônquicas , Carcinoma Neuroendócrino , Tumores Neuroendócrinos , Humanos , Neoplasias Brônquicas/diagnóstico , Neoplasias Brônquicas/terapia , Algoritmos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapiaRESUMO
SUMMARY: Neuroendocrine lung tumours are a group of different tumours that have similar morphological, immunohistochemical and molecular characteristics, and represents 1-2 % of all malignant lung tumours. Tumorlet carcinoids represent the nodular proliferation of hyperplastic neuroendocrine cells that is smaller than 5 mm in size. In this study, we reported the unusual finding of tumour carcinoid and endobronchial hamartoma in the same bronchus. A 49-year-old male patient with symptoms of prolonged severe cough and fever, and was treated for pneumonia. Since he did not adequately respond to antibiotic therapy lung CT scan was performed which showed middle lobe bronchus obstruction. Bronchoscopy revealed a lobulated whitish tumour which was biopsied and histopathological diagnosis was hamartoma. Tumour could not be completely removed during bronchoscopy, it was decided to surgically remove it. On serial section, during gross examination in the same bronchus, an oval yellowish area with a diameter of 3 mm was found along the bronchial wall. According to gross and histomorphological characteristics and immunophenotype of tumour cells, the diagnosis of tumour carcinoid was set. Diagnosis of carcinoids of the tumorlet type is usually an accidental finding and it can be seen on CT in the form of subcentimeter, single or multiple, nodular changes. Considering that the clinical picture is nonspecific, they should always be kept in mind as a possible differential diagnosis.
RESUMEN: Los tumores neuroendocrinos de pulmón son un grupo de tumores de diferentes características morfológicas, inmunohistoquímicas y moleculares similares, y representan el 1- 2 % de todos los tumores malignos de pulmón. Los carcinoides tumorales representan la proliferación nodular de células neuroendocrinas hiperplásicas de tamaño inferior a 5 mm. En este estudio reportamos el hallazgo inusual de tumor carcinoide y hamartoma endobronquial en el mismo bronquio. Un paciente varón de 49 años con síntomas de tos severa prolongada y fiebre fue tratado por neumonía. Al no responder adecuadamente a la terapia con antibióticos, se realizó una tomografía computarizada de pulmón que mostró obstrucción del bronquio del lóbulo medio. La broncoscopia reveló una tumoración blanquecina lobulada de la cual se tomó biopsia y el diagnóstico histopatológico fue hamartoma. No fue posible extirpar el tumor por completo durante la broncoscopia y se decidió extirparlo quirúrgicamente. En la sección seriada, durante el examen macroscópico en el mismo bronquio, se encontró un área amarillenta ovalada de 3 mm de diámetro a lo largo de la pared bronquial. De acuerdo a las características macroscópicas e histomorfológicas y de inmunofenotipo de las células tumorales, se estableció el diagnóstico de tumor carcinoide. El diagnóstico de carcinoides de tipo tumorlet suele ser un hallazgo accidental y se observan en la TC en forma de cambios nodulares subcentimétricos, únicos o múltiples. En consideración de que el cuadro clínico es inespecífico, siempre debe tenerse en cuenta como posible diagnóstico diferencial.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Brônquicas/patologia , Tumor Carcinoide/patologia , Hamartoma/patologia , Neoplasias Brônquicas/diagnóstico , Broncoscopia , Tumor Carcinoide/diagnóstico , Hamartoma/diagnósticoRESUMO
Los tumores traqueo bronquiales son extremadamente infrecuentes en la edad pediátrica e incluyen lesiones benignas y malignas. Por la baja frecuencia en niños y sintomatología respiratoria inespecífica, la sospecha diagnostica es habitualmente tardía. El tratamiento de elección en la mayoría de ellos es la resección quirúrgica abierta, sin embargo, la remoción endoscopia podría estar indicada es casos muy seleccionados con histología benigna y de localización accesible.
Tracheobronchial tumors are extremely rare in children and include benign and malignant lesions. Due to the low frequency in children and nonspecific respiratory symptoms, diagnostic suspicion is usually late. The treatment of choice in most of them is open surgical resection, however, endoscopy removal could be indicated in highly selected cases with benign histology and accessible location.
Assuntos
Humanos , Criança , Neoplasias Brônquicas/diagnóstico , Neoplasias Brônquicas/terapia , Neoplasias da Traqueia/diagnóstico , Neoplasias da Traqueia/terapia , Neoplasias Brônquicas/classificação , Neoplasias da Traqueia/classificaçãoRESUMO
NENs are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise across all sites, stages and grades. Although improved diagnostic techniques have led to earlier detection and stage migration, the improved prognosis documented over time for advanced gastrointestinal and pancreatic neuroendocrine tumors also reflect improvements in therapy. The aim of this guideline is to update practical recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification and therapeutic options are briefly discussed, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, and treatment algorithms are provided.
Assuntos
Neoplasias Brônquicas/terapia , Neoplasias Gastrointestinais/terapia , Tumores Neuroendócrinos/terapia , Neoplasias Pancreáticas/terapia , Guias de Prática Clínica como Assunto/normas , Neoplasias Brônquicas/diagnóstico , Ensaios Clínicos como Assunto , Terapia Combinada , Gerenciamento Clínico , Neoplasias Gastrointestinais/diagnóstico , Humanos , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Prognóstico , Sociedades MédicasRESUMO
Resumen El carcinoma mucoepidermoide bronquial es una neoplasia infrecuente, representando el 0,1 a 0,2% de los tumores malignos primarios del pulmón. En general tiene un buen pronóstico, sin embargo, existe un subtipo de alto grado de pronóstico más ominoso. En este artículo se presentan dos casos clínicos de carcinoma mucoepidermoide bronquial de bajo grado, enfocado en su diagnóstico y manejo quirúrgico.
ABSTRACT Bronchopulmonary mucoepidermoid carcinoma is an uncommon neoplasm, accounting for 0.1 to 0.2% of primary malignant tumors of the lung. In general it has a good prognosis, however there is a subtype of high grade of more ominous prognosis. In this paper we present two clinical cases of low grade pulmonary mucoepidermoid carcinoma, focused on their diagnosis and surgical management.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Neoplasias Brônquicas/cirurgia , Neoplasias Brônquicas/diagnóstico , Carcinoma Mucoepidermoide/cirurgia , Carcinoma Mucoepidermoide/diagnóstico , Prognóstico , Tórax/diagnóstico por imagem , Broncoscopia/instrumentação , Tomografia Computadorizada por Raios X , Microscopia/instrumentaçãoAssuntos
Aciclovir/uso terapêutico , Antivirais/uso terapêutico , Brônquios/patologia , Herpes Simples/diagnóstico , Idoso , Neoplasias Brônquicas/diagnóstico , Diagnóstico Diferencial , Herpes Simples/tratamento farmacológico , Humanos , Masculino , Simplexvirus/genética , Tomografia Computadorizada por Raios XRESUMO
Los tumores bronquiales son raros en los niños. La mayoría son malignos; el más común es el tumor carcinoide. Con menor frecuencia, se asienta en el árbol traqueobronquial el carcinoma mucoepidermoide. El carcinoma mucoepidermoide representa del 0,1% al 0,2% de los tumores malignos broncopulmonares. Se manifiesta con síntomas de obstrucción de la vía aérea y/o neumonías recurrentes. El diagnóstico precoz y el tratamiento adecuado son de gran importancia en el pronóstico. La broncoscopía con biopsia de la masa endobronquial es el método de elección para confirmar el diagnóstico. El tratamiento consiste en la resección quirúrgica de la lesión. Se presenta a una niña de 11 años con disnea de esfuerzo y tos debidas a un carcinoma mucoepidermoide bronquial de bajo grado de malignidad. Se describen las manifestaciones clínicas, los métodos diagnósticos y el tratamiento de esta rara neoplasia en pediatría.
Bronchial tumors are rare in children. Most of them are malignant, being the carcinoid tumor the most common. Less frequently the mucoepidermoid carcinoma is found in the tracheobronchial tree. Mucoepidermoid carcinoma accounts for 0.1 to 0.2% of all malignant bronchopulmonary tumors. It manifests with symptoms of airway obstruction and/or recurrent pneumonias. Early diagnosis and appropriate treatment are of great importance in the prognosis. Bronchoscopy with biopsy of the endobronchial mass is the method of choice to confirm the diagnosis. Treatment consists of surgical resection of the lesion. We present an 11-year-old girl with dyspnea on exertion and cough due to a low malignant mucoepidermoid carcinoma of the bronchi. We describe the clinical manifestations, the diagnostic methods and the treatment of this rare neoplasia in pediatrics.
Assuntos
Humanos , Feminino , Criança , Neoplasias Brônquicas/cirurgia , Neoplasias Brônquicas/diagnóstico , Carcinoma Mucoepidermoide/cirurgia , Carcinoma Mucoepidermoide/diagnósticoRESUMO
Bronchial tumors are rare in children. Most of them are malignant, being the carcinoid tumor the most common. Less frequently the mucoepidermoid carcinoma is found in the tracheobronchial tree. Mucoepidermoid carcinoma accounts for 0.1 to 0.2% of all malignant bronchopulmonary tumors. It manifests with symptoms of airway obstruction and/or recurrent pneumonias. Early diagnosis and appropriate treatment are of great importance in the prognosis. Bronchoscopy with biopsy of the endobronchial mass is the method of choice to confirm the diagnosis. Treatment consists of surgical resection of the lesion. We present an 11-year-old girl with dyspnea on exertion and cough due to a low malignant mucoepidermoid arcinoma of the ronchi. We describe the clinical manifestations, the diagnostic methods and the treatment of this rare neoplasia in pediatrics.
Los tumores bronquiales son raros en los niños. La mayoría son malignos; el más común es el tumor carcinoide. Con menor frecuencia, se asienta en el árbol traqueobronquial el carcinoma mucoepidermoide. El carcinoma mucoepidermoide representa del 0,1% al 0,2% de los tumores malignos broncopulmonares. Se manifiesta con síntomas de obstrucción de la vía aérea y/o neumonías recurrentes. El diagnóstico precoz y el tratamiento adecuado son de gran importancia en el pronóstico. La broncoscopía con biopsia de la masa endobronquial es el método de elección para confirmar el diagnóstico. El tratamiento consiste en la resección quirúrgica de la lesión. Se presenta a una niña de 11 años con disnea de esfuerzo y tos debidas a un carcinoma mucoepidermoide bronquial de bajo grado de malignidad. Se describen las manifestaciones clínicas, los métodos diagnósticos y el tratamiento de esta rara neoplasia en pediatría.
Assuntos
Neoplasias Brônquicas , Carcinoma Mucoepidermoide , Neoplasias Brônquicas/diagnóstico , Neoplasias Brônquicas/cirurgia , Carcinoma Mucoepidermoide/diagnóstico , Carcinoma Mucoepidermoide/cirurgia , Criança , Feminino , HumanosAssuntos
Neoplasias Brônquicas/complicações , Neoplasias Brônquicas/diagnóstico , Hemangioma/complicações , Hemangioma/diagnóstico , Síndrome do Desconforto Respiratório/etiologia , Neoplasias Brônquicas/terapia , Pré-Escolar , Feminino , Hemangioma/terapia , Humanos , Síndrome do Desconforto Respiratório/diagnóstico , Síndrome do Desconforto Respiratório/terapiaRESUMO
Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient's symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up