Clinical analysis of primary lung tumors in 56 children / 中华肿瘤杂志

Objective: To investigate the clinical manifestation, pathological type, treatment and prognosis of primary lung tumors in children. Methods: We collected and retrospectively analyzed the clinical manifestation, pathological type, therapeutic method and prognosis of 56 primary lung tumors patients who diagnosed from 2009 to 2019 in Guangzhou Women and Children Medical Center. Results: There were 56 patients identified as the primary lung tumors, including pleuropulmonary blastoma (PPB, n=28), pulmonary inflammatory myofibroblastic tumor(IMT, n=20), mucoepidermoid carcinoma(n=6), infantile hemangioma (n=1), pulmonary sclerosing hermangioma(n=1). Respiratory symptoms were the most manifestation at the time of diagnosis including 26 patients with cough, 3 with hemoptysis, and 17 with dyspnea. Others included 15 with fever, 3 with chest pain, and 2 with epigastiric pain. The primary tumor of 18 cases were located in the lower lobe of left lung, 11 cases in the lower lobe of right lung, 10 cases in the upper lobe of left lung, 7 cases in the upper lobe of right lung, 6 cases in the middle lobe of right lung, and 4 cases in pulmonary hilum. Among the 56 patients, 41 patients underwent thoracotomy, 13 thoracoscopy, and 2 fiberoptic bronchoscopy. Five patients with type Ⅰ PPB were still alive at the end of follow-up without chemotherapy. Among 5 patients with type Ⅱ PPB, 2 patients without chemotherapy died after recurrence, 3 patients suffered postoperative chemotherapy were still alive at the end of follow-up. All of the 18 patients with type Ⅲ PPB underwent postoperative chemotherapy with IVADo regimen. Recurrence occurred in 6 cases, distant metastasis occurred in 3 cases, and cancer-related deaths occurred in 8 cases. For 20 patients with IMT, recurrence occurred in 5 of 13 patients experienced wedge resection, 1 of 6 patients experienced lobectomy and 1 of 6 underwent fiberoptic bronchoscopy, respectively. For 6 mucoepidermoid carcinoma patients, lobectomy was carried on 5 patients, wedge resection on 1 patient, all of them were still alive at the end of follow-up. One hermangioma patient underwent fiberoptic bronchoscopy and other 1 sclerosing hermangioma patient underwent wedge resection, both of them were still alive at the end of follow-up. Conclusions: The clinical manifestations of the primary lung tumors in children are nonspecific. Complete resection and achieving negative marginattribute to the excellent outcome. Adjunctive treatment such as chemotherapy is necessary for patients with type Ⅱ and type Ⅲ PPB.

Pleuropulmonary blastoma in children: a case report

Pleuropulmonary Blastoma [PPB] is a very rare, highly aggressive and malignant tumor that originates from either the lungs or pleura. It occurs mainly in children aged less than five or six years. It has poor prognosis with three different subtypes: cystic [type I], combined cystic and solid [type II] and solid [type III]. PPB is treated with aggressive multimodal therapies including surgery and chemotherapy. We present a case of 3.5 years old boy with PPB type II successfully treated with complete surgical resection followed by neoadjuvant chemotherapy

Pleuropulmonary blastoma in an infant.

Pleuropulmonary blastomas (PPB) are rare and highly aggressive tumors. Herein, we report an infantile case of type III PPB. A 9-mth-old boy presented to our unit with a history of cough and tachypnea for 2 days. Chest computed tomography revealed a mass in the left upper lobe, emphysema in the left upper lung, and mediastinum and heart shifted towards the right. The mass was removed completely by left upper lobectomy and histology confirmed diagnosis of type III PPB. The immature blastematous tissue was positive for vimentin while benign epithelium was positive for epithelial membrane antigen and cytokeratin. No lymph nodule metastasis was found in the 7 lymph nodules obtained from the hilum of the lung near the tumor. Currently, the patient is under close follow-up and is doing well.

Pleuropulmonary blastoma: a rare intrathoracic childhood tumour

This report documents a case of pleuropulmonary blastoma presenting in a four year old boy. This is a rare and highly malignant childhood intrathoracic neoplasm that has certain clinical and histological features which differs from the adult form of pulmonary blastoma. The differential diagnosis of an intrathoracic childhood pulmonary blastoma includes other childhood solid small round cell tumours

Blastoma pulmonar: tratamento cirúrgico por lobectomia superior direita e broncoplastia / Pulmonary blastoma: treatment through sleeve resection of the right upper lobe

O blastoma pulmonar é um tumor maligno raro, de crescimento rápido, composto por uma mistura de células epiteliais e mesenquimais malignas. Relatamos o caso de uma paciente com hemoptise e uma massa no lobo superior direito. Apresentava uma reserva pulmonar limitada e achado fibrobroncoscópico de comprometimento do brônquio intermediário. O tratamento realizado através de lobectomia superior direita com broncoplastia foi inédito. A paciente encontra-se assintomática após 36 meses de seguimento. A literatura é revisada em relação a aspectos clínicos, anatomopatológico e tratamento.

Tumores malignos pleuropulmonares primitivos de la infancia / Malignant pleuropulmonary primary tumors in childhood

Se presentan 3 casos de tumores primitivos de origen pleuropulmonar:2 blastomas pleuropulmonares y un mesotelioma.Se obtuvieron inicialmente remisiones completas con quimioterapia convencional,cirugía y en uncaso se agregó radioterapia.Un paciente murió a los 14 meses y 2 están en progresión de enfermedad 12 y 14 meses después del diagnóstico.Terapia multimodal,cirugía,poliquimioterapia y radioterapia,juegan un rol importante en el tratamiento de estos tumores,sin embargo es insuficiente y no previenen la recurrencia local y/o metástasis.Se hace hincapié en el mal pronóstico de este tipo de tumores

Blastoma pulmonar. Reporte de un caso pediátrico / Pulmonary blastoma. A pediatric case

El blastoma pulmonar es una entidad esporádica en niños, siendo un tumor raro, con malignidad primaria para el pulmón y la pleura. Se presenta el caso de un preescolar masculino de tres años de edad cuyo cuadro clínico se manifestó por dolor abdominal, fiebre pérdida importante de peso, ataque al estado general y dificultad respiratoria, diagnosticándose en hospital de segundo nivel como neumonía y derrame pleural. Se discuten las características clínicas y el abordaje diagnóstico en relación a la literatura. Conclusión: nuestro caso demuestra la dificultad para establecer el diagnóstico sin el apoyo del tratamiento quirúrgico. La tomografía computada sugiere la extensión del tumor y la invasión mediastinal, pero es la toracotomía la que de hecho demuestra la posibilidad de resecarse si no existe compromiso mediastinal. Aunque es un tumor con baja frecuencia, las decisiones terapéuticas del blastoma pulmonar son difíciles, sobre todo si tomanos en cuenta que la quimioterapia y radioterapia posteriores al acto quirúrgico, aún están en el plano de la discusión

Blastoma Pulmonar. Presentacion de 1 Caso / Pulmonary blastoma. A case report

Se presenta el caso de un paciente de 18 anos de edad, de raza blanca y sexo masculino, con manifestaciones respiratorias de tos, expectoracion, dolor toracico y rayos X de torax con una masa del lobulo superior izquierdo. Despues de pleurotomia minima y biopsia se le diagnostico un blastoma pulmonar. Se le realizo neumectomia y posteriormente, se le administro poliquimioterapia