Namaoui Rabia Yasmine, Hadjaoui Wissam, Zerabib Asma, Belbekri Leila, Zar Fadéla, Berrah Hemana, Mouats Ali, Bessaïh Abdessamed
Service d'Anatomie et Cytologie Pathologiques, CLCC Béchar, Route Ouakda, N6, Béchar, Algérie.
Université de Béchar Tahri Mohammed, BP 417 Route Kenadsa, Bechar, Algérie.
Pan Afr Med J. 2022 Sep 6;43:8. doi: 10.11604/pamj.2022.43.8.33823. eCollection 2022.
Pleuropulmonary blastoma is a rare intrathoracic tumor in children. It is associated with poor prognosis and diagnosis is based on histological examination. We conducted a didactic study involving a 3-year-old child with severe acute respiratory distress associated with hemothorax; radiological and thoracoscopic examination suggested malignant pleuropulmonary process. Anatomopathological examination with radio-clinical comparison allowed for the diagnosis of solid-cystic pleuropulmonary blastoma type II. Unfortunately, given the severity of the clinical features, the child died within a few weeks due to multiple organ failure. Pathologist experience is very important to recognize the disease and to start adequate treatment as soon as possible. This allows for a tumor regression rate up to 90% after neoadjuvant treatment and a 5-year survival rate of at least 53% for aggressive forms: solid and solido-cystic tumors.
肺胚细胞瘤是儿童罕见的胸腔内肿瘤。其预后较差，诊断基于组织学检查。我们进行了一项教学研究，对象是一名3岁儿童，患有与血胸相关的严重急性呼吸窘迫；放射学和胸腔镜检查提示为恶性胸膜肺病变。通过解剖病理学检查及影像学与临床对照，诊断为II型实性-囊性肺胚细胞瘤。不幸的是，鉴于临床特征的严重性，该儿童因多器官功能衰竭在数周内死亡。病理学家的经验对于识别该疾病并尽快开始适当治疗非常重要。这使得新辅助治疗后肿瘤退缩率高达90%，对于侵袭性类型（实性和实性-囊性肿瘤）5年生存率至少为53%。
