Gutweiler J R, Labelle J, Suh M Y, Misra M V, Ahmed M, Lee E Y, Vargas S O, Kim H B, Weldon C B
Department of Surgery, Children's Hospital Boston, Boston, Massachusetts, USA.
Eur J Pediatr Surg. 2008 Jun;18(3):192-4. doi: 10.1055/s-2008-1038484.
Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm of early childhood arising in the lung or visceral pleura. Approximately 150 cases have been reported in the literature, with only one previously documented case of PPB in siblings.
We present the case of two brothers diagnosed with PPB.
A two month-old boy with an abnormal breathing pattern was referred for evaluation of a cystic mass discovered on chest radiograph. Computed tomography (CT) of the chest was performed at our institution which revealed findings compatible with congenital cystic adenomatoid malformation (CCAM) of the right middle and lower lobes. The patient underwent urgent thoracic exploration one week later after developing severe respiratory distress. Histological examination revealed PPB type I (cystic). The patient's 15-month-old brother was presumed to have a CCAM noted radiographically months earlier during an asthma exacerbation. He underwent elective cyst resection and was also found to have type I PPB. The index patient was treated with adjuvant chemotherapy due to the large size of the PPB and intraoperative spillage of cystic fluid during the emergent surgery. In contrast, the brother is being followed without adjuvant chemotherapy, given the much smaller size of the PPB, wide margins of resection, and lack of spillage. Family history included an uncle diagnosed at age 11 with an unusual form of T cell acute lymphoblastic leukemia.
Although PPB is known to have a familial association with other neoplasms, this case represents only the second report of PPB occurring in siblings. The importance of thoroughly investigating and resecting pulmonary cystic masses in the pediatric population is highlighted by these cases.
胸膜肺母细胞瘤（PPB）是一种罕见的儿童期胸腔内肿瘤，起源于肺或脏层胸膜。文献报道约150例，此前仅有1例同胞患PPB的记录。
我们报告两兄弟被诊断为PPB的病例。
一名呼吸模式异常的2个月大男孩因胸部X线片发现囊性肿块而前来评估。我院对其进行了胸部计算机断层扫描（CT），结果显示与右中、下叶先天性囊性腺瘤样畸形（CCAM）相符。该患者在出现严重呼吸窘迫1周后接受了紧急开胸探查。组织学检查显示为I型（囊性）PPB。该患者15个月大的兄弟在数月前哮喘发作时胸部X线检查推测有CCAM。他接受了择期囊肿切除术，也被发现患有I型PPB。由于PPB体积较大且急诊手术中囊液外溢，索引患者接受了辅助化疗。相比之下，其兄弟因PPB体积小得多、切除边缘宽且无外溢，未接受辅助化疗，正在接受随访。家族史包括一名11岁时被诊断为特殊形式T细胞急性淋巴细胞白血病的叔叔。
虽然已知PPB与其他肿瘤有家族关联，但该病例是同胞患PPB的第二例报道。这些病例凸显了对儿科人群肺囊性肿块进行全面检查和切除的重要性。
