Eur J Pediatr Surg 2008; 18(3): 192-194
DOI: 10.1055/s-2008-1038484
Case Report

© Georg Thieme Verlag KG Stuttgart · New York

A Familial Case of Pleuropulmonary Blastoma

J. R. Gutweiler1 , J. LaBelle2 , M. Y. Suh1 , M. V. Misra1 , M. Ahmed3 , E. Y. Lee4 , S. O. Vargas5 , H. B. Kim1 , C. B. Weldon1
  • 1Department of Surgery, Children's Hospital Boston, Boston, Massachusetts, USA
  • 2Department of Hematology/Oncology, Dana Farger Cancer Institute, Boston, Massachusetts, USA
  • 3Department of Radiology, Beth-Israel Deaconess Medical Center, Boston, Massachusetts, USA
  • 4Department of Radiology and Medicine, Pulmonary Division, Children's Hospital Boston, Boston, Massachusetts, USA
  • 5Department of Pathology, Children's Hospital Boston, Boston, Massachusetts, USA
Further Information

Publication History

received June 27, 2007

accepted after revision December 19, 2007

Publication Date:
21 May 2008 (online)


Purpose: 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. Patients and Methods: We present the case of two brothers diagnosed with PPB. Results: 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. Conclusion: 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.


Dr. Christopher B. Weldon

Department of Surgery
Children's Hospital Boston

300 Longwood Avenue, Fegan 3

Boston, MA 02115