Pleuropulmonary blastoma: a single-institution experience. J Pediatr Hematol Oncol 2012 Jul;34(5):e182-5
Date
05/16/2012Pubmed ID
22584785DOI
10.1097/MPH.0b013e3182546adfScopus ID
2-s2.0-84862868368 (requires institutional sign-in at Scopus site) 16 CitationsAbstract
Pleuropulmonary blastoma (PPB) is a rare primary intrathoracic mesenchymal malignancy that occurs exclusively in early childhood. Twelve patients were diagnosed with PPB (1 type I, 5 type II, and 6 type III) between 1979 and 2009 at our institution. Upfront complete tumor resection was successful in 5 of 6 patients. Six patients had biopsy followed by neoadjuvant chemotherapy, 2 had complete tumor resection, and 2 had microscopic residual disease after surgery. All patients received vincristine, dactinomycin, and cyclophosphamide chemotherapy. Eight received additional chemotherapy with doxorubicin, cisplatin, etoposide, or ifosfamide. Three patients received local irradiation. The 5-year event-free and overall survivals were 33% ± 14% and 42% ± 14%, respectively. Median time to progression was 8 months. Five of 9 patients with gross total resection survived, whereas all 3 with gross residual disease died. Three of 5 survivors did not receive radiation. A high index of suspicion for PPB must be maintained in all patients diagnosed with intrathoracic sarcoma in early childhood. Gross total resection is necessary for cure, and selected patients do not require radiation therapy.
Author List
Venkatramani R, Malogolowkin MH, Wang L, Mascarenhas LMESH terms used to index this publication - Major topics in bold
Child, PreschoolFemale
Humans
Infant
Infant, Newborn
Lung Neoplasms
Male
Pulmonary Blastoma
