Pleuropulmonary blastoma: A single-institution experience

Rajkumar Venkatramani, Marcio Malogolowkin, Larry Wang, Leo Mascarenhas

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

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.

Original languageEnglish (US)
JournalJournal of Pediatric Hematology/Oncology
Volume34
Issue number5
DOIs
StatePublished - Jul 1 2012
Externally publishedYes

Keywords

  • chemotherapy
  • microscopic residual disease
  • pleuropulmonary blastoma
  • radiation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

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