Open AccessPleuropulmonary blastoma
Author(s) -
Jamila Shuja,
Iftikhar Ahmad,
Khushnaseeb Ahmad,
Hina Manzoor,
Shahwali Kakar,
Muhammad Sadiq,
M. Ahmad,
Mehjabeen Marri,
Hamida Naheed
Publication year - 2017
Publication title -
journal of cancer research and practice
Language(s) - English
Resource type - Journals
eISSN - 2589-0425
pISSN - 2311-3006
DOI - 10.1016/j.jcrpr.2017.03.004
Subject(s) - ifosfamide , medicine , etoposide , pulmonary blastoma , malignancy , chemotherapy , cyclophosphamide , carboplatin , vincristine , mesenchyme , radiology , lung , surgery , pathology , mesenchymal stem cell , cisplatin
Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignancy that typically affects children. In general, PPB is a dysembryonic neoplasm of thoraco-pulmonary mesenchyme, which arises from the lung, pleural surface, or both. Herein we report a 1.5-year-old patient with PPB, where complete surgery was not possible due to involvement of vital structures (i.e., lungs, aorta, and pleura) by the tumor, as demonstrated by radiographic studies. Thereafter, neoadjuvant chemotherapy with six courses of ICE/VAC (= ifosfamide, carboplatin, etoposide/vincristine, actinomycin-D, cyclophosphamide) was implemented. Thereafter, We observed a significant 60% reduction in tumor burden. The patient survived for two years, manifesting marked improvement. Ultimately, our study confirmed that PPB is a rare childhood malignancy that appears to respond promisingly to neoadjuvant chemotherapy