Pleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam

Pediatr Hematol Oncol. 2020 Sep 27:1-9. doi: 10.1080/08880018.2020.1818905. Online ahead of print.


Pleuropulmonary blastoma (PPB) is a rare malignant tumor in childhood cancer. This type of tumor is difficult to identify and can easily be misdiagnosed. The International PPB protocol is a complicated and aggressive protocol. It is not easily applicable to developing countries where hospitals do not have enough resources. Here we present a challanging case of a patient successfully treated in Vietnam, using limited medical resources. The patient (22 month old, male) was diagnosed with congenital cystic adenomatoid malformation in his 1st hospital admission. After 6 months of onset, the patient was diagnosed with PPB type II in the fourth hospitalization following analysis of a lung CT scan and a pathology report. After the aggressive chemotherapy regimen, the patient had two episodes of severe neutropenia and infection from which he recovered. The patient received chemotherapy and surgery treatment at our hospital, but received radiation under general anesthesia and rehabilitation therapy to improve respiration at another hospital over 600 km away. It has been 1.5 years after entering remission, and he is starting kindergarten. Lung CT scan and pathology should be analyzed to avoid missing diagnosis of PPB in patients with cystic or mixed cystic and solid lung lesions. Biopsies from cases of suspected PPB should be sent for expert pathology review. Two factors important to the successful application of the protocol are good supportive care and the multidisciplinary collaboration between medical facilities to provide proper resources during treatment. We hope to recreate more successful outcomes not only in Vietnam but also in all developing countries.

PMID:32985315 | DOI:10.1080/08880018.2020.1818905