Biphasic Pulmonary Blastomas In The Context Of Neurofibromatosis 1

Pulmonary blastomas (PB) are rare malignant lung tumors. At present, there is no standard treatment for unresectable pulmonary blastomas. Neurofibromatosis 1 is an autosomal dominant genetic disease, characterized by neural crest cell prosoplasia, causing multi-system damage. To the best of our knowledge, pulmonary blastomas in the context of neurofibromatosis have not been previously examined. A 20-year-old man with a history of neurofibromatosis 1 was diagnosed with a pulmonary blastoma (stage IIIA). Liposomal doxorubicin and ifosfamide were administered for a total of six cycles. Apatinib was given as maintenance treatment. Brain metastasis was found 9 months after presentation. Brain tumors were treated with whole brain radiation, 30 Gy/10 fractions, and gross tumor volume 51 Gy/12 fractions. Relief of neurological symptoms was obtained via radiotherapy. The patient died a little less than 11 months after presentation. Advanced pulmonary blastomas are treated with chemotherapy or alternatively with Apatinib. For brain metastases, whole brain radiation can relieve neurological symptoms and prolong survival times.