Classic biphasic pulmonary blastoma: a case report and review of the literature

Abstract

Pulmonary blastoma (PB) is a very rare malignant lung tumor consisting of classic biphasic PB, well-differentiated fetal adenocarcinoma, and pleuropulmonary blastoma. We herein present an unusual case involving a patient with classic biphasic PB who underwent right upper lobe resection and subsequent treatment. No standard treatment guidelines are available for PB because of its rarity. Our patient received nedaplatin plus paclitaxel as adjuvant chemotherapy. After disease recurrence, the patient received two cycles of etoposide-cisplatin and six cycles of pemetrexed, bevacizumab, and carboplatin. Because of severe adverse effects of the chemotherapy, the patient was finally administered anlotinib, a new oral multikinase inhibitor. Both the tumor size and the serum tumor marker concentration decreased. In conclusion, surgical excision is the treatment of choice for PB. Chemotherapy in the present case resulted in PB activity that was consistent with the literature. Targeted therapies including antiangiogenic agents should be considered as a new treatment option for this rare disease.

Keywords: Pulmonary blastoma; alpha-fetoprotein; anlotinib; chemotherapy; surgical excision; targeted therapy.

Figure 1.

Contrast-enhanced chest computed tomography showed a mass of approximately 5 cm in diameter in the right upper lobe complicating a pulmonary infection.

Figure 2.

Bronchoscopy showed that the tumor was occluding the right upper apical bronchus.

Figure 3.

Histopathological examination demonstrated mixed epithelial and mesenchymal malignancies. The epithelial component formed glands composed of columnar cells with clear cytoplasm, whereas the mesenchymal component consisted of round and spindle cells with variable degrees of nuclear atypia. (a) Hematoxylin and eosin, 5×. (b) Hematoxylin and eosin, 20×.

Figure 4.

The patient’s clinical course showed a decreased alpha-fetoprotein (AFP) concentration after lobe resection and adjuvant chemotherapy with nedaplatin-paclitaxel. After recurrence 2 years later, the AFP concentration rapidly increased. After two cycles of etoposide-cisplatin and six cycles of pemetrexed plus bevacizumab plus carboplatin, the AFP concentration declined again. After the administration of anlotinib, the AFP concentration remained stable and low.

Figure 5.

Positron emission tomography scan with liver metastasis.