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We report a case of peripheral primitive neuroectodermal tumor (pPNET), which belongs to the pPNET/ Ewing's sarcoma family, arising in the chest wall of a 69-year-old man. He had high levels of serum neuronspecific enolase and pro-gastrin-releasing peptide, which are believed to be useful diagnostic blood markers for small cell lung carcinoma (SCLC). Microscopically, the tumor was composed of solid nests and sheets of monotous, primitive, small round cells with a few rosettes, making it difficult to distinguish from SCLC.

Pro-GRP were within the normal limits. A chest computed tomography revealed a tumor located in left S6 (Fig. 1). A yellow, soft and peduncular polypoid tumor protruding into the left main bronchus was observed at bronchoscope (Fig. 2). She underwent left lower lobectomy with bronchoplasty and lymph node resection. The size of the tumor was 32×32×35 mm. The length of the peduncular polypoid stalk was 25 mm. Histopatholologically, the tumor showed the epithelial components (Fig. 3A) and the mesenchymal components (Fig. 3B). The tumor was diagnosed as biphasic pulmonary blastoma (stage IIB, pT3N0M0).There has been no evidence of recurrence for 30 months after operation. Here, we reported a rare case of biphasic pulmonary blastoma presenting as an endobronchial polyp with a long stalk protruding into the left lower lobe.