Extraosseous benign notochordal cell tumor presenting as bilateral pulmonary nodules

Intraosseous benign notochordal cell tumors are rare and the likely precursors of chordoma. Extraosseous benign notochordal cell tumors have been reported in only 2 patients, and both presented as solitary pulmonary nodules. Here, we report a 53-year-old woman with an incidental finding of small nodules bilaterally in the lungs. The clinician suggested the tumors were metastases; however, histologic examination of both tumors showed benign notochordal cell tumor, characterized by adipocyte-like vacuolated cells with bland nuclei and lacking an intercellular myxoid matrix. Although extraosseous benign notochordal cell tumors are extremely rare, the diagnosis should be recognized by pathologists to avoid overtreatment of patients.