First Case of Metastasis of a Chordoma in the Abdominal Wall

Abstract

Chordomas are rare malignant primary bone tumors of ectodermal origin, that arise from notochordal remnants of the developing spine, which most often occur in the sacral area. It is an aggressive, locally invasive neoplasm, which carries a poor prognosis. Chordomas’ metastatic incidence ranges from 5 to 40%. It is generally believed that metastases without local recurrence of primary neoplasm are extremely rare. En-bloc excision with wide margins and postoperative radiation therapy remains the only curative treatment of primary chordoma. Its metastases should also be resected surgically. We present the first case of metastasis of a previously surgically treated primary sacrococcygeal chordoma. Metastatic lesions developed in the abdominal wall of a 42-year-old man without local recurrence and were discovered incidentally. We also describe the surgical management of this case. This phenomenon has not yet been described. We demonstrate that, incidentally discovered chordoma metastasis in the abdominal wall can occur and present a diagnostic challenge. This case report focuses on the importance of follow up with magnetic resonance imaging or computerized tomography scan after resection of sacral chordoma paying attention to the entire abdomen. After this experience we feel that surgery could be considered a valid option for the treatment of chordoma metastasis in this scenario.