Chordoma is a slowly growing malignant tumor of uncertain histogenesis thought to be derived from the embryonic notochord. It has a predilection for the cervical and lumbosacral spine. Although rarely, chordoma has been described to occur in the mediastinum as discrete masses with no apparent relationship with the adjacent vertebral bones (Ahrendt and Wesselhoeft 1992; Suster and Moran 1995). Presenting symptoms are related to local compression or invasion, mostly difficulty swallowing, chest pain, and shortness of breath.
Not applicable due to the limited number of reported cases.
Most published cases have been seen in late adulthood. Exceptional cases are seen in pediatric subjects.
No sex predilection.
Mediastinal chordomas arise in the posterior sector of the mediastinum.
Complete surgical excision is the treatment of choice for resectable tumor. Postoperative radiotherapy has been administered...