title: Ectopic pineal chordoma.
abstract: BACKGROUND
  Chordomas are rare tumors that arise from the remnants of embryonic notochord anywhere along the neuroaxis. Even though they may occur in an extraosseous intradural location, the most common sites include the sacrococcygeal and clivus regions. The authors report a unique presentation encompassing the pineal region with metastasis to the peritoneum after a ventriculoperitoneal (VP) shunt procedure and review the current knowledge about their pathophysiology and management. The presentation and clinical history endorse the idea that intradural extraosseous chordomas may be distinct from ecchordosis physaliphora and probably do not derive from it.
  
  
  METHODS
  An 18-year-old male with previous history of VP shunt presented to the emergency room with pain and abdominal distension. Computed tomography (CT) scans revealed a mass in the pineal region and in the abdominal cavity. Histopathologic exams showed chordoma in both abdominal and cranial samples. The patient died due to systemic complications.
  
  
  CONCLUSIONS
  The authors hypothesized that notochord remnants may subsist within the brain and occasionally may generate a neoplastic lesion.
authors: Division of Neurological Surgery, University of Sao Paulo, School of Medicine, Sao Paulo, Brazil.
authors: Eberval G
authors: EG
authors: Figueiredo
authors: Wagner M
authors: WM
authors: Tavares
authors: Leonardo
authors: L
authors: Welling
authors: Sergio
authors: S
authors: Rosemberg
authors: Manoel Jacobsen
authors: MJ
authors: Teixeira