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.Case Description: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.Conclusion:The authors hypothesized that notochord remnants may subsist within the brain and occasionally may generate a neoplastic lesion.

Chordomas

“…23 The last way of seeding is along a ventriculoperitoneal shunt; one case of pineal CH treated by radiotherapy and ventricular shunt was diagnosed after a seeding in the abdomen. 55 …”

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

“…It was a pineal chordoma with abdominal metastasis, due to previous ventriculoperitoneal shunt placement. The origin of such intraparenchymal tumours is not clear and it has been suggested that they arise from ecchordosis physaliphora (EP) or directly from notochord remnants that subsist within brain tissue [8,10]. The cases described suggest ectopic notochordal remnants may be located in different areas, even away from the neuroaxis.…”

“…The first case of an intracerebral hypothalamic chordoma was reported by Commins et al in 1994 [8]. The second case has been recently described by Figueiredo et al [10]. It was a pineal chordoma with abdominal metastasis, due to previous ventriculoperitoneal shunt placement.…”

“…The majority of these lesions were located in the prepontine space. Intradural extraosseous chordomas have occasionally been found within the brain, in hypothalamic and pineal regions [8,10]. Intradural chordomas are believed to be less aggressive and have a better prognosis for patients.…”

Reprint of “Chordoma in children: Case-report and review of literature”

“…15,16 Interestingly, multiple reports concern unusual presentations in youngsters, such as: extra clival cranial, intra cerebral, mediastinal, gluteal, etc. [17][18][19][20][21][22][23][24][25][26][27][28][29] Symptoms at presentation depend on the tumor site, and are not specific to children. If they are IC, cranial nerve palsies are seen in 60% or so.…”