- Division of Neurological Surgery, University of Sao Paulo, School of Medicine, Sao Paulo, Brazil
- Department of Pathology, University of Sao Paulo, School of Medicine, Sao Paulo, Brazil
Eberval G. Figueiredo
Division of Neurological Surgery, University of Sao Paulo, School of Medicine, Sao Paulo, Brazil
DOI:10.4103/2152-7806.85986Copyright: © 2011 Figueiredo EG. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
How to cite this article: Figueiredo EG, Tavares WM, Welling L, Rosemberg S, Teixeira MJ. Ectopic pineal chordoma. Surg Neurol Int 12-Oct-2011;2:145
How to cite this URL: Figueiredo EG, Tavares WM, Welling L, Rosemberg S, Teixeira MJ. Ectopic pineal chordoma. Surg Neurol Int 12-Oct-2011;2:145. Available from: http://sni.wpengine.com/surgicalint_articles/ectopic-pineal-chordoma/
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.
Keywords: Chordoma, metastasis, notochord, pineal region, ventriculoperitoneal shunt
Chordomas are rare aggressive tumors originating from the notochord. Primary sites include the clivus, vertebrae and sacrococcygeal region.[
An 18-year-old man presented with abdominal pain, distension and constipation. His past medical history included VP shunt and a craniotomy with a supracerebellar infratentorial access to a pineal mass that had a histological diagnosis of germinoma in April 1994. Postoperatively, he was submitted to radiotherapy (5400 rads).
Physical examination revealed stable vital signs. Heart and lung auscultation revealed no abnormalities. Abdominal exam showed a large, solid mesogastric mass associated with pain and distension. On neurological exam, the patient was alert, oriented, with Parinaud syndrome and left oculomotor nerve paresis. Abdominal ultrasonography revealed a large solid and cystic abdominal mass around the VP catheter. The tumor produced pyelocalyceal ectasia and bowel distention and was accompanied by moderate ascites. During the admission, peritoneocentesis was performed. Aspiration cytology revealed no abnormalities. Magnetic resonance imaging (MRI) showed a pineal irregular mass occupying the superior cerebellar and quadrigeminal cisterns with hyposignal on T1, medium signal on T2 and heterogeneous enhancement after gadolinium injection [
The histopathologic analysis revealed cells of various shapes, from polyhedral to prismatic, arranged in a lobular manner. Thin-walled blood vessels surrounded by a fibrous trabeculae framework with variable thickness were noticed. They were arranged in cords and sheets and exhibited an eosinophilic cytoplasm with intervening epithelial and intracellular mucin containing cells [
Chordomas are very rare tumors that arise from notochord embryonic remnants. They may be found anywhere along the axial skeleton between the skull base and coccyx, where the notochord vestiges may be encountered.[
The origin of intradural extraosseus chordoma is still debated and it is speculated that it may arise from an ecchordosis physaliphora.[
The notochord appears at nearly 3 weeks of fetal development.[
This is the second case of chordoma arising within the brain. Commins et al. reported an exceptional occurrence of a hypothalamic chordoma.[
Notwithstanding the low incidence of chordomas’ metastasis, the tumor presents a high rate of recurrence (85%) and recent publications have demonstrated a high frequency of tumor implants on the tumor surgical field.[
The current treatment for chordomas is surgical removal followed by radiotherapy (4500–5500 rads) on the tumor bed.[
The authors present their experience with an unusual occurrence of an intracerebral chordoma with a rare metastatic mechanism. The presentation and clinical history endorse the idea that intradural extraosseous chordomas are distinct from usual ecchordosis physaliphora and probably do not derive from it. We hypothesized that notochord remnants may subsist within the brain parenchyma and may occasionally undergo further transformation. Additionally, special attention should be given for the use of VP shunts in chordomas since the possibility of abdominal metastasis exists and treatment might be delayed as metastases may develop without early symptoms.
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