Ecchordosis physaliphora is a congenital benign hamartomatous lesions derived from notochord remnants. It is usually located in the retroclival prepontine region. It is due to remaining notochord cells along the axis of the spine after embryogenesis.
There has been some controversy as to whether intradural chordoma and large ecchordosis physaliphora are different entities. Some authors (Wolfe et al.) proposed the name intradural chordoma for all intradural notochord remnant lesions 8. Others (Rodriguez et al.) proposed than all intradural notochord remnant lesions should be called ecchodrosis physaliphora, until chordoma are pathologically proven to arise from the intradural compartment 9. However, they are currently considered distinct pathologies with common origin.
Contrarily to chordoma, patients with ecchordosis physaliphora are usually asymptomatic. It is found in approximately 2% of autopsies. Patients with chordoma present with brain stem or cranial nerves symptoms.
Ecchordosis physaliphora and chordoma are histologically indistinguishable, apart from an infiltrative growth in chordoma. They both have a common origin from the notochord.
CT-scan is generally not sensitive for such lesions, mainly because of posterior fossae artifcats.
Apart from the characteristic location (retroclival, prepontine & intradural), MRI findings are not specific:
- T1 : hypointense
- T2 : hyperintense
- T1 C+ : +/- enhancement (50 % in some series 1)
The differential diagnosis of retroclival intradural lesions consists mainly of 4:
- benign notochordal cell tumor (BNCT)
- skull base metastasis
- dermoid cyst
- epidermoid cyst
- arachnoid cyst
Hubert von Luschka (1820 - 1875), a german pathologist, first described the finding of pathologic ectopic notochordal tissue at the posterior clivus in 1856.
- 1. Mehnert F, Beschorner R, Küker W et-al. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol. 25 (10): 1851-5. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 2. Rotondo M, Natale M, Mirone G et-al. A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management. J. Neurol. Neurosurg. Psychiatr. 2007;78 (6): 647-9. doi:10.1136/jnnp.2006.109561 - Free text at pubmed - Pubmed citation
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- 5. Alkan O, Yildirim T, Kizilkiliç O et-al. A case of ecchordosis physaliphora presenting with an intratumoral hemorrhage. Turk Neurosurg. 2009;19 (3): 293-6. Turk Neurosurg (link) - Pubmed citation
- 6. Ciarpaglini R, Pasquini E, Mazzatenta D et-al. Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report. Neurosurgery. 2009;64 (2): E387-8. doi:10.1227/01.NEU.0000337064.57270.F0 - Pubmed citation
- 7. Srinivasan A, Goyal M, Kingstone M. Case 133: Ecchordosis physaliphora. Radiology. 2008;247 (2): 585-8. doi:10.1148/radiol.2472050101 - Pubmed citation
- 8. Wolfe JT, Scheithauer BW. "Intradural chordoma" or "giant ecchordosis physaliphora"? Report of two cases. Clin. Neuropathol. 6 (3): 98-103. - Pubmed citation
- 9. Rodriguez L, Colina J, Lopez J, Molina O, Cardozo J. Intradural prepontine growth: giant ecchordosis physaliphora or extraosseous chordoma? Neuropathology 1999;19:336–340.
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