Ecchordosis physaliphora is a congenital benign hamartomatous lesion derived from notochord remnants, usually located in the retroclival prepontine region, but can be found anywhere from the skull base to the sacrum.
There has been some controversy as to whether intradural chordoma and large ecchordosis physaliphora are different entities. Some authors (such as Wolfe et al.) proposed the name 'intradural chordoma' for all intradural notochordal remnant lesions 8. Others (such as Rodriguez et al.) proposed that all intradural notochordal remnant lesions should be called ecchordosis physaliphora, until chordoma are pathologically proven to arise from the intradural compartment 9. However, they are currently considered distinct pathologies with common origin.
Unlike chordomas which are often symptomatic due to brainstem or cranial nerve compression, patients with ecchordosis physaliphora are usually asymptomatic. They are found in ~2% of autopsies 1.
Ecchordosis physaliphora arise from remaining notochord cells along the axis of the spine after embryogenesis. Unfortunately, ecchordosis physaliphora and chordoma are histologically indistinguishable, other than by examining the margins, the latter demonstrating infiltrative growth.
CT is generally not sensitive for such lesions, mainly because of posterior fossa artifacts and the near CSF density of the mass. The bony clival defect is however visible as a well-demarcated smoothly corticated region, without aggressive features.
Occasionally an osseous stalk is seen at the base of the lesion which is said to be pathognomonic in this context 7.
A stalk-like connection to the clivus is usually seen if high resolution images are obtained.
Apart from the characteristic location (retroclival, prepontine, and intradural), MRI findings are non-specific, with signal similar to CSF:
- T1: hypointense
- T2: hyperintense
- T1 C+ (Gd): variable, however most cases have not shown substantial enhancement 1
History and etymology
Hubert von Luschka (1820-1875), a German pathologist, first described the finding of pathologic ectopic notochordal tissue at the posterior clivus in 1856.
The differential diagnosis of retroclival intradural lesions consists mainly of 4:
- 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
- 3. Takeyama J, Hayashi T, Shirane R. Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma? Pathology. 2006;38 (6): 599-600. Pathology (link) - Pubmed citation
- 4. Adamek D, Malec M, Grabska N et-al. Ecchordosis physaliphora - a case report and a review of notochord-derived lesions. Neurol. Neurochir. Pol. 45 (2): 169-73. Neurol. Neurochir. Pol. (link) - Pubmed citation
- 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. Ng SH, Ko SF, Wan YL et-al. Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol. 1998;71 (843): 329-31. Pubmed citation