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Ecchordosis physaliphora

MedGen UID:
473001
•Concept ID:
C0266493
•
Congenital Abnormality
Synonyms: Ecchordosis Physaliphora; Heterotopic notochordal tissue
SNOMED CT: Ecchordosis physaliphora (69664005); Heterotopic notochordal tissue (69664005)

Definition

A very rare, slow growing, usually asymptomatic hamartomatous lesion that arises from ectopic notochordal tissue. Morphologically it is characterized by the presence of typical physaliphorous cells in a myxoid background. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEcchordosis physaliphora

Recent clinical studies

Etiology

Evaluation of Long-Term Follow-Up in Ecchordosis Physaliphora versus Chordoma.
Stuebe CM, Rindler RS, Laack N, Carr CM, Choby G, Inwards CY, Van Gompel JJ
World Neurosurg 2023 Jun;174:157-168. Epub 2023 Mar 9 doi: 10.1016/j.wneu.2023.03.016. PMID: 36898627
Unrecognized notochordal lesions as a likely cause of idiopathic clival cerebrospinal fluid leaks.
Hasegawa H, Van Gompel JJ, Choby G, Raghunathan A, Little JT, Atkinson JL
Clin Neurol Neurosurg 2023 Jan;224:107562. Epub 2022 Dec 17 doi: 10.1016/j.clineuro.2022.107562. PMID: 36549221
Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.
Veiceschi P, Arosio AD, Agosti E, Bignami M, Pistochini A, Cerati M, Castelnuovo P, Locatelli D
Acta Neurochir (Wien) 2021 Sep;163(9):2475-2486. Epub 2021 Apr 26 doi: 10.1007/s00701-021-04857-5. PMID: 33900480
Ecchordosis physaliphora: typical and atypical radiologic features.
Park HH, Lee KS, Ahn SJ, Suh SH, Hong CK
Neurosurg Rev 2017 Jan;40(1):87-94. Epub 2016 May 21 doi: 10.1007/s10143-016-0753-4. PMID: 27206421
Ecchordosis physaliphora and its variants: proposed new classification based on high-resolution fast MR imaging employing steady-state acquisition.
Chihara C, Korogi Y, Kakeda S, Nishimura J, Murakami Y, Moriya J, Ohnari N
Eur Radiol 2013 Oct;23(10):2854-60. Epub 2013 Aug 1 doi: 10.1007/s00330-013-2888-9. PMID: 23903994

Diagnosis

Skull Base Tumors.
Forst DA, Jones PS
Continuum (Minneap Minn) 2023 Dec 1;29(6):1752-1778. doi: 10.1212/CON.0000000000001361. PMID: 38085897
Ecchordosis Physaliphora: Does It Even Exist?
Stevens AR, Branstetter BF 4th, Gardner P, Pearce TM, Zenonos GA, Arani K
AJNR Am J Neuroradiol 2023 Aug;44(8):889-893. Epub 2023 Jul 13 doi: 10.3174/ajnr.A7932. PMID: 37442592Free PMC Article
Endoscopic resection of a giant intradural retroclival ecchordosis physaliphora: surgical technique and literature review.
Choudhri O, Feroze A, Hwang P, Vogel H, Ajlan A, Harsh G 4th
World Neurosurg 2014 Nov;82(5):912.e21-6. Epub 2014 Jun 14 doi: 10.1016/j.wneu.2014.06.019. PMID: 24937599
Ecchordosis physaliphora - a case report and a review of notochord-derived lesions.
Adamek D, Malec M, Grabska N, Krygowska-Wajs A, Gałązka K
Neurol Neurochir Pol 2011 Mar-Apr;45(2):169-73. doi: 10.1016/s0028-3843(14)60029-3. PMID: 21574122
Retroclival ecchordosis physaliphora: MR imaging and review of the literature.
Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T
AJNR Am J Neuroradiol 2004 Nov-Dec;25(10):1851-5. PMID: 15569763Free PMC Article

Therapy

Ecchordosis physaliphora presenting with abducens nerve palsy.
Ahn SS, Han J
J AAPOS 2016 Jun;20(3):266-8. Epub 2016 Apr 7 doi: 10.1016/j.jaapos.2016.01.010. PMID: 27060668
Ecchordosis Physaliphora: Evaluation with Precontrast and Contrast-Enhanced Fast Imaging Employing Steady-State Acquisition MR Imaging Based on Proposed New Classification.
Özgür A, Esen K, Kara E, Yencilek E, Vayisoğlu Y, Kara T, Yıldız A
Clin Neuroradiol 2016 Sep;26(3):347-53. Epub 2014 Dec 10 doi: 10.1007/s00062-014-0361-z. PMID: 25491776
A case of ecchordosis physaliphora presenting with an intratumoral hemorrhage.
Alkan O, Yildirim T, Kizilkiliç O, Tan M, Cekinmez M
Turk Neurosurg 2009 Jul;19(3):293-6. PMID: 19621298

Prognosis

Evaluation of Long-Term Follow-Up in Ecchordosis Physaliphora versus Chordoma.
Stuebe CM, Rindler RS, Laack N, Carr CM, Choby G, Inwards CY, Van Gompel JJ
World Neurosurg 2023 Jun;174:157-168. Epub 2023 Mar 9 doi: 10.1016/j.wneu.2023.03.016. PMID: 36898627
Ecchordosis physaliphora: typical and atypical radiologic features.
Park HH, Lee KS, Ahn SJ, Suh SH, Hong CK
Neurosurg Rev 2017 Jan;40(1):87-94. Epub 2016 May 21 doi: 10.1007/s10143-016-0753-4. PMID: 27206421
Intraosseous benign notochordal cell tumor.
Amer HZ, Hameed M
Arch Pathol Lab Med 2010 Feb;134(2):283-8. doi: 10.5858/134.2.283. PMID: 20121620
Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report.
Ciarpaglini R, Pasquini E, Mazzatenta D, Ambrosini-Spaltro A, Sciarretta V, Frank G
Neurosurgery 2009 Feb;64(2):E387-8; discussion E388. doi: 10.1227/01.NEU.0000337064.57270.F0. PMID: 19190444
A rare symptomatic presentation of ecchordosis physaliphora and unique endoscope-assisted surgical management.
Cha ST, Jarrahy R, Yong WH, Eby T, Shahinian HK
Minim Invasive Neurosurg 2002 Mar;45(1):36-40. doi: 10.1055/s-2002-23584. PMID: 11932823

Clinical prediction guides

Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.
Veiceschi P, Arosio AD, Agosti E, Bignami M, Pistochini A, Cerati M, Castelnuovo P, Locatelli D
Acta Neurochir (Wien) 2021 Sep;163(9):2475-2486. Epub 2021 Apr 26 doi: 10.1007/s00701-021-04857-5. PMID: 33900480
Ecchordosis physaliphora: typical and atypical radiologic features.
Park HH, Lee KS, Ahn SJ, Suh SH, Hong CK
Neurosurg Rev 2017 Jan;40(1):87-94. Epub 2016 May 21 doi: 10.1007/s10143-016-0753-4. PMID: 27206421
Ecchordosis Physaliphora: Evaluation with Precontrast and Contrast-Enhanced Fast Imaging Employing Steady-State Acquisition MR Imaging Based on Proposed New Classification.
Özgür A, Esen K, Kara E, Yencilek E, Vayisoğlu Y, Kara T, Yıldız A
Clin Neuroradiol 2016 Sep;26(3):347-53. Epub 2014 Dec 10 doi: 10.1007/s00062-014-0361-z. PMID: 25491776
Benign notochordal cell tumors of the spine: natural history of 8 patients with histologically confirmed lesions.
Iorgulescu JB, Laufer I, Hameed M, Boland P, Yamada Y, Lis E, Bilsky M
Neurosurgery 2013 Sep;73(3):411-6. doi: 10.1227/01.neu.0000431476.94783.c6. PMID: 23719057
Retroclival ecchordosis physaliphora: MR imaging and review of the literature.
Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T
AJNR Am J Neuroradiol 2004 Nov-Dec;25(10):1851-5. PMID: 15569763Free PMC Article

Recent systematic reviews

Evaluation of Long-Term Follow-Up in Ecchordosis Physaliphora versus Chordoma.
Stuebe CM, Rindler RS, Laack N, Carr CM, Choby G, Inwards CY, Van Gompel JJ
World Neurosurg 2023 Jun;174:157-168. Epub 2023 Mar 9 doi: 10.1016/j.wneu.2023.03.016. PMID: 36898627
Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.
Veiceschi P, Arosio AD, Agosti E, Bignami M, Pistochini A, Cerati M, Castelnuovo P, Locatelli D
Acta Neurochir (Wien) 2021 Sep;163(9):2475-2486. Epub 2021 Apr 26 doi: 10.1007/s00701-021-04857-5. PMID: 33900480
Retroclival ecchordosis physaliphora: MR imaging and review of the literature.
Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T
AJNR Am J Neuroradiol 2004 Nov-Dec;25(10):1851-5. PMID: 15569763Free PMC Article

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