Case 14846 Retroclival Ecchordosis Physaliphora Dr Praveen .P.Wali Dr Harsha Chadaga Dr Anil .R Dr Madhukumar COLUMBIA ASIA HOSPITAL, COLUMBIA ASIA HOSPITAL, BRIGADE GATEWAY, RADIOLOGY; YESHWANTPUR 560022 BANGALORE, India; Email:
[email protected] COLUMBIA ASIA HOSPITAL Section: Neuroradiology Published: 2017, Aug. 9 Patient: 52 year(s), male
Clinical History 52 year male, doctor by profession c/o persistent headache 1year back , MRI performed outside was unremarkable except for incidental retroclival arachnoid cyst. Now comes for follow up imaging with no fresh complaints. Purpose of follow up was that being a doctor , was more anxious.
Imaging Findings A well defined 10 x 5.8 mm T2 hyperintense lesion which follows CSF signal intensity on all sequences seen in pre-pontine cistern to the left of basilar artery. Another similar lesion measuring 11.2 x 7.2 mm lesion seen to the right of midline in the pre pontine cistern. In all likelihood these two communicate in the midline. There are tiny T2 hyperintense lesions in the dorsal clivus similar in signal intensity to that of retroclival lesion. The retroclival lesion is connected to the dorsal clival lesions through a stalk.
Discussion Background:
Ectopic location of remnants of notochordal tissue in posterior wall of clivus, 1, 2was described by Lushka. Differentials considered for notochordal remnant derived mass in retro-clival location; ecchordosis physaliphora, benign notochordal cell tumors and neuroenteric-cyst (both benign) or chordomas(malignant)3, 4. Clinical Perspective: Notochordal development begins in 3rd-week of intra-uterine life and forms primitive skeleton of vertebrates5, 11 Adult counterpart of notochordal-remanant is nucleus pulposus of intervertebral disc. Occasional locations of notochordal-rests are craniospinal-axis, sacrococcygeal, dorsum of sella and clival region1, 2 Ecchordosis-physaliphora is rare benign gelatinous and hamartomatous tissue, typically situated intradurally and mostly attached to dorsal clivus through delicate pedicle.6, 7Rarely can be located in sacrococcygeal region2, 8Intracranial location is within subdural and subarachnoid space in prepontine cistern attached to dorsal clivus by pedicle and associated with bony defect9, 13, 14 Usually asymptomatic due to small size and slow growth.2Incidence~0.4-2% at autopsies.1, 2 Symptomatic Ecchordosis-physaliphora are very rare and only few cases reported, usually due to expansion and mass effect on adjacent structures and sometimes extratumoral hemorrhage.16, 17, 18 Histologic, immunohistochemistry and ultrastructural studies often ineffective in distinguishing small samples of Ecchordosis-physaliphora and chordomas1, 2 Imaging Perspective: MRI-Ecchordosis-physaliphora appears as well-circumscribed extra-axial non enhancing lesion in prepontine cistern.Hypointense on T1WI, hyperintense on T2WI.16, 19In contrast, chordomas usually enhance.11, 12, 15 Even intradural-chordomas show considerable enhancement.10 Hence, its postulated that if lesion enhances, it usually represents an extradural/intradural chordoma, while non-enhancing lesion represent an Ecchordosis-physaliphora. 12 However, T1-hyperintensity and non-enhancement points towards another benign condition i.e., neuroenteric-cyst.12, 13, 19Benign-Notochordal-Cell- Tumors are T1-hypointense, homogenously iso-hyperintense on T2-sequence.There should be no or minimal enhancement, however no extra-osseus extension of lesion should be seen.16 Presence of osseus-stalk connecting retro-clival mass with clival notochordal remnant is characteristic hallmark of Ecchordosis-physaliphora.1, 16 Other differential diagnoses include dermoid, epidermoid, arachnoid-cyst.Arachnoid-cysts are of CSF-signal on all sequences, sharp marginated homogeneous unilocular masses.Epidermoid cysts are ill-defined, located at cerebellopontine-angle cistern and reveal characteristic diffusion-restriction. Dermoids are heterogenous extra-axial masses containing fat/calcification and located in midline.15, 20, 21 CT is not advisable for diagnosis due to posterior fossa artifacts and also lesion density is almost similar to that of CSF22.However to demonstrate bony defect one can do CT, but most of times MRI does reveal bony defect as well. Conclusion:Since benign entity like Ecchordosis-physaliphora shares significant similarity histologically with malignant ones like clival-chordoma, diagnosis is challenging and this differentiation is extremely essentially as treatment differs significantly.Imaging modalities like MRI and CT-scans aid in identifying and distinguishing these lesions.
Final Diagnosis RETROCLIVAL ECCHORDOSIS PHYSALIPHORA
Differential Diagnosis List RETROCLIVAL ARACHNOID CYST, RETROCLIVAL EPIDERMOID
Figures Figure 1 Axial T2
Axial T2 - Well defined small T2 hyperintense lesions on either side of basilar artery ( red right , blue - left ) which suppress on FLAIR images ( lower row) © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts;
Axial T2 - Well defined small T2 hyperintense lesions on either side of basilar artery ( red right , blue - left ) which suppress on FLAIR images ( lower row) © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain;
Imaging Technique: MR; Procedure: Education; Special Focus: Cysts;
Axial T2 - Well defined small T2 hyperintense lesions on either side of basilar artery ( red right , blue - left ) which suppress on FLAIR images ( lower row) © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts;
Axial T2 - Well defined small T2 hyperintense lesions on either side of basilar artery ( red right , blue - left ) which suppress on FLAIR images ( lower row) © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts;
Figure 2 Axial CISS sequence
Axial CISS sequence -Thin walled retroclival T2 hyperintense lesions(yellow arrow) on either side of basilar artery (green arrow) with no mass effect. Small pedicle attaching the retro-clival lesion with the clivus seen (red arrow ) © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts;
Axial CISS sequence -Thin walled retroclival T2 hyperintense lesions(yellow arrow) on either side of basilar artery (green arrow) with no mass effect. Small pedicle attaching the retro-clival lesion with the clivus seen (red arrow )
© columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts; Figure 3 Axial CISS sequence
Axial CISS sequence - Magnified image of pedicle attaching the retroclival lesion to clivus demonstrating the bony defect ( violet arrow ). © columbia asia hospital bangalore
Area of Interest: Neuroradiology brain; Imaging Technique: MR; Procedure: Education; Special Focus: Cysts; Figure 4 Saggital reformat of CISS sequence
Saggital reformat of CISS sequence - well defined thin walled T2 hyperintense retroclival lesion ( green arrow ) with pedicle seen. © COLUMBIA ASIA HOSPITAL BANGALORE
Area of Interest: Neuroradiology brain; Imaging Technique: MR;
Procedure: Education; Special Focus: Cysts;
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a pictorial review. Journal of European Radiology S547-S602 [15] Kyriakos M, Totty GW, Lenke GL (2003) Giant vertebral notochordal rest: a lesion distinct from chordoma: discussion of an evolving concept American Journal of Surgical Pathology 27:396-406 [16] Corsi A, De Maio F, Mancini F, Ippolito E, Riminucci M, Bianco P (2008) Notochordal inclusion in the vertebral bone marrow. Journal of Bone and Mineral Research 23:572-575 [17] Boriani S, Bandiera S, Biagini R, et al (2006) Chordoma of the mobile spine: fifty years of experience Spine (Phila Pa 1976) 31:493-503 [18] Yamaguchi T, Watanabe-Ishiiwa H, Suzuki S, Igarashi Y, Ueda Y. (2005) Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors Modern Pathology 18:1005-1010 [19] Haasper C, Länger F, Rothenthal H, et al (2007) Coccydynia due to a benign notochordal cell tumor Spine 32:E394-E396. [20] Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O (2003) Comprehensive review of intracranial chordoma Radiographics 23:995-1009 [21] Bjornsson J, Wold LE, Ebersold MJ, Laws ER (1993) Chordoma of the mobile spine: a clinicopathologic analysis of 40 patients The American Journal of Cancer 71:735-740 [22] Srinivasan A(1), Goyal M, Kingstone M (2008) Ecchordosis physaliphora. Radiology 247(2):585-8
Citation Dr Praveen .P.Wali Dr Harsha Chadaga Dr Anil .R Dr Madhukumar COLUMBIA ASIA HOSPITAL, COLUMBIA ASIA HOSPITAL, BRIGADE GATEWAY, RADIOLOGY; YESHWANTPUR 560022 BANGALORE, India; Email:
[email protected] (2017, Aug. 9) Retroclival Ecchordosis Physaliphora {Online} URL: http://www.eurorad.org/case.php?id=14846
