Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Clinical Neuroradiology
Published in: Clinical Neuroradiology 4/2014

01-12-2014 | Review Article

Skull Base Chondroid Chordoma: Atypical Case Manifesting as Intratumoral Hemorrhage and Literature Review

Authors: Satoshi Tsutsumi, MD, Chihiro Akiba, Takamoto Suzuki, Hajime Nakanishi, Hiroshi Izumi, Yukimasa Yasumoto, Masanori Ito

Published in: Clinical Neuroradiology | Issue 4/2014

Login to get access

Abstract

Objective

Chondroid chordoma (CC) is a rare but commonest subtype of chordoma with little reported clinical information. The present study summarizes and updates present knowledge of CC.

Methods

Literature search for demographic data and clinical appearance of cranial CCs except for those entirely confined to the sinonasal region.

Results

A total of 48 English language papers published from 1968–2013 were retrieved describing 132 CCs as skull base tumors. The male-to-female ratio was 1:1. The mean age at diagnosis was 43 years, predisposing to the third to fifth decades of life. The clival (34 %) and spheno-occipital (29 %) regions were the most frequent sites of origin followed by the sellar (12 %) and sphenoid (5 %) regions. Intratumoral calcification and bony erosion were identified as the characteristic neuroimaging findings. Surgical resection by the transcranial, transsphenoidal, transnasal, transpharyngeal, or transpalatal route with or without adjuvant radiotherapy was the main treatment option. The initial treatment outcome was satisfactory in 82 % of cases with considerably better prognosis compared with typical chordomas.

Conclusion

CC is a distinct entity to be discriminated from the typical type of chordoma. There are no distinguishing features on magnetic resonance imaging between CC and typical chordoma. Intratumoral calcification and concurrent bony erosion on neuroimaging should suggest the possibility of CC. Extensive surgical resection and adjuvant radiotherapy can achieve satisfactory outcome.
Literature
1.
Okamoto K, Ito J, Tokiguchi S, Furusawa T. High-resolution CT findings in the development of the sphenooccipital synchondrosis. AJNR Am J Neuroradiol. 1996;17:117–20.PubMed
2.
Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors at the skull base. Cancer. 1973;32:410–20.PubMedCrossRef
3.
Jeffrey PB, Biava CG, Davis RL. Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol. 1995;103:271–9.PubMed
4.
Falconer MA, Bailey IC, Duchen LW. Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg. 1968;29:261–75.PubMedCrossRef
5.
Richter HJ Jr., Batsakis JG, Boles R. Chordomas: nasopharyngeal presentation and atypical long survival. Ann Otol Rhinol Laryngol. 1975;84:327–32.PubMedCrossRef
6.
Agrawal A. Chondroid chordoma of petrous temporal bone with extensive recurrence and pulmonary metastases. J Cancer Res Ther. 2008;4:91–2.PubMedCrossRef
7.
Akai T, Sonobe M, Sugita K, Suzuki K, Oyama K, Takahashi S. Intracranial chondroid chordoma. J Clin Neurosci. 1997;4:244–7.PubMedCrossRef
8.
Bastiaensen LA, Leyten AC, Tjan TG, Misere JF. Chondroid chordoma of the base of the skull: orbital and other neuro-ophthalmological symptoms. Doc Ophthalmol. 1983;55:5–15.PubMedCrossRef
9.
Brooks JJ, LiVolsi VA, Trojanowski JQ. Does chondroid chordoma exist? Acta Neuropathol. 1987;72:229–35.PubMedCrossRef
10.
11.
Drummond DS, Wenzel DM, Kudryk W. Spheno-occipital chondroid chordoma. J Otolaryngol. 1997;26:197–200.PubMed
12.
Harbour JW, Lawton MT, Criscuolo GR, Holliday MJ, Mattox DE, Long DM. Clivus chordoma: a report of 12 recent cases and review of the literature. Skull Base Surg. 1991;1:200–6.PubMedCentralPubMedCrossRef
13.
Hasegawa M, Nishijima W, Watanabe I, Nasu M, Kamiyama R. Primary chondroid chordoma arising from the base of the temporal bone. A 10-year post-operative follow-up. J Laryngol Otol. 1985;99:485–9.PubMedCrossRef
14.
Hirosawa RM, Santos AB, França MM, Fabris VE, Castro AV, Zanini MA, et al. Intrasellar chondroid chordoma: a case report. ISRN Endocrinol. 2011;2011:259392.PubMedCentralPubMed
15.
Ishida T, Dorfman HD. Chondroid chordoma versus low-grade chondrosarcoma of the base of the skull: can immunohistochemistry resolve the controversy? J Neurooncol. 1994;18:199–206.PubMedCrossRef
16.
Iwasaki S, Ito K, Takai Y, Morita A, Murofushi T. Chondroid chordoma at the jugular foramen causing retrolabyrinthine lesions in both the cochlear and vestibular branches of the eighth cranial nerve. Ann Otol Rhinol Laryngol. 2004;113:82–6.PubMedCrossRef
17.
Jiagang L, Yanhui L, Xueying S, Qing M. Intradural suprasellar chondroid chordoma. J Clin Neurosci. 2010;17:402–3.PubMedCrossRef
18.
Kaufman BA, Francel PC, Roberts RL, Argemand E, Park TS, Dehner LP. Chondroid chordoma of the lateral skull base. Pediatr Neurosurg. 1995;23:159–65.PubMedCrossRef
19.
Koshiyama H, Sakamoto M, Fujiwara K, Kim YC, Teraura T, Koh T. Chondroid chordoma presenting with hypopituitarism. Intern Med. 1992;31:1366–9.PubMedCrossRef
20.
Lee HJ, Kalnin AJ, Holodny AI, Schlder M, Grigorian A, Sharer LR. Hemorrhagic chondroid chordoma mimicking pituitary apoplexy. Neuroradiology. 1998;40:720–3.PubMedCrossRef
21.
22.
Komatsu M, Komatsu F, Tsugu H, Oshiro S, Nabeshima K, Inoue T. Endoscopic management of a lower clival chondroid chordoma: case report. Turk Neurosurg. 2012;22:123–6.
23.
Mohanty PP, Pasricha R, Datta NR, Jain M. Primary chondroid chordoma of the petrous part of the temporal bone. Clin Oncol (R Coll Radiol). 2003;15:365–6.CrossRef
24.
Moriki T, Takahashi T, Wada M, Ueda S, Ichien M, Miyazaki E. Chondroid chordoma: fine-needle aspiration cytology with histopathological, immunohistochemical, and ultrastructural study of two cases. Diagn Cytopathol. 1999;21:335–9.PubMedCrossRef
25.
Nabeshima K, Sato S, Sameshima T, Goya T, Ohno A, Hinoura Y, et al. Intraoperative squash and touch cytology of chondroid chordoma of the skull base. Report of a case with immunocytochemical and immunohistochemical studies. Acta Cytol. 1997;41:913–8.PubMedCrossRef
26.
Nguyen GK, Johnson ES, Mielke BW. Chondroid chordoma of the skull base diagnosed by squash-smear technique. Diagn Cytopathol. 1985;1:161–3.PubMedCrossRef
27.
Nishigaya K, Kaneko M, Ohashi Y, Nukui H. Intradural retroclival chordoma without bone involvement: no tumor regrowth 5 years after operation. Case report. J Neurosurg. 1998;88:764–8.PubMedCrossRef
28.
Persson S, Kindblom LG, Angervall L. Classical and chondroid chordoma. A light-microscopic, histochemical, ultrastructural and immunohistochemical analysis of the various cell types. Pathol Res Pract. 1991;187:828–38.PubMedCrossRef
29.
Rossiello R, Ferrara G, Varricchio A, Baldi A, Motta S, Motta G. Chondroid chordoma of the lateral skull base. ORL J Otorhinolaryngol Relat Spec. 2001;63:114–8.PubMedCrossRef
30.
Rupa V, Rajshekhar V, Bhanu TS, Chandi SM. Primary chondroid chordoma of the base of the petrous temporal bone. J Laryngol Otol. 1989;103:771–3.PubMedCrossRef
31.
Sagit M, Kuran G, Saka C, Akin I, Hucumenoglu S, Kacar A. Endonasal endoscopic approach for the resection of chondroid chordoma with skull base involvement. J Craniofac Surg. 2011;22:1720–1.PubMedCrossRef
32.
Salisbury JR. Demonstration of cytokeratins and an epithelial membrane antigen in chondroid chordoma. J Pathol. 1987;153:37–40.PubMedCrossRef
33.
Shinde SV, Monipanda K. Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med. 2005;51:220–2.PubMed
34.
Shintaku M, Maeno K, Okabe H. Chondroid chordoma of the skull base: immunohistochemical and ultrastructural study of two cases with special reference to microtubules within rough-surfaced endoplasmic reticulum. Med Mol Morphol. 2010;43:241–5.PubMedCrossRef
35.
Shotton JC, Kuhwoede R, Fisch U. Mesenchymal tumors of the skull base with particular reference to surgical management and outcome. Skull Base Surg. 1992;2:112–7.PubMedCentralPubMedCrossRef
36.
Spoden JE, Bumsted RM, Warner ED. Chondroid chordoma. Case report and literature review. Ann Otol Rhinol Laryngol. 1980;89:279–85.PubMedCrossRef
37.
38.
Takenaka K, Nishimura Y, Andoh T, Sakai N, Yamada H, Shimokawa K. Delayed postcontrast CT and MR imaging of chondroid chordoma—case report. Neurol Med Chir (Tokyo). 1992;32:28–31.
39.
Uggowitzer MM, Kugler C, Groell R, Lindbichler F, Radner H, Sutter B, et al. Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology. 1999;41:504–7.PubMedCrossRef
40.
Valderrama E, Kahn LB, Lipper S, Marc J. Chondroid chordoma. Electron-microscopic study of two cases. Am J Surg Pathol. 1983;7:625–32.PubMedCrossRef
41.
Walker WP, Landas SK, Bromley CM, Sturm MT. Immunohistochemical distinction of classic and chondroid chordomas. Mod Pathol. 1991;4:661–5.PubMed
42.
Watson D, Smith DR. Chondroid chordoma: case report and literature review. Indiana Med. 1991;84:18–21.PubMed
43.
Wenig BL, Sciubba JJ, Goldstein MN, Abramson AL. Chondroid chordoma of the skull base: a better prognosis based on histologic criteria. Otolaryngol Head Neck Surg. 1985;93:559–63.PubMed
44.
Wojno KJ, Hruban RH, Garin-Chesa P, Huvos AG. Chondroid chordomas and low-grade chondrosarcomas of the craniospinal axis. An immunohistochemical analysis of 17 cases. Am J Surg Pathol. 1992;16:1144–52.PubMedCrossRef
45.
Zemmoura I, Ben Ismail M, Travers N, Jan M, François P. Maxillary surgical seeding of a clival chordoma. Br J Neurosurg. 2012;26:102–3.PubMedCrossRef
46.
Brooks JJ, Trojanowski JQ, LiVolsi VA. Chondroid chordoma: a low grade chondrosarcoma and its differential diagnosis. Curr Top Pathol. 1989;80:165–81.PubMedCrossRef
47.
Jian BJ, Bloch OG, Yang I, Han SJ, Aranda D, Tihan T, et al. Adjuvant radiation therapy and chondroid chordoma subtype are associated with a lower tumor recurrence rate of cranial chordoma. J Neurooncol. 2010;98:101–8.PubMedCrossRef
48.
Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ. Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer. 1993;72:2943–9.PubMedCrossRef
49.
Rosenberg AE, Brown GA, Bhan AK, Lee JM. Chondroid chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol. 1994;101:36–41.PubMed
50.
Sorimachi T, Sasaki O, Nakazato S, Koike T, Shibuya H. Myxoid chondrosarcoma in the pineal region. J Neurosurg. 2008;109:904–7.PubMedCrossRef
51.
Krisht KM, Altay T, Couldwell WT. Myxoid meningioma: a rare metaplastic meningioma variant in a patient presenting with intratumoral hemorrhage. J Neurosurg. 2012;116:861–5.PubMedCrossRef
52.
Mangubat EZ, Byrne RW. Major intratumoral hemorrhage of a petroclival atypical meningioma: case report and review of literature. Skull Base. 2010;20:469–74.PubMedCentralPubMedCrossRef
53.
Asari S, Tsuchida S, Fujiwara A, Yabuno N, Furuta T, Ohmoto T. Trigeminal neurinoma presenting with intratumoral hemorrhage: report of two cases. Clin Neurol Neurosurg. 1992;94:219–24.PubMedCrossRef
54.
Kubota T, Hayashi M, Yamamoto S. Subarachnoid hemorrhage due to trigeminal neurinoma. Surg Neurol. 1981;16:157–60.PubMedCrossRef
55.
Lieu AS, Howng SL. Trigeminal neurinoma presenting as intratumoral hemorrhage—case report. Kaohsiung J Med Sci. 1998;14:181–5.PubMed
56.
Majoie CB, Hulsmans FJ, Castelijns JA, Sie LH, Walter A, Valk J, et al. Primary nerve-sheath tumours of the trigeminal nerve: clinical and MRI findings. Neuroradiology. 1999;41:100–8.PubMedCrossRef
57.
Ooi GC, Peh WC, Fung CF. Case report: Acute haemorrhagic presentation of trigeminal neuroma. Br J Radiol. 1996;69:363–5.PubMedCrossRef
58.
Sastri SB, Dwarakanath S, Chandramouli BA. Trigeminal schwannoma with intratumoral hemorrhage: report of a case and review of literature. Neurol India. 2011;59:773–5.PubMedCrossRef
59.
Shimabukuro H, Masuzawa T, Miyagi K, Sato F. Trigeminal neurinoma revealed by intratumoral hemorrhage following a minor head injury. Surg Neurol. 1983;19:346–50.PubMedCrossRef
60.
Harada K, Nakahata I, Hayashi J, Tanaka M, Akiyama Y, Iwamuro Y, et al. Chondrosarcoma of the clivus presenting with intratumoral hemorrhage: case report. J Neurooncol. 2006;77:221–3.PubMedCrossRef
61.
Kitai R, Yoshida K, Kubota T, Sato K, Handa Y, Kasahara K, et al. Clival chordoma manifesting as nasal bleeding. A case report. Neuroradiology. 2005;47:368–71.PubMedCrossRef
62.
Nakau R, Kamiyama H, Kazumata K, Andou M. Subarachnoid hemorrhage associated with clival chordoma—case report. Neurol Med Chir (Tokyo). 2003;43:605–7.
63.
Uda T, Ohata K, Takami T, Hara M. An intradural skull base chordoma presenting with acute intratumoral hemorrhage. Neurol India. 2006;54:306–7.PubMedCrossRef
Metadata
Title
Skull Base Chondroid Chordoma: Atypical Case Manifesting as Intratumoral Hemorrhage and Literature Review
Authors
Satoshi Tsutsumi, MD
Chihiro Akiba
Takamoto Suzuki
Hajime Nakanishi
Hiroshi Izumi
Yukimasa Yasumoto
Masanori Ito
Publication date
01-12-2014
Publisher
Springer Berlin Heidelberg
Published in
Clinical Neuroradiology / Issue 4/2014
Print ISSN: 1869-1439
Electronic ISSN: 1869-1447
DOI
https://doi.org/10.1007/s00062-014-0321-7

Other articles of this Issue 4/2014

Clinical Neuroradiology 4/2014 Go to the issue

Clinical Case

Freiburg Neuropathology Case Conference: Periorbital Bone Lesion Causing Proptosis in a 31-Year-Old Patient

Original Article

A Statistical Model for Intervertebral Disc Degeneration: Determination of the Optimal T2 Cut-Off Values

Original Article

Temporal Evolution of Intraparenchymal Hyperdensity After Intra-arterial Therapy in Patients with Ischemic Stroke: Optimal Discrimination Between Hemorrhage and Iodinated Contrast

Original Article

Correlation of Diffusion MRI Findings with Lesion Progression in Patients with Traumatic Intracerebral Hemorrhage

Information

Societies’ Communications

Original Article

Definition of Ktrans and FA Thresholds for Better Assessment of Experimental Glioma Using High-field MRI: A Feasibility Study