Spontaneous Shrinking and Growing Skull Base Chordoma

Abstract

Background

Chordomas are rare slow-growing tumors occurring in the axial skeleton and can demonstrate local aggressive behavior, typically extending from the median axis, compressing surrounding tissue. Complete surgical resection and adjuvant radiotherapy are the preferred treatments. We present an unusual case of a spontaneously shrinking and growing off-midline petroclival chordoma.

Case Description

A 23-year-old woman presented with right abducens nerve palsy. Computed tomography and magnetic resonance imaging (MRI) revealed an off-midline petroclival lesion compressing the abducens nerve with characteristics of a chondrosarcoma. Preoperative MRI indicated spontaneous lesion regression, and the abducens nerve showed clinical improvement. Hence, the planned surgery was canceled. During the wait-and-scan period, abducens nerve palsy recurred. MRI confirmed lesion growth and showed an intratumoral linear structure indicative of blood. Even though preoperative MRI again demonstrated shrinkage, the lesion was surgically removed. Despite the unusual presentation, histopathological examination diagnosed a conventional chordoma. A second surgery was required to remove the residual tumor, after which the patient received high-dose photon beam therapy.

Conclusion

This article discusses the uncommon presentation and behavior of a petroclival chordoma, showing fluctuating cycles of off-midline growth and spontaneous regression. While intratumoral hemorrhage is hypothesized to explain this tumor behavior, the exact etiology needs further investigation. The case presented here emphasizes the importance of considering chordoma in the differential diagnosis despite an atypical disease course.

Keywords

skull base chordoma - chondrosarcoma - spontaneous shrinkage - hemorrhage - endoscopic - skull base surgery

^† Both authors contributed equally.

Publication History

Received: 18 February 2025

Accepted: 09 April 2025

Article published online:
09 May 2025

• References

• 1 Koutourousiou M, Snyderman CH, Fernandez-Miranda J, Gardner PA. Skull base chordomas. Otolaryngol Clin North Am 2011; 44 (05) 1155-1171
• 2 Kyriakos M, Totty WG, Lenke LG. Giant vertebral notochordal rest: a lesion distinct from chordoma: discussion of an evolving concept. Am J Surg Pathol 2003; 27 (03) 396-406
• 3 Vujovic S, Henderson S, Presneau N. et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 2006; 209 (02) 157-165
• 4 McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control 2001; 12 (01) 1-11
• 5 Crockard HA, Steel T, Plowman N. et al. A multidisciplinary team approach to skull base chordomas. J Neurosurg 2001; 95 (02) 175-183
• 6 Almefty K, Pravdenkova S, Colli BO, Al-Mefty O, Gokden M. Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer 2007; 110 (11) 2457-2467
• 7 Wu Z, Zhang J, Zhang L. et al. Prognostic factors for long-term outcome of patients with surgical resection of skull base chordomas-106 cases review in one institution. Neurosurg Rev 2010; 33 (04) 451-456
• 8 Kishimoto R, Omatsu T, Hasegawa A, Imai R, Kandatsu S, Kamada T. Imaging characteristics of metastatic chordoma. Jpn J Radiol 2012; 30 (06) 509-516
• 9 Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 1979; 72 (05) 765-776
• 10 Stacchiotti S, Casali PG, Lo Vullo S. et al. Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers. Ann Surg Oncol 2010; 17 (01) 211-219
• 11 Fagundes MA, Hug EB, Liebsch NJ, Daly W, Efird J, Munzenrider JE. Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys 1995; 33 (03) 579-584
• 12 Bjornsson J, Wold LE, Ebersold MJ, Laws ER. Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer 1993; 71 (03) 735-740
• 13 Fatani JA, Arab A, Alkhalidi H, Ajlan A. Clival simple bone cyst: rare pathological entity (case report). Surg Neurol Int 2020; 11: 427
• 14 Shkarubo A, Kuleshov A, Semenova L. et al. Surgical treatment of clival and axial bone cysts. Neurosurg 2016;73
• 15 Gunawat P, Shaikh ST, Karmarkar V, Deopujari C, Shah N. Endoscopic excision of symptomatic simple bone cyst at skull base. J Clin Diagn Res 2016; 10 (10) PD03-PD04
• 16 Damante JH, Da S Guerra EN, Ferreira Jr O. Spontaneous resolution of simple bone cysts. Dentomaxillofac Radiol 2002; 31 (03) 182-186
• 17 Wilkins RM. Unicameral bone cysts. J Am Acad Orthop Surg 2000; 8 (04) 217-224
• 18 Uysal E, Cohen MA, Abou-Al-Shaar H, Palmer C, Couldwell W. Hemorrhagic skull base chordoma presenting as chordoma apoplexy. Cureus 2021; 13 (11) e19187
• 19 Santegoeds RGC, Temel Y, Beckervordersandforth JC, Van Overbeeke JJ, Hoeberigs CM. State-of-the-art imaging in human chordoma of the skull base. Curr Radiol Rep 2018; 6 (05) 16
• 20 Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O. Comprehensive review of intracranial chordoma. Radiographics 2003; 23 (04) 995-1009
• 21 Meyers SP, Hirsch Jr WL, Curtin HD, Barnes L, Sekhar LN, Sen C. Chordomas of the skull base: MR features. AJNR Am J Neuroradiol 1992; 13 (06) 1627-1636
• 22 Pamir MN, Özduman K. Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol 2006; 58 (03) 461-470
• 23 Vuong HG, Dunn IF. Chondrosarcoma and chordoma of the skull base and spine: implication of tumor location on patient survival. World Neurosurg 2022; 162: e635-e639
• 24 Brown RV, Sage MR, Brophy BP. CT and MR findings in patients with chordomas of the petrous apex. AJNR Am J Neuroradiol 1990; 11 (01) 121-124
• 25 Bander ED, Kocharian G, Liechty B, Tsiouris AJ, Schwartz TH. Spontaneous regression of a clival chordoma. Case report. Acta Neurochir (Wien) 2020; 162 (02) 433-436
• 26 Vellutini EAS, Stamm AEC, de Oliveira MF. Spontaneous regression of a clival chordoma: an unusual case report. J Neurol Surg A Cent Eur Neurosurg 2022; 83 (04) 388-390
• 27 Radl R, Leithner A, Kastner N, Bodo K, Nader A, Windhager R. Disappearance of a cervical spine chordoma after nonoperative treatment. A case report. J Bone Joint Surg Am 2005; 87 (08) 1822-1827
• 28 González TMC, Lam SW, van der Wal R, Navas A, van Langevelde K. Sacrococcygeal chordoma with spontaneous regression due to a large hemorrhagic component. Skeletal Radiol 2025; 54 (01) 159-163
• 29 Takagi K, Nagano A, Tsugita M, Nishimoto Y, Miyazaki T, Akiyama H. Spontaneous regression of multiple pulmonary metastasis of sacral chordoma: a case report. JBJS Case Connect 2021; 11 (04) e21
• 30 Lee HJ, Kalnin AJ, Holodny AI, Schulder M, Grigorian A, Sharer LR. Hemorrhagic chondroid chordoma mimicking pituitary apoplexy. Neuroradiology 1998; 40 (11) 720-723
• 31 Vilela MD, Pedrosa HAS, Dias Filho MA. A hemorrhagic clival chordoma with a long progression-free survival. World Neurosurg 2017; 105: 1042.e1-1042.e4
• 32 Tauziéde-Espariat A, Bresson D, Polivka M. et al. Prognostic and therapeutic markers in chordomas: a study of 287 tumors. J Neuropathol Exp Neurol 2016; 75 (02) 111-120
• 33 Li X, Ji Z, Ma Y, Qiu X, Fan Q, Ma B. Expression of hypoxia-inducible factor-1α, vascular endothelial growth factor and matrix metalloproteinase-2 in sacral chordomas. Oncol Lett 2012; 3 (06) 1268-1274
• 34 Cheney MD, Chen YL, Lim R. et al. [18F]-Fluoromisonidazole positron emission tomography/computed tomography visualization of tumor hypoxia in patients with chordoma of the mobile and sacrococcygeal spine. Int J Radiat Oncol Biol Phys 2014; 90 (05) 1030-1036
• 35 Mammar H, Kerrou K, Nataf V. et al. Positron emission tomography/computed tomography imaging of residual skull base chordoma before radiotherapy using fluoromisonidazole and fluorodeoxyglucose: potential consequences for dose painting. Int J Radiat Oncol Biol Phys 2012; 84 (03) 681-687