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Acta Neurologica Belgica

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14.10.2021 | Review article

Clinical management of pediatric chordomas: a comprehensive review

verfasst von: Taylor Reardon, Caleb Marsh, Preston Rippe, Donatas Ruzys, Benjamin Ayres, David Cline, Brian Fiani

Erschienen in: Acta Neurologica Belgica | Ausgabe 6/2021

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Abstract

Chordomas are malignant tumors derived from remnants of the notochord. These are extremely rare in pediatric patients, accounting for approximately 5% of all chordomas, with most lesions occupying the cranium. Chordomas also can occupy all levels of the spine, demonstrating a broad spectrum of neurologic presentation. Optimal treatment aims for gross total resection with accompanying radiation therapy to prevent recurrence. Their aggressive and infiltrative nature makes clinical management challenging, involving multiple disciplines and close monitoring to ensure optimal outcomes. This comprehensive review aims to cover the genetics, demographics, pathogenesis, neurologic sequelae, radiological considerations, chemotherapeutic management, surgical management, and post-operative considerations of pediatric chordoma patients.

Bailey P, Bagdasar D (1929) Intracranial chordoblastoma. Am J Pathol 5:439–450 (PMID: 19969867; PMCID: PMC2003507)PubMedPubMedCentral

Argaud R, Lestrade A (1926) Sur la précocité de certains chordomes sacrococcygiens. Bull Acad de méd 95:375–377

Occhipinti E, Mastrostefano R, Pompili A et al (1981) Spinal chordomas in infancy. Report of a case and analysis of the literature. Childs Brain 8(3):198–206 (PMID: 7238137)PubMed

Rassi MS, Hulou MM, Almefty K et al (2018) Pediatric clival chordoma: a curable disease that conforms to collins’ law. Neurosurgery 82(5):652–660. https://doi.org/10.1093/neuros/nyx254 (PMID: 28521059)CrossRefPubMed

Smoll N, Gautschi O, Radovanovic I et al (2013) Incidence and relative survival of chordomas: the standardized mortality ratio and the impact of chordomas on a population. Cancer 119(11):2029–2037. https://doi.org/10.1002/cncr.28032 (Epub 2013 Mar 15 PMID: 23504991)CrossRefPubMed

Borba L, Al-Mefty O, Mrak R et al (1996) Cranial chordomas in children and adolescents. J Neurosurg 84(4):584–591. https://doi.org/10.3171/jns.1996.84.4.0584 (PMID: 8613849)CrossRefPubMed

Bai J, Zhang S, Zhai Y et al (2020) A series of 62 skull base chordomas in pediatric and adolescent patients: clinical characteristics, treatments, and outcomes. Neurol India 68(5):1030–1036. https://doi.org/10.4103/0028-3886.294548 (PMID: 33109847)CrossRefPubMed

Beccaria K, Sainte-Rose C, Zerah M et al (2015) Paediatric chordomas. Orphanet J Rare Dis 22(10):116. https://doi.org/10.1186/s13023-015-0340-8 (PMID:26391590;PMCID:PMC4578760)CrossRef

Choi G, Yang M, Yoon D et al (2010) Pediatric cervical chordoma: report of two cases and a review of the current literature. Childs Nerv Syst 26(6):835–840. https://doi.org/10.1007/s00381-009-1076-3 (Epub 2010 Jan 22 PMID: 20094721)CrossRefPubMed

10.

Page MJ, Moher D, Bossuyt PM, et al. (2021) PRISMA 2020 explanation and elaboration: updated guidance and exemplars for reporting systematic reviews. bmj. 372

11.

Das P, Soni P, Jones J et al (2020) Descriptive epidemiology of chordomas in the United States. J Neurooncol 148(1):173–178. https://doi.org/10.1007/s11060-020-03511-x (Epub 2020 Apr 28 PMID: 32342333)CrossRefPubMed

12.

Cates JM. Chordoma [Internet] (2017) https://rarediseases.org/rare-diseases/chordoma/. (Accessed 28 Feb 2021).

13.

Wasserman JK, Gravel D, Purgina B (2018) Chordoma of the head and neck: a review. Head Neck Pathol 12(2):261–268. https://doi.org/10.1007/s12105-017-0860-8CrossRefPubMed

14.

Gröschel S, Hübschmann D, Raimondi F et al (2019) Defective homologous recombination DNA repair as therapeutic target in advanced chordoma. Nat Commun 10(1):1635. https://doi.org/10.1038/s41467-019-09633-9.PMID:30967556;PMCID:PMC6456501CrossRefPubMedPubMedCentral

15.

Sharifnia T, Wawer MJ, Chen T et al (2019) Small-molecule targeting of brachyury transcription factor addiction in chordoma. Nat Med 25(2):292–300. https://doi.org/10.1038/s41591-018-0312-3 (Epub 2019 Jan 21. PMID: 30664779; PMCID: PMC6633917)CrossRefPubMedPubMedCentral

16.

Gulluoglu S, Turksoy O, Kuskucu A, Ture U, Bayrak OF (2016) The molecular aspects of chordoma. Neurosurg Rev 39(2):185–196. https://doi.org/10.1007/s10143-015-0663-x (Epub 2015 Sep 12. PMID: 26363792)CrossRefPubMed

17.

de Bree K, de Bakker BS, Oostra RJ (2018) The development of the human notochord. PLoS ONE 13(10):e0205752. https://doi.org/10.1371/journal.pone.0205752 (PMID:30346967;PMCID:PMC6197658)CrossRefPubMedPubMedCentral

18.

Stratt B, Steiner RM (1980) The radiologic findings in posterior mediastinal chordoma. Skeletal Radiol 5(3):171–173. https://doi.org/10.1007/BF00347259 (PMID: 7209569)CrossRefPubMed

19.

Vujovic S, Henderson S, Presneau N et al (2006) Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 209(2):157–165. https://doi.org/10.1002/path.1969 (PMID: 16538613)CrossRefPubMed

20.

Ramesh T, Nagula SV, Tardieu GG et al (2017) Update on the notochord including its embryology, molecular development, and pathology: a primer for the clinician. Cureus 9(4):e1137. https://doi.org/10.7759/cureus.1137 (PMID:28480155;PMCID:PMC5418029)CrossRefPubMedPubMedCentral

21.

Heaton JM, Turner DR (1985) Reflections on notochordal differentiation arising from a study of chordomas. Histopathology 9(5):543–550. https://doi.org/10.1111/j.1365-2559.1985.tb02835.x (PMID: 4007792)CrossRefPubMed

22.

Sebro R, DeLaney T, Hornicek F et al (2016) Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas. BMC Med Imaging 16(1):53CrossRef

23.

Lau CS, Mahendraraj K, Ward A et al (2016) Pediatric chordomas: a population-based clinical outcome study involving 86 patients from the surveillance, epidemiology, and end result (SEER) database (1973–2011). Pediatr Neurosurg 51(3):127–136 (Epub 2016 Feb 17. doi: 10.1159/000442990. Epub 2016 Feb 17. PMID: 26881831)CrossRef

24.

Shih AR, Cote GM, Chebib I et al (2018) Clinicopathologic characteristics of poorly differentiated chordoma. Mod Pathol 31(8):1237–1245. https://doi.org/10.1038/s41379-018-0002-1 (Epub 2018 Feb 26. Epub 2018 Feb 26. PMID: 29483606)CrossRefPubMed

25.

Hofmann E (2012) The clivus: anatomy, normal variants and imaging pathology. Clin Neuroradiol 22(2):123–139. https://doi.org/10.1007/s00062-011-0083-4 (Epub 2011 Jun 28. PMID: 21710384)CrossRefPubMed

26.

Wold LE, Laws ER Jr (1983) Cranial chordomas in children and young adults. J Neurosurg 59(6):1043–1047. https://doi.org/10.3171/jns.1983.59.6.1043 (PMID: 6631498)CrossRefPubMed

27.

Wang L, Wu Z, Tian K et al (2017) Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients. J Neurosurg 127(6):1257–1267. https://doi.org/10.3171/2016.9.JNS16559 (Epub 2017 Jan 6 PMID: 28059654)CrossRefPubMed

28.

Brooks M, Kleefield J, O’Reilly GV et al (1987) Thoracic chordoma with unusual radiographic features. Comput Radiol 11(2):85–90. https://doi.org/10.1016/0730-4862(87)90016-3 (PMID: 3608452)CrossRefPubMed

29.

Kozlowski K, Barylak A, Campbell J et al (1990) Primary sacral bone tumours in children (report of 16 cases with a short literature review). Australas Radiol 34(2):142–149. https://doi.org/10.1111/j.1440-1673.1990.tb02830.x (PMID: 2241666)CrossRefPubMed

30.

Hamilton K, Rebsamen S, Salamat S et al (2019) Pediatric extraosseous sacral chordoma: case report and literature review of embryonic derivation and clinical implications. J Neurosurg Pediatr 22:1–6. https://doi.org/10.3171/2018.12.PEDS18544 (Epub ahead of print. PMID: 30797212)CrossRef

31.

Richards AT, Stricke L, Spitz L (1973) Sacrococcygeal chordomas in children. J Pediatr Surg 8(6):911–914. https://doi.org/10.1016/0022-3468(73)90010-9 (PMID: 4785565)CrossRefPubMed

32.

Agunbiade S, Nada A, Bhimaniya S et al (2020) Chordoma with lung metastases at initial presentation of a pediatric patient. Radiol Case Rep 15(4):382–386. https://doi.org/10.1016/j.radcr.2020.01.004 (PMID:32055265;PMCID:PMC7005512)CrossRefPubMedPubMedCentral

33.

Joyce EJ, Cohen MA, Ho W et al (2020) Extreme lateral transodontoid approach for resection of clival chordoma: 2-dimensional operative video. Oper Neurosurg (Hagerstown) 19(3):E298. https://doi.org/10.1093/ons/opz411 (PMID: 31943094)CrossRef

34.

Frankl J, Grotepas C, Stea B et al (2016) Chordoma dedifferentiation after proton beam therapy: a case report and review of the literature. J Med Case Rep 10(1):280. https://doi.org/10.1186/s13256-016-1076-3 (PMID:27729085;PMCID:PMC5059891)CrossRefPubMedPubMedCentral

35.

Habrand JL, Datchary J, Bolle S et al (2016) Chordoma in children: case-report and review of literature. Rep Pract Oncol Radiother 21(1):1–7. https://doi.org/10.1016/j.rpor.2015.10.007 (PMID: 26900351; PMCID: PMC4716407)CrossRefPubMed

36.

Miyahara H, Nodomi S, Umeda K et al (2020) Chemoradiotherapy for unresectable INI1-negative chordoma in a child. J Pediatr Hematol Oncol 42(1):65–68. https://doi.org/10.1097/MPH.0000000000001318 (PMID: 30281526)CrossRefPubMed

37.

Baliga S, Yock TI (2019) Proton beam therapy in pediatric oncology. Curr Opin Pediatr 31(1):28–34. https://doi.org/10.1097/MOP.0000000000000724 (PMID: 30531222)CrossRefPubMed

38.

Mizumoto M, Oshiro Y, Yamamoto T et al (2017) Proton beam therapy for pediatric brain tumor. Neurol Med Chir (Tokyo) 57(7):343–355. https://doi.org/10.2176/nmc.ra.2017-0003 (PMID: 28603224; PMCID: PMC5566707)CrossRef

39.

Di Maio S, Temkin N, Ramanathan D et al (2011) Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg 115(6):1094–1105. https://doi.org/10.3171/2011.7.JNS11355 (Epub 2011 Aug 5 PMID: 21819197)CrossRefPubMed

40.

Ahrendt MN, Wesselhoeft CW (1992) Chordoma presenting as a posterior mediastinal mass in a pediatric patient. J Pediatr Surg 27(12):1515–1518. https://doi.org/10.1016/0022-3468(92)90489-t (PMID: 1469556)CrossRefPubMed

41.

Tao ZZ, Chen SM, Liu JF et al (2005) Paranasal sinuses chordoma in pediatric patient: a case report and literature review. Int J Pediatr Otorhinolaryngol 69(10):1415–1418. https://doi.org/10.1016/j.ijporl.2005.03.027 (PMID: 15913796)CrossRefPubMed

42.

McDowell MM, Zwagerman NT, Wang EW et al (2020) Long-term outcomes in the treatment of pediatric skull base chordomas in the endoscopic endonasal era. J Neurosurg Pediatr 20:1–10. https://doi.org/10.3171/2020.6.PEDS19733 (Epub ahead of print. PMID: 33254137)CrossRef

43.

Bianchi PM, Marsella P, Masi R et al (1989) Cervical chordoma in childhood: clinical statistical contribution. Int J Pediatr Otorhinolaryngol 18(1):39–45. https://doi.org/10.1016/0165-5876(89)90229-2 (PMID: 2807753)CrossRefPubMed

44.

Soudack M, Guralnik L, Ben-Nun A et al (2007) Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol 37(5):492–497. https://doi.org/10.1007/s00247-007-0420-6 (Epub 2007 Mar 8 PMID: 17345078)CrossRefPubMed

45.

Castellano GC, Johnston HW (1975) Intrathoracic chordoma presenting as a posterior mediastinal tumor. South Med J 68(1):109–112. https://doi.org/10.1097/00007611-197501000-00029 (PMID: 1154050)CrossRefPubMed

46.

Selvaraj A, Wood AJ (2003) Superior mediastinal chordoma presenting as a bilobed paravertebral mass. Eur J Cardiothorac Surg 23(2):248–250. https://doi.org/10.1016/s1010-7940(02)00753-4 (PMID: 12559357)CrossRefPubMed

47.

Chau L, Malicki DM, Levy ML et al (2020) Unusual extradural chordoma in an adolescent presenting with lumbar radiculopathy. BMJ Case Rep 13(4):e234942. https://doi.org/10.1136/bcr-2020-234942 (PMID:32265215;PMCID:PMC7244276)CrossRefPubMed

48.

Al-Adra D, Bennett A, Gill R, Lees G (2011) Pediatric metastatic sacrococcygeal chordoma treated with surgery. Eur J Pediatr Surg 21(3):196–198. https://doi.org/10.1055/s-0031-1271635 (Epub 2011 Feb 21 PMID: 21341180)CrossRefPubMed

49.

Chandawarkar RY (1996) Sacrococcygeal chordoma: review of 50 consecutive patients. World J Surg 20(6):717–719. https://doi.org/10.1007/s002689900110 (PMID: 8662159)CrossRefPubMed

50.

Stapleton AL, Tyler-Kabara EC, Gardner PA et al (2017) Risk factors for cerebrospinal fluid leak in pediatric patients undergoing endoscopic endonasal skull base surgery. Int J Pediatr Otorhinolaryngol 93:163–166. https://doi.org/10.1016/j.ijporl.2016.12.019 (Epub 2016 Dec 26 PMID: 28109490)CrossRefPubMed

51.

Soudry E, Turner JH, Nayak JV et al (2014) Endoscopic reconstruction of surgically created skull base defects: a systematic review. Otolaryngol Head Neck Surg 150(5):730–738. https://doi.org/10.1177/0194599814520685 (Epub 2014 Feb 3 PMID: 24493791)CrossRefPubMed

52.

Hussein K, Bitterman R, Shofty B et al (2017) Management of post-neurosurgical meningitis: narrative review. Clin Microbiol Infect 23(9):621–628. https://doi.org/10.1016/j.cmi.2017.05.013 (Epub 2017 May 18 PMID: 28529027)CrossRefPubMed

53.

Dashti SR, Baharvahdat H, Spetzler RF et al (2008) Operative intracranial infection following craniotomy. Neurosurg Focus 24(6):E10. https://doi.org/10.3171/FOC/2008/24/6/E10 (PMID: 18518740)CrossRefPubMed

54.

Owens CD, Stoessel K (2008) Surgical site infections: epidemiology, microbiology and prevention. J Hosp Infect 70(Suppl 2):3–10. https://doi.org/10.1016/S0195-6701(08)60017-1 (PMID: 19022115)CrossRefPubMed

55.

Moran D, Zadnik PL, Taylor T et al (2015) Maintenance of bowel, bladder, and motor functions after sacrectomy. Spine J 15(2):222–229. https://doi.org/10.1016/j.spinee.2014.08.445 (Epub 2014 Sep 6 PMID: 25195977)CrossRefPubMed

56.

Fourney DR, Rhines LD, Hentschel SJ et al (2005) En bloc resection of primary sacral tumors: classification of surgical approaches and outcome. J Neurosurg Spine 3(2):111–122. https://doi.org/10.3171/spi.2005.3.2.0111 (PMID: 16370300)CrossRefPubMed

57.

Koutourousiou M, Gardner PA, Tormenti MJ et al (2012) Endoscopic endonasal approach for resection of cranial base chordomas: outcomes and learning curve. Neurosurgery 71(3):614–624. https://doi.org/10.1227/NEU.0b013e31825ea3e0 (PMID: 22592328)CrossRefPubMed

58.

Taneja B, Srivastava V, Saxena KN (2012) Physiological and anaesthetic considerations for the preterm neonate undergoing surgery. J Neonatal Surg 1(1):14 (PMID: 26023373; PMCID: PMC4420318)PubMedPubMedCentral

59.

Edomwonyi NP, Ekwere IT, Egbekun R et al (2006) Anesthesia-related complications in children. Middle East J Anaesthesiol 18(5):915–927 (PMID: 17094529)PubMed

Titel: Clinical management of pediatric chordomas: a comprehensive review
verfasst von: Taylor Reardon
Caleb Marsh
Preston Rippe
Donatas Ruzys
Benjamin Ayres
David Cline
Brian Fiani
Publikationsdatum: 14.10.2021
Verlag: Springer International Publishing
Erschienen in: Acta Neurologica Belgica / Ausgabe 6/2021
Print ISSN: 0300-9009
Elektronische ISSN: 2240-2993
DOI: https://doi.org/10.1007/s13760-021-01821-z

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