Review Article
Volume 9 Issue 4 - 2020
Sacrococcygeal Chordomas in Childhood, Adolescents and Young Adults: A Review
Mark Yoi Sun Soo*
Senior Clinical Lecturer, Faculty of Medicine, The University of Sydney and Radiology Department, Westmead Hospital, Westmead NSW, Australia
*Corresponding Author: Mark Yoi Sun Soo, Senior Clinical Lecturer, Faculty of Medicine, The University of Sydney and Radiology Department, Westmead Hospital, Westmead NSW, Australia.
Received: March 10, 2020; Published: March 23, 2020




Abstract

Background and objectives: Until the past two decades, childhood and adolescents sacrococcygeal chordoma was in general sparsely documented. Study of its biology and cyto-morphology in recent years has classified the tumour into several subtypes. This paper attempts to relate the morphology and histopathology of these subtypes in terms of prognosis and to gain insights on the tumour’s clinical behaviour and current management trends.

Material and Methods: The core material consists of seven articles on paediatric and adolescent sacrococcygeal chordoma and five review papers on the tumour’s biology and clinical behaviour in the paediatric and young adult population.

Results:

  1. The incidence of paediatric sacrococcygeal chordoma in comparison to its adult counterpart is low. In a series of 60 patients, only one belonged to the paediatric age. There are scant reports on patients with this condition in their second and third decade of life.
  2. The gross features on computed tomography and magnetic resonance imaging are mainly indistinguishable from other benign and malignant sacral tumours. Though internal septations on T2W magnetic resonance imaging are characteristic, these also feature in Ewing’s sarcoma and aneurysmal bone cysts. Among adolescents, computed tomography findings such as spotted calcifications and bone erosions are also found in chondrosarcoma, and sacral chondroblastic osteosarcoma.
  3. The highly malignant dedifferentiated sacral chordomas affect up to 50% of young adults in their thirties and forties.

Conclusion: Gross total resection with wide negative margins remains the treatment of choice for both children and adults while magnetic resonance imaging plays an indispensable role in surgical planning and post therapy follow-up. Poor differentiation, cellular atypia and SMARCB1 INI loss are unfavourable prognostic signs indicative of concomitant metastases.

Keywords: Adolescents; Childhood; Chordoma; Paediatric; Sacrococcygeal 

References

  1. Walcott BP., et al. “Chordoma: current concepts, management, and future directions”. Lancet Oncology 13.2 (2012): e69-e76. 
  2. Beccaria K., et al. “Paediatric Chordomas”. Orphanet Journal of Rare Diseases 10 (2015): 116. 
  3. Coffin CM., et al. “Chordoma in Childhood and Adolescence: A clinicopathologic Analysis of 12 Cases”. Archives of Pathology and Laboratory Medicine 117.9 (1993): 927-933. 
  4. Sebro R., et al. “Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas”. BMC Medical Imaging 16.1 (2016): 53. 
  5. Tsitouras V., et al. “Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature”. Journal of Clinical Neuroscience 34 (2016): 169-176. 
  6. Chandawarkar RY. “Sacrococcygeal chordoma: review of 50 consecutive patients”. World Journal of Surgery 20.6 (1996): 717-719. 
  7. Cable DG and Moir C. “Pediatric sacrococcygeal chordomas: a rare tumor to be differentiated from sacrococcygeal teratoma”. Journal of Pediatric Surgery 32.5 (1997): 759-761. 
  8. Horton KM., et al. “Metastasizing Chordoma in early Childhood: Report of a Case at 1.5T”. Magnetic Resonance Imaging 7.6 (1989): 689-691. 
  9. Schiavello E., et al. “Pediatric extraspinal sacrococcygeal ependymoma (ESE): an Italian AIEOP experience of six cases and literature review”. Child’s Nervous System 34.7 (2018): 1291-1298. 
  10. Soo MYS and Wong L. “Sacrococcygeal Chordoma”. Journal of Hong Kong College of Radiology 5.2 (2002): 117-125.
  11. Kumar N., et al. “Sacro-coccygeal teratoma in adult: Two rare case reports and review of literature”. International Journal of Applied and Basic Medical Research 4.2 (2014): 122-124. 
  12. Srinivasalu S and D’Souza A. “Sacral Ewing’s Sarcoma and Challenges in its Diagnosis on MRI”. Journal of Radiology Case Reports 3.1 (2009): 23-26. 
  13. Kupeli S., et al. “Sacral mesenchymal chondrosarcoma in childhood: a case report and review of the literature”. Pediatric Hematology and Oncology 27.7 (2010): 564-573. 
  14. Ilaslan H., et al. “Primary Ewing's sarcoma of the vertebral column”. Skeletal Radiology 33.9 (2004): 506-513. 
  15. Katonis P., et al. “Spinal Osteosarcoma”. Clinical Medicine Insights: Oncology 7 (2013): 199-208. 
  16. Singh AP., et al. “Sacrococcygeal Teratoma in an Adolescent: A Rare Case Report”. International Journal of Scientific Study 2.6 (2014): 157-159. 
  17. Al-Adra D., et al. “Pediatric metastatic sacrococcygeal chordoma treated with surgery”. European Journal of Pediatric Surgery 21.3 (2011): 196-198. 
  18. de Noronha L., et al. “Sacrococcygeal Chordoma in a 9-year old boy”. Arquivos de Neuro-Psiquiatria 53.3B (1995): 654-658. 
  19. Iwasa Y., et al. “Sacral chordoma in early childhood: clinicopathological and immunohistochemical study”. Pediatric and Developmental Pathology 1.5 (1998): 420-426. 
  20. Wu AM., et al. “Chondrosarcoma in a paediatric population: a study of 247 cases”. Journal of Children’s Orthopaedics 13.1 (2019): 89-99. 
  21. Shih AR., et al. “Clinicopathologic characteristics of poorly differentiated chordoma”. Modern Pathology 31.8 (2018): 1237-1245. 
  22. Beccaria K., et al. “Pediatric Chordomas: Results of a Multicentric Study of 40 Children and Proposal for a Histopathological Prognostic Grading System and New Therapeutic Strategies”. Journal of Neuropathology and Experimental Neurology 77.3 (2018): 207-215. 
  23. Pendharkar AV., et al. “Surgical management of Sacral Chordomas: Illustrative Cases and Current Management Paradigms”. Cureus 7.8 (2015): e301. 
  24. Fuchs B., et al. “Operative management of sacral chordoma”. Journal of Bone and Joint Surgery - American 87.10 (2005): 2211-2216. 
  25. Fourney DR and Gokaslan ZL. “Current management of sacral chordoma”. Neurosurgical Focus 15.2 (2003): E9. 
  26. McMaster ML., et al. “Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population”. Journal of Medical Genetics 48.7 (2011): 444-449. 
  27. Dahl NA., et al. “Chordoma Occurs in Young Children with Tuberous Sclerosis”. Journal of Neuropathology and Experimental Neurology 76.6 (2017): 418-423. 
  28. Chou WC., et al. “De Novo Dedifferentiated Chordoma of the Sacrum: A Case Report and Review of the Literature”. Chang Gung Medical Journal 32.3 (2009): 330-335. 
  29. Kim SC., et al. “Two cases of Dedifferentiated Chordoma in the Sacrum”. Korean Journal of Spine 12.3 (2015): 230-234. 
  30. Hamilton K., et al. “Pediatric extraosseous sacral chordoma: case report and literature review of embryonic derivation and clinical implications”. Journal of Neurosurgery Pediatrics 23.5 (2019): 628-633. 
  31. Akpolat N., et al. “Sacral Chondroblastic Osteosarcoma Misdiagnosed as Chondrosarcoma and Chordoma”. Turkish Journal of Medical Sciences 37.4 (2007): 243-249.
Citation: Mark Yoi Sun Soo. ““Sacrococcygeal Chordomas in Childhood, Adolescents and Young Adults: A Review”. EC Paediatrics 9.4 (2020): 88-93.

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