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


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.


  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 


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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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