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Child's Nervous System

, Volume 34, Issue 2, pp 367–371 | Cite as

A primary extradural malignant rhabdoid tumor at the craniovertebral junction in a 3-year-old boy

  • Takuma NishimotoEmail author
  • Sadahiro Nomura
  • Reiji Fukano
  • Tokuhiro Kimura
  • Eiji Ikeda
  • Michiyasu Suzuki
Case Report

Abstract

Introduction

Malignant rhabdoid tumor (MRT) is a highly aggressive childhood neoplasm and mainly presents in kidney and brain. We report the case of a patient with extrarenal extracranial MRT in the craniovertebral junction (CVJ).

Case report

A 3-year-old boy presented with tetraparesis that had rapidly developed for 2 weeks. The tumor was located in the ventral side of the C1 and C2 extradural space and had invaded the lower clivus. The symptom recovered after subtotal resection of the tumor through a lateral approach with removal of bilateral lateral masses at C1 and instrumental occipital-C2 posterior fusion. Definite histological diagnosis of the extradural tumor was difficult; however, a metastatic lesion in the rib showed a proliferation of INI1/SMARCB1-negative spindle and rhabdoid cells, indicating the tumor was MRT. Stereotactic CyberKnife radiotherapy with a marginal dose of 26 Gy contributed to control of local regrowth of the tumor before histopathological confirmation and induction of chemotherapy with ifosfamide, cisplatin, and etoposide. The patient survived for 29 months without local recurrence of the tumor and with independent activity, however then died of multiple metastases.

Conclusion

Treatment strategies for MRT in the CVJ should include an optimal surgical approach for reduction of tumor volume and stabilization of the spine, followed by high dose chemotherapy. Stereotactic radiotherapy may be useful for local control.

Keywords

Extradural tumor Posterior fusion INI1/SMARCB1/BAF47 Pediatrics 

Notes

Acknowledgements

We thank Dr. Tatsuo Akimura (Saint Hill Hospital), Professor Hideaki Iizuka (Kanazawa Medical University), Dr. Hisaharu Goto (Yamaguchi University Hospital), Dr. Kenichi Saito (Konan Saint Hill Hospital), and Professor Takashi Tamiya (Kagawa University) for assisting with surgery and irradiation with CyberKnife® and for much helpful advice in the course of treatment.

Compliance with ethical standards

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Supplementary material

381_2017_3631_MOESM1_ESM.docx (67 kb)
ESM 1 (DOCX 67 kb)

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of NeurosurgeryYamaguchi University School of MedicineYamaguchiJapan
  2. 2.Department of PediatricsYamaguchi University Graduate School of MedicineUbeJapan
  3. 3.Department of PathologyYamaguchi University Graduate School of MedicineUbeJapan

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