Malignant teratoma in Klippel–Feil syndrome: a case report and review of the literature

Journal of Medical Case Reports

F M Salpietro¹  G. Grasso³  F. Granata⁴  M. Scordino¹  D. Cafarella¹  F. Sanfilippo¹  C. Alafaci²  A. Adorno¹ 

[1] Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, Messina, 98158, Italy;Department of Neurosurgery - AOU Policlinico “G.Martino”, Via Consolare Valeria, Messina, 98125, Italy;Department of Neurosurgery - BIONEC, University of Palermo, Palermo, Italy;Department of Neuroradiology, University of Messina, Messina, Italy

关键词: Teratoma;    Posterior fossa;    Pathology;    Klippel–Feil syndrome;    Dermoid tumor;    Cervical spine;   

Others  :  1233281
DOI  :  10.1186/s13256-015-0700-y

 received in 2015-01-25, accepted in 2015-08-31,  发布年份 2015

【 摘 要 】

Introduction

Klippel–Feil syndrome is characterized by a congenital fusion of cervical vertebrae. Intracranial teratomas are nongerminomatous germ cell tumors and they account for 0.3 to 0.9% of all intracranial tumors. Teratomas with malignant transformation refer to lesions which give rise to malignant cancer of somatic type. The association between tumors of dermoid origin and Klippel–Feil malformation is extremely rare. Only 23 other cases have so far been reported, and only one case of dermoid tumor with areas of dedifferentiation on squamous cell carcinoma has been described.

Case presentation

We report the case of a 72-year-old white man with a 2-year history of gait and balance disturbances. A brain magnetic resonance imaging revealed a fourth ventricle neoplastic process with infiltrative features. He was operated through a suboccipital craniectomy with a C1 laminotomy and bilateral vertebral artery transposition. At 6-months follow-up, magnetic resonance imaging showed an early regrowth of the fourth ventricle tumor, with the same radiological features.

Conclusions

Patients with Klippel–Feil malformation could develop posterior fossa dermoid tumors. The malignant potential of such tumors must be considered and surgery is recommended. Particular attention must be focused on the histopathological analysis in order to identify possible foci of malignant transformation.

【 授权许可】

   
2015 Adorno et al.

【 图 表 】

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