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Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature.
J Med Case Rep 2015; 9:229JM

Abstract

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.

Authors+Show Affiliations

Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, 98158, Messina, Italy. aleadorno@yahoo.it.Department of Neurosurgery - AOU Policlinico "G.Martino", Via Consolare Valeria, 98125, Messina, Italy. calafaci@unime.it.Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, 98158, Messina, Italy. fra.sanfilippo@hotmail.com.Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, 98158, Messina, Italy. danielecaf@libero.it.Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, 98158, Messina, Italy. monica.scordino@libero.it.Department of Neuroradiology, University of Messina, Messina, Italy. fgranata@unime.it.Department of Neurosurgery - BIONEC, University of Palermo, Palermo, Italy. giovanni.grasso@unipa.it.Department of Neurosurgery - AOOR Papardo-Piemonte, Contrada Papardo, 98158, Messina, Italy. fsalp@unime.it.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

26438353

Citation

Adorno, A, et al. "Malignant Teratoma in Klippel-Feil Syndrome: a Case Report and Review of the Literature." Journal of Medical Case Reports, vol. 9, 2015, p. 229.
Adorno A, Alafaci C, Sanfilippo F, et al. Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature. J Med Case Rep. 2015;9:229.
Adorno, A., Alafaci, C., Sanfilippo, F., Cafarella, D., Scordino, M., Granata, F., ... Salpietro, F. M. (2015). Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature. Journal of Medical Case Reports, 9, p. 229. doi:10.1186/s13256-015-0700-y.
Adorno A, et al. Malignant Teratoma in Klippel-Feil Syndrome: a Case Report and Review of the Literature. J Med Case Rep. 2015 Oct 4;9:229. PubMed PMID: 26438353.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature. AU - Adorno,A, AU - Alafaci,C, AU - Sanfilippo,F, AU - Cafarella,D, AU - Scordino,M, AU - Granata,F, AU - Grasso,G, AU - Salpietro,F M, Y1 - 2015/10/04/ PY - 2015/01/25/received PY - 2015/08/31/accepted PY - 2015/10/7/entrez PY - 2015/10/7/pubmed PY - 2016/8/27/medline SP - 229 EP - 229 JF - Journal of medical case reports JO - J Med Case Rep VL - 9 N2 - 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. SN - 1752-1947 UR - https://www.unboundmedicine.com/medline/citation/26438353/Malignant_teratoma_in_Klippel_Feil_syndrome:_a_case_report_and_review_of_the_literature_ L2 - https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-015-0700-y DB - PRIME DP - Unbound Medicine ER -