6533b7d8fe1ef96bd126a437
RESEARCH PRODUCT
Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature
M. ScordinoF. M. SalpietroConcetta AlafaciD. CafarellaF. SanfilippoGiovanni GrassoA. AdornoFrancesca Granatasubject
MalePathologymedicine.medical_specialtyVertebral arteryKlippel–Feil syndromeCase ReportKlippel-Feil syndromeFourth ventricleKlippel–Feil syndromeMalignant transformationCervical spine Dermoid tumor Klippel–Feil syndrome Pathology Posterior fossa TeratomaPosterior fossamedicine.arteryCervical spinemedicinePathologyHumansAgedDermoid CystMedicine(all)medicine.diagnostic_testbusiness.industryCervical spine; Dermoid tumor; Klippel-Feil syndrome; Pathology; Posterior fossa; Teratoma; Aged; Cervical Vertebrae; Cranial Fossa Posterior; Dermoid Cyst; Gait Apraxia; Humans; Klippel-Feil Syndrome; Magnetic Resonance Imaging; Male; Teratoma; Tomography X-Ray Computed; Treatment Outcome; Medicine (all)Medicine (all)TeratomaMagnetic resonance imagingGeneral Medicinemedicine.diseaseMagnetic Resonance ImagingGait ApraxiaTreatment OutcomeDermoid cystCranial Fossa PosteriorCervical VertebraeGerm cell tumorsTeratomabusinessTomography X-Ray ComputedDermoid tumorHumandescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2015-01-01 |