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Case 209

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12-year-old female patient, previously healthy and with adequate vaccination, presented to her pediatrician with a story of progressive headache and neck pain in the last 2 months. There was loss of strength in the upper limb associated with nausea, vomits, lack of appetite and weight loss of 10 kg in the last month. Upon examination, there was neck stiffness, paralysis of the right arm, paresis of the left arm and need of support for ambulation.

Based on the clinical picture and the images, which one of the following is the most likely diagnosis?

a) Epidural abscess caused by bacterial meningitis

25%

b) Clival Chordoma

25%

c) Chondrosarcoma

25%

d) Metastasis

25%
   

Image analysis

 

Image 1: Magnetic resonance imaging, sagittal view , T2 weighted, showcases hyperintense expansive lesion (delimited in yellow) with its center in the clivus and extending inwards the vertebral canal. This compresses the spinal cord, which shows signal alteration compatible with oedema and compressive myelopathy.

 

Images 2: Magnetic resonance imaging, sagittal view , T1 weighted, shows a heterogeneous mass (delimited in green), but predominantly hypointense signal. 

Highlights

- Chordomas are malignant tumors derived from embryonic remnants of the notochord, and are considerate locally invasive.

- May appear in any age, but usually affect adults around the forth decade of life, with preference for males (2:1).

- The most common initial symptoms are diplopia and retro-orbital pain.

- In Computed Tomography it is presented as an expansive soft tissue mass , well delimited and with its center located in the clivus.

- The RMI is the best method for evaluation,  and shows a lesion with low intensity signal in T1 and high intensity in T2.

- The treatment is surgical removal. However, local recurrence is common. 

References

- Erdem E et al. Comprehensive review of intracranial chordoma. Radiographics, 2003; 23: 995-1009.

- Snyderman C, Lin D. Chordoma and chondrosarcoma of the skull base. UpToDate 2015. [Acesso em Outubro de 2015]. Disponível em: http://www.uptodate.com/contents/chordoma-and-chondrosarcoma-of-the-skull-base.

- Tunkel AR. Clinical features and diagnosis of acute bacterial meningitis in adults. UpToDate 2015. [Acesso em Outubro de 2015]. Disponível em: http://www.uptodate.com/contents/clinical-features-and-diagnosis-of-acute-bacterial-meningitis-in-adults.

- Rocha AJ et al. Colégio Brasileiro de Radiologia e Diagnóstico por Imagem: Encéfalo. Rio de Janeiro: Elsevier; 2010.

Author

Fábio Mitsuhiro Satake, 5th year medical student at UFMG.

E-mail: fabiosatake[at]gmail.com

Supervisor

Dra. Edrise Morici Lobo Mueller, Radiologist of Axial Medicina Diagnóstica and professor at Faculdade de Medicina da UFMG.

E- mail: edrisemueller[at]yahoo.com.br

Reviewers

Cairo Mendes, Fellype Borges, André Guimarães and Prof. Viviane Parisotto.

Translated by

Rafael Fusaro Aguiar Oliveira, 4th year medical student at UFMG.

E-mail: rafusaro[at]hotmail.com

 

 

 

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