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Case 191

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27-year-old female patient has evolved over 3 months with progressive history of dismay, drowsiness and loss of appetite, accompanied by tension-type headache. She also presented alteration of memory and writing, reduced visual acuity and abnormal wind chill, incompatible with the environment. No changes in the menstrual cycle. It was performed a Magnetic Resonance Imaging (MRI) of the head (below).

Considering the clinical and imaging studies presented, which is the most likely diagnosis?

a) Pituitary macroadenoma

25%

b) Intracranial germinoma

25%

c) Craniopharyngioma

25%

d) Pilocytic astrocytoma

25%
   

Image analysis

 

Image 1: Voluminous expansive lesion of the suprasellar cistern and hypothalamic region. It extends to the third ventricle - not being possible to identify the nerves and optical chiasms - and to the interpeduncular cistern, determining compression and remodeling of the midbrain (in red). It is characterized by hypointense signal on T1.

 

 

Image 2: By observing the T2-weighted MRI, we note that the expansive lesion is centered on the suprasellar cistern and hypothalamic region. It extends to the third ventricle (green arrow) - not being possible to identify the nerves and optical chiasms - and to the interpeduncular cistern, determining compression and remodeling of the midbrain (blue arrow). The lesion is clearly different from the pituitary gland (red arrow), which preserves dimensions and intensity of normal signal. It is characterized by hyperintense signal on T2.

 

 

Image 3: The lesion presents hyperintense signal in Flair-weighted MRI. Observe other aspects described in other images.

 

 

Image 4: Detail of Magnetic Resonance Imaging (MRI) of the head, sagittal section, T1-weighted, after contrast administration (Gadolinium). Observe that the lesion fills up heterogeneously by contrast, defining cystic-looking images inside. Pituitary signaled by the red arrow. Note the midbrain being compressed (blue arrow).

Highlights

- Craniopharyngiomas are rare solid or cystic-solid tumors that arise from remnants of Rathke's pouch along a line from the nasopharynx to the diencephalon.

- Although histologically benign, often result in high morbidity and mortality due to its location.
- The compression caused by supraselar extent of the tumor results in visual deficits, headache and endocrine abnormalities (GH, gonadotropins, TSH and CRH deficiencies).
- MRI is the best imaging test for diagnosis.
- The treatment is surgery and it can be complemented with radiotherapy.

References

Harsh, G. R.; Recht, L. D.   Marcus, K. J.Craniopharyngioma

In: UpToDate, Hirsch, MS, Kaplan, MS (Ed), UpToDate, 2014. Available at: http://www.uptodate.com/contents/craniopharyngioma

- Snyder, P. J. Causes, presentation, and evaluation of sellar masses. In: UpToDate, Hirsch, MS, Kaplan, MS (Ed), UpToDate, 2014. Available at: http://www.uptodate.com/contents/causes-presentation-and-evaluation-of-sellar-masses

- Recht,  L.D. Diagnosis and classification of low-grade gliomas. In: UpToDate, Hirsch, MS, Kaplan, MS (Ed), UpToDate, 2014. Available at: http://www.uptodate.com/contents/diagnosis-and-classification-of low-grade-gliomas

Author

Ana Júlia Furbino Dias Bicalho, 6th year medical student at UFMG.

E-mail: anajuliabicalho[at]gmail.com

Supervisor

Dr. Fernanda Moura Teatini, Neurologist, Preceptor of the Medical Residence of Neuroradiology of Radiology and Imaging Diagnosis of HC/UFMG.

E-mail: fernandateatini@ilive.com

Reviewers

André Guimarães, Bárbara Queiroz, Júlia Petrocchi e Professor Viviane Parisotto.

Translated by

Luísa Bernardino Valério, 3rd year medical student at UFMG.

E-mail: luisabernardino[at]gmail.com

 

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