Dados do Trabalho

Título

Pineocytoma presenting as a pituitary adenoma in a young woman: a case-based update

RESUMO

Case presentation: A 18-year-old female patient had a history of progressive frontal headache for about 1 year. The patient evolved with bilateral visual loss 1 month ago, accompanied by vertigo, galactorrhea, amenorrhea and paraparesis, with greater intensity in the left limb. Neurological exam revealed bilateral absence of direct and consensual photomotor reflexes. Brain Magnetic Resonance Imaging (MRI) showed the presence of an expansive lesion around the pineal gland, manifested with T1 hyposignal, T1 contrast-enhanced hypersignal, and low signal foci on T2 GRE. This lesion extends to the quadrigeminal plate, left fornix, interior of the fourth ventricle, left lateral ventricle and quadrigeminal cistern, causing tonsillar hernia and obstructive hydrocephalus of the sides of the ventricles and the third ventricle. After neurosurgical evaluation, a right ventriculoperitoneal shunt was performed to resolve intracranial hypertension and collection of cerebrospinal fluid for histopathological study, which confirmed the diagnosis of pineocytoma.
Discussion: Tumors of the pineal region are relatively rare, accounting for about 0.4-1% of intracranial tumors. These tumors are generally divided into pineocytoma (grade I), intermediately differentiating pineal parenchyma tumors (grade II or III), papillary tumor of the pineal region (grade II or III), and pineoblastoma (grade IV). Pineocytomas on MRI are usually smaller than <3 cm, well defined, iso or hypointense on T1 and hyperintense on T2. They usually do not seed cerebrospinal fluid and have slow growth associated with a good prognosis. The signs and symptoms depend on the tumor grade and its location, being mainly due to cranial hypertension due to obstructive hydrocephalus secondary to compression of the midbrain roof. In this sense, vomiting, behavioral changes and visual disturbances are also observed, which can characterize Parinaud's syndrome. Galactorrhea and amenorrhea are not usually associated with pineocytomas. However, they may be present rarely when there is coexistence with pituitary adenomas in bifocal tumors.

Palavras Chave

pineocytoma, differential diagnosis, pituitary adenoma