Spontaneous Remission of Pituitary Dysfunction in an Adolescent with Fluctuating Neurologic Symptoms

RODRIGUEZ, M; PASTOR RUEDA, J.M; BERGADÁ, I; CLÉMENT, F; ARAKAKI, N

Buenos Aires

Congreso; XXVI Reunion Anual de la Sociedad Latinoamericana de Endocrinología Pediatrica; 2016

Introduction: Pituitary dysfunction due to malignant cell infiltrationis an infrequent pathology in children and its spontaneouscomplete remission has not been yet described in pediatric patients.Case Report: We report a case of a 16-year-old boy with no remarkablepast medical history who was admitted to our hospitalafter complaining for two months of headaches and acute left handincoordination. An MRI showed two hyperintense lesions in theperiventricular regions, one in the left talamus and parietooccipitallobe and the other in corpus callosum. In addition, a marked thickenedpituitary stalk was observed. Routine laboratory workout wasnormal, tumor markers and serological test for human immunodeficiencyvirus were negative. He referred polyuria and polydipsiaand clinical examination showedright hemiparesis, short stature(height ? 2.6 SDS, target height ?1.8 SDS) and Tanner stage 5. Hormonalstudies showed low testosterone with low gonadotropins, awater deprivation test ruled out diabetes insipidus. A new MRI,one week after, showed a normal pituitary stalk. Symptoms disappearedspontaneously. Six months later without clinical symptoms,laboratory studies confirmed spontaneous partial recoveryof testicular function. A new MRI revealed only a small residuallesion. He never receivedsteroids or any other treatment. Twoyears later he suffered an acute aphasia. A MRI showed hyperintenselesions with well-defined borders located at the left frontallobe in the cortical and subcortical region. The patient presentedseizures and sudden neurological deterioration. Stereotactic biopsyshowed a B-primary central nervous system lymphoma (BPCNSL).Conclusions: PCNSL is extremely rare in the pediatric population.However, the incidence of this tumor has increased ten-foldover the last three decades in both immunocompetent and immunocompromisedindividuals. Pituitary infiltration has been describedas a rare presentation in adult patients. PCNSL spontaneousremission is exceedingly rare and is referred in the literatureas a type of brain vanishing tumor. We believe it is important toreport this case to pediatric endocrinologists to alert for patientswith neurological and endocrinological symptoms that wax andwane, in whom the diagnosis can be elusive.􀀀