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Bernardo Liberman, Arthur Cukiert, Elcio Machado, Alcione Sousa, Jose A Buratini, Joaquim Vieira, Jaime Goldman, Maria ER Silva, Katia Nogueira, Martha Huayllas, Luis R Salgado.
Departments of Endocrinology and Neurosurgery, Hospital Brigadeiro, Sao Paulo SP, Brazil.
INTRODUCTION: Lymphocytic inflamatory pathology of the hypothalamic-pituitary axis may yield variable endocrine deficits, including diabetes insipidus (DI). Inflamatory pathology of this region is more prevalent among women after the puerperium. Neuroimaging findings generally include thickening of the pituitary stalk (PS) and enlargement of the infundibulum and pituitary gland and intense contrast enhancement of these structures. A small number of patients with inflammation restricted to the infundibular region have also been described. This paper describes 2 additional patients with such localized inflamatory process.
MATERIAL: Patient I- Sixteen years old male, presented with DI 2 years ago. MR showed enlargement of the PS and infundibular region with intense contrast enhancement and a normally appearing pituitary gland and normal pituitary function. Patient II- 32 years old female, presented with DI and progressive hypopituitarism 3 years ago. MR follow-up showed progressive increase in the thickness and contrast enhancement of the PS and progressive pituitary atrophy. Both patients were submitted to transcranial resection of the PS through a cranial section made just above the infundibulum and a caudal one performed at the level of the diafragma sellae.
RESULTS: Pathological examination of both surgical specimens showed only intense lymphocytic infiltration. Both of them persist with DI postoperatively. Patient I showed a progressive hypopituitarism after surgery. There was no additional surgical morbidity. Postoperative MR scanning showed complete removal of the lesions and no additional areas of contrast enhancement.
DISCUSSION: Lymphocytic inflamatory pathology of the hypothalamic-pituitary axis may be restricted to the infundibular and PS regions. Even a normal or atrophic pituitary gland as demonstrated by MR is still compatible with the diagnosis. A progressively atrophic pituitary gland may also be seen as the inflamatory process evolves. Lymphocytic inflamatory pathology restricted to the infundibular and PS region may represent a different entity from infundibulo-hypophysitis or part of a spectrum of the inflamatory pathology of the region.