Macroadenoma da hipófise Associado a Sarcoidose Pulmonar e Cutânea.

Abstract

Sarcoidosis is a granulomatous disease of unknown etiology that can affect all organs and systems, including the central nervous system in approximately 5% of patients. Although the hypothalamo-hypophyseal system may be one of the areas affected by neurosarcoidosis, pseudotumoral lesions are very rare. This paper reports the case of a 42-year-old male Caucasian patient followed by the Pneumology and Dermatology services for pulmonary and cutaneous sarcoidosis. Corticotherapy was initiated five months from diagnosis and the patient showed clear clinical and imagiological signs of improvement. In the following two months, the patient experienced visual disturbances and a slight decrease in libido, but denied having other symptoms associated with hypothalamo-hypophyseal hypofunction. Laboratorial evaluation (basal determinations and stimulation tests) revealed panhypopituitarism. The EC-NMR showed a bulky intrasellar lesion extending into the suprasellar and right parasellar regions, with evidence of optic chiasm compression, erosion of the floor of the sella and cavernous sinus invasion. Campimetry showed a bitemporal hemianopsia. For the purposes of chiasmatic decompression and histological study of the lesion the patient underwent a right frontal craniotomy, with a subtotal resection of the hypophyseal lesion. The histological diagnosis revealed a pituitary adenoma with no immunohistochemical expression. The patient began radiotherapy six months after surgery. Although today's diagnostic tools allow for a high degree of accuracy, hypophyseal lesions still present problems to the endocrinologist as their identification is essential for determining the appropriate treatment. At times the final diagnosis is only achieved after a histological identification of the lesion (biopsy/surgery). It should be noted that the described cases of association between sarcoidosis and hypophyseal adenoma are very rare, making the differential diagnosis with neurosarcoidosis difficult--a condition which has a preferential medical approach and a different prognosis.