Extensive spherical amyloid deposition presenting as a pituitary tumor

• Published in: Journal of endocrinological investigation 2003-06, Vol.26 (6), p.552-555
• Main Authors: WIESLI, P, BRÄNDLE, M, BRANDNER, S, KOLLIAS, S. S, BERNAYS, R. L
• Format: Article
• Language: English
• Subjects: Aged; Amyloidosis - diagnosis; Amyloidosis - pathology; Biological and medical sciences; Diagnosis, Differential; Endocrinopathies; Humans; Hypothalamus. Hypophysis. Epiphysis (diseases); Magnetic Resonance Imaging; Male; Medical sciences; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Pituitary Diseases - diagnosis; Pituitary Diseases - pathology; Pituitary Neoplasms - diagnosis
• ISSN: 0391-4097; 1720-8386
• DOI: 10.1007/bf03345219

A 71-yr-old man was admitted for further evaluation and trans-sphenoidal surgery of a pituitary tumor. He complained of impotence and decreased libido over a period of about 40 yr. Thirty-eight yr ago he was treated for bilateral gynecomastia with galactorrhea. Endocrinological investigation at presentation revealed only mild hyperprolactinemia and hypogonadotropic hypogonadism. Pituitary magnetic resonance imaging (MRI) showed a tumor up to 2.5 cm in diameter with infiltration of the sphenoid sinus and right cavernous sinus. The tumor exhibited a heterogeneous hyperintense signal on T1-weighted images and hypointense signal on T2-weighted images. Standard trans-sphenoidal surgery was performed and a brownish mass was found inside the sella, which was removed. Histological examination of the mass revealed extensive spherical amyloid deposits with strongly positive immunohistochemical staining for prolactin. Therefore, a prolactinoma with extensive spherical amyloid deposition was diagnosed. Extensive spherical amyloid deposition is a rare finding in prolactin-secreting pituitary adenomas. So far, characteristic radiological findings by MRI have been described only twice. Due to characteristic MRI findings, the diagnosis of extensive intrasellar amyloid deposition can be entertained pre-operatively. Trans-sphenoidal surgical resection is essential to confirm the diagnosis histologically and because of the potential lack of tumor shrinkage under dopaminagonist therapy in this type of prolactinoma.