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'Extensive Spherical Amyloid Deposition Presenting As A View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by RERO DOC Digital Library J. Endocrinol. Invest. 26: 552-555, 2003 CASE REPORT Extensive spherical amyloid deposition presenting as a pituitary tumor P. Wiesli*, M. Brändle*,*****, S. Brandner**, S.S. Kollias***, and R.L. Bernays**** *Department of Internal Medicine, Division of Endocrinology and Diabetology, **Department of Pathology, ***Institute of Neuroradiology, ****Department of Neurosurgery, University Hospital of Zurich, Switzerland, *****Division of Endocrinology and Metabolism, Department of Internal Medicine, Taubman Center, University of Michigan, USA ABSTRACT. A 71-yr-old man was admitted for fur- the mass revealed extensive spherical amyloid de- ther evaluation and trans-sphenoidal surgery of a posits with strongly positive immunohistochemical pituitary tumor. He complained of impotence and staining for prolactin. Therefore, a prolactinoma decreased libido over a period of about 40 yr. with extensive spherical amyloid deposition was Thirty-eight yr ago he was treated for bilateral gy- diagnosed. Extensive spherical amyloid deposition necomastia with galactorrhea. Endocrinological in- is a rare finding in prolactin-secreting pituitary ade- vestigation at presentation revealed only mild hy- nomas. So far, characteristic radiological findings perprolactinemia and hypogonadotropic hypogo- by MRI have been described only twice. Due to nadism. Pituitary magnetic resonance imaging characteristic MRI findings, the diagnosis of ex- (MRI) showed a tumor up to 2.5 cm in diameter tensive intrasellar amyloid deposition can be en- with infiltration of the sphenoid sinus and right cav- tertained pre-operatively. Trans-sphenoidal surgi- ernous sinus. The tumor exhibited a heteroge- cal resection is essential to confirm the diagnosis neous hyperintense signal on T1-weighted images histologically and because of the potential lack of and hypointense signal on T2-weighted images. tumor shrinkage under dopaminagonist therapy in Standard trans-sphenoidal surgery was performed this type of prolactinoma. and a brownish mass was found inside the sella, (J. Endocrinol. Invest. 26: 552-555, 2003) which was removed. Histological examination of ©2003, Editrice Kurtis INTRODUCTION teristic clinical or biochemical features. Therefore, in- Extensive intrasellar spherical amyloid deposition is trasellar amyloid deposition is not usually recognized a rare finding in pituitary adenomas, with less than pre-operatively. Magnetic resonance imaging (MRI) 20 cases reported up to today (1). Spherical amyloid of intrasellar amyloid deposits have been described deposition is almost exclusively encountered in PRL only twice (3, 4). producing pituitary adenomas and only exception- We report a patient who was admitted for further ally occurs in GH and ACTH secreting or inactive evaluation and trans-sphenoidal surgery of a pitu- adenomas (1). Abnormal processing of a hormone itary tumor. Intra-operatively, parts of the sphenoid or prohormone by the adenoma cells has been sug- sinus and the sella were filled with a brownish wax- gested as the origin of the spherical amyloid forma- like mass of soft consistency. The histological in- tion (2). Amyloid deposits do not cause any charac- vestigation of this mass revealed almost exclusive- ly spherical amyloid bodies. CASE REPORT Key-words: Amyloid, pituitary, prolactin. A 71-yr-old man was referred for further evaluation Correspondence: Dr. Peter Wiesli. Department of Internal Medicine, Division of Endocrinology and Diabetology, University Hospital of Zurich, for an incidentally found pituitary tumor on a com- CH-8091 Zurich, Switzerland. puted tomography (CT), which was performed due E-mail: [email protected] to a syncope. The patient had had impotence and Accepted October 29, 2002. decreased libido for about 40 yr, but attributed them 552 Amyloid tumor of the pituitary to psychosocial factors. Bilateral mastectomy due to serum cortisol level of 480 nmol/l (reference 280- gynecomastia and galactorrhea was performed 38 yr 690 nmol/l), drawn at 08:00 h confirmed a normal ago. Laboratory results from that time were not avail- adrenal function. The IGF-I was low at 48 g/l (ref- able. Arterial hypertension was treated with a - erence for adults 100-300 g/l). Routine hemato- blocking agent. Physical examination disclosed pe- logical and chemical parameters including inflam- ripheral neuropathy with absence of ankle jerks and matory markers were within the normal range. impaired vibratory sense. The rest of neurological Pituitary MRI demonstrated a sellar mass of 2.5 x examination was normal including normal visual 1.8 x 1.8 cm diameter, without suprasellar exten- fields. Testes were soft, 14 and 10 ml in volume. Skin sion but with infiltration of the sphenoid sinus and was pale and showed fine wrinkles in the corners of right cavernous sinus (Fig. 1). The pituitary stalk was the eyes and mouth. Body and facial hair was sparse, shifted to the left side. This tumor mass showed and pubic hair showed a female distribution pattern heterogeneous hyperintensity on T1-weighted im- (Tanner P4-P5) suggesting hypogonadism. ages without iv contrast (Fig. 1A) and was hy- Laboratory investigations showed mildly elevated pointense on T2-weighted scans (Fig. 1D). prolactin levels of 72.5 g/l (normal 2.2-18.5). Total After a trans-nasal/trans-sphenoidal access to the T was low at 2.5 nmol/l (reference 8.2-35) and free sphenoid sinus, the sphenoid mucosa was found T was 7.2 pmol/l (reference 19-66 for men above to be normal, but the sellar floor was penetrat- 60 yr). FSH and LH serum levels were in the normal ed by a brownish, soft and wax-like, slightly dot- range (LH 2.4 IE/l, reference 2-12 and FSH 4.9 IE/l, ted mass. Exploration of the sella revealed no reference 2-12). Thyroid indices were normal and classical pituitary adenoma. The tumor was re- A C B Fig.1 - MRI findings. Sagittal (A) and coronal (B) T1-weighted MR images without iv contrast showing the heterogeneously hyperin- tense mass (arrow). On coronal T1-weighted MR images with iv contrast, the mass is hypointense in comparison with the hypophy- seal tissue (arrow) (C). On T2 coronal image (D) the mass is strongly hypointense as compared to the brain parenchyma. 553 P. Wiesli, M. Brändle, S. Brandner, et al. moved by ring curettes. The compressed pitu- negative (transabdominal us, chest radiography itary gland was pushed to the left side of the sel- and immunoelectrophoresis). Replacement ther- la turcica. Tuberculosis, bacterial and mycotic in- apy with testosterone was initiated postopera- fections were ruled out by negative microbio- tively, and resulted in improved well-being. The logical investigations of the material (Gram-stain- serum prolactin level slightly decreased (63.5 ing, culture and PCR). The post-operative course g/l, normal 2.2-18.5) postoperatively and re- was uneventful. mained in this range at 1-yr follow-up. Repeat MRI Paraffin-embedded tissue sections stained with of the sellar region showed tumor remnant. hematoxylin and eosin revealed that the removed mass was composed almost entirely of a spherical DISCUSSION eosinophilic material. This amorphous material stained positively and showed birefringence un- We report a man with a large pituitary tumor con- der polarized light with Congo red, indicating that taining mainly spherical amyloid deposits. The it represented amyloid (Fig. 2 A-D). Immunohisto- long history of impotence and decreased libido chemically, the extracellular amyloid spheroids and the early occurrence of gynecomastia with were positive for prolactin (Fig. 2E). Staining for galactorrhea were suggestive for hypogonadism growth hormone, FSH, LH, TSH, ACTH and - due to prolactinoma. Compression and apoptosis subunit as well as for -A4-amyloid was negative. of adenoma cells due to a local mass effect of the Further investigation for systemic amyloidosis was extensive intrasellar amyloid seem a possible ex- planation for the only mild hyperprolactinemia at diagnosis in described patient with long-standing disease. Immunohistochemical staining of the spherical amyloid deposits was strongly positive for PRL, indicating that the amyloid deposits were secreted directly by prolactin-secreting cells. In addition, extensive spherical amyloid deposition occurs typically in patients suffering from pro- lactinoma (5). Two different histological patterns of amyloid de- posits in pituitary adenomas have been de- scribed, the stellate and the spherical, respec- tively (6). Stellate amyloid is found perivascularly in a fibrillary pattern and small amounts may be frequently encountered in pituitary adenomas. No immunoreactive cytokeratin can be detected in these deposits. The type of spherical amyloid is rare. It is characterized by amorphous spheres ad- jacent to adenoma cells and containing im- munoreactive cytokeratin fibrils. Extensive depo- sition of amyloid spheroids in a pituitary adenoma is uncommon. In our patient, the amyloid mass penetrated the sellar floor and extended into the sphenoid sinus. This behavior would be expect- ed rather from a pituitary macroadenoma than from an amyloid mass. Fig. 2 - Pathology findings. The specimen consisted of several Abnormal processing of prolactin or the prohor- fragments of amorphous material. (A) Hematoxylin and eosin mone produced by the adenoma cells rather than stained fragments of amorphous material consisting of nu- merous small concentric portions. In addition, there are small a mesenchymal origin
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