Acromegaly and diabetes mellitus associated with hyperthyroidism.

A 66-year-old woman with acromegaly and diabetes mellitus as well as primary hyperthyroidism is described. Serum GH Levels were inappprpriately high.MRI revealed an enlarged sella turcica with intrasellar mass. Her HbAlc was 12.2% and fasting blood glucose 8.89 mmol/l. Thyroid hormone levels in serum and thyroidal radioiodine uptake values were elevated, while TSH measurements in serum were low. Anti TPO antibodies were negative, TSH receptor antibodies were normal. Thyrotoxicosis as the first presenting illness in acromegaly was particulary uncommon. An ultrasound thyroid scan showed a multinodular goiter. Histology of the pituitary lesion showed a typical eosinophilic adenoma which only secreted GH when tested with specific immunostain. Post-operatively, the patient's clinical conditions improved, however, secondary hypoadrenalism appeared.

Spontaneous resolution of pituitary apoplexy in a giant boy under 10 years old.

AIM: Pituitary gigantism is a very rare condition; the occurrence of pituitary apoplexy in children younger than 10 years old is even rarer. The aim of our study is to report this exceptional association. OBSERVATION: A boy aged 9 years and 6 months was hospitalized for the first time in November 2011 for symptoms suggesting pituitary apoplexy. The onset of his disease was difficult to determine as his health record has been poorly maintained. On October 10, 2011, he presented to an emergency department with a sudden drop of visual acuity with diplopia and retro-orbital headaches. An ophthalmological exam found very low visual acuity (1/20) with papillary edema. An MRI of the patient's brain revealed a hemorrhagic pituitary process reaching the chiasma, which was compressed, especially on the right side. Thereafter, the patient's vision improved spontaneously. Clinical examination was normal except for gigantism (+5 SD compared to the target stature). Hormonal assessment argued for mixed secretion [growth hormone (GH) = 39 ng/mL, n ≤ 5, prolactin ( PRL) = 470 ng/mL, n < 15]. Other pituitary functions were normal. Visual acuity normalized after 2 months, and an MRI showed a spontaneous reduction of the pituitary tumor. CONCLUSION: This unusual observation is a model of symptomatic pituitary apoplexy with spontaneous resolution in a boy with pituitary gigantism: phenomenon quite exceptional and worth to be reported.

Bilateral testicular enlargement and seminoma in a patient with acromegaly.

Growth hormone (GH) excess has been associated with soft tissue changes and an increase in the incidence of colonic polyps and gastrointestinal cancer. We describe a patient with endogenous GH excess caused by a pituitary tumour, resulting in acromegaly. The patient had bilateral testicular enlargement, with tumour of the right testicle. Pituitary MRI revealed a macroadenoma. After resection of the pituitary tumour, GH levels fell below 0.5 ng/ml, with acromegaly resolution. Testicular resection revealed seminoma. Following acromegaly resolution, the enlarged remaining testicle decreased in size. The implications of the testicular enlargement and seminoma in the presence of a GH-secreting tumour are discussed.

Treatment of an acromegalic cat with the dopamine agonist L-deprenyl.

Acromegaly was diagnosed in a 14-year-old domestic short hair cat presented for investigation and management of apparently insulin-resistant diabetes mellitus. Treatment with L-deprenyl and high doses of insulin was commenced. The L-deprenyl did not reduce the requirement for high doses of insulin and did not appear to reduce the clinical signs associated with the disease. The cat was euthanased one year after initial presentation.

Blastoma adrenal en adulto / Adrenal blastoma in an adult

El blastoma adrenal es una neoplasia excepcional de la que hay una única publicación en un niño de 21 meses. Se presenta un caso similar desarrollado en un varón de 68 años. Se revisan los criterios anatomopatológicos e inmunohistoquímicos y el diagnóstico diferencial. En conclusión, el blastoma adrenal no es un tumor exclusivo de los niños (AU)

Goldmann perimetry in acromegaly: a survey of 307 cases from 1951 through 1996.

OBJECTIVE: Because visual pathway lesions are a common complication of pituitary tumors, visual field examinations in patients with acromegaly were studied. Proportion and outcome of visual field defects in patients with acromegaly were evaluated. DESIGN: Large, retrospective case series. PARTICIPANTS: We reviewed 307 cases of acromegaly seen from 1951 through 1996 at a single referral center. METHODS: Kinetic visual field testing had been performed with the Goldmann perimeter, and the frequency of visual field defects and their correlation with other clinical manifestations and characteristics of the adenoma were examined. MAIN OUTCOME MEASURES: Repeat visual field examinations. RESULTS: Of the 307 patients included in the analysis, a visual field defect that could be attributed to the pituitary adenoma was observed in 62 (20.2%) during follow-up. Visual field defects were bilateral in 38 (61.3%) of these cases. Patients with visual field abnormalities were significantly younger (P = 0.04), had larger tumors (P < 0.001), had more suprasellar extensions (P < 0.001), and had higher levels of growth hormone in their serum (P = 0.04) than patients free of visual field defects. At the end of the follow-up period, visual field examination remained abnormal in 32 (10.4%). Return to a normal visual field examination after treatment was more frequently observed in patients who were less than 40 years of age at the time of diagnosis (P = 0.004). Secondary empty sella syndrome was the main cause of visual field defects after treatment. Abnormal visual field, either at the time of diagnosis or during follow-up, decreased from 27% of patients between 1951 and 1975 to 15.4% of patients between 1976 and 1996, when modern neuroimaging techniques became available. CONCLUSIONS: Endocrinologic and neuroimaging follow-up of patients with acromegaly should be accompanied by ophthalmic assessment. Factors predictive of visual field defects have been identified.

Lactation associated with acidophilic pituitary adenoma, pheochromocytoma, and cystic endometrial hyperplasia in two goats.

Two unbred adult female goats were examined for persistent, inappropriate lactation. Prostaglandin F2 alpha treatment was ineffective in relieving the condition. Over 5 months, 1 goat developed evidence of CNS disease; this same goat had persistently high serum prolactin concentrations. At necropsy, both goats had an acidophilic adenoma of the pars distalis, a condition that, to our knowledge, has not previously been reported in goats. In addition, both goats had pheochromocytomas and cystic endometrial hyperplasia, conditions that are rarely reported in small ruminants. The inciting cause of inappropriate lactation in goats can be difficult to determine. Pituitary adenomas should be suspected when treatment with prostaglandins is unsuccessful, signs of CNS disease develop, or persistently high serum prolactin concentrations are detected.

Acromegaly.

Acromegaly can be a serious, chronic, debilitating condition. The disease is often missed for years, resulting in significantly delayed diagnosis. This article reviews recent advances in our understanding of acromegaly and the impact of current approaches on the diagnosis and monitoring of patients with this disorder.

Diabetes mellitus in a dog with a growth hormone-producing acidophilic adenoma of the adenohypophysis.

A 9-year-old male Doberman Pinscher was referred to the Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, for polyuria/polydipsia, anorexia, and vomiting. Laboratory examination of blood and urine revealed hyperglycemia, glucosuria, and acidosis. Diabetes mellitus was diagnosed but was very resistant to subsequent insulin treatment. At the owners' request, the dog was euthanatized and a postmortem examination was performed. In addition to hepatic, pancreatic, and renal changes compatible with diabetes mellitus, an acidophilic adenoma of the adenohypophysis was found. Immunohistochemical staining for growth hormone, adrenocorticotropic hormone, and prolactin showed a strong immunolabeling for growth hormone within the cytoplasm of the tumor cells. Although growth hormone level was not measured in the plasma, our findings suggest that the diabetes mellitus in this dog was caused by excess growth hormone secreted by the pituitary neoplasm.

Prolactin-secreting pituitary adenomas with mammary dysplasia in New Zealand white rabbits.

Nine aged (mean age = 3.2 years) nulliparous New Zealand white rabbit does were found to have markedly enlarged teats. The teats were frequently engorged with fluid but were not hot and did not cause signs of pain. The number of affected teats per animal ranged from 1 to 8 (mean = 4). The teats and associated glandular tissue were typically discolored grey, blue, or greenish black (n = 6). Prolactin concentrations were evaluated by radioimmunoassay. Serum prolactin concentrations ranged from 22.4 ng/ml to 2.21 micrograms/ml (mean = 397.3 ng/ml), which was 10- to 1000-fold greater than normal values in nonpregnant rabbits. Conventional radiography of the skull of six rabbits did not reveal pituitary enlargement. Necropsy revealed an enlarged pituitary gland and sella turcica in six of nine does. The diaphragma sellae had ruptured in two rabbits. All nine rabbits had pituitary acidophil adenomas. The neoplastic portions of the pituitaries were diffusely immunoreactive when stained immunohistochemically for prolactin. In contrast, only small clusters of five to seven cells stained positively in normal pituitaries selected as controls. Histologic examination of the mammary glands revealed numerous large dilated cystic spaces containing proteinaceous fluid. Many cysts had numerous papillary epithelial infoldings. The cystic dilations extended into and included the teat canal producing the gross appearance. Prolactin-secreting acidophil adenomas have not been previously reported in the rabbit, and the association with mammary dysplasia is unique.

Sudden blindness from pituitary apoplexy: a report of two cases.

Two cases of acidophil adenoma of the pituitary causing sudden blindness from pituitary apoplexy are presented. The tumours had been clinically silent, without producing any symptoms of endocrine dysfunction. Radiological evidence was very conclusive. Transfrontal craniotomy with decompression resulted in quick and dramatic visual improvement. The interesting syndrome of clinical manifestations is discussed.

Pathological and experimental investigations in a case of gigantism.

A pituitary adenoma was transsphenoidally removed from a 4.5-year-old girl suffering from gigantism. Prior to the operation both the growth hormone (GH) and the prolactin (PRL) levels in the serum were elevated. By light microscopy the tumor appeared to be an acidophilic adenoma. Two distinct cell types, the densely granulated and the sparsely granulated cells, could be distinguished by electron microscopy. Double immunolabeling revealed the presence of GH alone in some densely granulated cells and PRL alone in some sparsely granulated cells, as well as GH and PRL co-localized in both of the morphologically distinguished cell types. Both cell types were identified in the monolayer and the suspension cultures by electron microscopy. GH and PRL concentrations in the culture media were measured by radioimmunoassay. The basal secretion of growth hormone was almost uniform during the 3-week cell culture period. GH and PRL release was significantly inhibited by bromocriptine. Our studies revealed a bimorphous and bihormonal mixed adenoma in childhood.

[Ocular findings in pituitary adenoma].

Of 315 cases (629 eyes) of surgically confirmed pituitary adenoma, 487 eyes (77.4%) suffered diminished visual acuity, 393 eyes (62.5%) primary optic atrophy and 500 eyes (79.5%) visual field defects. Ophthalmoplegia and papilledema were also observed. The ocular changes and visual prognosis were discussed.

Effect of long-term treatment with somatostatin analogue (SMS 201-995) on pituitary tumor shrinkage in acromegaly--report of two cases.

The effect of long-term somatostatin analogue (SMS 201-995) treatment in two acromegalic patients is reported. Continuous tumor shrinkage was observed even after 129 and 139 weeks of treatment with 600 micrograms of SMS 201-995 daily. A huge and firm adenoma underwent shrinkage during treatment with SMS 201-995. No serious side effect appeared during 160 weeks of treatment. SMS 201-995 has a longterm tumor shrinkage effect and improves endocrinopathies.

Thoracic disc herniation in acromegaly.

Herniation of a thoracic disc in an acromegalic giant is reported. Degenerative changes in the spine in association with dorsal kyphosis, and the additional strain, resulted in the disc prolapse.

[A case of male acromegaly with galactorrhea].

A 43-year-old man was admitted to our clinic with complaints of headache and nasal obstruction. He has noted increasing hand and foot size with decreasing libido and pollakiuria for ten years. On admission, he showed galactorrhea. His visual symptoms were slightly decreased. Endocrinological evaluation revealed high serum levels of GH and prolactin. A plain skull X-ray showed acromegalic features with remarkable destruction of dorsum sellae. A contrast enhanced CT demonstrated an intrasellar high density mass extending to the sphenoid sinus and the right ethmoidal sinus. A cerebral angiogram was nor.al. Surgery was then performed with the transsphenoidal approach. A soft reddish brown mass was found in the sphenoid sinus and the bilateral cavernous sinus extending from the sella turcica. Histologically the tumor was eosinophilic adenoma. There were numerous cells exhibiting immunostaining for both GH and PRL in the immunocytochemical study. Postoperative course was uneventful. His visual impairment improved soon after the operation. Serum GH and PRL levels decreased to 38 and 130 ng/ml. He was treated with conventional irradiation (500 rads), so remained galactorrhea and hyperhidrosis. One year after the operation, there is no regrowth of the residual tumor.