Amyloidoza plamkowata jako powikłanie makrogruczolaka prolaktynowego przysadki — nowy związek kliniczny

  Amyloid deposition in the pituitary gland is a rare localised form of amyloidosis, and most commonly reported with prolactinoma. Macular amyloidosis is a rare form of localised cutaneous amyloidosis of obscure aetiology. In contrast to most localised amyloidosis, the precursor protein(s) of both macular amyloidosis and prolactinoma are unknown. A 35-year-old man with chronic headache (six years), blurring of vision (three years), and hyperpigmented macular lesion involving arms, legs, and back (two years) was diagnosed to have hyperprolactinaemia (8927 ng/mL) and secondary adrenal insufficiency. MRI revealed pituitary macroadenoma compressing the optic chiasma, encasing the right carotid artery and extending into the sphenoid sinus. A biopsy of skin from the right upper arm revealed thickened stratum corneum, acanthosis, and deposition of pale eosinophilic material in papillary dermis that gave a rose pink colour under methyl-violet and appeared congophilic with Congo red stain, which under polarised light showed green birefringence, diagnostic of macular amyloidosis. Headache, bitemporal haemianopia, and skin lesion improved following cabergoline therapy. Temporal profile of the disease characterised by symptoms of macroprolactinoma preceding onset of macular amyloidosis with resolution of symptoms of macroprolactinoma, accompanied by reductions in prolactin, and concomitant improvement in macular amyloidosis with cabergoline therapy may suggest some link between macroprolactinoma and macular amyloidosis. This report intends to highlight this novel association of macular amyloidosis and macroprolactinoma. (Endokrynol Pol 2015; 66 (6): 555–558)    Złogi amyloidu w przysadce to rzadka forma lokalizacji amyloidozy. Najczęściej występuje razem z gruczolakiem prolaktynowym przysadki. Amyloidoza plamkowata to rzadka forma skupionej amyloidozy skórnej o niewyjaśnionej etiologii. Przeciwnie do większości amyloidoz występujących w jednym miejscu, białko prekursora zarówno amyloidozy plamkowatej, jak i gruczolaka prolaktynowego pozostają nieznane. U 35-letniego mężczyzny cierpiącego na przewlekły ból głowy (od 6 lat), nieostre widzenie (od 3 lat) oraz plamkowe zmiany pigmentacyjne na rękach, nogach i plecach (od 2 lat) zdiagnozowano hiperprolaktynemię (8927 ng/ml) i wtórną niedoczynność kory nadnerczy. Badanie rezonansem magnetycznym ujawniło makrogruczolaka przysadki mózgowej, uciskającego skrzyżowanie wzrokowe, prawą tętnicę szyjną i ekspansją/rozrostem do zatoki klinowej. Biopsja skóry z górnej części prawego ramienia wykazała pogrubienie warstwy rogowej naskórka, akantozę, złogi bladego materiału kwasochłonnego w warstwie brodawkowatej skóry właściwej, które barwiły na różowo przy zetknięciu z fioletem metylowym i wydawały się podatne na barwienie czerwienią kongo, które w świetle spolaryzowanym ujawniało zieloną dwójłomność, wskazując na występowanie amyloidozy plamkowatej. Ból głowy, niedowidzenie połowicze dwuskroniowe, a także zmiany skórne uległy poprawie po zastosowaniu leczenia kabergoliną. Czas wystąpienia choroby, charakteryzującej się objawami makrogruczolaka prolaktynowego przysadki, poprzedzającymi rozpoczęcie amyloidozy plamkowatej wraz ze złagodzeniem objawów makroprolaktynomy oraz redukcją stężenia prolaktyny, a także jednoczesnej poprawie amyloidozy plamkowatej przy terapii kabergoliną może sugerować, że istnieje związek między makrogruczolakiem prolaktynowym i amyloidozą plamkowatą. Niniejszy raport ma na celu naświetlenie nowego związku między amyloidazą plamkowatą i makrogruczolakiem prolaktynowym przysadki. (Endokrynol Pol 2015; 66 (6): 555–558)

A case report of reactive hypoglycemia in a patient with pheochromocytoma and it′s review of literature

Pheochromocytoma, a tumor characterized by catecholamine excess, is usually associated with impaired glucose tolerance. Hypoglycemia may occur after the abrupt withdrawal of catecholamines in the postoperative period. Rarely, insulin secretion by stimulation of β-2 adrenoreceptors may overwhelm the glucagon production, thereby causing hypoglycemia. Here, we describe a female with pheochromocytoma, who presented with postprandial hypoglycemia

Prolonged adrenal insufficiency after unilateral adrenalectomy for Cushing′s Syndrome

The contralateral healthy adrenal in patients undergoing unilateral adrenalectomy for Cushing′s is known to be suppressed temporarily and forms the basis of peri and postoperative steroids. We present four cases of Cushing′s who had prolonged adrenal insufficiency with continued requirement for steroids for periods ranging 1-4 years after unilateral adrenalectomy for Cushing′s. We further review literature regarding the recovery of the hypothalamo pituitary adrenal axis postsurgery in patients with Cushing′s syndrome

Progression of puberty after initiation of androgen therapy in patients with idiopathic hypogonadotropic hypogonadism

Background: Onset of puberty in boys usually occurs by 14 years of age. Some boys may exhibit delayed sexual maturation till about 17-18 years of age. However, pubertal onset beyond 18 years of age is exceedingly rare. Materials and Methods: Patients diagnosed as idiopathic hypogonadotropic hypogonadism (IHH) who had onset of puberty (increase in testicular volume >10 ml) while on androgen therapy were studied. These patients were evaluated prospectively. Results: There were nine subjects that were included in the study. The pre-therapy testicular volumes ranged from 3 to 6 ml. Luteinizing hormone (LH) levels increased from 1.2 ± 0.96 to 2.8 ± 1.0 IU/L, follicular stimulating hormone (FSH) levels increased from 1.5 ± 0.79 to 3.5 ± 1.9 IU/L, and testosterone increased from 0.36 ± 0.16 to 3.4 ± 2.1 ng/ml. Three out of nine patients had testosterone levels below 3 ng/ml. Conclusion: Our present study indicates that pubertal development can occur in patients presenting with hypogonadotropic hypogonadism after 18 years of age. However, acquired pubertal status may be subnorma

Behavioral aggressiveness in boys with sexual precocity

Background: Some boys with sexual precocity are known to have behavioral problems like increased physical and verbal aggression and school and social maladjustments. It is believed to be due to premature androgen exposure. However, it is not clear why only some develop this problem, difference in etiology could be one explanation. Aim: The aim of the study is to assess behavioral aggression in boys with sexual precocity due to different disorders. Materials and Methods: Seven children, ages three to seven years, were enrolled for this study. Two were diagnosed to have congenital adrenal hyperplasia (CAH), three had testotoxicosis, while two had central precocious puberty. Parents of children with precocious puberty underwent the (CASP) questionnaire (children′s aggression scale-parent version). Results: Testosterone levels were high in all patients. Parents denied any history of physical or verbal aggression in the two boys with CAH. Their CASP rating was 0. In contrast, the CASP ratings in the two boys with testotoxicosis and the two with precocious puberty for five domains ranged from 3.1 - 24.2, 2.6 - 8.3,1-5.6,0 - 7.1, and 0 - 1, respectively. In the present study, increased aggression was seen among all the patients with testotoxicosis and both with precocious puberty. In contrast, there were no symptoms of either increased verbal or physical aggression in either of the two patients with CAH. Conclusions: The hormonal milieu in the boys with CAH versus those with sexual precocity due to other causes differed in terms of cortisol and androgen precursors. The androgen excess in CAH children was a consequence of cortisol deficiency. It is possible that cortisol sufficiency is required for androgen-mediated behavioral effects

Spontaneous reduction of prolactinoma post cabergoline withdrawal

Prolactinomas are common pituitary tumors usually highly responsive to dopamine agonists. Around 70-90% of the prolactinomas exhibit decrease in tumor size, though variably with these agents. Uncommonly, there may be little or no shrinkage in pituitary tumor. In the absence of medical therapy, pituitary apoplexy may also result in tumor shrinkage, albeit rarely. We report here a case showing only modest reduction in prolactinoma with cabergoline given for a period of one and a half years. Surprisingly, this tumor showed a 40% reduction in the tumor size 3 months after cabergoline withdrawal in the absence of clinical or radiological evidence of apoplexy