合并糖尿病的垂体腺瘤的外科治疗

目的：总结分析合并糖尿病的垂体腺瘤的外科治疗效果。方法：回顾分析1990年至2000年收治的43例合并糖尿病的垂体腺瘤患者的资料,生长激素腺瘤29例,泌乳素腺瘤8例,无功能性腺瘤6例;经额入路11例,翼点入路28例,蝶窦入路5例;围手术期严密监测并控制血糖。结果：肿瘤全切除31例（72．1％,次全切除8例（20．9％）,大部分切除4例（7％）;术后32例血糖恢复正常;绝大多数患者手术后恢复良好,仅1例死于尿崩症。结论：合并糖尿病的垂体腺瘤患者的病情复杂,选择合适的手术方法,力争全切肿瘤,以及围手术期的正确处理,是获得良好预后的关键。     

An R201H activating mutation of the GNAS1 (Gsalpha) gene in a corticotroph pituitary adenoma.

In the pituitary gland, activating mutations of the GNAS1 (Gsalpha) gene at Gln227 have been identified in adrenocorticotrophin secreting, growth hormone secreting, and prolactin secreting adenomas. To date, mutations at the codon encoding R201, typically underlying the McCune-Albright syndrome and isolated fibrous dysplasia of bone, have been demonstrated only in growth hormone secreting pituitary adenomas. In this study, a polymerase chain reaction amplified target sequence in exon 8 of the GNAS1 gene was sequenced, identifying the first R201 mutation seen in an isolated basophilic adenoma which generated Cushing's disease in a child. This case adds Cushing's disease to the range of human diseases caused by R201 mutations of the GNAS1 gene.     

Unusual double pituitary adenoma: a case report

We report the case of a 60-year-old woman with Cushing disease. Magnetic resonance imaging (MRI) revealed a large sellar and suprasellar mass involving the right cavernous sinus, consistent with pituitary macroadenoma. It was resected by transsphenoidal surgery. Light microscopy revealed two separate pituitary adenomas with different histologic and immunohistochemical features. One was amphophilic and strongly Periodic Acid-Schiff (PAS) positive, the other chromophobic and PAS negative. The former tumor was immunopositive for adrenocorticotropic hormone (ACTH); approximately 30% tumor cells were immunopositive for MGMT (O6-Methylguanine-DNA Methyl-Transferase). The second tumor was a PAS negative, luteinizing hormone (LH) and alpha subunit immunopositive gonadotroph adenoma. In this tumor, about 90% of the cells were immunopositive for MGMT. The Ki-67 nuclear indices of the two tumors were 6% and 2%. Our case represents a rare combination of two morphologically different pituitary adenomas, one producing ACTH and the other LH and alpha subunit. The two tumors differed not only in Ki-67 labeling indices but in MGMT immunoexpression as well.     

Lipoid pneumonia associated with diabetes mellitus type II in a cat

Our purpose is to report a rare case of lipoid pneumonia found in a cat with diabetes mellitus (DM) type II. The cat is an aged, mixed-breed male with a 3-year history of having DM. During the last 16?weeks of its life, the cat presented signs of dyspnea and weakness and was continuously treated by nebulization. Thoracic radiography showed an increased radiopacity of the alveoli. The histopathological findings showed typical lipoid pneumonia, proliferative bronchitis, and diffuse suppurative pneumonia. Chronic pancreatitis with islet amyloidosis and arteriosclerosis was also detected. Immunohistochemistry with anti-Amylin antibody showed a strong positive result in the islet of Langerhans, confirming type II DM in this case. A diagnosis of death from respiratory failure associated with idiopathic lipoid pneumonia was achieved.     

Silent pituitary corticotroph carcinoma in a young dog

An 11-month-old neutered female weimaraner was humanely destroyed 6 days after an acute onset of neurological signs. At necropsy examination the pituitary gland was replaced by a large neoplastic mass that compressed and infiltrated the overlying hypothalamus. Small nodules were detected in the spleen, kidneys and stomach. Adrenal, thyroid and parathyroid glands were normal in size. The primary pituitary mass, visceral nodules and microscopical metastases detected within the ventricles and leptomeninges of the brain comprised polygonal, chromophobic neoplastic cells, which labelled strongly for adrenocorticotrophic hormone (ACTH) on immunohistochemical examination. These findings, in the absence of clinical or pathological evidence of pituitary-dependent hyperadrenocorticism, support a diagnosis of endocrinologically-inactive ('silent') pituitary corticotroph (ACTH-containing) carcinoma.     

Morphology of the pituitary gland in ferrets (Mustela putorius furo) with hyperadrenocorticism

Pituitary tumours are the cause of hyperadrenocorticism in a variety of species, but the role of the pituitary gland in hyperadrenocorticism in ferrets is not known. In this species, the disease is mediated by the action of excess gonadotrophins on the adrenal cortex and is characterized by an excessive secretion of sex steroids. In this study, the pituitary gland of four healthy control ferrets, intact or neutered, and 10 neutered ferrets with hyperadrenocorticism was examined histologically following immunohistochemical labelling for adrenocorticotrophic hormone, alpha-melanocyte-stimulating hormone, growth hormone, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone, and prolactin. Immunohistochemistry revealed that somatotrophs, thyrotrophs and lactotrophs were the most abundant cell types of the pars distalis of the pituitary gland in the healthy ferrets. The distribution of corticotrophs was similar to that in the dog and man. In ferrets, as in dogs, the melanotrophic cell was almost the only cell type of the pars intermedia. Gonadotrophs were found in the pars distalis of neutered, but not intact ferrets. All the ferrets with hyperadrenocorticism had unilateral or bilateral alterations of the adrenal gland. In addition, in the pituitary gland of two of these ferrets a tumour was detected. These tumours were not immunolabelled by antibodies against any of the pituitary hormones, and had characteristics of the clinically non-functional gonadotroph tumours seen in man. In some of the other ferrets low pituitary immunoreactivity for gonadotrophic hormones was detected, which may have been due to the feedback of autonomous steroid secretion by the neoplastic transformation of the adrenal cortex. It is concluded that initially high concentrations of gonadotrophins resulting from castration may initiate hyperactivity of the adrenal cortex. The low incidence of pituitary tumours and the low density of gonadotrophin-positive cells in non-affected pituitary tissue in this study suggest that persistent hyperadrenocorticism is not dependent on persistent gonadotrophic stimulation.     

垂体腺瘤中垂体激素与S-100蛋白免疫组化双重染色观察

目的：探讨滤泡星状细胞在垂体腺瘤分类中的意义及滤泡星状细胞与内分泌细胞之间的关系。方法：应用免疫组化双重染色方法,对４２ 例人重体腺瘤的垂体激素与 Ｓ１００ 蛋白表达进行对照观察。结果：垂体腺瘤组织中的滤泡星状细胞有两种情况,一种为腺瘤组织中可见散在分布的滤泡星状细胞,并可见１ 个瘤细胞既有 Ｓ１００ 蛋白表达,又含激素分泌颗粒;另一种为滤泡星状细胞构成了腺瘤的一种主要的细胞成分。结论：滤泡星状细胞与内分泌细胞的功能密切相关,可能在调整内分泌细胞的产生和激素释放方面起一定的作用;滤泡星状细胞腺瘤应作为垂体无功能腺瘤的一个单独类型。     

Long-term insulin-dependent diabetes mellitus with secondary pituitary insufficiency and regression of retinopathy

Three women with insulin-dependent diabetes mellitus (IDDM) from childhood and early development of diabetic retinopathy are described. Insulin requirement was reduced to 5-12 IU daily in all three after relatively uncomplicated births and all had very brittle diabetes on this dosage. At re-examination 16-22 years after these births and after 34-42 years of IDDM, regression of retinopathy was observed in two patients, while the third had a light retinopathy at the same level as initially. Other diabetic complications were few and none of the patients had nephropathy. Pituitary examination revealed incomplete hypopituitarism in all cases, human growth hormone (HGH) being the sole common factor lacking. These findings and a review of four similar cases reported previously lend some support to the hypothesis of HGH as a possible pathogenetic co-determinant in the development of diabetic retinopathy.     

垂体腺瘤术后尿崩症的预防与处理

目的;探讨垂体腺瘤术后尿崩症的预防处理方法,提高垂体腺瘤的外科治疗水平。方法：回顾性总结近10年收治的127例手术治疗的垂体腺瘤患者的资料,并复习相关文献。结果：127例术后并发尿崩症64例,发生率为50．5％,尿崩死亡1例,死亡率为1．6％,其中39例（60．9％）在术后1周内恢复正常;11例（17．2％）在2周内恢复正常;3例（4．7％）在4周内恢复正常;4例（6．3％）持续1年;4例（6．3％）持续1年以上;3例（4．7％）持续3年以上。结论：术中保护好垂体柄、下丘脑和避免细小穿通动脉的损伤,是防治垂体腺瘤术后尿崩症的关键。     

Changes in hormone production of a recurrent silent corticotroph adenoma of the pituitary: a histologic, immunohistochemical, ultrastructural, and tissue culture study.

A 19-year-old man with blurred vision, headache, and no signs or symptoms of hormone excess was found to have a pituitary adenoma. The tumor was removed by a transfrontal approach. He had postoperative radiation therapy, but subsequently had three recurrences, all removed surgically. By histology, the tumor was a chromophobic, slightly acidophilic pituitary adenoma. Immunohistochemistry revealed the presence of adrenocorticotropin (ACTH) in all four biopsies, alpha-subunit of glycoprotein hormones, and, to a lesser extent, follicle-stimulating hormone (FSH) and luteinizing hormone (LH) in the third and fourth tumor resection specimens. Ultrastructurally, the tumor had typical features of a silent corticotroph adenoma subtype 2. In tissue culture, the second, third, and fourth specimens released ACTH, alpha-subunit, FSH, and LH and responded to corticotropin-releasing hormone with increased release of ACTH, alpha-subunit, FSH, and LH. To our knowledge only one silent corticotroph adenoma has been reported previously which expressed plurihormonality. Change in immunohistochemical profile in malignant tumors is a well-known phenomenon; however, it was not reported previously in benign pituitary adenomas. The factors accounting for changing tumor phenotype are unknown.     

Takotsubo cardiomyopathy in a patient with pituitary adenoma and secondary adrenal insufficiency

We describe a case of Takotsubo cardiomyopathy in a case of pituitary macroadenoma in acute adrenal crisis. A 48-year-old man presented with acute onset altered sensorium, vomiting, and gasping. On admission, he was unresponsive and hemodynamically unstable. He was intubated and ventilated and resuscitated with fluids and inotropes. The biochemical evaluation revealed hyponatremia, hyperkalemia, and hypocortisolism. Hyponatremia was corrected with 3% hypertonic saline. Contrast enhanced computed tomography (CT) scan of the brain revealed a sellar-suprasellar mass with hypothalamic extension with no evidence of pituitary apoplexy. A diagnosis of invasive pituitary adenoma with the Addisonian crisis was made and steroid replacement was initiated. Despite volume resuscitation, he had persistent refractory hypotension, recurrent ventricular tachycardia, and metabolic acidosis. Electrocardiogram (ECG) showed ST elevation and T-wave inversion in lateral leads; cardiac-enzymes were increased suggestive of acute coronary syndrome. Transthoracic echocardiography showed severe regional wall motion abnormalities (RWMAs) involving left anterior descending territory and low ejection fraction (EF). Coronary angiogram revealed normal coronaries, apical ballooning, and severe left ventricular dysfunction, consistent with a diagnosis of Takotsubo''s cardiomyopathy. Patient was managed with angiotensin-converting enzyme inhibitors and B-blockers. He improved over few days and recovered completely. At discharge, ECG changes and RWMA resolved and EF normalized to 56%. In patients with Addisonian Crisis with persistent hypotension refractory to optimal resuscitation, possibility of Takotsubo''s cardiomyopathy should be considered. Early recognition of association of Takotsubos cardiomyopathy in neurological conditions, prompt resuscitation, and supportive care are essential to ensure favorable outcomes in this potentially lethal condition.     

GH and PRL-producing pituitary adenoma with neuron-like differentiation

A pituitary adenoma with neuron-like differentiation in the sella turcica is reported. Sections of the tumor showed a mixture of adenoma cells, ganglionic cells, and neuropil-like structures by light microscopy. Both pituitary adenoma cells and large cells recognized as ganglionic cells by H&E were strongly immunoreactive for both growth hormone (GH) and prolactin (PRL), which indicated that these large cells had properties similar to those of pituitary adenoma cells. Furthermore, electron microscopy (EM) revealed characteristic low electron-dense secretory granules as well as GH-type large electron-dense secretory granules in adenoma cells, neuropils, and swollen bulbs of neuronal endings, which indicated that these three populations may be of the same origin. Furthermore, we could not find typical cell bodies of ganglionic cells by EM. These results are consistent with a hypothesis that attempts to explain the origin of the neuronal components by the neuronal differentiation of adenoma cells. Thus, the best designation of our tumor may be “pituitary adenoma with neuron-like differentiation.”     

Pregnancy in a patient with hypopituitarism following surgery and radiation for a pituitary adenoma

This is a case of partial hypopituitarism resulting from surgery and radiation for a non- functioning pituitary macroadenoma. The patient had amenorrhea which was secondary to hypogonadotrophic hypogonadism and had been on L-thyroxine for central hypothyroidism. For pregnancy, ovulation was induced by gonadotrophins and this was followed by an intrauterine insemination. The antenatal period was uneventful and a Caesarean section was done at 33 weeks when the patient presented with preterm labour. Both infant and mother are well, eight months after delivery.