Superiority of magnetic resonance imaging (MRI) over computerized tomography (CT) in diagnosis of hypopituitarism]

Hypopituitarism can be a result of various lesions of hypothalamus, pituitary stalk, or of the pituitary gland itself. The aim of the study was to assess the value of CT and MRI examinations in determination of the cause of hypopituitarism. Seventeen patients with hypopituitarism (9 women and 8 men) aged 22 to 61 years have been examined. In three cases growth deficiency was observed, 4 women had galactorrhoea, 4 patients had diabetes insipidus, 16 patients had supra-adrenal insufficiency, 14 had signs of hypogonadism and 10 hypothyroidism. In each case plasma concentrations of LH, FSH, PRL, TSH, alpha-subunit, ACTH before and after appropriate stimulation with TRH, metoclopramid, LH-RH, GRF or metyrapon were determined with RIA. Every patient was examined both with CT and MRI (0.5 T Toshiba MRT 50a). All 17 patients had abnormal MR images of hypothalamo-pituitary area, while only 10 of them had abnormalities in their CT scans. In remaining 7 patients the MRI revealed: three cases of congenital malformation of hypophyseal stalk, two cases of empty sella, one posttraumatic lesion of the stalk and one case of granulomatous infiltration of the stalk. We found MRI superior to CT in establishing the case of hypopituitarism.     

Insulin hypoglycemia test and releasing hormone (corticotropin-releasing hormone and growth hormone-releasing hormone) stimulation in patients with pituitary failure of different origin

To investigate the efficacy of endocrine evaluation in diagnosing and localizing the cause of anterior pituitary failure, 17 patients with suprasellar space-occupying lesions, 4 patients with intrasellar tumors, 8 patients with no detectable anatomical lesion, 1 patient with posttraumatic failure and 1 patient with septooptical dysplasia were investigated. Endocrine evaluation consisted of measuring adrenocorticotropic hormone (ACTH), cortisol, and growth hormone (GH) levels during insulin hypoglycemia test (IHT) and after administration of corticotropin-releasing hormone (CRH) and growth hormone-releasing hormone (GRH). In addition, basal prolactin levels, gonadal and thyroid function were evaluated. The results showed that 4 of 17 patients with suprasellar tumors had normal ACTH and GH responses during IHT and after releasing hormone (RH) administration. Five of these patients had a normal ACTH or cortisol rise but no GH response during IHT. All 5 had a normal ACTH and 3 had normal GH rise after RH. Seven patients with suprasellar tumors had no ACTH or GH response during IHT, but all had an ACTH response to CRH. Only 3 of this group had a GH response to GRH. There was one exception of a patient who showed a GH and ACTH rise during IHT but only a blunted ACTH and no GH rise after RH administration. Four patients with pituitary failure and no demonstrable lesion had an ACTH rise after CRH but no GH rise after GRH, whereas in 3 patients with isolated ACTH deficiency no ACTH rise after CRH was seen. In 4 patients with nonsecreting pituitary tumors normal ACTH responses to IHT and CRH were seen, whereas GH rose during IHT only in 1 patient.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cushing Disease Revealed by Bilateral Atypical Central Serous Chorioretinopathy: Case Report

ObjectiveWe report the case of a patient with Cushing disease revealed by bilateral central serous chorioretinopathy (CSCR).MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year- old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed.MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year- old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed.ResultsA 53-year-old patient initially presented to the Department of Ophthalmology with a 4-week history of decreased vision in the left eye. Standard ophthalmologic examination and fluorescein angiography established the diagnosis of bilateral CSCR. Systemic clinical signs and biochemical analysis indicated hypercortisolism. Magnetic resonance imaging (MRI) of the pituitary gland showed a left-side lesion compatible with a microadenoma. The diagnosis of Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome secondary to a pituitary microadenoma was selected. Endoscopic endonasal transsphenoidal surgery was performed and the pituitary adenoma was successfully removed. The histology confirmed the presence of ACTH-immunopositive pituitary adenoma. Early postoperative morning cortisol levels indicated early remission. At 6 weeks postoperatively, the patient’s morning cortisol remains undetectable, and serous retinal detachments had regressed.ConclusionCSCR is an uncommon manifestation of endogenous Cushing syndrome. It can be the first presentation of hypercortisolism caused by Cushing disease. CSCR should be considered when assessing patients with Cushing syndrome complaining of visual disorders. On the other hand, it is useful in patients with an atypical form of CSCR to exclude Cushing’s syndrome. (Endoer. Praet. 2013;19:el29-el33)     

Ectopic Acth-Secreting Syndrome: A Single-Center Experience

ObjectiveEctopic adrenocorticotropic hormone (ACTH)-secreting syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze clinical, biochemical, and imaging characteristics; management strategies; and outcomes of EAS patients.MethodWe screened the records (1993-2012) of ACTH-dependent endogenous hypercortisolism cases managed at a tertiary care center.ResultsOf the 218 patients, 17 were diagnosed with EAS. The median 8:00 AM serum cortisol was 36 μg/dL (11.4-82.7 μg/dL), and the median basal plasma ACTH was 156 pg/mL (53.5-468 pg/mL). Notably, ACTH levels below 100 pg/mL were found in 4 patients. Suspicious microadenoma was found on magnetic resonance imaging (MRI) of the pituitary in 5 patients, and all of them underwent transsphenoidal surgery (TSS). Inferior petrosal sinus sampling (IPSS) was performed in 8 patients, and the results were suggestive of a peripheral source in all 8. Computed tomography (CT) localized the lesion in 15/17 patients. In 2 patients with negative CTs, gallium DOTATATE positron emission tomography (PET) scans localized the lesion. Despite difficulties localizing bronchial carcinoids, the cure rate was high (72%). In contrast, thymic carcinoids were easily localized but had poor outcomes.ConclusionEAS cannot be ruled out on the basis of marginally elevated ACTH. In cases with an equivocal MRI pituitary finding, prior IPSS can help avoid unnecessary TSS. CT is a useful modality for localization of an ectopic source. Functional imaging may help in cases where anatomical imaging fails. (Endocr Pract. 2013;19:1007-1014)     

Intraoperative cytologic diagnosis of symptomatic carcinoma (pulmonary small cell carcinoma) metastatic to the pituitary gland: a case report

BACKGROUND: Carcinoma metastatic to the pituitary gland is infrequent and has been reportedly detected in approximately 1% of pituitary surgical cases. It may masquerade as a pituitary adenoma both clinically and radiologically. CASE: A 49-year-old man presented with a 1-month history of severe headache, diplopia and blurred vision. Neurologic examination revealed bitemporal hemianopsia and left sixth nerve palsy. The initial radiologic diagnosis based on magnetic resonance imaging was pituitary adenoma. A biopsy of the lesion was performed. While intraoperative frozen section examination could not completely exclude an "atypical" pituitary adenoma, cytologic touch imprint findings were diagnostic of metastatic small cell carcinoma. Subsequently, additional workup revealed that the patient had a mass lesion in the right lung and right-sided mediastinal lymphadenopathy on chest computed tomography. This was a rare case of pituitary metastasis as the first manifestation of an occult malignancy. CONCLUSION: For intraoperative diagnosis at the time ofpituitary surgery, cytologic imprints can be used reliably to make a diagnosis not only of pituitary adenoma but also of metastatic lesions. It is appropriate in current neuropathology practice that the imprint method be used as the sole modality for intraoperative consultation for pituitary lesions.     

Transformation of a Pituitary Macroadenoma into to a Corticotropin-Secreting Carcinoma Over 16 Years

ObjectiveTo describe a case of a pituitary macroadenoma that differentiated into a corticotropin (ACTH)-secreting carcinoma with metastasis to the thigh.MethodsWe present a case report with a 16-year follow-up that includes anatomic and endocrine documentation of the history of an ACTH-secreting carcinoma.ResultsA 32-year-old woman presented for evaluation in 1989 because of visual feld defects. Magnetic resonance imaging revealed a locally invasive 3-cm macroadenoma. She had no clinical signs of cortisol excess. The patient underwent surgical debulking followed by a course of radiation directed to the pituitary. Results from retrospective immunohistochemical staining with antibodies against ACTH, prolactin, and MIB-1 were negative. Postoperatively, she could not be weaned from exogenous steroids without developing symptoms of adrenal insuffciency. In 1995, she developed left facial palsy and diplopia caused by tumor growth. In 1997, the patient developed progressive symptoms of cortisol excess, which continued after exogenous steroid replacement was discontinued. The patient’s clinical status continued to deteriorate because of local mass effect from tumor growth and uncontrolled hypercortisolism. She underwent bilateral adrenalectomy in 2003. The patient remained debilitated in a long-term care facility for 2 years when she was found to have a mass on her left hip. Biopsy results of the obturator muscle revealed metastatic tumor of neuroendocrine origin with strong reactivity to ACTH antibodies and MIB-1 labeling in 8% of tumor cell nuclei.ConclusionA pituitary tumor can transform into an ACTH-secreting carcinoma in an indolent manner. Patients with invasive pituitary adenomas require long-term surveillance to monitor for differentiation into pituitary carcinoma. (Endocr Pract. 2007;13:463-471)     

Pituitary adenoma showing intermittent secretion of high molecular weight adrenocorticotropin without evidence of Cushing's disease

A 29-year-old woman was admitted in March 1998 due to high plasma ACTH levels, amenorrhea and uncontrolled diabetes mellitus (DM) which had persisted since 1991. Plasma ACTH levels showed a wide range of changes: they were usually high (59-240 pg/ml), intermittently very high (336-942 pg/ml), and sometimes normal or low. Plasma cortisol levels were usually normal but were sometimes high when the ACTH levels were very high. However, even when the plasma ACTH levels were very high, she did not show any cushingoid features. DM was diagnosed as non-insulin-dependent DM. Plasma ACTH showed an excessive response to CRH, while cortisol showed a delayed response. Plasma cortisol showed a poor response to ACTH-(1-24). ACTH receptor gene analysis revealed no mutations in the ACTH receptor-coding region. MRI showed a nonenhancing mass on the left side of the pituitary. Cavernous sinus sampling showed a very high plasma ACTH level in the left cavernous sinus compared with the levels in the right cavernous sinus and peripheral blood. Sephadex G-75 gel filtration of plasma ACTH immunoreactivity in plasma obtained by cavernous sinus sampling showed mainly high molecular forms of ACTH, probably proopiomelanocortin and ACTH-beta-lipotropin. This case is a very rare form of pituitary adenoma showing intermittent secretion of high molecular ACTH unaccompanied by cushingoid features.     

Reduction of False-Negative Results in Inferior Petrosal Sinus Sampling with Simultaneous Prolactin and Corticotropin Measurement

ObjectiveTo investigate the value of prolactin as an independent marker of catheter placement to improve the diagnostic accuracy of inferior petrosal sinus sampling (IPSS) in patients with corticotropin-dependent Cushing syndrome.MethodsIn this retrospective cohort study, we reviewed hospital records of patients who underwent IPSS procedures at the Cleveland Clinic between 1997 and 2009. Serum prolactin and plasma corticotropin levels were measured prospectively in peripheral and inferior petrosal sinus (IPS) samples.ResultsForty-one patients underwent 42 IPSS procedures at our institution during the study period. Among35 patients with Cushing disease, 1 patient had erroneous IPSS results: all pre-corticotropin-releasing hormone (CRH) and post-CRH IPS to peripheral (IPS:P) ACTH ratios were less than 2 and less than 3, respectively. Despite radiologic evidence of appropriate IPS catheter placement, concurrent IPS:P prolactin ratios indicated that successful IPS venous sampling was not achieved. A second case with equivocal IPSS results could also be explained by corresponding IPS:P prolactin ratios. During IPSS, all patients with an identifiable ACTH-staining adenoma localizing to 1 side of the pituitary gland (n = 22) who demonstrated absent IPS:P ACTH gradients (< 2 before or < 3 after CRH administration) on the ipsilateral side of the corticotroph adenoma had corresponding IPS:P prolactin ratios less than 1.3.ConclusionsMeasurement of prolactin during IPSS testing may reduce false-negative results in patients with Cushing disease who do not demonstrate an appropriate central-to-peripheral ACTH gradient. In our series, all false-negative IPS:P ACTH ratios had a corresponding IPS:P prolactin ratio less than 1.3. (Endocr Pract. 2011;17:33-40)     

May primary empty sella turcica be a cause of isolated ACTH deficiency? A case report and the review of related literature

Isolated ACTH deficiency is an uncommon cause of secondary adrenocortical insufficiency and accompaniment with primary empty sella has been reported in several cases. We present a case of isolated ACTH deficiency associated with empty sella. A sixty-two year old woman was admitted to our endocrine clinic with complaints of weakness, fatigue, weight loss, nausea, vomiting, and lack of appetite for about one month. Physical examination indicated orthostatic hypotension and epigastric tenderness. Laboratory investigations revealed hypoglycemia, hyponatremia and anemia, in addition low plasma cortisole and ACTH levels. Serum cortisole responses to short and prolonged ACTH stimulation were tested and partial and accurate responses were obtained, respectively. Plasma ACTH and serum cortisole levels failed to respond after intravenous injection of human corticotropin releasing hormone. Other hypophysial hormone levels were within the normal reference ranges. Although cranial and abdominal computerized tomography images were evaluated as normal, cranial magnetic resonance imaging of the pituitary gland revealed 'primary empty sella turcica'. Replacement therapy with methylprednisolon resulted in the improvement of hypoglycemia, hyponatremia and clinical symptoms. Based on these results, the patient was diagnosed as isolated ACTH deficiency and was scheduled for follow up by our outpatient clinic. Our report is consistent with other reports pointing out that primary empty sella may be responsible for pathogenesis of isolated ACTH deficiency.     

Apoplexy of clinically silent pituitary adenoma during prostate cancer treatment with LHRH analog

LHRH analogs have become a promising modality in prostate cancer therapy as an alternative to surgical castration, and the use of these agents is generally considered to be safe. Since now, only few cases of an apoplexy of previously undiagnosed pituitary adenoma (usually gonadotropinoma) at the beginning of therapy have been described in the medical literature. We present a case of a 74 year old patient who was diagnosed of prostate cancer at the age of 68. There was no evidence of metastatic disease. Radical prostatectomy was performed and LHRH analog gosereline (Zoladex 3.6 mg s.c.) was administered. During the first day after gosereline injection the patient developed headaches that became more severe over the next 3 days. Then the patient experienced nausea and vomiting, double vision and eyelid ptosis. On the 5th day the patient temporarily lost consciousness and was admitted to hospital. Imaging (computerized tomography, magnetic resonance imaging) revealed the presence of a pituitary tumor and hemorrhage within the gland. There was no evidence of pituitary dysfunction in hormonal studies. Neurosurgical intervention was postponed for 5 days after admission. Pathological mass with signs of recent hemorrhage was removed via transsphenoidal route. The tumor had negative immunohistochemical GH, ACTH and PRL staining. Neurological impairment resolved within 9 months after the operation. As a result the patient required adrenal and thyroid replacement. During 6 years of follow-up there was no evidence of prostate cancer recurrence.     

Two-site immunoradiometric assay for adrenocorticotrophin: a cautionary study about the reactivity to its precursor molecules

The specificity of a "two-site" immunoradiometric assay (IRMA) has been reevaluated by examining its ability to detect heterogeneous adrenocorticotrophin-like immunoreactivity (ACTH-LI) separated by gel column chromatography. Plasma samples from patients with Addison's disease, Nelson's syndrome and ectopic ACTH syndrome and tissue extract of human anterior pituitary were subjected to ACTH-IRMA and the levels of ACTH-LI were compared with those measured by conventional ACTH-radioimmunoassay (RIA). The level of ACTH-LI measured by IRMA was considerably lower than that measured by RIA in the plasma of a case of ectopic ACTH syndrome and the ACTH-LI did not show a dilution curve parallel with that of the standard. Gel exclusion chromatography revealed that the plasma contained a relatively large quantity of "big ACTH" which was found to be poorly detected by the IRMA. In the plasma of Addison's disease or the extract of pituitary gland in which "big ACTH" constituted a small portion, whole ACTH-LI was apparently diluted in parallel with the ACTH standard, although the "big ACTH" also did not show full parallelism with the ACTH standard in the IRMA. These data suggest that "big ACTH" derived not only from an ectopic ACTH-producing tumour but also from a normal human pituitary gland cannot be detected as well as authentic ACTH by the ACTH-IRMA system. Therefore, samples which contain a relatively large proportion of "big ACTH" in the total ACTH-LI should be carefully evaluated by ACTH-IRMA.     

ACTH- and prolactin-producing pituitary gland microadenoma with biphasic features of atypia and intermediate filament expression

Herein, we report the case of a 28-year old woman clinically presenting with unclear weight gain over the last years. The patient displayed facial and neck edema in combination with unobtrusive striae distensae. Endocrinological examinations led to the diagnosis of Cushing's disease. Neuroradiological examination revealed an intrasellar tumor mass of 7 mm in diameter. Subsequently, transsphenoidal tumor resection was performed. Histological and immunohistochemical investigations revealed a pituitary gland adenoma showing a biphasic tumor growth pattern with two morphologically different tumor areas producing ACTH and prolactin respectively. Co-expression of ACTH and prolactin is exceedingly rare in pituitary adenoma. To our surprise, both tumor areas exhibited features of atypia consisting in elevated MIB-1 proliferation index in the ACTH-producing portion as well as p53 expression selectively in the prolactin-producing tumor parts. To our knowledge, this is the first case of an ACTH- and prolactin-producing pituitary gland adenoma exhibiting biphasic features of atypia.     

PROP1与联合垂体激素缺乏症

垂体特异性转录因子祖先蛋白(PROP l),是成对同源转录因子,在垂体腺中呈特异性表达,参与早期胚胎垂体的发育,因此,PROP1基因对于垂体前叶的发育是必需的。PROP1启动胚胎期垂体特异性转录因子(PIT-1)的起始表达并维持个体出生后的持续表达,且可直接促使PIT-1细胞系的前体分化为促性腺细胞系。其基因突变可使人、鼠患有联合垂体激素缺乏症(CPHD),表现为生长激素(GH)、促乳素(PRL)、促甲状腺素(TSH)以及促黄体激素(LH)、促卵泡激素(FSH)或促肾上腺皮质激素(ACTH)缺乏,垂体核磁共振成像显示垂体萎缩。在其它哺乳动物中PROP1突变也会引起垂体和性腺激素异常。就PROP1基因的结构与功能,以及与CPHD间的关系作一综述。     

Cytopathologic features of pituitary carcinoma with cervical vertebral bone metastasis: a case report

BACKGROUND: Pituitary carcinomas are extremely rare tumors of the adenohypophysis. The presence of craniospinal and/or systemic extracranial metastases is the only reliable criterion for the diagnosis of pituitary carcinoma. To date, only 2 cases have been reported correctly by fine needle aspiration biopsy (FNAB). We present an additional case of pituitary carcinoma with FNAB features. CASE: A 60-year-old woman presented with clinical features of Cushing's disease and a pituitary tumor. She underwent transsphenoidal resection of the tumor. The initial diagnosis was an adrenocorticotrophic hormone (ACTH)-producing invasive pituitary adenoma. The patient presented again with neck pain 6 years after the operation. Magnetic resonance imaging revealed metastatic tumor masses at the level of C5-C6 of the cervical vertebrae. Intraoperative fine needle aspiration and incomplete excision of metastatic tumors were performed. Cytologically, tumor cells were composed of a combination of loose groups and single cells. Neoplastic cells had a relatively monotonous appearance and displayed characteristic neuroendocrine tumor features. Immunocytochemistry from cell block sections revealed AE1/ AE3, synaptophysin chromogranin A and ACTH positivity in the tumor cells. CONCLUSION: Pituitary carcinoma with extracranial systemic metastases demonstrates typical neuroendocrine features on fine needle aspiration. In the differential diagnosis, metastatic neuroendocrine carcinomas should be kept in mind. In the absence of sufficient clinical data, these 2 entities cannot be distinguished correctly through the cytologic features.     

Immunohistochemical studies on the development of avian embryo pituitary corticotrophs under normal and experimental conditions

Summary Immunofluorescent ACTH cells are present in the developing chick pituitary gland from the 9th day of incubation.Rathke pouch grafts from 4–5 day or 5.5 and 6.5 day-old chicks, grafted into chick chorioallantoic membrane and grown for 12 days, gave rise to tinctorially normal pituitary glands in both cases.The early grafts were of pouch epithelium alone, separated from mesenchyme by trypsinization. The later grafts were surrounded by their attached mesenchyme, from which they are virtually inseparable.In 17 out of 18 of the 4.5 day grafts no immunofluorescent ACTH cells developed. (In the 18th case a few feebly stained single cells). In 16 out of 30 of the 5.5 and 6.5 day grafts ACTH cells were present in normal numbers.Of the 3 hypotheses put forward to explain these findings only one appears valid. This is that the ACTH cells are contributed directly by the mesectoderm (neural crest) surrounding the 5.5 and 6.5 day pituitary primordia.     

Pulmonary Atypical Carcinoid Tumor with Metastatic Involvement of the Pituitary Gland Causing Functional Hypopituitarism

ObjectiveTo report a unique case of a peripherally located pulmonary atypical carcinoid tumor with metastatic involvement of the pituitary, manifesting with evidence of functional hypopituitarism and compressive symptoms of dysarthria, bitemporal loss of vision, and ataxia.MethodsWe present a case report, including detailed laboratory, radiologic, and pathologic findings in a 50-year-old woman with a peripherally located pulmonary atypical carcinoid tumor and a lesion metastatic to the pituitary gland. The pertinent literature is also reviewed.ResultsA 50-year-old woman with a medical history of metastatic pulmonary atypical carcinoid tumor presented with symptoms of bitemporal hemianopia, ataxia, and dysarthria. Laboratory evaluation revealed functional hypopituitarism, and magnetic resonance imaging of the brain with use of gadolinium contrast demonstrated a pituitary lesion measuring 3.5 by 2.5 by 2.5 cm. Visual field testing revealed bitemporal superior quadrantanopia, consistent with a pituitary lesion of the chiasm. Computed tomographic angiography excluded the presence of an aneurysm of the internal carotid artery. Subsequently, the patient underwent subtotal transsphenoidal hypophysectomy. Findings on pathology examination were consistent with a lesion metastatic from the primary pulmonary atypical carcinoid tumor. The patient subsequently received monthly octreotide injections as well as chemotherapeutic regimens of capecitobine and temozolomide. At 18-month follow-up, disease had not recurred.ConclusionThis case of metastatic involvement of the pituitary gland from a peripherally located pulmonary atypical carcinoid tumor manifesting with evidence of functional hypopituitarism is highly uncommon. To our knowledge, such a case has not been previously reported. The presentation of an advanced pituitary metastatic lesion can be particularly dramatic, and the course can be potentially complicated. Therefore, heightened awareness of the possibility of a pituitary lesion metastatic from a pulmonary atypical carcinoid tumor is important. (Endocr Pract. 2007;13:291-295)     

Moyamoya syndrome following growth hormone-secreting pituitary adenoma

We report a case of Moyamoya syndrome developing in association with growth hormone-secreting pituitary adenoma. A 31-year-old female presented with acromegalic features. Magnetic resonance imaging revealed a 1 × 2 cm tumor in the sella turcica and MR angiography demonstrated unremarkable findings. Blood growth hormone and insulin-like growth factor I levels were elevated to 74.1 ng/ml and over 1 575 ng/ml, respectively. The diagnosis was growth hormone-secreting pituitary adenoma, and the tumor was removed through a transsphenoidal approach. Four years after surgery, she visited the outpatient department due to left side weakness for 2 months. Magnetic resonance images showed acute and old infarcted lesions in the basal ganglia and subcortical area and residual small pituitary adenoma in the sellar area. MR angiography demonstrated stenosis of the bilateral distal internal carotid arteries with basal collateral vessels. Conventional cerebral angiography showed complete obstruction in the right internal carotid artery and severe stenosis of the left internal carotid artery, middle cerebral artery, and anterior cerebral artery with basal collateral vessels. Her blood growth hormone and insulin-like growth factor I levels were 15.3 ng/ml and 1 055 ng/ml, respectively. We believe that excess systemic exposures of growth hormone and insulin-like growth factor I may participate in the development of Moyamoya syndrome.     

Ectopic corticotrophin-releasing-factor and growth hormone releasing factor secretion: diagnosis using human pituitary cell culture

Cell culture of human pituitary tissue has been used to diagnose a patient with Cushing's syndrome due to ectopic secretion of corticotrophin-releasing factor (CRF; case 1) and a case of acromegaly associated with ectopic secretion of a growth-hormone releasing factor (GRF; case 2). In both patients a pituitary tumour was not detected. Case 1 had a small cell carcinoma and symptoms of the ectopic ACTH syndrome, but in culture the carcinoma failed to secrete detectable ACTH. However, the culture medium used to maintain this carcinoma in vitro was found to contain a substance which stimulated ACTH secretion by human pituitary corticotrophs in cell culture. Radioimmunoassays and HPLC indicated that this substance had similar elution characteristics to human CRF and cross-reacted with antiserum to ovine CRF. Case 2 was found to have a lung tumour, the removal of which led to regression of her acromegalic symptoms. In culture, this tumour did not secrete GH, but did secrete a GRF. We conclude that the Cushing's syndrome and acromegaly, in cases 1 and 2, respectively, were due to ectopic secretion of CRF and GRF leading to hyperstimulation of the pituitary gland.     

朗格罕细胞组织细胞增生症颅内垂体影像表现及相关临床表现

目的:探讨朗格罕细胞组织细胞增生症累及垂体的MR表现及相关临床表现。方法:搜集了6例确诊为朗格罕细胞组织细胞增生症并垂体表现异常的患儿,男5例,女1例,年龄2～11岁,平均(6±3)岁,对其影像及临床表现进行回顾性分析。结果:临床患儿主要以头面部包块,多饮、多尿等就诊。头颅MR平扫(T1WI)表现6例患儿神经垂体高信号全部消失,垂体柄增粗5例,垂体柄著征1例,垂体饱满1例,其中3例治疗后复查垂体及垂体柄均有变化。结论:神经垂体高信号消失,垂体柄增粗或著征为朗格罕细胞组织细胞增生症累及垂体的头颅MR表现。累及垂体者临床几乎都有多饮、多尿表现。目前,MRI是诊断朗格罕细胞组织细胞增生症累及垂体的唯一可靠的影像学检查方法,并对治疗后病情随访有重要作用。     

Sex differences in adrenocortical structure and function. XXVIII. ACTH and corticosterone in intact, gonadectomised and gonadal hormone replaced rats

Plasma ACTH and corticosterone (B) concentration, ACTH content in the anterior pituitary gland and B content in the adrenals were measured in intact, gonadectomised and testosterone or estradiol replaced rats. Plasma ACTH and B levels and adrenal B content were higher in female than male rats. Neither orchiectomy nor testosterone replacement had an effect on plasma ACTH and B concentration. Orchiectomy did not affect adrenal B content and decreased pituitary ACTH while testosterone significantly lowered ACTH and B content in studied glands. On the other hand ovariectomy did not change pituitary ACTH and adrenal B content and notably lowered concentrations of these hormones in the blood. Estradiol replacement resulted in an increase in plasma ACTH and B concentrations, an effect accompanied by a marked drop in pituitary ACTH and an increase in adrenal B. These findings indicate the distinct sex differences in basal plasma ACTH and B concentrations with higher values in female rats, an effect dependent on the stimulatory action of estradiol on pituitary-adrenocortical axis.