1/36. acromegaly associated with a granular cell tumor of the neurohypophysis: a clinical and histological study. Case report.acromegaly is usually caused by a growth hormone (GH)-secreting pituitary adenoma, and hypersecretion of GH-releasing hormone (GHRH) from a hypothalamic or neuroendocrine tumor accounts for other cases. The authors report on the unusual association of acromegaly with a granular cell tumor of the neurohypophysis. A 42-year-old woman with a 10-year history of acral enlargement, headache, and menstrual abnormalities was referred to our department for a suspected GH-secreting pituitary adenoma. The patient's basal GH levels were mildly elevated at 4.8 microg/L, were not suppressed in response to an oral glucose tolerance test, and increased paradoxically after administration of thyrotropin-releasing hormone. The patient's insulin-like growth factor-1 (IGF-1) level was elevated at 462 microg/L, whereas a magnetic resonance image of the sella turcica revealed an intra- and suprasellar lesion that was compatible with a diagnosis of pituitary adenoma. A transsphenoidal approach to remove the lesion, which was mainly suprasellar, was successful during a second operative attempt, resulting in the clinical and biochemical regression of the patient's acromegaly. Four months postoperatively, the patient's basal GH level was 0.9 microg/L and her IGF-1 level was 140 microg/L. Histological analysis of the operative specimen demonstrated a granular cell tumor of the neurohypophysis, which when stained proved negative for pituitary hormones and GHRH. This case represents the first reported association between a granular cell tumor of the neurohypophysis and acromegaly. granular cell tumor of the neurohypophysis could be added to the restricted list of neoplastic causes of acromegaly secondary to hypersecretion of a GH-releasing substance.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
2/36. Acute leukaemia in acromegaly patients.acromegaly patients are known to have an increased risk of malignancies, especially colonic adenocarcinoma. This may be as a result of the growth-stimulating effect of growth hormone (GH). The clustering of leukaemia in children treated with GH has also caused concern. There have been a few reports of leukaemia in acromegaly patients. We report two patients with acute lymphoblastic leukaemia and one patient with acute myeloid leukaemia among 106 acromegaly patients treated over a 15-year period. Two of the cases received radiotherapy as part of their treatment. Adjusted for age and follow-up years, the incidence of leukaemia in this cohort is significantly higher than the general population. The incidence is also higher than would be expected as a result of radiotherapy alone, suggesting that GH may play a synergistic role.- - - - - - - - - - ranking = 0.016455383087179keywords = carcinoma (Clic here for more details about this article) |
3/36. Carcinoid syndrome, acromegaly, and hypoglycemia due to an insulin-secreting neuroendocrine tumor of the liver.We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor's humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor i due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor i and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and c-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation.- - - - - - - - - - ranking = 5keywords = neuroendocrine (Clic here for more details about this article) |
4/36. Hemorrhagic gastric carcinoma in an acromegalic patient.A rare case of hemorrhagic gastric carcinoma in an acromegalic patient is reported. A 79-year-old Japanese man was referred to our hospital with diagnoses of upper gastrointestinal hemorrhage and angina pectoris. This patient showed typical clinical features of acromegaly, with increased serum growth hormone (GH) and insulin-like growth factor i (IGF-I) level. A high titer of serum anti-helicobacter pylori (H. pylori) IgG was also observed. After percutaneous transluminal coronary angioplasty treatment for stenosis of the right coronary artery, the patient underwent distal gastrectomy. Gastric cancer was Type 2 macroscopically and was diagnosed histologically as a papillary and well to moderately differentiated tubular adenocarcinoma. reverse transcription-polymerase chain reaction analysis estimated that the amount of IGF-I receptor mRNA expression in the gastric cancer tissue was 1.6 times higher than that in the adjacent atrophic mucosa, whereas the amount of IGF-I mRNA expression in the cancer tissue was only half that in the atrophic mucosa. Both the stimulatory effects of GH and/or IGF-I on cell proliferation and H. pylori infection in gastric tumorigenesis may have been responsible for the development and growth of gastric carcinoma in this patient.- - - - - - - - - - ranking = 0.11518768161025keywords = carcinoma (Clic here for more details about this article) |
5/36. growth hormone-releasing hormone (GHRH) and GHRH receptor (GHRH-R) isoform expression in ectopic acromegaly.Bronchial endocrine neoplasms causing acromegaly due to ectopic production of growth hormone (GH)-releasing hormone (GHRH) have been reported. We describe the case of a 39-year-old man with clinical and biochemical acromegaly. magnetic resonance imaging revealed an enlarged pituitary, which was confirmed histologically to harbour somatotroph hyperplasia. Further investigations identified a circumscribed central mass in the right lung which was surgically resected and histologically confirmed to be an endocrine tumour with strong immunopositivity for GHRH, synaptophysin and chromogranin; the lesion also exhibited mild positivity for peptide yy, calcitonin gene-related peptide (CGRP), glucagon-like peptide (GLP)-1, corticotrophin-releasing hormone (CRH), tyrosine hydroxylase, vasoactive intestinal peptide (VIP) and enkephalin. S100 protein was identified in stellate cells surrounding nests of epithelial tumour cells. The MIB-1 antibody labelled about 10% of the tumour cells. We established that the tumour not only produced GHRH but the GHRH-receptor (GHRH-R) as well. GHRH and GHRH-R mRNA were identified and the latter was characterized as two variants, a full-length transcript and a truncated splice variant that has been described in human pituitary somatotroph adenomas. We suggest that GHRH expression by this tumour and the presence of its receptor may be responsible for enhanced growth. The expression of a truncated splice variant that is unable to transduce GHRH signalling may be implicated in the less aggressive behaviour of well-differentiated endocrine tumours that produce GHRH compared with small-cell lung carcinomas that are very responsive to GHRH growth stimulation.- - - - - - - - - - ranking = 0.016455383087179keywords = carcinoma (Clic here for more details about this article) |
6/36. acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report.OBJECTIVE AND IMPORTANCE: We describe a patient with acromegaly and pituitary hyperplasia secondary to a growth hormone-releasing hormone-secreting gastrointestinal carcinoid tumor. This case report illustrates the importance of including this rare clinical syndrome in the differential diagnosis of acromegaly for patients with suspected or known neuroendocrine tumors. CLINICAL PRESENTATION: A 19-year-old, Asian-American, male patient with a 2-year history of a nonresectable, metastatic, intestinal carcinoid tumor presented with complaints of headaches, arthralgias, sweats, and changing features. The examination revealed a young subject with acromegalic features, without visual field deficits. magnetic resonance imaging revealed a diffuse sellar mass that extended suprasellarly to compress the optic chiasm. Endocrinological studies demonstrated a growth hormone level of more than 100 ng/ml and an inappropriately elevated growth hormone-releasing hormone level. INTERVENTION: The patient underwent transsphenoidal resection of the pituitary mass for diagnostic and decompressive purposes. The pathological examination revealed pituitary hyperplasia, without evidence of an adenoma. Therapy with long-acting repeatable octreotide (Sandostatin LAR; Novartis AG, Basel, switzerland) was initiated postoperatively, to further control the acromegaly and carcinoid tumor. The soft-tissue swelling resolved, and the patient remained free of headaches, arthralgias, and sweats at the 6-month follow-up examination. CONCLUSION: Ectopic acromegaly is a rare syndrome that must be recognized by neurosurgeons because its treatment differs from that of classic pituitary acromegaly. We describe a patient for whom this syndrome was documented with magnetic resonance imaging, endocrinological testing, and pathological examinations.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
7/36. Hepatocellular carcinoma in a patient with acromegaly and high serum levels of insulin-like growth factor i: report of a case.A 64-year-old woman with high serum levels of growth hormone, insulin-like growth factor-I (IGF-I), and alpha-fetoprotein resulting from partially treated acromegaly was found to have a tumor under the left diaphragm. The patient also had a history of type C viral hepatitis. laparotomy revealed that the tumor was fixed to the diaphragm and connected to the liver and spleen. The tumor was excised with partial resection of the diaphragm, liver, and spleen, and a diagnosis of left-sided pedunculated hepatocellular carcinoma (HCC) was made. Further examination showed a higher level of IGF-I receptor mRNA in the tumor than in the normal liver parenchyma. We believe it is likely that the high serum levels of IGF-I may have played a role in the development of the pedunculated HCC in this patient.- - - - - - - - - - ranking = 0.082276915435894keywords = carcinoma (Clic here for more details about this article) |
8/36. Growth hormone secreting pituitary carcinoma: a case report and literature review.Only five cases of growth hormone secreting pituitary carcinoma have been documented. We present a 49-year-old West Indian male with grossly elevated plasma growth hormone (760-10,400 mU/l), and a large aggressive pituitary tumour that continued to grow despite repeated pituitary surgery, radiotherapy and medical therapy (bromocriptine and somatostatin analogue). Thirteen years after diagnosis the patient died secondary to left ventricular failure. A post-mortem revealed a large locally invasive pituitary tumour, but in addition numerous tumour seedlings within the cerebrospinal fluid space, and a solitary intraparenchymal tumour deposit within the right temporal lobe, clearly separate from the primary tumour. Pituitary carcinoma should be considered in any acromegalic with grossly elevated plasma growth hormone levels who fails to respond to conventional therapy.- - - - - - - - - - ranking = 0.098732298523073keywords = carcinoma (Clic here for more details about this article) |
9/36. A meiotic recombination in a new isolated familial somatotropinoma kindred.We report here the genetic findings of a new isolated familial somatotropinoma (IFS) kindred in which the mother (subject I:2) and one daughter (subject II:2) are affected; their ages at diagnosis were 25 and 14 years respectively. Additionally, patient I:2 developed virilization due to an androgen-secreting adrenocortical mass, presenting clinical and molecular features of sporadic adrenal carcinoma. To genotype this family and to narrow down the candidate interval of the putative IFS gene at 11q13, we performed haplotyping on the dna from all five members of the family and allelotyping of one available somatotropinoma using polymorphic microsatellite markers from chromosome region 11q12.1-11q13.5. Results indicated that the disease haplotype, between markers D11S956 and D11S527, was transmitted from subject I:2 only to subject II:2. A meiotic recombination event was detected in the fraternal twin sister of II:2 (subject II:1), but her disease status is unknown. Since she is only 18 years old this genetic event cannot yet narrow down the area involved in the pathogenesis of IFS. Allelotyping of the somatotropinoma from II:2 revealed loss of the chromosome carrying the wild-type copy of the putative IFS gene inherited from her father. These results support the involvement of a tumor suppressor gene at 11q13.1-q13.3 in the pathogenesis of IFS.- - - - - - - - - - ranking = 0.016455383087179keywords = carcinoma (Clic here for more details about this article) |
10/36. Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells in a patient with acromegaly.Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells (IDHPNC) is a rare disorder that most often presents clinically as obliterative bronchiolitis, but has also been associated with ectopic corticotropin syndrome. We describe a 49-year-old lifetime non-smoking female with longstanding cough, progressive exertional dyspnoea, and fixed airflow limitation, who presented with acromegaly. Head magnetic resonance imaging revealed a pituitary microadenoma and open lung biopsy revealed a multifocal proliferation of neuroendocrine cells which were immunopositive for both corticotropin and growth hormone-releasing hormone. To our knowledge, this is the first report of acromegaly associated with IDHPNC, and supports the possibility that IDHPNC may fall at one extreme of the spectrum of disorders encountered in multiple endocrine neoplasia type 1.- - - - - - - - - - ranking = 6keywords = neuroendocrine (Clic here for more details about this article) |
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