Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. There are presently no markers that can reliably distinguish benign and malignant neuroendocrine tumors. Here we describe a 63-year-old woman who developed sudden chest pain and hypertension combined with increased stool frequency. An incidental adrenal mass 5 cm in size with a bright signal on T2-weighted magnetic resonance imaging was discovered. Biochemical evaluation and (131)I-metaiodobenzylguanidine (MIBG) scintigraphy were negative. Histopathological examination revealed a mature adrenal GN. Neuroblastoma, the immature form of a GN, is known for deletions on chromosomal locus 1p36, and adrenal tumors frequently show allele loss on 17p. To further elucidate the histo- and pathogenesis of adrenal GN, we performed loss of heterozygosity studies on chromosomal loci 1p34-36 and 17p13 (the p53 gene locus) after careful microdissection of tumor and normal tissue. We did not detect allelic losses at these loci with the informative polymorphic markers used, suggesting that these loci are not involved in tumorigenesis. In addition, immunohistochemical investigation of the GN was positive for vasoactive intestinal peptide, a hormone commonly expressed in ganglion cells. We suggest that in our patient with an adrenal GN, the combination of biochemical, scintigraphic, molecular, immunohistochemical, and histopathological findings are all consistent with the benign morphology of this tumor.
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PMID:Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. 1265 73

A 4-cm paravertebral mediastinal tumor was resected in a 70-year-old male patient treated for hypertension. The tumor displayed both paraganglioma and ganglioneuroma areas that were in equal proportion and often merged one into the other. Paraganglioma areas contained synaptophysin and chromogranin-positive chief cells and PS100-positive sustentacular cells. Ganglioneuroma areas contained neurofilament-positive mature ganglion cells and PS100-positive Schwann cells. Such pheochromocytoma-ganglioneuroma has not been previously reported in the mediastinum and appears as the adrenal and aorticosympathetic counterpart of gangliocytic paraganglioma described in other anatomic sites.
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PMID:Paraganglioma with ganglioneuromatous component located in the posterior mediastinum. 1580 20

A 35-year-old man presented with a 17-month history of abdominal distension and left upper quadrant pain. He had no episodes suggesting the presence of hypertension. Hormonal studies were insignificant. Imaging studies demonstrated a bulky tumor with cyst formation measuring 22 cm. Preoperative differential diagnoses included an adrenal tumor, extra-adrenal retroperitoneal tumor and pancreatic mucinous tumor. Intraoperatively, no abnormality was observed in the pancreas. A tight adhesion of the tumor to the left kidney necessitated an en bloc resection of the tumor with the left kidney. The resected specimen, 22 x 20 x 8 cm, weighed 5,050 g. Pathologically, the tumor was ganglioneuroma originating from the left adrenal gland. Convalescence was uneventful, and abdominal symptoms disappeared. The patient has been doing well without evidence of recurrece 48 months after the operation.
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PMID:[A case of cystic ganglioneuroma of adrenal gland presenting as a huge retroperitoneal mass]. 1691 May 88

A majority of incidentally found adrenal tumors derive from the adrenal cortex. The aim of our study was evaluation of the incidence of chromaffin tumors in a group of 1,111 patients with adrenal incidentalomas. In this group there were 803 women and 308 men, aged 10-87 years. Clinical examination, imaging studies (ultrasound scans, CT, and MRI if necessary), and hormonal determinations (cortisol, ACTH and androgens in the blood, dexamethasone suppression test, urinary excretion of 17-OHCS, aldosterone and 17-KS, as well as PRA/aldosterone stimulation test and metanephrines in hypertensive patients and those with density in CT over 20 HU) were used. In 380 patients treated by surgery (mainly by laparoscopic approach), histological and immunocytochemical examinations were performed. Clinical examination revealed hypertension in 25% of the patients under study. Chromaffin tumors were detected in 43 patients, 33 women, and 10 men aged 20-75 years: pheochromocytoma in 36 (malignant in 3); chromaffin cells hyperplasia in 2; paraganglioma in 3; ganglioneuroblastoma in 1; ganglioneuroma in 1; and schwannoma in 2. The tumors' diameter ranged between 1.1 and 20.0 cm, density=25 Hounsfield units (HU) or more before contrast medium injection. Hypertension was present in 53% of these patients. The urinary metanephrines excretion was elevated in 31 of 38 patients, in whom the determinations were done. Chromaffin tumors were detected in 4% (pheochromocytomas in 3%) of 1,111 patients with adrenal incidentalomas. Malignancy was present in 9% of 43 patients with chromaffin tumors. High density in CT was a very important diagnostic finding in the incidentally found medullary tumors.
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PMID:1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. 1710 70

Ganglioneuromas (GN) are neural crest cell-derived tumors which may coexist with pheochromocytomas, secrete various neuropeptides or the symptoms may mimic that of a pheochromocytoma, producing hypertension or a hypotensive crisis during anesthesia for these tumors. We report here the case of a 7-year-old female child with an adrenal tumor suspected to be a pheochromocytoma, later confirmed by histology as a GN. This child presented with episodic headache, anxiety, palpitations and 3D helical (spiral) computed tomography of the abdomen revealed an adrenal tumor. In addition, the child was diagnosed to have a seizure disorder. She was managed as a pheochromocytoma. Although the child's preoperative catecholamine levels were normal, tumor manipulation caused a hypertensive crisis. We discuss the clinical characteristics of this unusual case, anesthesia management, and the postoperative course.
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PMID:Anesthesia management of a ganglioneuroma with seizures presenting as pheochromocytoma. 1747 56

Watery diarrhoea, hypokalaemia and achlorhydria (WDHA) syndrome was caused by vasoactive intestinal polypeptide (VIP)-producing tumour. A 3-year-old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal VIP-secreting ganglioneuroma is reported. The girl coughed, fevering up to 39 degrees C after a flu-like episode. She had eight to ten abundant stools daily which is not improved by dietary treatment, resulting in an important weight loss. She weighed 6.8 kg (nl P50 at 6 months of age) and is 76 cm (nl P50 at 9 months of age) in height. Blood electrolytes showed 129 mmol/L sodium, 2.42 mmol/L potassium, 94 mmol/L chloride and 18.6 mmol/L bicarbonate; urinary catecholamines were normal. Computed tomography scan evidenced a left side paravertebral mass of 4 x 6 cm in the lower thoracic region leading to the blood determination of vasoactive intestinal polypeptide which amounted 830 pmol/L(normal < 25 pmol/L). Surgical removal showed a ganglioneuroma of 160 g and was associated with disappearance of the diarrhoea and normalization of VIP level below 20 pmol/L. Review of the 63 reported cases in children with WDHA showed that many of the cases presented with non-treatable watery diarrhoea, hypokalaemia. Achlorhydria is not necessarily part of the WDHA syndrome. The male to female ratio is 1:1.5. Ganglioneuroblastoma and ganglioneuroma are the commonest tumours. Location of the tumour is variable: abdomen, chest or neck. Abdominal distension, flushing, episodic hypertension and colonic dilatation, constipation and ataxia were the other associated features. Surgical resection is the treatment of choice of VIP-producing tumours.
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PMID:Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl. 1910 28

A 64-year-old man presented with gross painless hematuria. Cystoscopy revealed a submucosal bladder neck mass covered by normal urothelium. During transurethral resection, the patient developed hemodynamic crisis including sinus bradycardia. Histopathologic examination revealed a primary bladder composite paraganglioma-ganglioneuroma (CPG). The patient underwent partial cystectomy and is symptom-free after one year. Bladder CPGs are extremely rare neoplasms that may result in life-threatening catecholamine secretion, especially during tumour manipulation. These tumours require complete surgical excision and should be included in the differential diagnosis of any solitary bladder mass covered by normal urothelium, especially when there is a history of hypertension or micturition attacks.
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PMID:Composite paraganglioma-ganglioneuroma of the urinary bladder: a rare neoplasm causing hemodynamic crisis at tumour resection. 1982 17

A 28-year-old woman was referred to our hospital complaining of upper abdominal discomfort. The patient had been receiving medical treatment for hypertension. Computed tomography revealed a 30 mm solid tumor with calcification in the left adrenal gland and a 8 mm nodule in the right adrenal gland. Endocrinological examinations revealed no activity of either adrenal mass. The left adrenal tumor was extirpated, because malignancy of the tumor was not ruled out. Histopathological examination proved that the tumor was ganglioneuroma arising from the extra-adrenal retroperitoneum.
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PMID:[Ganglioneuroma with calcification mimicking adrenal tumor: a case report]. 2118 6

The ganglioneuroma is a tumor of the sympathetic nervous system and may be associated with hypersecretion of vasoactive substances responsible for various symptoms and signs such as hypertension. The authors report a case of ganglioneuroma and a literature review, focusing on the most important aspects of diagnosis and therapy. The patient had recurrent symptomatic hypertensive crises, having performed an imaging study that found a nodule in the right adrenal gland. As neuroblastic tumors are radiologically undistinguishable, the patient underwent excision, confirming the diagnosis by pathology analysis. This case demonstrates the clinical variability of ganglioneuroma, the usefulness of imaging and the importance of histological study.
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PMID:Secondary hypertension to rare adrenal gland tumor. 2127 Nov 85

We report the case of a 45-year-old female referred to us with progressive shortness of breath and a huge left mediastinal mass. Computed tomography of the chest revealed a mass occupying the posterior mediastinum and extending from the apex caudally to the left hemidiaphragm. Further magnetic resonance imaging demonstrated tumor extension to the left interverteberal foramina of T5-T6 and T6-T7. Excision of the mass was performed through a left posterolateral thoracotomy. Histology confirmed a mediastinal ganglioneuroma. This is an unusual tumor with more than one extension in the spine. Ganglioneuromas are rare tumors of the peripheral nervous system. Most of these tumors are, however, retroperitoneal and are more common in children and young adults. Ganglioneuromas arise from neural crest cells. These tumors are mostly asymptomatic, but some may present with hypertension and flushing. Massive tumors can present with pressure symptoms.
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PMID:Giant ganglioneuroma of the posterior mediastinum. 2169 53


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