Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027497 (nausea)
 23,468 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 20-year-old woman died suddenly in a hospital emergency room after presenting with nausea, vomiting, back pain, and hypertension. At autopsy, an extra-adrenal pheochromocytoma (paraganglioma) of the organs of Zuckerkandl was found, with microscopic focal myocardial necrosis similar to that described in death from adrenal pheochromocytomas. Tumors of the organs of Zuckerkandl are extremely rare; less than 100 such cases have been reported in the world's literature, and only six, including the present case, have presented as a sudden, unexpected death. The symptoms of catecholamine storm may mimic those of acute drug intoxications, leading to misdiagnosis by both clinical physicians and pathologists.
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PMID:Sudden death due to a paraganglioma of the organs of Zuckerkandl. 301 64

Twelve patients with advanced apudomas--six with carcinoid tumors, two with chemodectomas, two with pancreatic islet cell tumor, and one each of medullary carcinoma of the thyroid and paraganglioma of unknown primary--were treated with a combination of doxorubicin 50 mg/m2 and cisplatin 50 mg/m2 every 3 to 4 weeks. Biochemical markers were present in 8 of the 12 patients. Five of the 12 patients (3 with carcinoid and 2 with chemodectomas) responded with more than 50% regression of tumor size measured as hypothetical area. Three others (two with islet cell tumors and one with carcinoid) had clinical and/or biochemical improvements. A median duration of response was 6 months. Nausea, vomiting, and alopecia were universal. Mild or moderate leukopenia was the most frequent toxicity. No sustained nephrotoxicity was seen. The combination of doxorubicin and cisplatin provides a new palliative therapy for patients with APUD tumors.
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PMID:Doxorubicin plus cisplatin in the treatment of apudomas. 403 25

Gangliocytic paraganglioma (GPG) with local lymph node metastasis was found in the pancreas of a 74-yr-old female who presented with diarrhea, steatorrhea, vomiting, nausea, and abdominal pain. A Whipple procedure led to a complete resolution of these symptoms and a return of an elevated stomatostatin level to normal. This is the first description of GPG in this location and the first endocrinologically active GPG.
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PMID:Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases. 863 24

The incidence of paraganglioma in the paediatric population is exceedingly rare, accounting for < 0.1% of childhood cancers. We report here the response and toxicity profile in a case of malignant paraganglioma which was treated with what is currently perceived as an unconventional and non-standard approach, using three consecutive low doses of 131I-MIBG (a cumulative dose of 11 647.6 MBq). The patient had a stable disease at the end of 42 months follow-up following the first treatment with 131I-MIBG. Excellent symptomatic and hormonal responses were observed. The only adverse effect was mild nausea in the first 24 h after therapy. In addition to the potentially primary end point of radiological and biochemical response measurement, we, in this paper, endeavoured to look for a quality of life evaluation for this form of treatment. Given the rarity of this condition, the experience gained by this therapeutic approach is intriguing from response and toxicity standpoints and may be extrapolated to malignant pheochromocytoma as well. An apparent "reverse discordance" between 131I-MIBG scintigraphy and 99Tc(m)-MDP bone scan encountered during pre-therapy work up is also described with possible explanations. This draws attention to an important clinical issue in selecting patients for high dose targeted therapy.
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PMID:Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy. 1686 19

A 19-year-old female patient presented with headache, nausea, hypertension, visual impairment of the left eye and exertion-related complaints of palpitations since 1 year. Fundoscopy showed severe hypertensive retinopathy grade IV. A paraganglioma in the left para-aortic region was diagnosed by urinary screening and magnetic resonance imaging scanning. The tumor was resected by a robot-assisted laparoscopic procedure. The surgical operating time was 250 minutes and the blood loss was 150 mL. After surgery the blood pressure normalized and antihypertensive drugs were no longer required. Oral intake was resumed on the first postoperative day and the hospital stay was 3 days. Final pathology examination revealed a paraganglioma with pheochromocytomal features of 7x5.1x3.5 cm. Reports on laparoscopic resection of large paragangliomas are scarce. This case has demonstrated that the procedure is feasible and associated with rapid recovery from surgery. The surgical team experienced explicit support by the robot in the dissection of this highly vascularized tumor.
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PMID:Robot-assisted laparoscopic resection of a large paraganglioma: a case report. 1705 84

Several studies have shown that patients with succinate dehydrogenase subunit B (SDHB) mutations have a very high risk for developing malignant paragangliomas. However, there is no consensus of what age screening for paragangliomas should start. We report a case of an 8-year-old white girl with a 3-year history of catecholamine excess-related complaints who was diagnosed with a malignant SDHB-associated mediastinal paraganglioma. The patient presented with intermittent sweating, headache, nausea, vomiting, fatigue and weight loss that had been present since she was 5 years of age. A large posterior mediastinal mass measuring 6.4 cm x 3.1 cm x 4.6 cm was discovered on computed tomography (CT) and magnetic resonance imaging (MRI). Laboratory data included an elevated level of urine normetanephrine of 45,400 microg/g creatinine (upper reference limit 718 microg/g) and elevated level of plasma normetanephrine of 62.4 nmol/l (upper reference limit <0.90 nmol/l). She was diagnosed with a thoracic paraganglioma and subsequently underwent surgical removal of the tumor and two lymph nodes. Histopathologic examination confirmed metastatic paraganglioma. Postoperatively, her blood pressure normalized and plasma normetanephrine levels remained normal. Our patient first presented with paraganglioma-associated signs and symptoms at the young age of 5 years. This case clearly illustrates the need for increased vigilance and screening for paragangliomas in families with SDHB at a younger age than previously thought.
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PMID:Malignant paraganglioma associated with succinate dehydrogenase subunit B in an 8-year-old child: the age of first screening? 1918 36

We report on the efficacy and safety of short-term administration of temozolomide, an inhibitor of nucleoside incorporation, in a 60-year-old woman with widespread hepatic metastases from a malignant paraganglioma. Temozolomide was orally administered in daily doses of 250 mg on days 1 to 5 and repeated every 28 days for five cycles. Clinical improvement was immediate and associated with weight gain, and further reduction in blood pressure without ortho-static intolerance. In addition, abnormal hepatic function was normalized and catecholamine production was significantly reduced. Except for mild nausea, adverse effects were virtually absent. Bone marrow function, renal function, and serum electrolytes remained normal; hemoglobin remained above 9 g/dl through treatment. The platelet count decreased but not to clinically meaningful levels. These responses allowed for a surgical debulking procedure to be performed safely without complications. The results suggest that temozolomide may be useful in presurgical preparation of patients with pheochromocytoma especially in those with widespread metastatic disease and poor physical condition. However, the present findings need confirmation in a larger study and the role of temozolomide in the long-term treatment of malignant paraganglioma/pheochromocytoma remains to be established.
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PMID:Clinical utility of temozolomide in the treatment of malignant paraganglioma: a preliminary report. 1953 32

In this case report, we document the clinical course, laboratory findings, and imaging findings of a 21-year-old pregnant woman with multiple paragangliomas due to a succinate dehydrogenase B (SDHB) mutation. We also review the literature on previously reported cases. The patient presented with nausea, palpitations, angina, and amenorrhea. Her blood pressure was 170/100 mm Hg and her beta-human chorionic gonadotropin was positive. Her blood pressure remained high despite phenoxybenzamine titration. A 24-hour urine analysis revealed elevated plasma metanephrines. Imaging was initially deferred due to early gestational age. After the patient terminated the pregnancy, magnetic resonance imaging revealed a left suprarenal mass, a mass at the aortic bifurcation, and a left periaortic mass. Her blood pressure was controlled on phenoxybenzamine and labetalol. The masses were excised. Pathology revealed paragangliomas. Genetic testing revealed mutation in the SDHB gene. One month later, her blood pressure was 122/86 mm Hg off of medication. Paraganglioma/pheochromocytoma should be suspected when hypertension occurs early in pregnancy. Genetic testing is important, as this may impact future offspring.
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PMID:Multiple paragangliomas in a pregnant patient with a succinate dehydrogenase B mutation. 2108 81

Paragangliomas are tumors arising from sympathetic and parasympathetic tissues. The classic associated syndromes are neurofibromatosis type 1, multiple endocrine neoplasia type 2 and von Hippel-Lindau. Germline mutations of succinate dehydrogenase subunits genes, are associated with familial paraganglioma syndromes 1,2,3 and 4. We report a 29-year-old woman with a family background of pheochromocytoma and history of paroxysmal headache, nausea, sweating, palpitations, associated with severe hypertension. The patient had elevated plasma noradrenalin and urinary normetanephrines. Imaging studies revealed three retroperitoneal extra-adrenal masses. The clinical and laboratory study of classic syndromes associated with paraganglioma was negative. The patient was operated and the pathological study of the surgical specimen was consistent with paragangliomas. The genetic study showed a mutation in the SDHB succinate dehydrogenase gen, Exon 2 of CCTCA c.300_304 (p.P56delYfsX5).
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PMID:[Multiple paragangliomas associated to a SDHB gene mutation: report of one case]. 2244 54

Extra-adrenal paraganglioma with isolated localization in the urinary bladder is a rare neuroendocrine tumor. Although the typical symptoms like headache, nausea, weight loss, flushing, heart palpitation or paroxysmal hypertension during micturition are well established, we present an unusual case of bladder paraganglioma, 'misdiagnosed' with basilar-type migraine due to headache for the past 8 years. As urologists linked the presence of a tumor (by CT) and symptoms connected with micturition, no cystoscopy and no transurethral resection of the bladder was performed prior to detailed diagnostic workup. After diagnosis of an extra-adrenal paraganglioma, the patient was scheduled for open partial cystectomy. In consideration of the fact that bladder paraganglioma is an infrequent genitourinary cancer, this case report clearly points out the importance of an exact anamnesis and clinical examination to minimize the probability of misdiagnosis with possible fatal consequences in any case with clinical suspicion of bladder paraganglioma.
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PMID:Unrecognized paraganglioma of the urinary bladder as a cause for basilar-type migraine. 2373 39


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