Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with a malignant extraadrenal retroperitoneal paraganglioma had elevated levels of immunoreactive neuropeptide Y (NPY) in the peripheral blood (5988 pg/ml; normal, 123 +/- 30 pg/ml [mean +/- standard error of the mean]). A 6-month course of chemotherapy allowed surgical removal of the previously unresectable primary tumor. Postoperatively, the plasma NPY level initially fell to 1089 pg/ml; continued chemotherapy caused an additional decrease to 440 pg/ml. Four months after surgery, the plasma NPY level increased to 940 mg/ml, coincident with hepatic metastases. This case is the first report of a NPY-secreting clinically nonfunctional malignant extraadrenal paraganglioma. Determination of circulating NPY levels may be useful in the diagnosis and follow-up of patients with neuroendocrine tumors.
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PMID:Neuropeptide Y secretion from a malignant extraadrenal retroperitoneal paraganglioma. 132 96

The bone metastases of a malignant, non-secreting paraganglioma were treated with [131I]metaiodobenzylguanidine (131I-MIBG) over a 10-year period. Initial treatment (131I-MIBG: 9.6 GBq) resulted in a decrease in the number of bone metastases from 16 to 2. At three years, a relapse with primary tumor regrowth and liver metastasis was again treated with 131I-MIBG (22.2 GBq). A decrease in the number of bone metastases and MIBG uptake was again observed.
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PMID:[131I]metaiodobenzylguanidine treatment of a malignant paraganglioma. 182 44

Isolated vertebral body metastases from paraganglioma are exceedingly rare. They have been reported to occur in the presence of active primary tumor in the neck, local recurrence, or widespread metastases. A unique case of carotid body tumor (paraganglioma) is reported with the following features: (1) multiple vertebral body metastases (C6, T9, and L3) presenting with spinal cord compression, and no evidence of local recurrence or other metastatic disease; (2) absence of mitoses on the original specimen or the metastatic deposit; and (3) a prolonged interval (9 years) to the development of symptomatic metastases.
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PMID:Multiple spinal metastases from paraganglioma. 222 78

A prosthesis was designed to protect the intestinal loop from external beam radiation therapy when post-operative radiation is indicated. It is a silicone inflatable balloon, which, when implanted displaces the intestinal loops out of the pelvic irradiation field. The prosthesis can be deflated between each course of irradiation, without surgery. The device has been used in 8 patients: 6 patients with recurrent pelvic tumor (2 rectal cancers, 1 anal cancer, 1 cancer of the endometrium, 1 cervical carcinoma, 1 ovarian carcinoma), 2 patients with primary tumor (1 malignant paraganglioma, 1 cervical carcinoma). Radiotherapy was administered by means of high power appliances. After radiotherapy, the prosthesis was deflated, then removed through a 3 cm incision under local or peridural anesthesia. The tolerance of the small intestine to the radiation therapy has been satisfactory in each case with no bowel injury due to radiation. Therefore, this simple device might be useful to prevent bowel injury during postoperative radiation in the treatment of abdominal and retroperitoneal tumor masses.
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PMID:[New surgical procedure for the protection of the small intestine before postoperative pelvic irradiation]. 237 97

This case report describes the treatment of the bone metastases of a nonfunctioning sympathetic paraganglioma, with [131I]MIBG. After primary tumor excision and unsuccessful external radiotherapy, the patient received three therapeutic doses of [131I]MIBG, resulting in a reduction of the number and volume of metastases, and an improvement of the general condition. At 3 yr following [131I]MIBG therapy, the patient remained in remission. [131I]MIBG appears to be an efficient and safe agent for treating malignant sympathetic paraganglioma.
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PMID:Therapeutic effectiveness of iodine-131 MIBG metastases of a nonsecreting paraganglioma. 319 14

A case of nonfunctioning paraganglioma, initially qualified as benign, is described. Two years following extirpation, functioning bone and lung metastases were manifested by hypertensive crises and increased levels of vanilmandelic acid and catecholamines in urine. The criteria for benign and malignant paragangliomas are discussed with regard to functional transformation of the tumor described in this case. It is concluded that the phenotypic heterogeneity of the primary tumor cells could explain the difference in biological behavior of the primary tumor and its metastases.
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PMID:Functioning metastases of a nonfunctioning paraganglioma. 335 78

The occurrence is reported of a bronchial epidermoid cancer appearing 13 years after irradiation for an intra-thoracic paraganglioma. Four criteria are required when considering the carcinogenic effects of radiation: a documented history or irradiation, a latent period of 8 to 20 years, histological evidence of the development of a malign tumor within the irradiated zone and a different histological type from that of the primary tumor. All these criteria are satisfied in this case. The occurrence of post radiation fibrosis and the knowledge of a cancer developing in primary or secondary pulmonary fibrosis are considered in discussing carcinogenesis. Radiation induces fibrous and an oncogenic mutation which by the secretion of certain proteins, notably a growth factor, favours the development of a cancer.
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PMID:[Epidermoid bronchial cancer induced by radiotherapy]. 372 60

Two patients, presenting with single mass lesions involving the posterior fossa and temporal bone, respectively, are reported. Clinically, both were thought to have primary tumors in those locations, hemangioblastoma in one and glomus jugulare paraganglioma in the other. Morphologic evaluation was compatible with adenocarcinoma of renal origin and large renal tumors were subsequently found in both patients. Electron microscopy was useful in eliminating the possibility of a primary tumor in those respective locations by demonstrating focal, but conspicuous dense arrays of microvilli at intercellular areas or along the free surfaces of plasma membranes. Microvilli do not, by themselves, signify any specific primary organ site. However, taken in the clinical and histologic contexts of a given case, ultrastructural observations can assist in establishing a definitive diagnosis.
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PMID:Renal adenocarcinoma presenting as a solitary metastasis: contribution of electron microscopy to diagnosis. 729 12

The clinical and pathologic features of 12 extra-adrenal paragangliomas of the retroperitoneum are presented. The patients (eight men, four women) had an average age of 43 years at the time of diagnosis; three had symptoms related to excess catecholamine secretion. Complete surgical resection was attempted in each case but was possible for only four patients. Five tumors were malignant; four patients died from regional and/or distant metastases 17 months to 10 years after surgery. One patient was alive with metastatic paraganglioma 25 years after initial treatment. Each patient with metastatic or locally recurrent paraganglioma had incomplete resection of the primary tumor. An important microscopic feature of each paraganglioma was the presence of cytoplasmic argyrophilia. Three of the five metastasizing paragangliomas showed readily identifiable mitoses or evidence of vascular invastion; the two other cases and all of the benign tumors lacked these features. Our findings suggest that in some cases histology may prove helpful in evaluating the malignant potential of these tumors.
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PMID:Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors. 737 61

During 35 years we operated on 44 patients with pheochromocytoma, 16 of them had extraadrenal tumors (36%). We operated on 8 men and 8 women. In 5 men there was primary extraadrenal tumor, the same in 7 women. In 3 men and in one woman we operated on recurrence of tumor on another site. Mean age of the whole group was 38.3 years, in patients with recurrence the mean age was 44 years. Time distance between primary tumor and relapse was 3 to 16 years. We operated on 6 tumors on the right side, 10 on the left side. Paragangliomas on the right side were localized as follows: three tumors were under the vena cava, one tumor was dispersed multifocally, two tumors were localized before the vena cava. On the left side there was the following distribution of tumors: two tumors beneath the bifurcation of the aorta, 5 tumors paraaortally, one tumor on the wall of the aorta in the region of renal arteries, three tumors were localized in the hilus of the kidney.
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PMID:Extraadrenal pheochromocytoma. 756 27


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