Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty patients, 16 males and 4 females, aged 11-76 yr, were treated for a metastatic pheochromocytoma at our institution between 1985 and 1990. A neurofibromatosis was associated in 4. Thirteen patients had a unilateral adrenal tumor, 3 had an extraadrenal retroperitoneal tumor, 2 had a bilateral adrenal pheochromocytoma, one had a unilateral tumor with a contralateral medullary hyperplasia and one an adrenal and an extraadrenal pheochromocytoma. Metastases occurred in all patients, at presentation in 11, 10 to 30 months later in 7, and 9 and 28 yr later, respectively in two. Histology did not afford conclusive evidence for malignancy. Catecholamine hyperproduction was present in all, predominantly affecting norepinephrine. Neuron Specific Enolase level was elevated in 11, Neuro-Peptide Y level in 9 and procalcitonin level in 11/18. High dopamine, methoxytyramine and homovanillic acid excretion levels seemed to correlate with large tumors or terminal stage. MIBG uptake was found in 16 after a diagnostic dose and in 1 only after a therapeutic dose. Surgery was performed on primary tumor in 18 and on distant metastase in 10. Iodine-131 MIBG therapy was performed in 11, among whom 9 were evaluable. Cumulative activity ranged from 100 to 711 mCi, in 1 to 6 courses. Symptomatic improvement occurred in 5 patients, stabilization was observed in 3 and tumor partial response in two, which lasted for 28 and 9 months, respectively terminating in a rapidly progressing disease with bone marrow involvement. Moderate myelosuppression occurred in 4 patients. Chemotherapy gave no response in 7 evaluable patients. Fourteen patients died with a median survival of 16 months from diagnosis of metastases (range 3-60). Response to therapy was poor and warrants further cooperative trials.
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PMID:Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases. 147 46

Twenty patients, 16 male and 4 female (aged 11-76 years), with metastatic pheochromocytoma were treated in our Institution between 1985 and 1990. Metastases occurred in all patients: at presentation in 11 patients, with a 10 to 30 month delay in 7 patients, and 9 and 28 years later respectively in 2 patients. Catecholamines hyperproduction was present in all patients. Metaiodobenzylguanidine (MIBG) uptake was found in 16 patients after a diagnostic dose and only after a therapeutic dose in 1 patient. Surgery was performed on primary tumor (18 patients) and on distant metastases (10 patients). 131I-MIBG therapy was performed in 11 patients, 9 of whom were evaluable. The cumulative activity ranged from 3.7 to 26.3 GBq (100 to 711 mCi) in 1 to 6 courses. We observed symptomatic improvement (5 patients) and partial tumor response in 2 patients, which lasted for 28 and 9 months respectively, terminating with a rapidly progressing disease involving the bone marrow. Stabilisation was observed in 3 patients. Moderate myelosuppression occurred in 4 patients. Fifteen patients died with a median survival of 16 months (range 3-60). Response to therapy was poor and further evaluation of the presently available therapeutic approaches is needed.
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PMID:[131I]metaiodobenzylguanidine therapy in 20 patients with malignant pheochromocytoma. 182 38

Computed tomography (CT) and 123I- or 131I-meta-iodo-benzyl-guanidine (MIBG) scintigraphy were compared for accuracy in tumor detection in 47 patients with neuroectodermal neoplasms. MIBG concentration was found in 12 of 13 pheochromocytomas, 12 of 12 neuroblastomas, 5 of 9 carcinoids, and 1 of 4 glomus tumors. MIBG uptake was not observed in medullary thyroid carcinomas, oat-cell carcinomas, Merkel tumors, 1 gastrinoma, and 2 unclassified neuroectodermal neoplasms. With regard to the different tumor manifestations, the sensitivity in detecting pheochromocytomas, neuroblastomas, and carcinoids was 87%, 77%, and 100% for CT, and 83%, 100% and 71% for MIBG scintigraphy, MIBG scintiscan was superior in the detection of small adrenal pheochromocytomas (less than 1 cm diameter) and in the depiction of small bone metastases and bone marrow infiltration from neuroblastoma. In all, 25 cycles of high-dose MIBG therapy were performed in eight patients with surgically incurable tumors (4 malignant pheochromocytomas, 1 neuroblastoma, 3 carcinoids). The total therapeutic activity applied was 3.55-43.29 GBq 131I-MIBG. Tumor kinetics of MIBG were investigated before and during treatment. One patient with metastatic pheochromocytoma has been in complete remission for a follow-up period of 36 months since completion of treatment, and another patient is in partial remission. Tumor reduction or no change was observed in four patients. Two patients died of non-concentrating recurrence and metastases.
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PMID:[Diagnosis and therapy of neuroectodermal tumors]. 253 1

Bone is the most common site of metastasis from pheochromocytoma. Now that the effects of hypercatecholaminemia can be adequately controlled with adrenergic blockade, pathologic fractures are becoming an increasingly significant cause of morbidity in patients with metastatic pheochromocytoma. Bone metastases from pheochromocytoma have not been extensively reevaluated since the advent of computed tomography (CT), high-resolution bone scintigraphy, and iodine 131 MIBG scintigraphy. Plain radiographs, CT scans, bone scans, and I-131 MIBG scans of 38 patients with pheochromocytoma bone metastasis were reviewed. The axial skeleton was the most common site of metastasis. Metastases typically appeared expansile and mixed lytic-sclerotic on radiographs. Bone scintigraphy was the most sensitive modality for detecting bone metastasis, with 74% of all alleged lesions being identified. In screening for bone metastasis from pheochromocytoma, bone scanning in conjunction with I-131 MIBG scanning is recommended, followed by scan- and symptom-directed radiography and - where a question still exists - CT.
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PMID:Bone metastases in pheochromocytoma: comparative studies of efficacy of imaging. 373 9

The author reports a case of metastatic pheochromocytoma to bone imaged with 1-131 MIBG. The "MIBG super scan" pattern is described and is characterized by marked increased uptake in bone with essentially complete absence of activity in the liver and bladder. The avid bony metastases "steal" activity from the liver, as well as from the kidneys and soft tissues. This is felt to be comparable to the "super scan" in bone scintigraphy, whereby bony metastases "steal" activity from the kidneys and soft tissues.
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PMID:The MIBG super scan. Description and therapeutic implications. 829 25

A 50-year-old man had metastatic pheochromocytoma to the left frontal bone. The primary adrenal tumor was removed 12 years previously. Bone scanning and diagnostic I-131 and I-123 MIBG imaging showed metastatic lesions in the right femur and midthoracic spine. However, post-therapy I-131 MIBG scanning showed extensive and widespread metastatic disease. Post-therapeutic I-131 MIBG whole-body scanning was necessary to more fully assess the extent of metastatic pheochromocytoma.
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PMID:Improved detection of disseminated pheochromocytoma using post therapy I-131 MIBG scanning. 895 12

Plasma levels of chromogranin A (CgA) were measured by ELISA in 22 patients with pheochromocytoma (18 non-metastatic, 3 metastatic, and 1 mixed neuroendocrine-neural tumor), 9 patients with primary hyperparathyroidism, and 9 patients with pituitary adenoma. The plasma levels of CgA were compared with norepinephrine, epinephrine, parathyroid hormone and pituitary hormones, i.e., growth hormone and prolactin. In pheochromocytoma, CgA in preoperative plasma of the patients without metastasis was 228 +/- 38 U/L (mean +/- SEM) and significantly higher than healthy controls (30 +/- 11 U/L, n = 40). Plasma CgA was decreased after removal of the tumors (28 +/- 6.0 U/L), except in three patients with metastatic pheochromocytoma and a mixed neuroendocrine neural tumor. The concentration of CgA in the patients with non-metastatic pheochromocytoma was significantly correlated with that of plasma norepinephrine (P < 0.005, r = 0.68) and urinary norepinephrine (P < 0.05, r = 0.65), but not with that of epinephrine. There was an exceptional case in which CgA was extremely high, but the CA level was normal. This tumor was a highly malignant pheochromocytoma with extensive metastases composed of small tumor cells which were occasionally positive for tyrosine hydroxylase immunohistochemically. These cells were considered to be poorly differentiated tumor cells and synthesized a very small amount of norepinephrine. Plasma levels of the patients with primary hyperparathyroidism and the patients with pituitary adenoma were 44 +/- 4 U/L and 48 +/- 8 U/L, respectively. Only one patient with a growth hormone-producing pituitary adenoma had a high level of CgA. Plasma CgA is a useful tumor marker for pheochromocytoma, even for malignant pheochromocytoma without elevated CA level, but not for hyperparathyroidism, or pituitary adenoma.
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PMID:Plasma chromogranin A in pheochromocytoma, primary hyperparathyroidism and pituitary adenoma in comparison with catecholamine, parathyroid hormone and pituitary hormones. 922 69

The prognosis of metastatic pheochromocytoma is poor in general. There have been few instances of long-term survival reported. We report a case of a 44-year-old woman who has survived for 26 years after bone metastasis. She was diagnosed as having pheochromocytoma arising in the left adrenal medulla in 1974. Metastasis of pheochromocytoma in the first and third lumbar vertebrae and the right ilium was observed at the same time. The primary lesion was removed, and posterior lumbar spinal fusion was performed for immobilization. The metastatic lesion in the ilium was left untouched. After 26 years, she is well despite a recurrence of the tumors in the skull and a new metastasis in the left abdomen.
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PMID:Twenty-six-years' survival with multiple bone metastasis of malignant pheochromocytoma. 1176 44

Secondary to the paucity of pheochromocytoma, very limited data exist regarding the optimal treatments of metastatic disease. Malignant pheochromocytomas are often considered unresponsive to radiotherapy, but this decision is based on the few case reports performed before 1970. There have been a handful of reports about metastatic resolution and palliation from radiation therapy. Nevertheless, radiotherapy is not considered a mainstay of pheochromocytoma treatment. In this case report, we describe a patient with a malignant extraadrenal pheochromocytoma, metastatic to the right humerus, and her treatment course of surgery and radiotherapy.
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PMID:Radiation treatment of recurrent pheochromocytoma of the bladder: case report and review of literature. 1182 94

Pheochromocytoma is a rare but clinically important tumor of catecholamine-secreting chromaffin cells. This tumor constitutes a surgically curable cause of hypertension. Therefore, correct localization of pheochromocytoma is essential for effective management of this tumor. Several conventional and nuclear imaging modalities are currently available to localize pheochromocytoma. Computed tomography (CT) and magnetic resonance imaging (MRI) have good sensitivity but poor specificity for detecting pheochromocytoma, and nuclear imaging approaches such as (131)I-metaiodobenzylguanidine scintigraphy or [(111)In]-DTPA-D-Phe-pentetreotide (Octreoscan) have limited sensitivity. However, specificity of (131)I-metaiodobenzylguanidine scintigraphy is very good and this means of imaging provides a method for confirming that a tumor is a pheochromocytoma and rules out metastatic disease. Recently, we introduced a new imaging method, 6-[(18)F]fluorodopamine positron emission tomography, that can be used successfully for the detection of solitary and metastatic pheochromocytomas. Our preliminary data suggest that this method is superior to other nuclear imaging methods including metaiodobenzylguanidine and octreotide scintigraphy. In this report we provide an update regarding nuclear imaging of primary and metastatic pheochromocytoma, particularly using 6-[(18)F]fluorodopamine positron emission tomographic scanning.
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PMID:Diagnostic localization of pheochromocytoma: the coming of age of positron emission tomography. 1238 52


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