Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031511 (pheochromocytoma)
 14,622 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three patients with malignant pheochromocytoma were treated with intravenous infusion of 131I-MIBG. The dose per therapy ranged from 2.33 to 4.03 GBq. The repeated therapies were performed at intervals of 6, 11, 10 and 13 months in the first patient, 17 and 11 months in the second patient and 9 months in the third patient. Lumbago disappeared but little objective improvement was achieved in the first patient. The second patient exhibited a gradual decrease in catecholamine values with no change in tumor size. Remarkable decreases in tumor size and catecholamine values were observed in the third patient. No side effect was observed in any patient. The radiation dose absorbed by the main tumor was the highest at the first therapy and decreased with the number of therapies: 42, 26, 19, x and 9.0 Gy in the first patient, 63, 20 and 8.8 Gy in the second patient, and 81 and 40 Gy in the third patient. This was due mainly to the decrease in % uptake by the tumor of the 131I-MIBG dose administered. Therefore the increase in the dose of 131I-MIBG administered at the first therapy and/or shorter interval therapies seems to be important to obtain more therapeutic effects on malignant pheochromocytoma.
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PMID:Radionuclide therapy of malignant pheochromocytoma with 131I-MIBG. 770 71

A 71-year-old woman was admitted with the chief complaint of headache, lumbago and slight fever. Computerized tomographic (CT) scan demonstrated a large soft tissue mass with multiple cystic necrosis in the right adrenal region. The plasma norepinephrine concentration was excessive and serum levels of neuron-specific enolase (NSE), calcitonin and parathormone were elevated. MIBG scintigraphy showed a high uptake in the same region. Under the diagnosis of pheochromocytoma without distant metastasis, right adrenalectomy was performed. The tumor was removed en bloc with right kidney and a part of the liver because of inflammatory adhesion. The histological examination revealed benign pheochromocytoma. After the operation, norepinephrine and calcitonin decreased to normal but the levels of NSE and PTH remained high. One year after operation, chest X-ray revealed multiple lung metastases and after 1.5 years she died of respiratory failure. Autopsy revealed multiple lung and bone metastases and a liver metastasis, parathyroid glands showed hyperplasia but the thyroid gland showed no abnormal change. This clinical course suggests that serum NSE might be a useful tumor marker for differentiating malignant pheochromocytoma from benign one, and this tumor producing calcitonin caused secondary hyperparathyroidism.
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PMID:[A case of malignant pheochromocytoma with high levels of serum neuron-specific enolase (NSE) and calcitonin]. 780 42

Phaeochromocytoma or paraganglioma that exclusively secretes dopamine is very rare. This case illustrates its atypical presentation and the importance of interpretative reporting for urine catecholamines leading to the diagnosis and subsequent management of a patient with this condition. We report a 71-year-old Chinese woman with a large dopamine-secreting paraganglioma. She presented with low back pain for six months. On examination, a right abdominal mass was palpable incidentally. Her blood pressure was normal throughout. Serial 24-h urine collections for catecholamines showed enormous elevation of urine dopamine excretion to 80.7 micromol/day (normotensive:<2.6 m mol/day). However, the daily excretions of urine adrenaline and noradrenaline, as well as their metabolites were within their respective reference intervals. Good communication between chemical pathologists and physicians prompted the arrangement of the whole body 131I-meta-iodobenzylguanidine (MIBG) scintigraphy, which showed a large signal in the right upper quadrant of the abdomen corresponding to a large extra-adrenal tumour detected by both ultrasonography and computerized tomography (CT) of the abdomen. Histological section of the tumour tissue revealed paraganglioma, which stained positive for chromogranin and neuron-specific enolase. After four months, the patient presented with chest symptoms and CT of the thorax revealed multiple nodules. Lung metastases were suspected. However, follow-up urine catechola- mine and dopamine excretions were again within their respective normotensive reference intervals. A second MIBG scintigraphy was performed, but no specific uptake at either the thorax or the abdomen could be demonstrated. Fine-needle aspiration cytology using the thoracoscopic technique was performed and immunochemical staining of the biopsy specimen showed the presence of non- small-cell carcinoma of the lung.
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PMID:The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma. 1580 39

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant inherited cancer syndrome that expresses nonendocrine and endocrine tumors. Here, we describe a 42-year-old man with an initial presentation of low back pain and hypertension. Clinical assessments revealed pheochromocytoma, medullary thyroid carcinoma with bone metastasis, and parathyroid hyperplasia. MEN 2A was diagnosed, and a family history of pheochromocytoma was traced. Surgical resection of the pheochromocytoma of the adrenal gland resulted in a cure of the patient's hypertension. He received systemic chemotherapy with the "MAID" regimen (mesna, doxorubicin, ifosfamide, and dacarbazine) over three cycles of 3 weeks each, and showed a partial response.
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PMID:Multiple endocrine neoplasia type 2A. 2263 91

Pheochromocytoma and Ganglioneuroblastoma are separate diseases and a rare combination in which the diagnosis can only be confirmed by pathological examination after tumor excision. We reported here a case of ganglioneuroblastoma encapsulated in pheochromocytoma. The patient is a woman, 73 years old, hypertensive, with hypothyroidism, associated for 15 years with hypercholesterolemia and hypertriglyceridemia, which had frequent complaints of low back pain. She underwent magnetic resonance and the findings were consistent with the diagnosis of pheochromocytoma. After surgery, anatomic, pathologic and immunohistochemistry analysis confirmed the diagnosis of pheochromocytoma composed by small ganglioneuroblastoma representation with the identification of small focus of infiltration of the adrenal capsule and adipose tissue by pheochromocytoma. This rare association can instigate the discussion of methods of diagnosis, more effective and more appropriate treatments for each patient.
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PMID:A rare case of Ganglioneuroblastoma Encapsulated in Pheochromocytoma. 2752 9