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

Malignant pheochromocytoma may present as a widespread metastatic disease, which is little or non-responsive to external beam radiotherapy and chemotherapy. The prognosis of these patients is bad due to both the progressive metastasis and the secretion of excess catecholamines which may cause hypertensive episodes. For these conditions [131I]metaiodobenzylguanidine (131I-MIBG) therapy may be an alternative treatment modality to induce both tumor remission and reduction of hormonal activity of the disease. The experience with 131I-MIBG therapy in four patients with metastatic malignant pheochromocytoma at The Netherlands Cancer Institute is reviewed. One patient with abdominal tumor recurrence and metastases to the lymph nodes and lungs had a partial remission of disease for 3 years; a second had a mixed response together with palliation and two other patients had stable disease, but were relieved of bone pain and severe hypertension, respectively. It is essential to be aware of the medication the patient is using, as many drugs are known or may be expected to interfere with the uptake and/or retention of 131I-MIBG by the tumor cells. The case of a significant reduction of 131I-MIBG uptake and retention by Labetalol in one of the patients is discussed. It is concluded that 131I-MIBG therapy may induce objective remission in patients with malignant pheochromocytoma and is certainly meaningful in the reduction of hormonal activity, the control of hypertension and the relief of pain from metastases.
 ...
PMID:[131I]metaiodobenzylguanidine therapy of malignant pheochromocytoma: interference of medication. 182 43

Malignant phaeochromocytoma is a rare tumour and experience in its management is therefore limited. Five patients are discussed in whom the development of metastases was associated with rapidly progressive disease.
 ...
PMID:Malignant phaeochromocytoma. 402 7

Malignant pheochromocytoma has a low incidence. Metastatic disease with skeletal spread is a known entity while osseous involvement of the spine is uncommon. Therefore the case of a 27 year of female with successive, polytopic spinal metastases is reported who underwent surgical resection and stabilisation. 6 years after onset of spinal metastases and operative treatment the patient is free of complaints according to the spinal column.
 ...
PMID:[Surgical therapy of polytopic vertebral metastasis in malignant pheochromocytoma]. 775 66

Malignant pheochromocytoma is an exceptional complication in patients with Multiple Endocrine Neoplasia Type 2a (MEN2a). In this paper, we report on a 53-year-old male patient with an evident RET gene germline mutation, who simultaneously developed hepatic metastases of medullary thyroid carcinoma (MTC) and pheochromocytoma. Comprehensive immunohistochemical investigations were performed to elaborate markers which could be useful for differentiating between MTC metastases and pheochromocytoma, respectively. Calcitonin and CEA, in particular cytokeratins and trefoil factor family 1 (TFF1), were detectable exclusively in MTC, whereas all the other markers revealed a comparable expression in both MTC and pheochromocytoma. The only clues that could indicate a potential malignant course were size, a lack of sustentacular cells and hyaline globules, and a focal spindle cell pattern in pheochromocytoma. Owing to a wide agreement in cellular differentiation and a lack of specific, routinely applicable markers for pheochromocytomas, a comprehensive and goal-directed immunohistochemistry is required to rule out pheochromocytoma metastasis in patients with MEN2a. A misinterpretation could lead to harmful clinical complications, as shown in the present case.
 ...
PMID:Simultaneously occurring liver metastases of pheochromocytoma and medullary thyroid carcinoma--a diagnostic pitfall with clinical implications for patients with multiple endocrine neoplasia type 2a. 1092 25

Malignant primary adrenal tumors are rare forms of cancer with an estimated incidence of two to ten new cases per one million inhabitants per year. The 5-year survival rate for adrenocortical carcinoma is approximately 35%, whereas the 10-year survival rate of malignant pheochromocytoma reaches 40%. Clinical studies support repeated surgery as the mainstay of treatment, either with curative or palliative intention. For adrenocortical carcinoma, adjunctive treatment with oral mitotane leads to well-documented improvement of survival. Rare malignant pheochromocytomas with distant metastases are preferably treated by 131I-MIBG. Chemotherapy is reserved for unresectable tumors without sufficient response to mitotane or 131I-MIBG, respectively. Cisplatin and etoposide as single therapy, or in combination with doxorubicin or etoposide, appear to be effective in adrenocortical carcinoma. Malignant pheochromocytoma may be treated with vincristine, dacarbazine, and cyclophosphamide. Treatment with octreotide is currently being evaluated. Radiotherapy is indicated if unresectable tumor masses cause local symptoms. If symptoms of endocrine activity are not sufficiently controlled by measures aiming at tumor mass reduction, specific inhibitors of hormone synthesis or action are available. Ketoconazole is widely used for adrenocortical carcinoma, and phenoxybenzamine and metyrosine are available for malignant pheochromocytoma. This review provides guidelines for rational disease management based on still scanty clinical evidence.
 ...
PMID:Clinical management of malignant adrenal tumors. 1126 Aug 59

Endosonography enables imaging of the adrenal glands, the mediastinum, and the epigastric retroperitoneal area. In this study, the diagnostic power of endosonography regarding the detection and localization of pheochromocytomas and the differentiation between benign and malignant lesions and their metastases and recurrences was investigated. Endosonography was performed using a Pentax FG 32 UA endosonoscope with a longitudinal 7.5-MHz sector array from the esophagus, stomach, and duodenum. A total of 22 pheochromocytomas in 11 patients were studied. All these tumors, recurrences, and metastases were histologically proven except in one single patient where pheochromocytoma had been diagnosed histologically in the past, and actual findings were obvious local recurrence and four metastases. Malignant pheochromocytoma (n = 10) tended to be larger at the time of examination than benign pheochromocytoma (n = 12; P = 0.069). No significant differences between benign and malignant pheochromocytomas regarding echogeneity and echostructure could be detected. However, hyperechoic echogeneity was seen only in benign lesions, which, however, had variable echogeneity. If confirmed by future observations, hyperechoic echogeneity may be considered to be suggestive of a benign nature. In several cases, endosonography detected small lesions that had been missed by routine diagnostic procedures and yielded helpful information for planning surgical strategy. In conclusion, endosonography is considered to be useful in early detection of pheochromocytomas, and in malignant disease of recurrence and metastases.
 ...
PMID:Endosonographic imaging of benign and malignant pheochromocytomas. 1507 Sep 32

Pheochromoblastoma (PHB) is a tumor developing from adrenal medulla and characterized by severe course and high mortality level (15%). The severity of patients' state determined not only by presence of cancerous growth, but also due to catecholamine hyperproduction. The author has analyzed the results of surgical treatment of 60 patients with PHB, which were in surgical clinic during 1968-2002 y.y. The most effective method of the treatment is surgery. The method of choice is a lumbotomic extraperitoneal approach. The eradication of malignant growth requires not only complete tumor removal in single capsule with the adrenal gland but also thorough revision of paranephric, paracaval and paraaortal lymphnodes. 38 patients (63.3%) from 60 underwent radical surgery followed by long-term remission. The remission of the disease in 3 patients (5%) was achieved only after reoperations. The disease persists in two patients though it is better controlled after the operation than earlier with medicine. 8 (13.3%) patients were diagnosed to have widespred metastases and were referred to symptomatic treatment. The recurrence of the disease after the primary surgery is a bad forecasting factor. The majority of the patients (55.8%) with the recurrence were incurable. In the rest of the cases the operative treatment led to the longterm remission only in 71.9% of the patients.
 ...
PMID:[The surgical treatment of malignant adrenal chromaphinomas]. 1572 15

Malignant pheochromocytoma is a rare disease with a high mortality. Surgical resection is the only effective treatment if extensive metastatic disease is not present. However, differentiating between benign and malignant pheochromocytoma is impossible in the absence of locoregional invasion or distant metastasis. This diagnostic dilemma has several drawbacks, including later detection and treatment of recurrence than if malignancy is determined at the original operation. With emerging molecular markers of malignant disease, optimal extent and approach for surgical treatment and appropriate extent of follow up could be established based on specific tumor behavior and the need for additional systemic therapy.
 ...
PMID:Malignant pheochromocytoma. 1688 97

Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment. There are no definitive histological or cytological criteria of malignancy, as it is impossible to determine this condition in the absence of advanced locoregional disease or metastases. We report a case of a patient with a giant retroperitoneal tumour, the second largest to be published, which was diagnosed as a malignant pheochromocytoma; it was treated with surgery. The literature is reviewed to evaluate tumour features and criteria to distinguish between benign and malignant pheochromocytomas.
 ...
PMID:Malignant giant pheochromocytoma: a case report and review of the literature. 2001 63

Malignant pheochromocytoma is a neuroendocrine tumor that originates from chromaffin tissue. Although osseous metastases are common, metastatic dissemination to the spine rarely occurs.Five years after primary diagnosis of extra-adrenal, abdominal pheochromocytoma and laparoscopic extirpation, a 53-year old patient presented with recurrence of pheochromocytoma involving the spine, the pelvis, both proximal femora and the right humerus. Magnetic resonance imaging and computed tomography revealed osteolytic lesions of numerous vertebrae (T1, T5, T10, and T12). In the case of T10, total destruction of the vertebral body with involvement of the rear edge resulted in the risk of vertebral collapse and subsequent spinal stenosis. Thus, dorsal instrumentation (T8-T12) and cement augmentation of T12 was performed after perioperative alpha- and beta-adrenergic blockade with phenoxybenzamine and bisoprolol.After thorough preoperative evaluation to assess the risk for surgery and anesthesia, and appropriate perioperative management including pharmacological antihypertensive treatment, dorsal instrumentation of T8-T12 and cement augmentation of T12 prior to placing the corresponding pedicle screws did not result in hypertensive crisis or hemodynamic instability due to the release of catecholamines from metastatic lesions.To the authors' knowledge, this is the first report describing cement-augmentation in combination with dorsal instrumentation to prevent osteolytic vertebral collapse in a patient with metastatic pheochromocytoma. With appropriate preoperative measures, cement-augmented dorsal instrumentation represents a safe approach to stabilize vertebral bodies with metastatic malignant pheochromocytoma. Nevertheless, direct manipulation of metastatic lesions should be avoided as far as possible in order to minimize the risk of hemodynamic complications.
 ...
PMID:Cement-augmented dorsal instrumentation of the spine as a safe adjunct to the multimodal management of metastatic pheochromocytoma: a case report. 2222 47


1 2 Next >>