NEW YORK (Reuters Health) Nov 29 - Chemotherapy shrinks lesions, relieves symptoms, and prolongs survival in patients with metastatic pheochromocytoma or sympathetic extra-adrenal paraganglioma, according to a report online October 17th in Cancer.
"Chemotherapy could be offered to patients with progressive disease, in particular to patients with rapidly progressive disease," said senior author Dr. Camilo Jimenez in email to Reuters Health.
"In addition, chemotherapy should be considered in patients with unresectable tumors and overwhelming symptoms related to tumor burden and hormone excess," added Dr. Jimenez, from The University of Texas MD Anderson Cancer Center in Houston.
These rare neuroendocrine tumors together have an estimated incidence of roughly one per 10,000 person-years in the U.S. About 17% become metastatic.
Dr. Jimenez and colleagues evaluated the clinical benefits of systemic chemotherapy in a retrospective study of 52 patients with metastatic pheochromocytoma or sympathetic extra-adrenal paraganglioma.
Seventeen patients, or one third, responded to treatment (i.e., with any objective reduction in tumor size on cross-sectional imaging studies during the first chemotherapy regimen).
All 17 responders had received cyclophosphamide and dacarbazine. Twelve had also received doxorubicin, and 14 of 17 also received vincristine.
Thirty-one patients had catecholamine excess, adrenergic symptoms, and hypertension at baseline. Six who responded had their antihypertensive medication doses reduced by more than 50%, and three of these six had complete responses and were able to stop all their antihypertensive medications.
Overall survival at five years was 51%, and median overall survival was longer (albeit nonsignificantly) in responders vs. nonresponders (6.4 vs. 3.7 years).
Patients had significantly longer overall survival if they had metachronous rather than synchronous metastatic disease (median 9.9 vs. 5.1 years) and if they had surgery and chemotherapy rather than chemotherapy alone (6.5 vs. 3.0 years).
In a multivariate model, the favorable impact of chemotherapy on overall survival at one year was significant among patients with synchronous metastatic disease after adjustment for tumor size.
"Because these tumors are scarce, it is imperative to involve expert centers and international networks in order to achieve both expertise and statistical power" in clinical trials, Dr. Jimenez said.
But, Dr. Jimenez added, "Many patients may have indolent and asymptomatic disease and may not need systemic treatment. Appropriate multidisciplinary consultations between physicians familiar with this disease are important before embarking on a treatment plan."
SOURCE: http://bit.ly/tdEiSJ
"Chemotherapy could be offered to patients with progressive disease, in particular to patients with rapidly progressive disease," said senior author Dr. Camilo Jimenez in email to Reuters Health.
"In addition, chemotherapy should be considered in patients with unresectable tumors and overwhelming symptoms related to tumor burden and hormone excess," added Dr. Jimenez, from The University of Texas MD Anderson Cancer Center in Houston.
These rare neuroendocrine tumors together have an estimated incidence of roughly one per 10,000 person-years in the U.S. About 17% become metastatic.
Dr. Jimenez and colleagues evaluated the clinical benefits of systemic chemotherapy in a retrospective study of 52 patients with metastatic pheochromocytoma or sympathetic extra-adrenal paraganglioma.
Seventeen patients, or one third, responded to treatment (i.e., with any objective reduction in tumor size on cross-sectional imaging studies during the first chemotherapy regimen).
All 17 responders had received cyclophosphamide and dacarbazine. Twelve had also received doxorubicin, and 14 of 17 also received vincristine.
Thirty-one patients had catecholamine excess, adrenergic symptoms, and hypertension at baseline. Six who responded had their antihypertensive medication doses reduced by more than 50%, and three of these six had complete responses and were able to stop all their antihypertensive medications.
Overall survival at five years was 51%, and median overall survival was longer (albeit nonsignificantly) in responders vs. nonresponders (6.4 vs. 3.7 years).
Patients had significantly longer overall survival if they had metachronous rather than synchronous metastatic disease (median 9.9 vs. 5.1 years) and if they had surgery and chemotherapy rather than chemotherapy alone (6.5 vs. 3.0 years).
In a multivariate model, the favorable impact of chemotherapy on overall survival at one year was significant among patients with synchronous metastatic disease after adjustment for tumor size.
"Because these tumors are scarce, it is imperative to involve expert centers and international networks in order to achieve both expertise and statistical power" in clinical trials, Dr. Jimenez said.
But, Dr. Jimenez added, "Many patients may have indolent and asymptomatic disease and may not need systemic treatment. Appropriate multidisciplinary consultations between physicians familiar with this disease are important before embarking on a treatment plan."
SOURCE: http://bit.ly/tdEiSJ
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