There is no standard staging system for pheochromocytoma and paraganglioma. Patients have traditionally been divided into one of three categories:
Localized (apparently benign) disease.
Metastatic disease. The most common sites of metastasis for pheochromocytoma or extra-adrenal paraganglioma are lymph nodes, bones, lungs, and liver.
Reinduction chemotherapy followed by allogeneic bone marrow transplantation (alloBMT).
Palliative radiation therapy (for patients with symptomatic recurrence).
Dasatinib (for patients with Philadelphia chromosome [Ph1]-positive ALL).
Patients with ALL who experience a relapse following chemotherapy and maintenance therapy are unlikely to be cured by further chemotherapy alone. These patients should be considered for reinduction chemotherapy followed by alloBMT.
Palliative radiation therapy
Low-dose palliative radiation therapy may be considered in patients with symptomatic recurrence either within or outside the central nervous system.
Patients with Ph1-positive ALL will often be taking imatinib at the time of relapse and thus will have imatinib-resistant disease. Dasatinib, a novel tyrosine kinase inhibitor with efficacy against several different imatinib-resistant BCR-ABL mutations, has been approved for use in Ph1-positive ALL patients who are resistant to or intolerant of imatinib. The approval was based on a series of trials involving patients with chronic myelogenous leukemia, one of which included small numbers of patients with lymphoid blast crisis or Ph1-positive ALL.
In one study, ten patients were treated with dose-escalated dasatinib. Seven of these patients had a complete hematologic response (<5% marrow blasts with normal peripheral blood cell counts), three of whom had a complete cytogenetic response.
The common toxicities were reversible myelosuppression (89%) and pleural effusions (21%).
Virtually all of these patients relapsed within 6 months of the start of treatment with dasatinib.
Treatment Options Under Clinical Evaluation for Recurrent Adult ALL
Patients for whom an HLA-matched donor is not available are excellent candidates for enrollment in clinical trials that are studying the following:[3,4,5,6,7,8,9]
Novel chemotherapeutic or biological agents.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent adult acute lymphoblastic leukemia. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
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WebMD Public Information from the National Cancer Institute
September 04, 2014
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