Childhood low-grade astrocytomas may recur many years after initial treatment. Recurrent disease is usually at the primary tumor site, although multifocal or widely disseminated disease to other intracranial sites and to the spinal leptomeninges has been documented.[1,2] Most children whose low-grade fibrillary astrocytomas recur will harbor low-grade lesions; however, malignant transformation is possible. Surveillance imaging will frequently identify asymptomatic recurrences.
At the time of recurrence, a complete evaluation to determine the extent of the relapse is indicated. Biopsy or surgical resection may be necessary for confirmation of relapse because other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The need for surgical intervention must be individualized on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the mass lesion, and the clinical picture.
There are several types of plasma cell neoplasms. These diseases are all associated with a monoclonal (or myeloma) protein (M protein). They include monoclonal gammopathy of undetermined significance (MGUS), isolated plasmacytoma of the bone, extramedullary plasmacytoma, and multiple myeloma.
(Refer to the Lymphoplasmacytic Lymphoma (Waldenström Macroglobulinemia) section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.)
Incidence and Mortality
An individual plan needs to be tailored based on patient age, tumor location, and prior treatment. If patients have not received radiation therapy, local radiation therapy is the usual treatment, although further chemotherapy in lieu of radiation may be considered, depending on the child's age and the extent and location of the tumor.[Level of evidence: 3iA]; [Level of evidence: 3iiiDi] For children with low-grade glioma for whom radiation therapy is indicated, conformal radiation therapy approaches appear effective and offer the potential for reducing the acute and long-term toxicities associated with this modality.[8,9] In patients treated with surgery alone whose disease progresses, chemotherapy and/or radiation therapy are options. If recurrence takes place after irradiation, chemotherapy should be considered. Chemotherapy may result in relatively long-term disease control.[10,11] Vinblastine alone, temozolomide alone, or temozolomide in combination with carboplatin and vincristine may be useful at the time of recurrence for children with low-grade gliomas.[10,11,12]; [Level of evidence: 3iiDi]
Patients with low-grade astrocytomas who relapse after being treated with surgery alone should be considered for another surgical resection. If this is not feasible, local radiation therapy is the usual treatment. If there is recurrence in an unresectable site after irradiation, chemotherapy should be considered.