Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Option Overview
Many of the improvements in childhood cancer survival have been made using combinations of known and/or new agents that have attempted to improve the best available, accepted therapy. Clinical trials in pediatrics are designed to compare potentially better therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with standard therapy.
All children with non-Hodgkin lymphoma (NHL) should be considered for entry into a clinical trial. Treatment planning by a multidisciplinary team of cancer specialists with experience treating tumors of childhood is strongly recommended to determine, coordinate, and implement treatment to achieve optimal survival. Children with NHL should be referred for treatment by a multidisciplinary team of pediatric oncologists at an institution with experience in treating pediatric cancers. Information about ongoing clinical trials is available from the NCI Web site.
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NHL in children is generally considered to be widely disseminated from the outset, even when apparently localized; as a result, combination chemotherapy is recommended for most patients.
In contrast to the treatment of adults with NHL, the use of radiation therapy is limited in children with NHL. Early studies demonstrated that the routine use of radiation had no benefit for low-stage (I or II) NHL. It has been demonstrated that prophylactic central nervous system (CNS) radiation can be omitted in lymphoblastic lymphoma.[3,4] It has also been demonstrated that CNS radiation can be eliminated for patients with anaplastic large cell lymphoma and B-cell NHL, even for patients who present with CNS disease.[5,6] Further data to support the limited use of radiation in pediatric NHL comes from the Childhood Cancer Survivor Study. This analysis demonstrated that radiation was a significant risk factor for secondary malignancy and death in long-term survivors.
Treatment of NHL in childhood and adolescence has historically been based on clinical behavior and response to treatment. A study by the Children's Cancer Group demonstrated that the outcome for lymphoblastic NHL was superior with longer acute lymphoblastic leukemia–like therapy, while nonlymphoblastic NHL (Burkitt lymphoma) had superior outcome with short, intensive, pulsed therapy.
There are two potentially life-threatening clinical situations that are often seen in children with NHL: (1) mediastinal masses and (2) tumor lysis syndrome, most often seen in lymphoblastic and Burkitt or Burkitt-like NHL. These emergent situations should be anticipated in children with NHL and addressed immediately.
Patients with large mediastinal masses are at risk of cardiac or respiratory arrest during general anesthesia or heavy sedation. Due to the risks of general anesthesia or heavy sedation, a careful physiologic and radiographic evaluation of the patient should be carried out and the least invasive procedure should be used to establish the diagnosis of lymphoma.[10,11] Bone marrow aspirate and biopsy should always be performed early in the workup of these patients. If a pleural effusion is present, a cytologic diagnosis is frequently possible using thoracentesis. In those children who present with peripheral adenopathy, a lymph node biopsy under local anesthesia and in an upright position may be possible. In situations in which the above diagnostic procedures are not fruitful, consideration of a computed tomography (CT)–guided core needle biopsy should be contemplated. This procedure can frequently be carried out using light sedation and local anesthesia before proceeding to more invasive procedures. Care should be taken to keep patients out of a supine position. Most procedures, including CT scans, can be done with the patient on their side or prone. Mediastinoscopy, anterior mediastinotomy, or thoracoscopy are the procedures of choice when other diagnostic modalities fail to establish the diagnosis. A formal thoracotomy is rarely, if ever, indicated for the diagnosis or treatment of childhood lymphoma. Occasionally, it will not be possible to perform a diagnostic operative procedure because of the risk of general anesthesia or heavy sedation. In these situations, preoperative treatment with steroids or localized radiation therapy should be considered. Since preoperative treatment may affect the ability to obtain an accurate tissue diagnosis, a diagnostic biopsy should be obtained as soon as the risk of general anesthesia or heavy sedation is thought to be alleviated.