Treatment

Treatment of NHL is based upon histology, immunophenotype and clinical stage.

Unlike HL, NHL in children is considered to be widely disseminated from the beginning, even when the disease appears localized. Combination chemotherapy is recommended for all patients.

Radiation therapy is sometimes added for children with: primary NHL of the bone, CNS involvement (sometimes emergency therapy), testicular involvement, or severe mass effect (i.e., superior vena caval compression or airway obstruction).

Children with localized disease do not benefit from radiation therapy, which, in this setting, only increases toxicity.

Patients with Stage I/II disease, regardless of its histological subgroup, are treated with 6 cycles of COMP (cyclophosphamide, vincristine, methotrexate and prednisone) or 3 cycles of COPA (cyclophosamide, vincristine, doxorubicin and prednisone) followed by 6 months of mercaptopurine and methotrexate. This regimen has achieved a 90% cure rate.

Children with advanced stage disease (III/IV), however, are treated based on the NHL's histological subtype.

Initial surgical management includes incisional biopsy for diagnosis, followed by intense, multiagent chemotherapy, except for small, easily resectable lesions. Resection of massive retroperitoneal or mediastinal masses is not indicated. Complete surgical resection of well-localized tumors, particularly if confined to the bowel, may be beneficial to overall survival and reduces complications such as tumor lysis syndrome or bowel perforation following chemotherapy.

Children with large mediastinal tumors are at significant risk of cardiac or respiratory arrest during general anesthesia. For this reason, the least invasive procedure possible is used to establish the diagnosis of lymphoma. Bone marrow biopsy and aspiration often provide the diagnosis and should be done early. Peripherally involved lymph nodes are biopsied using local anesthesia and light sedation in most instances. Pleural effusions also can provide the correct histologic diagnosis. If less invasive measures are not able to make the diagnosis, computed tomography guided core needle biopsy of the mediastinal mass is considered. Finally, if necessary, mediastinoscopy, thoracoscopy, or anterior mediastinotomy may be required to establish the diagnosis.

If the risk of anesthesia or heavy sedation is too great, preoperative treatment with radiation therapy or steroids without biopsy is considered.