Outcomes

Treatment

The role of surgery in the initial diagnosis and staging for HL has been reduced. With the wide application of chemotherapy in all stages of HL, surgical staging has become irrelevant, because the additional information it provides does not alter treatment. The surgeon's primary role is to obtain tissue for diagnosis. Biopsies should be taken from the most easily accessible site, and adequate tissue must be obtained and sent fresh to pathology for immunohistochemistry, immunopheno-typing, cytogenetics, and flow cytometry. Fine-needle aspiration is generally discouraged, because it is inaccurate and inadequate tissue is obtained to properly stage and classify the patient.

Children and adolescents with HL are divided into three risk categories: low-, intermediate-, and high-risk disease-based on clinical and pathologic staging data, histology, stage at presentation, presence or absence of B symptoms, number of involved sites, and/or presence of bulky disease (>10 cm). The exact definitions of each stage will often change between studies and clinical trial consortiums, such as the Children's Oncology Group (COG).

Treatment for HL is based largely on the stage of the disease. But other factors, including a person’s age and general health, and the type and location of the disease, may also affect treatment options.

Several types of treatment can be used for Hodgkin disease:

· Chemotherapy

· Radiation therapy

· Monoclonal antibodies

· High-dose chemotherapy and stem cell transplant

The 2 main methods of treating HL are chemotherapy and radiation therapy. Depending on the situation, one or both of these treatments might be used.

Chemotherapeutic drug regimens include MOPP (mechlorethamine, vincristine, procarbazine, prednisone) and ABVD (adriamycin, bleomycin, vinblastine, dacarbazine). Combinations of MOPP and ABVD with lower radiation doses has reduced treatment toxicity. Combined chemotherapy and lower dose radiation therapy is effective and achieves local control in over 95% of cases.

Monoclonal antibodies and high-dose chemotherapy with stem cell transplants may be used for certain patients, especially if other treatments haven’t worked. Except for biopsy and staging, surgery is rarely used to treat Hodgkin disease.

Newer protocols obtain close to 95% overall five-year survival in children. The youngest patients have the best prognosis. Histologically, the lymphocyte depleted subtype has the worst prognosis, but it is very unusual in children. The lymphocyte predominant subtype has the best prognosis.

There are significant complications of treatment related to both chemotherapy and radiation. The complications of chemotherapy depend on the specific agents used but include myelosuppression, cardiovascular changes, pulmonary problems, gonadal problems and neurologic impairment.

Mantle radiation causes adverse effects such as hypothyroidism, myelosuppression, pericarditis, pneumonitis, nephritis, skeletal hypoplasia, osteonecrosis, gonadal dysfunction, increased risk of breast cancer and growth retardation.

Secondary neoplasms (acute nonlymphocytic leukemia, thyroid carcinoma, parathyroid adenoma, soft tissue sarcoma, osteogenic sarcoma, Non-Hodgkin lymphomas, etc.) are probably radiation induced. The incidence of a secondary leukemia in children treated with MOPP is about 5-7% and increases to about 10% ifradiation therapy is administered. The peak incidence for leukemia following treatment for Hodgkin's disease occurs at 5 years following therapy. Solid tumors develop in about 4-5% of patients.

Non-Hodgkin's Lymphoma

Non-Hodgkin lymphomas (NHLs) comprise a heterogeneous group of tumors that has a constantly evolving classification system. The current World Health Organization (WHO) pathologic classification identifies almost 60 unique subtypes based on morphologic, immunophenotypic, and genetic differences, as well as clinical behaviour.

NHL can be broadly divided based on the cell of origin (B-cell or T-cell) or on clinical behavior (indolent, aggressive, or highly aggressive).

There are distinct differences between adult and pediatric NHL, with a strong bias toward precursor B-lymphoblastic and T-lymphoblastic lymphoma, anaplastic large cell lymphoma, and Burkitt lymphoma in childhood.

Indolent lymphomas are slowly progressive but incurable diseases, with a median survival time of 8 to 10 years. Aggressive lymphomas, such as Burkitt and Burkitt-like lymphomas, are rapidly progressive at presentation but curable in 70% to 90% of patients, with outcome strongly dependent on clinical and biological features (as identified by current molecular and immunologic approaches) at presentation.