Joseph Junewick, MD FACR
over 7 years ago
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Stage IIA Hodgkin Disease
Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 7 years ago
|Updated: over 7 years ago
16 year old female with enlarging painless right cervical and supraclavicular adenopathy.
Nodular Sclerosing Hodgkin Disease
No fever, night sweats or weight loss.
CT – Numerous enlarged lymph nodes in the right neck, right greater than left supraclavicular regions, right axilla, and mediastinum.
PET – Maximum intensity 3D reformat demonstrates marked F-18 FDG avidity.
Lymphoma (Hodgkin disease and non-Hodgkin lymphoma) is a neoplastic proliferation of immune cells and represents the third most common pediatric malignancy. While lymphoma is not just a pediatric disease, there are unique features of the disease presumably because of the age related differences in the immune system and unique considerations related to treatment.
Hodgkin disease has a bi-modal incidence with one age peak in adolescence and young adulthood and the other in late adulthood. Hodgkin disease is rare in children less than 5 years of age. Under the age of 10 years, males are more commonly affected, whereas in adolescence males and females are equally affected. Patients most often present with painless cervical or supraclavicular adenopathy. The mediastinum is involved at diagnosis in nearly 2/3 of patients. There are several subtypes of Hodgkin disease with the nodular sclerosing histological subtype being the most common, followed by mixed cellularity, lymphocyte predominance, and others. Histology predicts sites of involvement, stage, co-morbid conditions and clinical course.
Hodgkin disease usually spreads in a contiguous lymphatic pattern. Extranodal dissemination (typically stage IV) occurs as a result of direct extension or hematogenous spread, with the lung, liver, bones and bone marrow being the most common sites. Staging for Hodgkin disease is based on clinical and imaging evaluations. Clinical parameters include physical examination, sedimentation rate, liver function tests, and bone marrow aspiration and biopsy. Bone marrow aspiration and biopsy are performed in patients with stage III or IV disease or in patients exhibiting “B” symptoms. Imaging includes chest radiography, CT and/or MR examinations, Gallium-67 or FDG PET scan, and occasionally bone scan. CT and MR examinations predominantly determine anatomic extent of disease based on enlarged lymph nodes and visceral infiltration. FDG PET is now the preferred modality to assess metabolic activity although gallium-67 scanning is an acceptable alternative. Chest radiography remains an important method to assess for the presence of bulky disease (mediastinal mass > 33% of the transverse diameter of the chest at the level of the diaphragm). Bone scan may be necessary if there is bone pain or an elevated alkaline phosphatase level.
Treatment strategies are determined based on risk. Low-risk disease represents stage I or IIA with no bulk or B symptoms. High-risk disease is stage IIIB and IVB. Intermediate-risk disease is composed of all other stages. Most pediatric patients with Hodgkin’s disease receive combined chemotherapy as initial treatment. Chemotherapy is tailored based on risk stratification to minimize known side effects of diminished fertility, leukemia, pulmonary fibrosis, and cardiovascular damage. Radiation therapy (treatment volume and radiation dose) is usually protocol-specific. If radiation therapy is utilized in the treatment protocol it is usually low dose involved field radiation therapy because growth disturbance, breast vulnerability and cardiovascular complications are associated with standard dose radiation therapy. High dose chemotherapy with autologous or allogenic bone marrow transplantation may be used for refractory disease. The goal of treatment is complete response; any other outcome is considered a failure.
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