Introduction

Lymphoma is the third most common malignancy of childhood, with an incidence of 30 per one million children age zero to 19 years [1]. From near universal mortality 60 years ago, there is now greater than 90% 10-year survival for Hodgkin lymphoma (HL) and greater than 70% long term survival for nonHodgkin lymphoma (NHL) [2].

As outcomes have improved, emphasis has been placed on maintaining high rates of long term disease free survival while minimizing morbidity from second malignancies and treatment associated cardiovascular disease. Modern protocols are risk based and response adapted. In practice, this means that most children receive systemic chemotherapy tailored to their disease stage while radiation therapy is given selectively to involved fields in HL and rarely in children with NHL. In many cases, assessment of treatment response by interim 18F-FDG positron emission tomography (PET-CT) imaging allows for response based treatment adaptation [3][4] with reduced treatment for good responders and intensified therapy for poor responders.

How has the role of surgery in pediatric lymphoma changed?

The role of surgery for diagnosing, treating and managing the complications of lymphoma has evolved significantly over the past four decades alongside the innovations in diagnostic imaging and medical therapy. Into the 1990s, laparotomy with surgical staging of Hodgkin disease was considered the standard of care in the United States. At that time, children with early stage disease were treated with radiation therapy alone. Prior to the widespread adoption of PET-CT, laparotomy altered staging in 25% of patients [5]. Unfortunately, in addition to the risk of postsplenectomy sepsis, staging laparotomy was associated with a 17% risk of subsequent bowel obstruction requiring additional laparotomy [6]. With the introduction of PET-CT and the switch to multimodal therapy including systemic chemotherapy for early stage disease, the benefits of staging laparotomy were negated. A randomized trial showed no difference in overall and relapse free survival in early stage patients who underwent clinical versus surgical staging [7]. Staging laparotomy has therefore been eliminated from North American HL protocols since the mid 1990s.

Although staging laparotomy is no longer performed, the surgeon still plays a critical role in establishing the diagnosis of lymphoma and managing the complications of the disease. There are also several rare but distinct scenarios in which surgical excision can minimize or eliminate systemic therapy. A working understanding of the current lymphoma protocols is also critical for maximizing diagnostic yield while avoiding unnecessary surgical morbidity.

content in this topic is referenced in SCORE Lymphoma/Leukemia overview