Vol. 7, Issue 1, Part A (2025)

Hodgkin lymphoma (HL) is a highly curable lymphoid malignancy characterized by the presence of Hodgkin and Reed-Sternberg (HRS) cells in a reactive background. While the disease typically presents with contiguous lymph node involvement, extranodal presentation is uncommon and often mimics infections, autoimmune disorders, or other neoplasms, making diagnosis and management more challenging. This study aims to review and analyze the clinical features, diagnostic strategies, and therapeutic outcomes of Hodgkin lymphoma with extranodal involvement, emphasizing its rarity, diagnostic pitfalls, and optimal management approaches. Extranodal HL most commonly involves bone, skin, and soft tissue. Clinical presentations are variable and often lead to misdiagnosis. Imaging (particularly PET-CT), tissue biopsy, and immunohistochemical analysis (e.g., CD30+, CD15+, PAX5+) are critical for accurate diagnosis. Standard ABVD chemotherapy remains the backbone of treatment and is effective even in rare extranodal sites. Newer therapies such as brentuximab vedotin and immune checkpoint inhibitors show promise in relapsed or refractory cases. We concluded that, extranodal presentation of HL requires heightened clinical suspicion and a multidisciplinary approach for early diagnosis. With timely intervention, including standard chemotherapy and targeted agents when needed, patients with extranodal HL can achieve excellent outcomes. Increased awareness of atypical presentations is essential for improving prognosis and avoiding delays in treatment.