Primary Cutaneous Lymphomas in Children: A Case Series Review with Emphasis on Diagnostic Imaging and Multidisciplinary Management.

INTRODUCTION

Primary Cutaneous Lymphomas (PCLs) are rare extranodal non-Hodgkin lymphomas that present in the skin without extracutaneous disease at diagnosis; they are exceptionally uncommon in children and frequently mimic benign dermatoses, delaying recognition. We report a case series of three pediatric patients managed at a national referral center.

CASE PRESENTATION: Case 1: A 16-year-old with erythrodermic mycosis fungoides (stage IIIA) refractory to multiple systemic and skin-directed therapies who achieved remission after haploidentical allogeneic hematopoietic stem-cell transplantation. Case 2: A 3-year-old with aggressive cytotoxic CD8-positive Tcell lymphoma with EBV-associated disease and severe infectious complications during CHOP-based therapy, culminating in death despite salvage treatments and EBRT. Case 3: A 10-year-old with CD30-positive primary cutaneous anaplastic large cell lymphoma with a relapsing course, treated with BFM-90 chemotherapy, gemcitabine, pegylated doxorubicin plus total skin electron beam therapy, and ongoing PUVA with good dermatologic control. Across cases, diagnosis relied on clinicopathologic correlation with immunohistochemistry and staging CT; serum IgE was elevated in all three children.

CONCLUSION

Pediatric PCLs show heterogeneous behavior and therapeutic responses. Early biopsy of atypical or treatment-refractory eruptions, comprehensive histopathology and immunophenotyping, targeted EBV testing when suggested, and appropriate use of skin-directed radiotherapy (EBRT/TSEBT) and transplantation in selected refractory disease are essential. Multidisciplinary management and equitable access to specialized therapies are critical to optimize outcomes.