Sarcoma de células dendríticas foliculares hepáticas primarias: estudio de caso y revisión de la literatura

Follicular dendritic cell sarcoma, a rare malignant tumor arising from specialized antigen-presenting cells, typically occurs in lymph nodes but can also develop at extranodal sites, including the liver. We present a case of a woman in her late 20s with systemic lupus erythematosus who presented with fever as the chief complaint. Imaging revealed a hepatic mass, and pathology confirmed the diagnosis of hepatic follicular dendritic cell sarcoma. The patient showed no recurrence 1 year post-radical surgery. We analyzed a total of 43 cases from 32 previous reports and the present case. Among these, the mean age at onset was 48 years, the median maximum tumor diameter was 87.5 mm, and there was a female predominance. Survival analysis showed that the 5-year disease-free survival of patients was 63.4%. This case and literature review highlight the rarity and diagnostic challenges of primary hepatic follicular dendritic cell sarcoma, especially in patients with atypical presentations such as fever of unknown origin. Early recognition and radical surgery are crucial for a favorable prognosis.