Abstract
BACKGROUND: Histiocytic sarcoma (HS) is a rare lymphohematopoietic malignancy with nonspecific clinical manifestations, diagnostic challenges, high aggressiveness, and a poor prognosis. Primary HSs arising in the spleen are extremely uncommon, with few cases reported globally. Here, we present the clinical course of a patient with splenic HS. CASE SUMMARY: A 67-year-old woman was admitted to our hospital because of a large splenic mass that was detected during a routine health examination 1 month before presentation. Abdominal computed tomography revealed a large occupying lesion in the spleen, which was possibly a lymphangioma. The patient underwent splenectomy, and postoperative pathological examination confirmed the diagnosis of splenic HS. At the 6-month telephonic follow-up, the patient reported feeling well. CONCLUSION: Given the paucity of cases and the poor prognosis of splenic HS, whose definitive diagnosis hinges exclusively on pathology, and given that all current therapeutic strategies are based on isolated case reports, it is imperative to enhance our understanding of this disease to improve patient diagnosis and management.