Splenic histiocytic sarcoma: Disease progression from the perspective of pathophysiology.

Abstract

Splenic histiocytic sarcoma (SHS) is a rare, aggressive hematological malignancy with unclear progression and management. Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis, treatment and management of SHS. A 60-year-old female with incidentally detected splenic mass (6.0 cm × 5.7 cm) underwent splenectomy, confirmed as SHS in 2020. Post-op imatinib therapy was given. In 2022, hepatic metastases (2.4 cm × 2.9 cm) with pancytopenia led to supportive care. Lesions enlarged to 4.3 cm × 2.7 cm, leading to multi-organ failure and death at 33 months. The case was categorized into three distinct stages based on the pathophysiology of SHS: Early-stage splenic tumor growth, mid-stage liver metastasis with hematological abnormalities, and late-stage tumor infiltration leading to multiorgan failure. For SHS, this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.