Case Report of Primary Splenic Angiosarcoma in a Young Patient: A Very Rare and Challenging Disease.

INTRODUCTION: Primary splenic angiosarcoma (PSA) is a rare type of angiosarcoma often associated with an unfavorable prognosis. The presenting symptoms are usually nonspecific, adding to the diagnostic difficulty of this disease. Limited cases have been described in younger adults, as most cases have been reported in individuals between 50 and 70 years old.

PRESENTATION OF CASE: We report the case of a 25-year-old patient with cerebral palsy who initially presented for high-grade fever and left upper quadrant (LUQ) pain. On computed tomography (CT) scan, a large heterogenous mass was discovered in the spleen, and the patient underwent total splenectomy with pathological analysis confirming the diagnosis of PSA. The patient had no further complications and is still clinically stable.

DISCUSSION: PSA's pathogenesis is not fully understood, though potential risk factors include exposure to chemicals or radiation, none of which were present in this case. Diagnosing PSA is challenging due to nonspecific symptoms and imaging findings that overlap with benign splenic lesions. Treatment primarily involves splenectomy, but prognosis remains poor due to the high likelihood of early metastasis. Adjuvant therapies, including chemotherapy and targeted agents, are under investigation.

CONCLUSION: PSA is a rare and aggressive malignancy with significant diagnostic and therapeutic challenges. Early recognition and timely management may be important in selected cases, and outcomes remain poor, highlighting the need for further research to optimize treatment strategies.