Malignant Tumors of the Spleen

Malignant tumors of the spleen are rare lesions that can be divided into three broad categories: lymphoproliferative diseases, metastatic lesions, and primary splenic (nonlymphoid) malignant neoplasms. Additionally, although myeloproliferative diseases are not tumors of the spleen, they do have the potential to either directly or indirectly affect splenic function, often manifesting as splenomegaly, hypersplenism, or both. Lymphoproliferative and myeloproliferative diseases are almost always systemic diseases. Consequently, medical therapy is the primary treatment, and splenectomy is currently reserved for patients with hematologic disorders refractory to medical management—for palliation of symptoms caused by splenomegaly or hypersplenism—and in the rare case of the pure splenic form of the disease. Patients affected by malignant splenic lesions often present with vague symptomatology, including generalized malaise, fatigue, weight loss, and fever. These symptoms may be accompanied by abdominal pain, a palpable left upper quadrant mass, or overt splenomegaly. Radiologic imaging, including ultrasonography, computed tomography, and magnetic resonance imaging, is mandatory in the evaluation of splenic tumors and may narrow the differential diagnosis. Management of splenic metastases should be individualized and, whenever possible, determined in a multidisciplinary setting. Splenectomy is the treatment of choice for patients with primary malignant tumors of the spleen in the absence of metastatic disease.  This review contains 10 figures, 8 tables and 51 references Key words: angiosarcoma, leukemia, malignant splenic tumor, metastatic splenic tumor, spleen, splenectomy, splenic lymphoma