This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Littoral cell angioma of the spleen (LCA) is a very rare, vascular tumor of the spleen. Most reported cases have been non-cancerous (benign), but an LCA can become cancerous (malignant). In many cases, LCA does not cause any symptoms. It may be discovered due to abdominal pain or by accident (incidentally) when having a physical exam or during tests performed for other reasons. Besides abdominal pain, LCA may cause an enlarged spleen (splenomegaly), anemia, or thrombocytopenia. Case reports have associated LCA with various other conditions including portal hypertension, Crohn’s disease, Gaucher disease, lymphoma, aplastic anemia, colon cancer, pancreatic cancer, lung cancer, and myelodysplastic syndrome. LCA affects men and women equally and can occur at any age, but it mostly occurs in middle-aged adults and occasionally in children.
LCA is difficult to diagnose with imaging studies because of its similar appearance to other types of benign splenic tumors as well as malignant tumors such as lymphoma and Kaposi sarcoma. For this reason, the specific tumor type often cannot be confirmed until it is biopsied or removed, and then tested. Currently, the main treatment for LCA is open splenectomy (spleen removal) or hand-assisted laparoscopic total splenectomy, a less-invasive technique. Some authors have suggested only continued surveillance of the tumor may be appropriate if no symptoms are present, because the chance for malignancy in an asymptomatic person is thought to be low, and splenectomy leads to impairment of immune function.
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