Littoral cell angioma of the spleen is a rare, benign primary vascular tumor of the spleen.
Littoral cell angiomas may occur at any age and have no gender predilection.
Littoral cell angiomas have been diagnosed in association with various malignancies outside the spleen 10,12. The condition has also been linked to Crohn disease 13,14.
The lesion is often detected incidentally in asymptomatic patients. Symptomatic patients may present with abdominal pain, splenomegaly on physical examination, and/or laboratory evidence of hypersplenism including anemia and thrombocytopenia.
Littoral cell tumors arise from the littoral cells lining the sinusoids of splenic red pulp. Littoral cell angiomas are the benign type, as opposed to the less common, low-grade malignant littoral cell hemangioendothelioma and the rare and aggressive littoral cell angiosarcoma 9.
The spleen is enlarged with multiple nodules, the color of which varies by age of blood products 7.
Histopathology demonstrates a proliferation of anastomosing vascular channels congested with blood and lined with tall endothelial cells that show hemophagocytosis 7,8.
The tumor cells express both vascular endothelial markers (such as CD31 and factor VIII) and histiocytic markers (such as CD68) 7,8.
Imaging usually demonstrates splenomegaly and multiple mass lesions.
The masses are hypoattenuating compared to normal spleen 2-8. They demonstrate progressive homogeneous contrast enhancement, later than normal splenic parenchyma 2.
The masses are often low in T1 and T2 signal intensity due to hemosiderin 7.
Treatment and prognosis
As many patients are symptomatic and imaging findings are non-specific, splenectomy is usually performed for definitive histological diagnosis and treatment. Littoral cell angiomas are considered benign.
The imaging differential includes
- other primary splenic vascular tumors, such as hemangioma, hamartoma, hemangioendothelioma, angiosarcoma
- 1. Abbott RM, Levy AD, Aguilera NS et-al. From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics. 24 (4): 1137-63. doi:10.1148/rg.244045006 - Pubmed citation
- 2. Levy AD, Abbott RM, Abbondanzo SL. Littoral cell angioma of the spleen: CT features with clinicopathologic comparison. Radiology. 2004;230 (2): 485-90. doi:10.1148/radiol.2302030196 - Pubmed citation
- 3. Grantham M, Einstein D, Mccarron K et-al. Littoral cell angioma of the spleen. Abdom Imaging. 23 (6): 633-5. Abdom Imaging (link) - Pubmed citation
- 4. Cheng SP, Yang TL, Chen BF et-al. Image of the month. Littoral cell angioma. Arch Surg. 2005;140 (11): 1127-8. doi:10.1001/archsurg.140.11.1127-a - Pubmed citation
- 5. Abbott RM, Levy AD, Aguilera NS et-al. From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics. 24 (4): 1137-63. doi:10.1148/rg.244045006 - Pubmed citation
- 6. Goldfeld M, Cohen I, Loberant N et-al. Littoral cell angioma of the spleen: appearance on sonography and CT. J Clin Ultrasound. 2002;30 (8): 510-3. doi:10.1002/jcu.10101 - Pubmed citation
- 7. Bhatt S, Huang J, Dogra V. Littoral cell angioma of the spleen. (2007) AJR. American journal of roentgenology. 188 (5): 1365-6. doi:10.2214/AJR.06.1157 - Pubmed
- 8. Dascalescu CM, Wendum D, Gorin NC. Littoral-cell angioma as a cause of splenomegaly. (2001) The New England journal of medicine. 345 (10): 772-3. doi:10.1056/NEJM200109063451016 - Pubmed
- 9. Sarandria JJ, Escano M, Kamangar F, Farooqui S, Montgomery E, Cunningham SC. Massive splenomegaly correlates with malignancy: 180 cases of splenic littoral cell tumors in the world literature. (2014) Minerva chirurgica. 69 (4): 229-37. Pubmed
- 10. Peckova K, Michal M, Hadravsky L, Suster S, Damjanov I, Miesbauerova M, Kazakov DV, Vernerova Z, Michal M. Littoral cell angioma of the spleen: a study of 25 cases with confirmation of frequent association with visceral malignancies. (2016) Histopathology. 69 (5): 762-774. doi:10.1111/his.13026 - Pubmed
- 11. Cai YQ, Wang X, Ran X, Liu XB, Peng B. Laparoscopic splenectomy for splenic littoral cell angioma. (2015) World journal of gastroenterology. 21 (21): 6660-4. doi:10.3748/wjg.v21.i21.6660 - Pubmed
- 12. Bisceglia M, Sickel JZ, Giangaspero F, Gomes V, Amini M, Michal M. Littoral cell angioma of the spleen: an additional report of four cases with emphasis on the association with visceral organ cancers. (1998) Tumori. 84 (5): 595-9. Pubmed
- 13. Cappello M, Bravatà I, Cocorullo G, Cacciatore M, Florena AM. Splenic littoral cell hemangioendothelioma in a patient with Crohn's disease previously treated with immunomodulators and anti-TNF agents: a rare tumor linked to deep immunosuppression. (2011) The American journal of gastroenterology. 106 (10): 1863-5. doi:10.1038/ajg.2011.204 - Pubmed
- 14. Sarandria JJ, Escano M, Kamangar F, Farooqui SO, Montgomery E, Cunningham SC. Littoral cell angioma: gastrointestinal associations. (2014) Gastrointestinal cancer research : GCR. 7 (2): 63-4. Pubmed
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