Littoral cell angioma of the spleen: a study of 25 cases with confirmation of frequent association with visceral malignancies

Abstract

The cohort of 25 patients is the largest series of LCAs published to date. By using antibodies against recently introduced endothelial markers, we have expanded the immunoprofile of LCA. We have further highlighted the clinical significance of LCA, as more than half of the patients in this study also harboured a coexisting visceral malignancy. Therefore, we conclude that the finding of splenic LCA mandates a thorough clinical evaluation for a concomitant malignancy.

Littoral cell angioma of spleen

“…These include colorectal cancer, renal cancer, pancreatic cancer, haematological cancer, ovarian cancer and testicular cancer. In one study, colorectal cancer was reported to be the most common . Our patient had clear cell renal cell carcinoma.…”

“…Patient usually presents with splenomegaly, abdominal pain, pyrexia of unknown origin, anaemia and thrombocytopenia. The incidence of LCA is unknown; however, incidence of splenic haemangioma varies from 0.03% to as high as 14% in one autopsy series . It often coexists with a broad spectrum of internal malignant neoplasms and occasional immunological and non‐neoplastic haematological disorders .…”

“…The incidence of LCA is unknown; however, incidence of splenic haemangioma varies from 0.03% to as high as 14% in one autopsy series . It often coexists with a broad spectrum of internal malignant neoplasms and occasional immunological and non‐neoplastic haematological disorders . These include colorectal cancer, renal cancer, pancreatic cancer, haematological cancer, ovarian cancer and testicular cancer.…”

Optimum immunohistochemical procedures for analysis of macrophages in human and mouse formalin fixed paraffin-embedded tissue samples

“…Littoral cell angioma is a rare primary splenic tumor arising from endothelial cells, and CD163 is a useful marker for the diagnosis of littoral cell angioma. 22,30 In conclusion, we summarized and listed the best methods for detecting macrophage-specific antigens in the present study. Antibodies listed in this article is also suggested to be useful for pathological diagnosis of HS and HLH/HPS, or for the assessment of TAMs.…”

A Novel Presentation of Littoral Cell Angioma and Lymphatic Malformations in a Neonate

“…9 Approximately 27% of patients who are diagnosed with LCA have a coexistent visceral malignancy, and 17% have immune dysregulation in the form of chronic immunosuppression or an autoimmune condition (such as Crohn disease, Epstein syndrome, Castleman Disease, Evans Syndrome, Gaucher Disease, and Wiskott-Aldrich Syndrome). 6,9,13,14 Thus, there is speculation that an underlying immune dysregulation contributes to the development of LCA. However, the rarity of LCA has limited research to test this hypothesis.…”