What are the conditions associated with LCA?
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. [2] [3] LCA affects men and women equally and can occur at any age, but it mostly occurs in middle-aged adults ...
Why is LCA difficult to diagnose?
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. [1] . For this reason, the specific tumor type often cannot be confirmed until it is biopsied or removed, and then tested.
What is a LCA?
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. [1] [2] [3] Besides abdominal pain, LCA may cause an enlarged spleen (splenomegaly), anemia, or thrombocytopenia. [4] 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. [2] [3] LCA affects men and women equally and can occur at any age, but it mostly occurs in middle-aged adults and occasionally in children. [1]
Introduction
Littoral cell angioma (LCA) is a rare splenic vascular neoplasm originating from the littoral cell lining the red sinuses ( 1 – 4 ). Falk was the first to describe and name this entity; since its initial description, more and more studies have been conducted to investigate LCA ( 1, 5 – 9 ).
Materials and Methods
Two researchers independently searched the PubMed, Embase, WanFang, and CNKI databases from the inception to May 2021 to find published articles related to LCA. The search terms used included littoral cell angioma or littoral cell tumor or littoral cell without restrictions.
Results
A total of 189 studies comprising 435 patients met the inclusion criteria ( Figure 1, Table 1, and Table S1 ), namely, 171 foreigners and 264 Chinese individuals. The statistical analysis showed a male-to-female ratio of 0.90 in 405 patients with LCA, which indicated the absence of gender predilection.
Discussion
To the best of our knowledge, this was the first systematic review and analysis of all observational studies to describe the clinical landscape of LCA. Due to the low incidence of LCA, studies on LCA published in English and Chinese were collected to summarize the epidemiological characteristics.
Data Availability Statement
The original contributions presented in the study are included in the article/ Supplementary Material. Further inquiries can be directed to the corresponding author.
Author Contributions
WW and YS designed the study. WW, RZ, and RL collected clinical data. WW, GQ, XZ, and YZ wrote the manuscript. All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.
Funding
The study was supported by the National Natural Science Foundation of China (81900558) and the Henan Medical Science and Technique Foundation (SBGJ202003035).
What is littoral cell angioma?
Littoral cell angioma (LCA) is a rare primary splenic hemangioma, which can appear in the red sinus shore cells of the reticuloendothelial system (RES) in the spleen. Falk et al. were the first to describe and name LCA in 1991. Most of the reported cases are that of benign tumors and can present as a single lesion as well as multiple recurrent lesions. [1] The cells express both endothelial as well as histiocytic antigens, a characteristic feature of double differentiation of littoral cells, in contrast to the splenic red pulp cells that only express endothelial markers. The diagnosis of LCA relies on morphology and immunohistochemical studies. Fine needle aspiration cytology can aid in some cases in narrowing the differential before the operative excision of the tumor. [2]
What are the complications of littoral cell angioma?
The complications of littoral cell angioma could be secondary to hypersplenism leading to anemia, thrombocytopenia, abdominal pain, and weight loss. Additionally, the patient can develop early postoperative complications of splenectomy performed for treatment of LCA like bleeding, incision site infection, hernia, atelectasis, pulmonary and abdominal sepsis, pancreatitis, pulmonary embolism. [5] Asplenic patients are at an increased lifelong risk of developing post-splenectomy septic syndrome and infections with encapsulated organisms. The administration of appropriate immunizations does not eliminate the risk (for example, pneumococcal vaccination). [12]
What is differential diagnosis of vascular neoplasms of the spleen?
The differential diagnosis includes other vascular neoplasms of the spleen, including splenic hemangioma, lymphangioma, hamartoma, angiosarcoma. The LCA can be differentiated from these lesions based on the histopathological and immunophenotyping findings, as detailed above. Imaging studies (MRI, CT scan, ultrasound) have not demonstrated usefulness in differentiating between these.
Can LCA be diagnosed by FNAC?
The diagnosis of LCA is only possible by histopathological examination. Fine needle aspiration cytology (FNAC) is an option for splenic masses; however, it is not very specific and is not recommended given the risk of bleeding and tumor cell dissemination if the tumor is malignant. [2]
Is LCA a congenital disease?
The exact pathogenesis mechanism leading to the development of LCA is not known. However, the association of the LCA with congenital and immunological disorders does point towards the possible role immune dysregulation plays in the pathogenesis of these tumors. There have been case reports describing the association of cancers of the lung, colon, pancreas, kidney, and ovary with LCA. Also rarely reported are leiomyosarcoma, lymphoma, and melanoma. Therefore, one should always be mindful of the possibility of and rule out other neoplasms in all patients diagnosed with LCA. [6] In a retrospective case study conducted in 17 cases of LCA by Bi et al., 17% of the cases were found to correlate with congenital and immunological disorders like ankylosing spondylitis, aplastic anemia, myelodysplastic syndrome, chronic glomerulonephritis, Gaucher disease, Crohn disease, Wiskott-Aldrich syndrome, lymphocytic colitis, and Epstein syndrome. [7] The case study also proposed that the hyperplasia and anastomosis changes in the littoral cells could be secondary to hemodynamic disturbances in the spleen. These disturbances lead to hemangioma-like growth in the littoral cells along with histiocytic reaction.
Is LCA a malignant tumor?
Littoral cell angioma (LCA) is a rare splenic tumor. Both benign and malignant cases have been reported. This activity reviews the clinical presentation, evaluation, and treatment of LCA and explains the role of the interprofessional healthcare team in diagnosing and managing patients with this condition.
Can a splenectomy be performed for LCA?
It should only be performed by an experienced surgeon with particular attention to prevent splenic capsule rupture and, consequently, any tumor cell dissemination. A study done by Cai et al. demonstrated that it is feasible and safe to do LS for patients with LCA. Conversion to hand-assisted laparoscopic splenectomy (HALS) or even open surgery may be necessary when dealing with cases having extensive adhesions or massive splenomegaly, especially in patients with malignant tumors. [5]
Spleen-vascular tumors
Cite this page: Tsang P. Littoral cell angioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenlittoralcellangioma.html. Accessed February 20th, 2022.
Littoral cell angioma
Cite this page: Tsang P. Littoral cell angioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenlittoralcellangioma.html. Accessed February 20th, 2022.
