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Disease Profile

Littoral cell angioma of the spleen

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Littoral cell angioma

Summary

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]

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.[1][5] Currently, the main treatment for LCA is open splenectomy (spleen removal) or hand-assisted laparoscopic total splenectomy, a less-invasive technique.[1] 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.[6][7]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Littoral cell angioma of the spleen. Click on the link to view a sample search on this topic.

References

  1. Du J, Shen Q, Yin H, Zhou X, Wu B. Littoral cell angioma of the spleen: report of three cases and literature review. Int J Clin Exp Pathol. July 1, 2015; 8(7):8516-8520. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4555755/.
  2. Gupta, P., et al.. Littoral Cell Angioma of Spleen: An Uncommon Presentation of a Rare Neoplasm. J. Clin Imaging Sci. November 30, 2012; 2:69:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3551492/.
  3. Rana, N. et al.. Case Report: Littoral cell angioma of spleen. Indian J Radiol Imaging. Aug 19, 2009; 19(3):210-212. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2766879/.
  4. Anbardar MH, Kumar PV, Forootan HR. Littoral cell angioma of the spleen: Cytological findings and review of the literature. J Cytol. April-June, 2017; 34(2):121-124. https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/28469325/.
  5. 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. Minerva Chir. August, 2014; 69(4):229-237. https://www.ncbi.nlm.nih.gov/pubmed/24987971.
  6. Cui XW, Ignee A, De Molo C, Schreiber-Dietrich D, Woenckhaus M, Dietrich CF. Littoral cell angioma of the spleen. Z Gastroenterol. February, 2013; 51(2):209-212. https://www.ncbi.nlm.nih.gov/pubmed/23417366.
  7. Leung VA, Tang S, Mahe E, Patlas MN. Littoral Cell Angioma: Diagnosis by Image-Guided Biopsy. Ann Clin Lab Sci. 2012; 42(4):417-421. https://www.annclinlabsci.org/content/42/4/417.full.pdf.