Littoral cell angioma: A rare cause of pediatric thrombocytopenia

ObjectiveLittoral cell angioma (LCA) is currently considered to be a rare splenic tumor with malignant potential. As the epidemiology, pathogenesis, clinical manifestation, treatment, and prognosis remain unclear, the clinical diagnosis and treatment of LCA have not been standardized. Hence, we performed a comprehensive analysis of 189 observational studies comprising 435 patients to improve the current status of diagnosis and treatment.MethodsPubMed, Embase, WanFang and CNKI were searched from inception to May 2021 to identify LCA studies that were published in English and Chinese. The clinical information of LCA patients were extracted and analyzed.ResultsThe LCA has a male-to-female ratio of 0.90 and a solitary-to-multiple ratio of 0.31. In terms of clinical features, 69.7% of the patients showed splenomegaly, 49.7% were asymptomatic, and 39.2% experienced epigastric discomfort. As the imaging findings of patients with LCA were nonspecific, an image-guided biopsy (10/12) was a safe and effective method for diagnosing in this condition. Notably, results of the prognostic analysis indicated that LCA has a lower risk of recurrence and metastasis. The patient may develop a stable disease or the tumor will grow but will not metastasize. Besides, the novel immunohistochemical pattern of LCA was described as CD31+/ERG+/FVIII Antigen+/CD68+/CD163+/lysozyme+/CD8−/WT1−.ConclusionLCA should be reconsidered as a benign primary splenic vascular neoplasm, which is more like an intra-splenic manifestation of abnormal body function. Image-guided biopsy with follow-up might be a beneficial choice for LCA patients. For LCA patients with abdominal discomfort, pathological uncertainty or continuous tumor enlargement, splenectomy remains the preferred treatment.

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Case report and literature review: Asymptomatic littoral cell angioma in a 3-year-old girl

Mou,

Yang,

Wang

et al. 2024

Front. Pediatr.

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BackgroundLittoral cell angioma (LCA) is an extremely uncommon benign vascular tumor of the spleen. Cases of LCA in infants are rarely reported, and due to the rarity of the tumor and non-specific symptoms, the diagnosis of LCA is often overlooked in clinical practice.Case reportWe present a 3-year-old girl with pulmonary inflammation who was admitted to the hospital due to the discovery of a space-occupying lesion in the spleen. Pathology after splenectomy confirmed LCA, and there was no recurrence observed at the 5-month follow-up examination.ConclusionLCA should be considered when a child shows asymptomatic splenomegaly, with antigen expression indicating dual positivity of endothelial and histiocytic markers. Laparoscopic splenectomy remains the primary method of treating LCA.

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Littoral cell angioma: A rare cause of pediatric thrombocytopenia

Cited by 3 publications

References 7 publications

Littoral cell angioma of the spleen in a child with Cornelia De Lange syndrome

Littoral cell angioma of the spleen in a child with Cornelia De Lange syndrome

Clinical Landscape of Littoral Cell Angioma in the Spleen Based on a Comprehensive Analysis

Case report and literature review: Asymptomatic littoral cell angioma in a 3-year-old girl

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