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

Abstract

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.

Littoral Cell Angioma of the Spleen: A Case Report

“…Around 150 cases have been reported worldwide. An association between LCA and autoimmune or metabolic disorders has been reported [5][6][7][8][9][10][11]. Additionally, LCA has been shown to recur after surgical excision and leads to distant metastasis [13][14][15][16].…”

“…Around 150 cases have been reported worldwide with most reported cases being benign but with unspecified malignant potential [1][2][3][4][5][6]. LCA is more prevalent in Asia and South America [5][6][7][8]. It usually presents as isolated splenomegaly with a single lesion or multiple recurrent lesions.…”

“…It usually presents as isolated splenomegaly with a single lesion or multiple recurrent lesions. Although the etiology of LCA remains mostly unknown, an association between LCA and autoimmune or metabolic disorders has been reported [5][6][7][8][9][10][11]. There are no specific imaging findings of LCA, and the differential diagnosis after imaging includes hamartoma, lymphoma, Kaposi's sarcoma, lymphangioma, and hemangioma.…”

Case Report: Littoral Cell Angioma in a Patient with Rectal Carcinoma

“…That then would allow diagnosing it by biopsy without mandatory having to perform a splenectomy given the benign nature of these tumors. A new study published in February 2022 suggested that an image-guided biopsy with follow-up be the optimal diagnostic choice for patients with Littoral Cell Angioma, but a pathological uncertainty is in the cytology always possible, and standard recommendations still need to be made for the diagnostic procedure [8].…”

“…Disseminated disease is reported in rare cases and is a sign of atypical but not frankly malignant course of this entity [7]. According to a new study comprising the analysis of 435 cases of Littoral Cell Angioma, this entity should be considered as a benign vascular tumor, which is the intrasplenic manifestation of abnormal body function (due to the high rate of coexisting malignancy or immune dysfunction) [8].…”