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. 2022 Feb 8:12:790332.
doi: 10.3389/fonc.2022.790332. eCollection 2022.

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

Weijie Wang  1 , Guangzhao Qi  2 , Xiangtian Zhao  3 , Yanping Zhang  4 , Rongtao Zhu  1 , Ruopeng Liang  1 , Yuling Sun  1
Affiliations

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

Weijie Wang et al. Front Oncol. .

Abstract

Objective: Littoral 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.

Methods: PubMed, 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.

Results: The 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-.

Conclusion: LCA 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.

Keywords: diagnosis; littoral cell angioma; prognosis; systematic review; treatment.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Selection of studies for inclusion in the systematic review of LCA.
Figure 2
Figure 2
General characteristics of LCA patients. (A) Age distribution of LCA. (B) Statistics on various malignant tumors co-occurred with LCA. (C) Statistics on comorbid diseases of immune dysfunction. (D) Statistics on comorbid benign diseases except for immune dysfunction.
Figure 3
Figure 3
Representative imaging of LCA. (A) Ultrasound imaging often showed hyperechoic lesions. (B, C) The PET/CT showed no abnormal uptake of 18F-FDG. (D) On nonenhanced CT, LCA often manifested as single or multiple hypodense nodules. (E, F) On enhanced CT, LCA characterized by delayed reinforcement. (G) In T2WI, the splenic lesions mostly manifested as a high signal intensity. (H, I) Similar to contrast-enhanced CT, Enhanced MRI showed a delayed enhancement.
Figure 4
Figure 4
The typical pathological features of LCA. (A) (×40) & (B) (×200). HE staining of LCA showed sinus like anastomosing channels with an irregular lumen, which was lined with tall, plump endothelial cells. Of the vascular markers, LCA reacted positively with (C) CD31, (D) factor VIII, and (E) CD34. Of the histiocytic markers, LCA typically expressed with (F) CD68 and (G) CD163. (H) A positivity rates of Ki67 was approximately 5%.
Figure 5
Figure 5
A process of diagnosis and treatment for LCA.
See this image and copyright information in PMC

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