Littoral Cell Angioma Of The Spleen:10 Cases Series And Collective Review Of 346 Cases In Literature

Background:Littoral cell angioma(LCA)is a rare vascular tumor of the spleen.It is a benign neospasm which is thought to rise from the splenic sinus.LCA was initially described in 1991 and much is unknown about this entity because of its rarity.Since more and more cases accumulate,its association with malignancies and immunological disorders has been reported.Therefore,it is necessary to update our information to make a better understanding of this tumor by statistical analysis of pathological characteristicsPurpose:The purpose of this study were to present a series of 10 patients with LCA in Qilu hospital and research the previous published literature about LCA to summarize the information of all the patients.Then we could elaborate our understanding of LCAMethod:We present a report of 10 cases in our institute and make a comprehensive review of literature till 30 November 2019.All relative individual patient data(including demographics and history,imaging,pathology,diagnosis and therapeutics,prognosis and comorbidities)were retrieved from the original reports,and hot topics were summarized and discussed accordinglyResults:346 cases from the literature and 10 cases from our hospital were systematically reviewed and analyzed.Gender was almost equally distributed and the median age was 49.6±17.1 years.50.0%(123/246)of the cases were asymptomatic,about 37%(91/246)of the cases were abdominal pain and discomfort.Splenomegaly and hypersplenism were reported in 59.5%(209/351)and 50.7%(74/142)of cases,respectively.Ultrasonography(US)showed a hypoechonic mass in 30 of 51 patients.58.9%(30/51)patients' tumors presented low-density in plain CT scan,and about 87.4%(76/87)of them presented delayed enhancement in enhanced CT.There was no specificity in MRI.And the leisions usually showed positive immunohistochemical staining for CD68(99.6%,227/228)and factor ?(95.6%,152/159).The diagnosis of LCA mainly depends on postoperative pathological examination,and percutaneous biopsy can also be useful before operation.And concerns also arise recently about association between LCA and coexisting malignancies and immunological disorders.There are 75 various malignancies concomitant with LCA reported in 70 patients.Meanwhile,there are also more and more cases of LCA with autoinmune disorders discribed in literature,such as Gaucher's disease and Crohn's disease,as well as renal transplantation and systemic lupus erythematosus(SLE)in which patients need to take chronic immunosuppression therapy.About the treatment,splenectomy was the most common and splenic arterial embolization was also worthy of considerationConclusion:Littoral cell angioma typically exhibits a benign clinical course,but rare cases with malignant potentials were reported,such as recurrence and metastasis after operation.There was no specificity in clinical symptoms,imaging findings and laboratory examination in preoperative diagnosis.Percutaneous biopsy may be cautiously recommended.And its association with malignancies and immunological disorders has been reported,therefore a long-term follow-up is necessary.Etiology and long-term follow-up for natural course and prognosis of LCA should be focused on for future studies and investigations.