Published online Feb 16, 2023. doi: 10.12998/wjcc.v11.i5.1188
Peer-review started: November 14, 2022
First decision: November 30, 2022
Revised: December 9, 2022
Accepted: January 16, 2023
Article in press: January 16, 2023
Published online: February 16, 2023
Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen. Given its rarity, standard diagnostic and therapeutic recommendations have yet to be developed for reported cases. Splenectomy is the only method of obtaining a pathological diagnosis and providing treatment to obtain a favorable prognosis.
A 33-year-old female presented with abdominal pain for one month. Computed tomography and ultrasound revealed splenomegaly with multiple lesions and two accessory spleens. The patient underwent laparoscopic total splenectomy and accessory splenectomy, and splenic LCA was confirmed by pathology. Four months after surgery, the patient presented with acute liver failure, was readmitted, rapidly progressed to multiple organ dysfunction syndrome and died.
Preoperative diagnosis of LCA is challenging. We systematically reviewed online databases to identify the relevant literature and found a close relationship between malignancy and immunodysregulation. When a patient suffers from both splenic tumors and malignancy or immune-related disease, LCA is possible. Due to potential malignancy, total splenectomy (including accessory spleen) and regular follow-up after surgery are recommended. If LCA is diagnosed after surgery, a comprehensive postoperative examination is needed.
Core Tip: Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen. No standard diagnostic and therapeutic recommendations are available. We report a patient with LCA and without comorbidities who died of multiple organ dysfunction syndrome 4 mo after surgery, which is extremely rare. Systematic analysis of relevant cases in the PubMed, Embase, Web of Science and the Cochrane Library databases revealed that LCA has a close relationship with malignancy and immunodysregulation. The possibility of LCA should not be overlooked when a patient presents with splenic tumors and malignancy or immune-related disease. Considering its potential malignant behavior, total splenectomy (including accessory spleen) and regular follow-up after surgery are recommended.