Spleen angiosarcoma: a world review

Introduction: Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and ma...

Full description

Saved in:
Bibliographic Details
Main Authors: Lee, Brian Juin Hsien, Shelat, Vishal Girishchandra
Other Authors: Lee Kong Chian School of Medicine (LKCMedicine)
Format: Article
Language:English
Published: 2022
Subjects:
Online Access:https://hdl.handle.net/10356/159278
Tags: Add Tag
No Tags, Be the first to tag this record!
Institution: Nanyang Technological University
Language: English
Description
Summary:Introduction: Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and management. In this study, we aim to provide a comprehensive review of SA. Areas covered: On 27 February 2021, a literature search was done in PubMed and Embase database. The search yielded 122 articles involving 205 patients. The focus was on patient demographics, risk factors, clinical presentations, investigation results, preliminary diagnoses, therapies provided, and patient outcomes. These factors were analyzed to identify possible risk factors, diagnostic modalities, and therapeutic principles that were not mentioned before. Expert opinion: The clinical presentation or investigation results of patients with SA are often nonspecific. Hence, they may not be sufficient to clinch the diagnosis of SA if used alone. The authors recommend a triple assessment of clinical examination, imaging findings, and pathology to diagnose SA with high accuracy. Splenectomy should be the mainstay of management, with chemotherapy and radiotherapy considered as adjuncts, especially in the presence of metastases.