Zhou Y, Sun YW, Liu XY, Shen DH. Primary ovarian angiosarcoma: Two case reports and review of literature. World J Clin Cases 2023; 11(21): 5122-5128 [PMID: 37583851 DOI: 10.12998/wjcc.v11.i21.5122]
Corresponding Author of This Article
Dan-Hua Shen, Doctor, Chief Physician, Department of Pathology, Peking University People’s Hospital, No. 11 Xizhimen South Street, Beijing 100044, China. shendanhua@pkuph.edu.cn
Research Domain of This Article
Oncology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Ying Zhou, Yi-Wen Sun, Xiao-Yang Liu, Dan-Hua Shen, Department of Pathology, Peking University People’s Hospital, Beijing 100044, China
Author contributions: Zhou Y and Sun YW designed the research study; Zhou Y, Sun YW and Liu XY performed the research; Zhou Y and Shen DH wrote the manuscript; and all authors have read and approve the final manuscript.
Informed consent statement: Written informed consent has been obtained from the patient for publication of this case report.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Dan-Hua Shen, Doctor, Chief Physician, Department of Pathology, Peking University People’s Hospital, No. 11 Xizhimen South Street, Beijing 100044, China. shendanhua@pkuph.edu.cn
Received: April 8, 2023 Peer-review started: April 8, 2023 First decision: May 31, 2023 Revised: June 5, 2023 Accepted: June 27, 2023 Article in press: June 27, 2023 Published online: July 26, 2023 Processing time: 109 Days and 12 Hours
Abstract
BACKGROUND
Angiosarcoma (AS) is a rare and highly aggressive soft tissue disease that most commonly arises in deep soft tissues. There are only a few reported cases of AS involving the ovary and even fewer reports of the underlying molecular abnormalities. Here, we briefly review two cases of primary ovarian AS (oAS) with specific molecular events and immune checkpoints. The clinical features and prognosis of the disease, diagnosis, differential diagnosis, and new treatment approaches are discussed based on a literature review.
CASE SUMMARY
Case 1: A 51-year-old female patient was admitted with right lower limb pain for 5 mo, and lower abdominal pain with hematuria for 1 mo. Partial removal of rectus abdominis muscle and fascia, partial hysterectomy, bilateral salpingo-oophorectomy, and inguinal and pelvic lymphadenectomy were performed. Pathology revealed primary oAS. Fluorescence in situ hybridization revealed c-MYC gene amplification. MESNA + ADM + IFO + DTIC (MAID) regimen was administered, but stable disease was achieved. The patient died 1 mo later. Case 2: A 41-year-old female patient presented with fatigue, nausea, decreased appetite, and diffuse abdominal pain. On physical examination, the abdomen was distended and a complex cystic mass was palpable in the right pelvic cavity. Pathology revealed primary oAS. MAID chemotherapy was administered and programmed death ligand 1 (PD-L1) staining was performed on the tumor samples. The patient benefited from anti-PD-1 immunotherapy and is alive without any evidence of disease 27 mo off therapy in follow-up.
CONCLUSION
Long-term survival benefit for primary oAS can be achieved by alternative therapeutic strategies using pathological indicators to inform treatment.
Core Tip: Angiosarcoma (AS) is a rare and highly aggressive type of soft tissue that most commonly arises in deep soft tissues. Alternative therapeutic strategies such as targeted therapies and anti-programmed cell death 1/programmed cell death ligand 1 therapy may be promising for primary ovarian AS.
