Case Report
Low-Grade Endometrial Stromal Sarcoma with Intravenous and
Intracardiac Extension: A Multidisciplinary Approach
Wataru Kudaka,
1
Hitoshi Inafuku,
2
Yuko Iraha,
3
Tomoko Nakamoto,
1
Yusuke Taira,
1
Rie Taira,
1
Hisashi Kamiya,
3
Maho Tsubakimoto,
3
Yuichi Totsuka,
2
Yukio Kuniyoshi,
2
Tomoko Tamaki,
4
Hajime Aoyama,
4
Masanao Saio,
4
Naoki Yoshimi,
4
and Yoichi Aoki
1
1
Department of Obstetrics and Gynecology, Graduate School of Medicine, University of the Ryukyus, 207 Uehara, Nishihara,
Okinawa 903-0215, Japan
2
Department of Toracic and Cardiovascular Surgery, Graduate School of Medicine, University of the Ryukyus, 207 Uehara,
Nishihara, Okinawa 903-0215, Japan
3
Department of Radiology, Graduate School of Medicine, University of the Ryukyus, 207 Uehara, Nishihara, Okinawa 903-0215, Japan
4
Department of Pathology and Oncology, Graduate School of Medicine, University of the Ryukyus, 207 Uehara, Nishihara,
Okinawa 903-0215, Japan
Correspondence should be addressed to Yoichi Aoki; yoichi@med.u-ryukyu.ac.jp
Received 25 March 2016; Accepted 14 April 2016
Academic Editor: Yoshio Yoshida
Copyright © 2016 Wataru Kudaka et al. Tis is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background. A rare case of low-grade endometrial stromal sarcoma (LG-ESS) extending to inferior vena cava (IVC) and cardiac
chambers. Case Report. A 40-year-old woman had IVC tumor, which was incidentally detected by abdominal ultrasonography
during a routine medical checkup. CT scan revealed a tumor in IVC, right iliac and ovarian veins, which was derived from
the uterus and extended into the right atrium and ventricle. Te operation was performed, the heart and IVC were exposed,
and cardiopulmonary bypass was initiated. A right atriotomy was performed, and the intracardiac mass was removed. Ten
the tumor in IVC and the right internal iliac vein were removed afer longitudinal venotomies in the suprarenal and infrarenal
vena cava, the right common iliac vein. Next the pelvis was explored. Tumors were found originating from the posterior wall of
the uterus and continuing into both the right uterine and ovarian vein. Te patient underwent total hysterectomy with bilateral
salpingooophorectomy. Complete tumor resection was achieved. Histopathological analysis confrmed a diagnosis of LG-ESS. She
showed no evidence of disease for 2 years and 3 months. Conclusions. Our case highlights the importance of a multidisciplinary
approach in treating this rare cardiovascular pathological condition through preoperative assessment to fnal operation.
1. Introduction
Low-grade endometrial stromal sarcoma (LG-ESS) has indo-
lent clinical progression with repeated local recurrence
within 10 years up to 50% of cases. Late mortality because
of lung metastases occurs in 15% of cases [1, 2]. ESS tends
to spread throughout the lymph nodes and venous system
but rarely involves large vessels or the heart. In general,
the intracardiac extension of LG-ESS is rare and most cases
derive from renal cell carcinoma, nephroblastoma, colon
adenocarcinoma, melanoma, hepatocellular carcinoma, or
bronchogenic carcinoma [3]. Only 22 cases of advanced
LG-ESS have been reported in which the great vessels
were invaded and a tumor of the inferior vena cava (IVC)
formed [3–6]. However, earlier studies show that >50% of
intravenous LG-ESS cases exhibit intracardiac extension [7].
Here, we report a rare case of LG-ESS extending to IVC
and cardiac chambers, which was treated with a multidis-
ciplinary approach. Written informed consent was obtained
from the patient for the publication of her medical details.
2. Case Presentation
A 40-year-old woman had received regular follow-up for a
fatty liver, and inferior vena cava (IVC) tumor, which was
initially thought to be a blood thrombus, was incidentally
Hindawi Publishing Corporation
Case Reports in Obstetrics and Gynecology
Volume 2016, Article ID 3467849, 6 pages
http://dx.doi.org/10.1155/2016/3467849