Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Multidisciplinary Approach

Abstract

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. The 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. Then the tumor in IVC and the right internal iliac vein were removed after 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. The patient underwent total hysterectomy with bilateral salpingooophorectomy. Complete tumor resection was achieved. Histopathological analysis confirmed 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 final operation.

Rare case of high-grade endometrial stromal sarcoma metastasising to inferior vena cava and right atrium

“…ESS is known to metastasise and recur in lymph nodes, bone, peritoneum, retroperitoneum, lung, bowel, heart and skin 8–10. There have been numerous case reports and case series about the extension of LG-ESS tumour thrombus into the IVC, extending to the heart and even to the pulmonary vasculature 11–13. However, there are no cases of HG-ESS showing such a pattern of spread.…”

Recurrent malignant peripheral nerve sheath tumor presenting as an asymptomatic intravenous thrombus extending to the heart: a case report

“…Sarcomas with vascular tissue origins, such as intimal sarcoma [ 21 ] and angiosarcoma [ 22 , 23 ], reportedly extend into large vessels. However, there are a few reports on sarcoma from non-vascular tissues extending into large vessels, including cases of low-grade endometrial stromal sarcoma [ 24 ], clear cell sarcoma of the kidney [ 25 ], osteosarcoma [ 26 ], and renal Ewing’s sarcoma [ 27 ]. MPNSTs extending to the great vessels are extremely rare, with only one other case reported in the literature [ 28 ].…”

Recurrent Malignant Peripheral Nerve Sheath Tumor Presenting As an Asymptomatic Intravenous Thrombus Extending to the Heart: A Case Report

“…Sarcomas with vascular tissue origins, such as intimal sarcoma [21] and angiosarcoma [22,23], reportedly extend into large vessels. However, there are a few reports on sarcoma from non-vascular tissues extending into large vessels, including cases of low-grade endometrial stromal sarcoma [24], clear cell sarcoma of the kidney [25], osteosarcoma [26], and renal Ewing's sarcoma [27]. MPNSTs extending to the great vessels are extremely rare, with only one other case reported in the literature [28].…”