Removal of an endometrioid stromal sarcoma from the inferior vena cava and right atrium

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A 30-year-old female patient underwent surgical removal of an endometrioid stromal sarcoma located in the inferior vena cava and right atrium following a history of ovarian endometrioid stromal sarcoma.

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The paper reports a case of a 30-year-old woman with a mass located in the inferior vena cava and right atrium, who underwent surgical removal using cardiopulmonary bypass and deep hypothermic circulatory arrest; the mass was diagnosed as an endometrioid stromal sarcoma. The case includes her history of endometriosis and a prior low-grade ovarian endometrioid stromal sarcoma diagnosed four years earlier. The authors note, generally for endometrioid stromal sarcomas, that despite a better prognosis than other sarcomas, relapse can occur in up to 56% of cases and even as late as 20 years after surgery. This paper is centrally about endometriosis — it describes endometrioid stromal sarcoma arising in an individual with a documented endometriosis history and previous ovarian endometrioid stromal sarcoma.

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Abstract

Entometrioid stromal sarcomas are seen in extra-uterine as well as extra-gonadal sites and have a strong association with endometriosis. Although having better prognosis than other sarcomas, yet these tumors may relapse (whether local or distant) in up to 56% of cases, even as late as 20 years after surgery. We report a case of a 30-year-old female patient with a mass in the inferior vena cava and right atrium which was surgically removed using cardiopulmonary bypass and deep hypothermic circulatory arrest and turned to be an entometrioid stromal sarcoma. The patient gave a history of endometriosis followed by the appearance of a low-grade ovarian endometrioid stromal sarcoma 4 years before the development of the mass in the IVC and right atrium. Similar content being viewed by others

References

Diesing D, Cordes T, Finas D, Löning M, Mayer K, et al. Endometrial stromal sarcomas—a retrospective analysis of 11 patients. Anticancer Res. 2006;26:655–61. Wong J, Tan G, Chia C, Selvarajan S, Teo M. A rare case of multiple malignancies arising in endometriosis: diffuse extra-uterine high-grade endometrial stromal sarcoma and ovarian carcinoma. Am J Cancer Case Rep. 2015;3:1–8. Mourra N, Tiret E, Parc Y, de Saint-Maur P, Parc R, et al. Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis. Arch Pathol Lab Med. 2001;125:1088–90. Alaaraj R, Lataifeh I, Elsaadi J, Salameh M, Sughayer M. Primary low-grade endometrioid stromal sarcoma of the ovary with multiple omental metastases. Gulf Med J. 2016;5:69–73. Ali RH, Rouzbahman M. Endometrial stromal tumors revisited: an update based on the 2014 WHO classification. J Clin Pathol. 2015;68:325–32. Al-Ebrahim KE. Right atrial mass: the dilemma of diagnosis and when not to operate (letter). Ann Thorac Surg. 2009;87:2005. Lauten A, Strauch JT, Wippermann J, Wahlers T. A rare type of right atrial tumor in a 66-year-old woman. J Thorac Cardiovasc Surg. 2007;133:251–2. Chiappini B, Savini C, Marinelli G, Martin Suarez S, DiEusanio M, et al. Cavoatrial tumor thrombus: single-stage surgical approach with profound hypothermia and circulatory arrest, including a review of the literature. J Thorac Cardiovasc Surg. 2002;124:684–8. Author information Authors and Affiliations Corresponding author Ethics declarations Conflict of interest The authors have no conflict of interest to declare. Rights and permissions About this article Cite this article Alkady, H., Abouramadan, S., Nagy, M. et al. Removal of an endometrioid stromal sarcoma from the inferior vena cava and right atrium. Gen Thorac Cardiovasc Surg 67, 324–327 (2019). https://doi.org/10.1007/s11748-018-0902-5 Received: Accepted: Published: Version of record: Issue date: DOI: https://doi.org/10.1007/s11748-018-0902-5

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MeSH descriptors

Heart Atria Heart Neoplasms Ovarian Neoplasms Sarcoma, Endometrial Stromal Vena Cava, Inferior Adult Cardiopulmonary Bypass Circulatory Arrest, Deep Hypothermia Induced Diagnosis, Differential Female Heart Atria Heart Neoplasms Heart Neoplasms Heart Neoplasms Heart Neoplasms Humans Ovarian Neoplasms Ovarian Neoplasms Ovarian Neoplasms Ovarian Neoplasms

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