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Right atrial and right ventricular obstruction by recurrent stromomyoma
I Kronzon1, G M Goodkin, A Culliford
1Department of Medicine, New York University School of Medicine, NY.
Summary
A recurrent low-grade endometrial stromal sarcoma (stromatosis) presented as a large cardiac tumor, necessitating complex surgical resection. This case highlights the rare cardiac manifestation of uterine-derived sarcomas.
Area of Science:
- Cardiovascular Pathology
- Gynecologic Oncology
- Surgical Oncology
Background:
- A 30-year-old woman with a history of uterine fibroids presented with recurrent cardiac tumors.
- Previous resections were performed for a right atrial mass diagnosed as leiomyoma, with pathology revealing low-grade endometrial stromal sarcoma (stromatosis) with features of intravenous leiomyomatosis.
Observation:
- The patient developed facial and lower extremity swelling, and ascites.
- Echocardiography showed a large tumor extending from the inferior vena cava into the right atrium and ventricle.
- Lower extremity ischemia secondary to compartment syndrome was noted.
Findings:
- Successful surgical resection of the extensive tumor was performed.
- The tumor involved the right side of the heart, inferior vena cava, and mesenteric and renal veins.
Implications:
- This case underscores the potential for rare, aggressive behavior of low-grade endometrial stromal sarcoma.
- It highlights the importance of comprehensive imaging and surgical planning for complex cardiac and venous tumor involvement.
- Early diagnosis and intervention are crucial for managing such rare oncologic emergencies.