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GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report
A Mitsuhashi1, Y Nagai, M Sugita
1Department of Obstetrics and Gynecology, Chiba University School of Medicine, Japan.
Insights
Intravenous leiomyomatosis with cardiac extension is rare. Long-term gonadotropin-releasing hormone (GnRH) agonist treatment effectively prevented recurrence in a patient with estrogen receptor-positive disease.
Area of Science:
- Cardiovascular Surgery
- Gynecologic Oncology
- Endocrinology
Background:
- Intravenous leiomyomatosis (IVL) with cardiac extension is an exceedingly rare condition.
- IVL involves benign smooth muscle tumors that invade vascular spaces.
Observation:
- A case of IVL with inferior vena cava extension into the right atrium was surgically managed.
- Three surgical procedures were performed: laparotomy, pelvic mass debulking, and intracardiac tumor resection.
Findings:
- Postoperative administration of a gonadotropin-releasing hormone (GnRH) agonist (leuprorelin acetate) was initiated due to estrogen receptor-positive tumor cells.
- While tumor regrowth occurred after initial cessation of GnRH agonist therapy, readministration completely inhibited residual pelvic mass enlargement for 15 months.
Implications:
- This case suggests that IVL is hormone-dependent, similar to uterine leiomyomas.
- Long-term GnRH agonist therapy may be a valuable strategy for preventing recurrence in cases of incompletely resected IVL, especially when functioning ovarian tissue remains.
Background:
Intravenous leiomyomatosis with cardiac extension is an extremely rare disease.
Case:
We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing.
Conclusion:
Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.