Intravascular Lipoleiomyoma With Expansion Into the Right Atrium and Subsequent Debulking Surgery.

Area of Science:

• Cardiovascular Medicine
• Oncology
• Pathology

Background:

• Lipoleiomyomas are rare benign tumors composed of smooth muscle and adipose tissue, predominantly occurring in the uterus.
• Intravenous leiomyomas are a distinct entity, originating from vascular smooth muscle and capable of extensive growth.

Observation:

• A 61-year-old woman with a history of uterine leiomyoma presented with syncopal episodes due to a large right atrial mass.
• The mass, originating from the iliac veins, required partial resection and was pathologically identified as an angioleiomyomatous tumor.
• Three years later, recurrence was observed, extending from the iliac vessels to the right atrium, necessitating complex thoracoabdominal surgery.

Findings:

• The recurrent tumor was hormone receptor-positive.
• Adjunctive therapy with letrozole, an aromatase inhibitor, was initiated post-surgery.
• Follow-up imaging demonstrated significant tumor regression with the patient remaining asymptomatic.

Implications:

• Aromatase inhibitors may be a valuable therapeutic option for managing recurrent, hormone receptor-positive intravenous leiomyomas, especially when complete surgical resection is not feasible.
• This case highlights the importance of a multidisciplinary approach in managing complex cases of intravenous leiomyomas involving cardiac structures.
• Long-term surveillance is crucial for patients with a history of extensive intravenous leiomyomas due to the potential for recurrence.