One-stage surgical removal of paraovarian inner-vessel angioleiomyoma with intercaval and intracardiac localization (Rare clinical case)

Abstract

Introduction: The inner-vessel angioleiomyoma (IAL) is an extremely rare version of leiomyoma. According to the literature it happens in 0,4% of the leiomyoma cases. IAL is a benign tumor which invades the vessels in the pelvis. It causes symptoms similar to those of leiomyoma, but sometimes the first symptoms are of cardiac character. Goal: To present a rare clinical case of a patient with intracardiac IAL of genital origin. To introduce a suitable one-stage surgical treatment for intracardiac IAL. Clinical case: The patient is 72 years old in overall good condition. She is diagnosed with chronic heart deficiency – II functional class. She has one single complaint of palpitation when having a rest. The computer tomography shows a tumor formation in the left ovary from which an inner-vessel heterogenic structure starts and fills the lumen of the left ovarian vein. The latter goes through the inferior vena cava, reaching the right atrium and prolapses into the right ventricle. The cardiac and gynecology teams consulted with each other and decided to do a combined one-stage removal of the tumor formation. Under the condition of a total cardiopulmonary bypass and “empty beating heart”, a long formation with 20 cm length and 2 cm width was extirpated from the right atrium and inferior vena cava. The next stage was to remove the left ovary with all adnexa. The early post-surgery period went smoothly with stable hemodynamic values. The post-surgery transthoracic echocardiogram (TTE) established preserved cardiac function and absence of IAL. The patient was dismissed from the hospital in good overall condition on the tenth day after surgery. The histological result showed a tumor formation with bundle construction, composed of spindle-shaped cells with smooth muscle characteristics. There is an interstitial fibrosis and multiple thin-walled and thick-walled vessels with muscle layer. Diagnosis: paraovarian angioleiomyoma. Conclusion: The combined one-stage surgery treatment under the protocol of our institution is a safe method of treatment of intracardiac inner-vessel angioleiomyoma. A condition for its implementation is the interdisciplinary approach.