P1696 Intravascular leiomyomatosis with inferior vena cava and intracardiac extension, with severe obstruccion to the right ventricle.The fundamental role of transesophageal echocardiography

Abstract

Abstract Introduction Intravascular leiomyomatosis (IL) is a rare type of tumor common and was first described by Birch-Hirschfeld in 1896. There are currently few cases reported in the literature involving this type of vena cava tumor and the right atrium. This tumors are characterized by nodular, intravascular, histologically benign masses of smooth muscle with variable extensión. The most frequently affected women are pre-menopausal and multiparous. There are no specific clinical manifestations of vascular leiomyomas. Clinical case This is 51-year-old woman with a history of morbid obesity (body mass index of 47kg / m2SC) in bariatric surgery protocol, mixed dyslipidemia, is presented to the emergency service of our Institute, with uterine myomatosis diagnosed 6 months prior to admission. She was admitted for a 2-week evolution characterized by dyspnea of medium efforts that progressed at rest on the day of his admission with acute heart failure. A transthoracic and transesophageal echocardiograhy three-dimensional (TTE, TEE 3D) was made in which a solid-appearing, regular-border, heterogeneous tumor was reported from the inferior vena cava to the right atrium without being attached to the interatrial septum or other structures, with severe protrusion to the right ventricle, highly mobile, heterogeneous, without vascularity with color doppler, causing intermittent obstruction and tricuspid stenosis with a mean gradient of 8mmHg, pulmonary artery systolic pressure calculated at 46 mmHg. TAC showed invasion from the uterine leiomyoma to the inferior vena cava and right atrium. The patient was taken to emergency surgery, the vascular surgery and cardiothoracic surgery performed a long intervention. Macroscopic description reported uterine tumor of 25x19x18cm. Weight of 3.90Kg, with multiple nodular lesions, the largest being 18x15cm. Intracavitary tumor with fragment of tissue. The pathological diagnosis was uterine leiomyomatosis, intravascular leiomyomatosis in right periadnexal vessels. The patient was taken to intensive therapy, although the postoperative period was prolonged, she was discharged in functional class II NYHA. Discussion This case demonstrates the importance of recognizing this rare disease and suspecting it in patients who present with the a symptoms and the role of the echocardiography is fundamental for the characterization of the tumor and the invasion of neighboring structures. The reported cases of intravascular leiomyomas with intracardiac extension are approximately 10% and to date, less than 300 cases have been reported in the literatura. Vascular leiomyomas arise from uterine leiomyomas with vascular invasión, the etiology still remains unknown, it has been associated with high levels of estrogen. The key in its treatment is the complete resection. The prognosis is good after its resection. The recurrence rate is up to 30%. Anti-estrogenic therapy has been considered but its efficacy is controversial. Abstract P1696 Figure. Intravascular leiomyomatosis