Intravenous Leiomyomatosis: A Report Of Two Cases With Literature Review

Objective:To investigate the clinicopathological characteristics, diagnosis, differential diagnosis, treatment and prognosis of intravenous leiomyomatosis (IVL) in order to enhance the understanding of this special disease as well as to reduce the misdiagnosis and missed-diagnosis.Materials and Methods:(1) The clinical data of two cases of IVL patients was retrospectively analyzed, and relevant literatures were reviewed. (2) Given the extreme rarity and unfavorable prognosis of IVL with intracardiac extension, here we performed an electronic literature search for intracardiac leiomyomatosis (ICL) in PubMed and Web of Science updated to March 2016, and then provide a detailed and comprehensive review of the demographics, clinical presentation, preoperative assessment, diagnosis, operative approach, and postoperative issues of this disorder.Results:(1) Both two patients were women of reproductive age. One patient was hospitalized for uterine leiomyoma with the clinical symptoms of increase of menstrual volume and extended menstrual period. Because of the involvement of bilateral vena iliaca and inferior vena cava, she was preliminarily diagnosed as IVL by CT and MRI before the operation. Another patient was hospitalized for uterine leiomyoma without any clinical symptoms, preoperatively misdiagnosed as uterine leiomyoma with degeneration, uterine sarcoma cannot be excluded. Finally this patient was diagnosed as IVL intraoperatively. Two patients were eventually diagnosed with IVL by pathological examination. (2) A total of 175 articles that met the characterization of ICL were included, consisting of 278 cases. According to our analysis, the mean age of detection is 46.0 years (range 20-81years).246 patients out of 278 (88.6%) had undergone previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma when admitted. The most common clinical presentations were dyspnea (44.3%), syncope/presyncope (25.2%), palpitations/tachycardia (23.2%), edema of the lower extremities (22.8%) and abdominal discomfort/ascites (21.5%). The route of tumor extension to the caval system was described in 225 cases, most commonly (65.3%) through the iliac venous system, less common (30.7%) through the ovarian venous system, rarely (4.0%) through both iliac and ovarian venous routes. Distant locations were recorded in 259 patients, most commonly (60.2%) extension into the right atrium, less common (33.2%) into right ventricle, rarely (6.6%) into pulmonary artery. Of 163 patients for whom a preoperative diagnosis was made, ICL was correctly diagnosed in 106 (65.0%), while 36 (22.1%) were misdiagnosed as right atrium myxoma, followed by thrombus in 12 (7.4%). Surgical excision was performed in 254 patients (91.4%), including a single stage procedure (45.7%) through a combined sternolaparotomy or a two stage approach (34.9%).The follow-up time was provided for 148 patients, with an average of 27.2 months (range 1 months to 25 years). Follow-up of complete removal was recorded for 113 patients with 3 recurrences (2.7%). Follow-up of incomplete removal was recorded for 40 patients, and of these, recurrence occurred in 14 (35%).Conclusions:(1) IVL is a rare manifestation of a benign uterine tumor. Owing to the diversified and non-specific clinical manifestations of IVL without extension to major vein and heart, the preoperative diagnosis rate is extremely low, mostly diagnosed intraoperatively and postoperatively by pathological examination; (2) When an enlarged uterus and intrauterine mass with rich blood flow are shown in ultrasound examination, especially when the mass grows beyond uterus; when uterine leiomyoma or pelvic mass with intravenous "thrombus" are shown in CT, both malignant tumor as well as IVL should be considered; (3) Complete tumor resection together with total hysterectomy and bilateral salpingoophorectomy are crucial for successful treatment of IVL, and bilateral salpingoophorectomy can obviously reduce the recurrence rate. (4) ICL mainly involves premenopausal women, and most patients had undergone a previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma; the tumor extending into the right cardiac chambers most commonly through the iliac venous system. (5) When a right atrial mass is identified in middle-aged women with previous hysterectomy/myomectomy or uterine leiomyoma, a high degree of ICL suspicion should be maintained, then an echocardiogram and abdominal CT/MRI should be arranged to confirm the origin and evaluate the extension route; (6) Complete removal guarantees an excellent outcome of ICL with rare recurrence, while incomplete removal leads to recurrence in one-third of patients.