Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

Abstract

Background Extra-pelvic intravenous leiomyomatosis (IVL) extending into inferior vena cava (IVC) or heart (i.e. intracardiac leiomyomatosis, ICL) is an extremely rare benign disease. No consensus has been reached on the optimal surgical strategy. The aim of this study is to introduce four types of one-stage surgical strategies including less invasive options and a guideline to select patient-specific strategy for this disease. Methods Twenty-four patients of extra-pelvic IVLs receiving one-stage resections at the Zhongshan Hospital from July 2011 to November 2019 were reviewed retrospectively. Base on the initial experiences of the indiscriminate choices of tumor thrombectomies through sterno-laparotomy under cardiopulmonary bypass (CPB) in 6 ICLs, an anatomy-based guideline for four types of surgical strategies was developed and applied for the next 18 patients. Results Under the direction of guideline, tumor thrombectomies through single laparotomy were applied without CPB in 2 ICLs and 4 IVLs confined in IVC, or with CPB in 7 ICLs. Guideline-directed double-incisions with CPB were applied in only 5 ICLs, including 1 receiving mini-thoracotomy and 4 receiving sternotomy because of tumor adherences with right atriums in 2 and with pulmonary arteries in 2. All 24 patients accomplished one-stage panhysterectomy, bilateral adnexectomy and complete resections of intracaval and intracardiac tumors. For residual pelvic intravenous tumors in 19 patients, 17 received macroscopically complete resections while the other 2 failed because of high risk of hemorrhage. Intraoperative blood losses, operation time and hospitalization expense in the single-laparotomy non-CPB group were significantly lesser than the other groups. In CPB groups, inpatient stay and hospitalization expense in the single-incision group were significantly lesser than the double-incisions group. All patients were alive and free of recurrences during a mean follow-up of 35.4±27.2 months (range, 1–100 months). The pelvic tumor residues in 2 patients remained unchanged for 51 and 52 months since operation, respectively. Conclusions For various extra-pelvic IVLs, the 4 types of surgical strategies including less invasive options are feasible, providing these are selected by a guideline base on the tumor extension and morphology. The proposed guideline is believed to accommodate more patients receiving less invasive surgery without compromising the curative effect.

The role of estrogen deprivation therapy in premenopausal women with primary unresectable intracardiac leiomyomatosis: a systematic review and meta-analysis

“…Consequently, the incidence and the recurrence rate, the morbidity, and mortality in the whole world could be higher [38]. Moreover, it still remains controversial on the treatment strategy for the patients with ICLM, especially for those in developing areas or with less medical insurance, who may face unaffordable medical expenses and the shortage of medical resources [2,26,39]. Therefore, Li et al emphasized that less invasive surgery without compromising the curative effect would be the main goal [39].…”

“…Moreover, it still remains controversial on the treatment strategy for the patients with ICLM, especially for those in developing areas or with less medical insurance, who may face unaffordable medical expenses and the shortage of medical resources [2,26,39]. Therefore, Li et al emphasized that less invasive surgery without compromising the curative effect would be the main goal [39]. Complete removal of the ICLM plus BSO was the most optimal and efficient treatment in preventing recurrences (Table 4), which was consistent with other studies [17,18,23,38,40].…”

Management and prognosis comparison between incidental and nonincidental intravenous leiomyomatosis: a retrospective single-center real-life experience

“…Primary treatment of IVL includes surgery, with either a 1-or 2-stage approach, and adjuvant therapy. Complete resection of IVL tumor lesions can effectively reduce the recurrence risk (6,7), but the extent of surgery and surgical pattern still lack conformity (8)(9)(10). Some studies have also stressed that a precise and full-scale preoperative evaluation is crucial for surgical planning and achieving successful outcomes (11,12).…”