Resection of Intracaval Leiomyomatosis Using Abdominal Approach and Venovenous Bypass

et al. 2020

Orphanet J Rare Dis

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.

Section: Discussionmentioning

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

et al. 2020

Orphanet J Rare Dis

“…Although deep hypothermia circulatory arrest provided a perfectly bloodless field, patients were at risk of related complications such as organ dysfunction, severe coagulation disorder and neurological damage. There were some individual less invasive attempts [15][16][17][18][19][20] and a few reports [6][7]10] have proposed surgical guidelines covering part of these strategies, however, in our opinion, these proposals were not ideal and excluded some patients who would have received less invasive surgery. The present study introduces 4 types of surgical strategies (Fig.…”

Section: Discussionmentioning

confidence: 93%

“…In most reports [5,7,[13][14] , one-stage resection of ICL utilized sternolaparotomies, cardiopulmonary bypass (CPB) and even circulatory arrest. For certain types of ICL, several less invasive strategies have been proposed in individual cases, such as simple abdomen incision [15][16][17][18][19], avoidance of CPB [15,[17][18][19] or endoscopic surgery [20]. Due to the extreme paucity and wide heterogeneity of extra-pelvic IVL, no consensus has been reached on the optimal surgical strategy.…”

Section: Introductionmentioning

confidence: 99%

Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

et al. 2020

Preprint

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.

“…In most reports [7,15,[21][22], one-stage resection of ICL utilized sternolaparotomies, cardiopulmonary bypass (CPB) and even circulatory arrest. For certain types of ICL, several less invasive strategies have been proposed in individual cases, such as simple abdomen incision [23][24][25][26][27], avoidance of CPB [23,[25][26][27] or endoscopic surgery [28]. Due to the extreme paucity and wide heterogeneity of extra-pelvic IVL, no consensus has been reached on the optimal surgical strategy.…”

Section: Introductionmentioning

confidence: 99%

Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

et al. 2020

Preprint

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.