Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

Laterally Extended Endopelvic Resection Versus Chemo or Targeted Therapy Alone for Pelvic Sidewall Recurrence of Cervical Cancer

BackgroundLaterally extended endopelvic resection (LEER) has been introduced for treatment of pelvic sidewall recurrence of cervical cancer (PSRCC), which occurs in only 8% of patients with relapsed cervical cancer. LEER can only be performed by a proficient surgeon due to the high risk of surgical morbidity and mortality, but there is no evidence as to whether LEER is may be more effective than chemo or targeted therapy alone for PSRCC. Thus, we aimed to compare the efficacy and safety between LEER and chemo or targeted therapy alone for treatment of PSRCC.MethodsWe prospectively recruited patients with PSRCC who underwent LEER between December 2016 and December 2019. Moreover, we retrospectively collected data on patients with PSRCC who received chemo or targeted therapy alone between January 2000 and December 2019. We compared treatment-free interval (TFI), progression-free survival (PFS), treatment-free survival (TFS), overall survival (OS), tumor response, neurologic disturbance of the low extremities, and pelvic pain severity in the different patient groups.ResultsAmong 1295 patients with cervical cancer, we included 28 (2.2%) and 31 (2.4%) in the prospective and retrospective cohorts, respectively. When we subdivided all patients into two groups based on the median value of prior TFI (PTFI, 9.2 months), LEER improved TFI, PFS, TRS and OS compared to chemo or targeted therapy alone (median, 2.8 vs. 0.9; 7.4 vs. 4.1; 30.1 vs. 16.9 months; P ≤ 0.05) in patients with PTFI < 9.2 months despite no difference in survival in those with PTFI ≥ 9.2 months, suggesting that LEER may lead to better TFI, PFS, TRS and OS in patients with PTFI < 9.2 months (adjusted hazard ratios, 0.28, 0.27, 0.44 and 0.37; 95% confidence intervals, 0.12-0.68, 0.11-0.66, 0.18-0.83 and 0.15-0.88). Furthermore, LEER markedly reduced the number of morphine milligram equivalents necessary to reduce pelvic pain when compared with chemo or targeted therapy alone.ConclusionCompared to chemo or targeted therapy alone, LEER improved survival in patients with PSRCC and PTFI < 9.2 months, and it was effective at controlling the pelvic pain associated with PSRCC.Trial RegistrationClinicalTrials.gov, identifier NCT02986568.

Robotically Assisted Laterally Extended Endopelvic Resection (LEER) of an Isolated Ovarian Cancer Recurrence

A 64-year-old woman with recurrent ovarian cancer isolated to the left pelvic sidewall underwent robotic resection of a tumor adherent to the external iliac vein and encasing the ureter and internal iliac vessels. A narrated video-clip of the case is included.

Efficacy and safety of laterally extended endopelvic resection for the pelvic side wall gynecologic tumors: A four-year prospective cohort study with historical comparison.

6092 Background: Although laterally extended endopelvic resection (LEER) has been introduced to control the pelvic sidewall tumors, there is a lack of evidence about its efficacy and safety despite high skillful procedure, compared with the other treatments. Thus, we performed a prospective cohort study with historical comparison for four years. Methods: One gynecologic oncologist performed LEER consecutively for patients with the pelvic sidewall tumors between March 2014 and July 2018. We compared clinicopathologic characteristics and survival between patients who received primary LEER and with those treated with other treatments. Results: We enrolled 37 patients treated with LEER. Among them, 22 (59.5%) and 15 (40.5%) had recurrent and primary disease. Among perioperative outcomes, there was more estimated blood loss, and hospitalization was longer in recurrent disease and previous surgery (p < 0.05). In recurrent disease, previous progression-free survival < 8 months was related to poor recurrence-free survival after LEER (median, 5.4 vs. 10.2 months; p < 0.05). When LEER was applied for the first recurrence of cervical cancer, recurrence-free survival and overall survival after treatment seemed to be longer in LEER (n = 9) than in palliative chemotherapy (n = 27) without statistical significance (median, 12.2 vs. 4.7 months and 23.2 vs. 12.4 months; p = 0.13 and p = 0.63). In 15 patients with primary locally advanced cervical cancer, LEER after partial response to neoadjuvant chemotherapy showed longer progression-free survival than LEER after stable or progressive disease to neoadjuvant chemotherapy and primary radiotherapy (p = 0.012). After LEER, grade 3 and 4 complications developed in 15 (23.1%) and 2 (3.1%) patients. Conclusions: Compared with palliative chemotherapy, LEER followed by palliative chemotherapy may improve progression-free survival in patients with recurrent cervical cancer located in the pelvic sidewall. If possible, it is more effective to apply LEER without preceding palliative chemotherapy for recurrent cervical cancer located in the pelvic sidewall.

Laparoscopic laterally extended endopelvic resection procedure for gynecological malignancies

ObjectivesPelvic side wall infiltration by gynecological malignancies has been considered for a long time an absolute contraindication to curative resection. The development of the laterally extended endopelvic resection (LEER) has challenged this surgical paradigm. Although the LEER has been standardized in open surgery, only small studies have been published about its endoscopic feasibility. The objective of this study is to analyze the safety of LEER in patients with gynecological malignancies involving the pelvic side wall.MethodsWe retrospectively evaluated a consecutive series of patients who underwent a laparoscopically modified LEER between July 2014 and November 2018. This indicated gynecological tumors involving the pelvic sidewall and surgeries were conducted in two Italian institutions. All patients underwent pre-operative CT scan or PET to evaluate for distant metastases. Patients without suspicioun of distant metastasis underwent pelvic MRI and examination under anesthesia to establish the resectability of the disease and concomitant diagnostic laparoscopy to exclude intraperitoneal dissemination. All women with disease-free interval <6 months, and/or performance status >2 ECOG were excluded. Type of resection was defined based on the status of the pathologic margins: R0, microscopically negative (free margin <5 mm); R1, microscopically positive; and R2, macroscopically (grossly) positive. Disease-free survival was calculated from the date of primary surgery to the time of recurrence. Overall survival was defined as the time from primary surgery to death.ResultsOverall, 39 patients underwent a laparoscopic LEER and 18 (46.2%) patients were eligible for a laparoscopic approach. Laparoscopic LEER was performed as primary treatment for newly diagnosed tumors in eight patients (44.4%), and for recurrences in the other 10 patients (55.6%). No laparotomic conversions were registered. R0 resection was achieved with negative margins in all patients. The median operative time was 415 min (range, 285–615), median estimated blood loss was 285 mL (range, 100–600), and the median length of hospital stay was 10 days (range; 4–22). Only four patients (22.2%) needed blood intraoperative transfusion. In seven patients (38.9%), post-operative admission to intensive care unit was required. There were three (16.7%) intraoperative complications, all managed laparoscopically. In total there were six (33.3%) major postoperative complications: three patients (16.7%) experienced moderate hydronephrosis with normal renal function, which required temporary placement of nephrostomy; one patient (5.6%) had permanent urinary retention; and two patients (11.1%) had a reoperation, one for post-operative hemoperitoneum and another for complete vaginal cuff dehiscence.DiscussionLaparoscopic LEER can be safely performed by experienced laparoscopic surgeons, in carefully selected patients with gynecological malignancies involving the lateral pelvic side wall, even for those in which a bladder and rectum sparing surgery appears possible. Further larger prospective trials are needed to evaluate the oncological and the long-term functional outcomes.