DICER1-mutated rhabdomyosarcoma of the ovary with teratoid features.

DICER1-mutated rhabdomyosarcoma is a rare, emerging entity with a predilection for the gynecologic and genitourinary tracts. We report here a case of DICER1-mutated rhabdomyosarcoma of the ovary in a 14 years old girl which displayed interspersed mature teratoid glands, neuroectodermal rosettes and immature blastematous-like tubes. Morphologically the sarcomatous component predominated, corresponding to a high grade spindle cell rhabdomyosarcoma with botryoid features. Islets of cartilage were present. The sarcomatous proliferation encased the teratoid glands, forming cambium layer-like arrangements. The sarcoma cells were Myogenin and MYOD1 positive, the neuroectodermal rosettes expressed SALL4 along with cytokeratins and EMA and were negative for Inhibin; immature blastematous-like tubes were negative for SALL4 and Inhibin. Whole RNA- and targeted DNA-sequencing revealed two DICER1 mutations in exon 26: c.5113G>A: p.(Glu1705Lys) and exon 12: c.1642C>T: p.(Gln548X). The sarcomatous component harbored a complex genetic profile while the teratoid component was diploid, none of the above displayed abnormality of 12p. DICER1-mutated sarcomas display pathological features similar to embryonal rhabdomyosarcomas, botryoid type. They also display heterogeneous features combining cartilage foci, teratoid mature glands, immature blastematous-like tubes and/or neuroectodermal components. Molecular testing remains necessary to confirm the diagnosis. Further studies need to clarify the nosology of DICER1-mutated sarcomas and devise specific therapeutic strategies.

Predictive risk factors of acute kidney injury after cytoreductive surgery and cisplatin-based hyperthermic intra-peritoneal chemotherapy for ovarian peritoneal carcinomatosis.

OBJECTIVE: The aim of our study was to assess the incidence and identify the predictive risk factors of acute kidney injury after cytoreductive surgery and cisplatin-based hyperthermic intra-peritoneal chemotherapy. METHODS: This is a retrospective study from two centers evaluating patients with advanced or recurrent ovarian cancer who underwent cytoreductive surgery followed by cisplatin-based hyperthermic intra-peritoneal chemotherapy from January 2007 to December 2013. Patients were classified into two groups according to the occurrence of acute kidney injury, defined as a glomerular filtration rate at post-operative day 7 25% lower than at day 0. We also evaluated acute kidney injury following Risk, Injury, Failure, Lost and End-stage kidney function criteria. Univariate and multivariate analyses were conducted in order to assess the association between different variables and the occurrence of acute kidney injury. RESULTS: Sixty-six patients were included: 29 (44%) underwent first-line treatment and 37 (56%) were treated for recurrent disease. The incidence of post-operative acute kidney injury was 48%. After multivariate analysis, hypertension (OR 18.6; 95% CI 1.9 to 182.3; p=0.012) and low intra-operative diuresis (OR 0.5; 95% CI 0.4 to 0.8; p=0.001) were associated with acute kidney injury. CONCLUSION: The incidence of acute kidney injury after cytoreductive surgery and cisplatin-based hyperthermic intra-peritoneal chemotherapy was high. Hypertension and low intra-operative diuresis were independent risk factors for this complication. Adequate peri-operative hydration, in order to maintain correct diuresis, could decrease the occurrence of acute kidney injury in patients undergoing cytoreductive surgery plus hyperthermic intra-peritoneal chemotherapy.

Are we performing episiotomies correctly? A study to evaluate French technique in a high-risk maternity unit.

INTRODUCTION: The aim of this study was to evaluate episiotomy technique, in particular suture angles, and any correlation between suture angle and severe perineal tears. MATERIAL AND METHODS: An observational questionnaire-based study was conducted between 01 August 2015 and 30 April 2016 among accoucheurs performing episiotomies in a French maternity unit with facilities for high-risk pregnancies. For each patient included, accoucheurs were asked to measure the episiotomy suture angle, and to record the angle at which they thought they had cut, the length of the episiotomy, its distance from the anus, and whether the woman sustained a sphincter injury. RESULTS: The centre's episiotomy rate during the study period was 15%. We analyzed the characteristics of episiotomies performed on 89 women (68 by doctors and 21 by midwives). Only 43% of suture angles were between 45° and 60° (45.6% of those performed by doctors vs 38.1% by midwives, p=0.8623), whereas 91% of accoucheurs thought they had cut within the correct range. Doctors made longer incisions than midwives (4 [4.2-5.0] vs 3 [2.5-3.5] cm, p=0.0006). Only 40.5% of accoucheurs correctly estimated the incision angle. Twelve (13.64%) of the 88 women sustained a third-degree perineal tear. The risk of sphincter injury was higher with suture angles <45° (odds ratio 5.46 [1.11-26.75], p=0.037). After multivariate analysis, this result was no longer significant (p=0.079). CONCLUSION: It appears that many accoucheurs have difficulty estimating episiotomy incision angles correctly and that education and training in this domain requires improvement.

Synthetic mesh repair of an anterior perineal hernia following robotic radical urethrocystectomy

ABSTRACT Introduction: Perineal hernia is a protrusion of intra-abdominal viscera through a defect in the pelvic floor and is a rare but challenging complication after extensive abdominoperineal surgery. There have been small series published after colorectal exenteration, but no cases have been reported after radical cystectomy and urethrectomy. Case Presentation: A 68 years old woman developed an anterior perineal hernia, with no vaginal prolapse, after an anterior exenteration for bladder cancer. A perineal approach with the use of a synthetic polypropylene mesh was chosen to resolve the condition. After 6 months of follow-up, the patient has no symptoms or recurrence of the anterior perineal hernia. Conclusion: To our knowledge, this case is the first report of perineal hernia after radical urethrocystectomy. Although being a case report, this article describes a potential and challenging complication after extensive anterior pelvic surgery, that could increase its incidence in the future. Literature review shows that whether perineal, abdominal or combined approach is chosen, surgery must respect hernia repair principles.

Synthetic mesh repair of an anterior perineal hernia following robotic radical urethrocystectomy.

INTRODUCTION: Perineal hernia is a protrusion of intra-abdominal viscera through a defect in the pelvic floor and is a rare but challenging complication after extensive abdominoperineal surgery. There have been small series published after colorectal exenteration, but no cases have been reported after radical cystectomy and urethrectomy. CASE PRESENTATION: A 68 years old woman developed an anterior perineal hernia, with no vaginal prolapse, after an anterior exenteration for bladder cancer. A perineal approach with the use of a synthetic polypropylene mesh was chosen to resolve the condition. After 6 months of follow-up, the patient has no symptoms or recurrence of the anterior perineal hernia. CONCLUSION: To our knowledge, this case is the first report of perineal hernia after radical urethrocystectomy. Although being a case report, this article describes a potential and challenging complication after extensive anterior pelvic surgery, that could increase its incidence in the future. Literature review shows that whether perineal, abdominal or combined approach is chosen, surgery must respect hernia repair principles.

Treatment of neovaginal prolapse: case report and systematic review of the literature.

INTRODUCTION AND HYPOTHESIS: Neovaginal prolapse (NP) is a rare event as few cases have been reported in the literature. Its management is complex and depends on the initial pathology, the vaginoplasty technique and the patient's history. We present a review the literature on this rare event. METHODS: We describe the case of a 72-year-old woman who presented with NP 1 year after pelvic exenteration and radiotherapy for recurrent cervical carcinoma associated with vaginal reconstruction by shaped-tube omentoplasty. She had undergone two previous surgical procedures (posterior sacrospinous ligament suspension and partial colpocleisis), but NP recurred each time within a few months. We performed an anterior approach to the sacrospinous ligament and inserted a mesh under the anterior wall of the neovagina, with the two mesh arms driven through the sacrospinous ligament in a tension-free manner (Uphold Lite® system). The MEDLINE, Cochrane Library, ClinicalTrials and OpenGrey databases were systematically searched for literature on the management of NP following bowel vaginoplasty, mechanical dilatation, graciloplasty, omentoplasty, rectus abdominis myocutaneous flap and the Davydov procedure. RESULTS: The postoperative course in the patient whose case is described was uneventful and after 1 year of follow-up, the anatomical results and patient satisfaction were good. The systematic search of the databases revealed several studies on the treatment of NP using abdominal and vaginal approaches, and these are reviewed. CONCLUSIONS: Overall, sacrocolpopexy would appear to be a good option for the treatment of prolapse after bowel vaginoplasty, but too few cases have been reported to establish this technique as the standard management of NP.

Application of a hyaluronic acid gel after intrauterine surgery may improve spontaneous fertility: a randomized controlled trial in New Zealand White rabbits.

OBJECTIVE: Intrauterine adhesions (IUAs) are the most common complication after hysteroscopy in patients of reproductive age. Intra-abdominal anti-adhesion gel reduces the incidence of adhesions, but effects on fertility after uterine surgery are not known. The objective of our work was to evaluate the effect of intrauterine anti-adhesion gel on spontaneous fertility after repeated intrauterine surgery with induced experimental synechiae in the rabbit model. MATERIALS AND METHODS: Twenty New Zealand White rabbits underwent a double uterine curettage 10 days apart and were randomized into two groups. Each rabbit served as its own control: one uterine tube was the treatment group (A), the second uterine tube was the control group (B) to avoid bias through other causes of infertility. Group A received a post curettage intrauterine instillation of anti-adhesion gel whereas group B, the control group, underwent curettage without instillation of the gel. After a recovery period, the rabbits were mated. An abdominal ultrasound performed 21 days after mating allowed us to diagnose pregnancy and quantify the number of viable fetuses. RESULTS: There was a significant difference in total fetuses in favor of group A, with an average of 3.7 (range, 0-9) total fetuses per tube against 2.1 (0-7) in group B (p = .04). The number of viable fetuses shows a trend in favor of group A, with an average of 3.4 (0-7) viable fetuses per tube against 1.9 (0-6) viable fetuses per tube in group B (p = .05). CONCLUSION: The use of immediate postoperative anti-adhesion gel improved fertility in an animal model after intrauterine surgery likely to cause uterine synechiae. This experimental model will permit comparison of different anti-adhesion solutions, including assessment of their tolerance and potential mucosal toxicity on embryonic development.