Surgical Management of Hereditary Breast Cancer.

The identification that breast cancer is hereditary was first described in the nineteenth century. With the identification of the BRCA1 and BRCA 2 breast/ovarian cancer susceptibility genes in the mid-1990s and the introduction of genetic testing, significant advancements have been made in tailoring surveillance, guiding decisions on medical or surgical risk reduction and cancer treatments for genetic variant carriers. This review discusses various medical and surgical management options for hereditary breast cancers.

Gynecologic Considerations for the Urologic Surgeon.

While gynecologic malignancy is uncommon in women with conditions such as pelvic organ prolapse and bladder cancer, urologists should be acquainted with the relevant gynecologic literature as it pertains to their surgical care of female patients. While taking the patient history, urologists should be aware of prior cervical cancer screening and ask about vaginal bleeding, which can be a sign of uterine cancer. Urologic surgeons should also discuss the role of concomitant prophylactic oophorectomy and/or salpingectomy for ovarian cancer risk reduction at the time of pelvic surgery. An understanding of basic tests, such as a transvaginal sonogram, can help urologists provide comprehensive care.

WHO recommendation on prophylactic antibiotics for women undergoing caesarean section

Direct maternal infections around the time of childbirth account for about one tenth of the global burden of maternal death. Women who develop peripartum infections are also prone to severe morbidity, long-term disabilities such as chronic pelvic pain, fallopian tube blockage and secondary infertility. Maternal infections before or during childbirth are also associated with an estimated 1 million newborn deaths annually. Several factors increase the risk of maternal peripartum infections, including pre-existing maternal conditions (e.g. malnutrition, diabetes, obesity, severe anaemia, bacterial vaginosis and group B streptococcus infections), as well as prolonged prelabour rupture of membranes, multiple vaginal examinations, manual removal of the placenta, operative vaginal birth and caesarean section. As such, the strategies to reduce maternal peripartum infections and their short- and long-term complications have been directed at improving infection prevention and control practices. Globally, an effective intervention for preventing morbidity and mortality related to maternal infection is the prophylactic and therapeutic use of antibiotics. However, the misuse of antibiotics for obstetric conditions and procedures is common in many settings. Inappropriate antibiotic use has implications for the global effort to prevent and reduce antimicrobial resistance. The WHO global strategy for containment of antimicrobial resistance underscores the importance of appropriate use of antimicrobials at different levels of the health system to reduce the impact of antimicrobial resistance, while ensuring access to the best treatment available. WHO guidelines for health professionals and policy-makers on the need for antibiotics ­ and the type of antibiotics ­ for the prevention and treatment of maternal peripartum infections align with the WHO strategy and, if implemented, will improve maternal and newborn outcomes. In 2019, the Executive Guideline Steering Group (GSG) for World Health Organization (WHO) maternal and perinatal health recommendations prioritized updating of the existing WHO recommendation on prophylactic antibiotics for women undergoing caesarean section in response to the availability of new evidence. The recommendation in this document thus supersedes the previous WHO recommendation on prophylactic antibiotics for women undergoing caesarean section as published in the 2015 guideline WHO recommendations for the prevention and treatment of maternal peripartum infections.

Prophylactic procedures associated with gynecological surgery for the management of superficial endometriosis and adhesions. Clinical practice guidelines from the French College of Gynecologists and Obstetricians (CNGOF)

To provide guidelines from the French College of Obstetricians and Gynecologists (CNGOF), based on the best currently available evidence, for the prophylactic procedures associated with gynecological surgery for benign disease such as superficial endometriosis lesions and adhesions. The CNGOF has decided to adopt the AGREE II and GRADE systems for grading scientific evidence. Each recommendation for practice was allocated a grade that reflects the quality of evidence (QE) (clinical practice guidelines). Endometriosis and pelvic pain Superficial endometriosis can be entirely asymptomatic. Surgical treatment of asymptomatic superficial peritoneal endometriosis is not recommended in women of childbearing age for the prevention of pelvic pain, especially in case of proximity to noble organs (e.g., the ureters, rectum and sigmoid, and ovaries in nulligravida) as there is no evidence that the disease will progress to become symptomatic (low level of evidence). In case of accidental discovery of superficial endometriosis in women of childbearing age with pelvic pain, it is recommended that the lesions are excised, if surgically accessible. Removal of superficial endometriosis lesions in patients with painful symptoms improves quality of life and pain (low level of evidence). Endometriosis and infertility It appears that women with isolated superficial endometriosis diagnosed by laparoscopy with histological confirmation have a significantly higher incidence of primary infertility than patients without endometriosis. However, there is no data regarding the impact of treatment of these lesions on the fertility in these women or on the natural course of their disease (low level of evidence). It is recommended that excision is performed rather than monopolar coagulation of superficial endometriosis lesions in infertile women, as this results in a higher spontaneous pregnancy rate (low level of evidence). Adhesions and pelvic pain There is limited data in the literature regarding the benefit of performing systematic adhesiolysis during laparoscopy to prevent pelvic pain when incidental pelvic adhesions are discovered. For patients with pelvic pain, it is probably better not to perform adhesiolysis to prevent pelvic pain, although this can be decided on a case-by-case basis depending on the extent of the adhesions, the topography, and the type of surgery considered (low level of evidence). For asymptomatic patients, it is recommended not to perform adhesiolysis to prevent pelvic pain due to the lack of clear efficacy both short- or long-term and due to the increased risk of surgical injuries (low level of evidence). Adhesions and infertility There is limited data in the literature regarding the potential benefit of performing systematic adhesiolysis when there is an incidental discovery of pelvic adhesions during laparoscopy to prevent infertility. For infertile women, in the event of fortuitous discovery of adhesions at laparoscopy, it is probably better not to perform complex adhesiolysis. Only adhesiolysis of tubo-ovarian adhesions that are minimal or slight in terms of their extension and/or their nature may be useful to improve the chances of spontaneous pregnancy. However, it remains to be decided on a case-by-case basis depending on other potential causes of infertility (low level of evidence). For women without known infertility issues, it is probably better not to perform systematic adhesiolysis in order to improve their pregnancy chances, considering the balance between the unknown benefit and the risks of complications inherent to surgery (low level of evidence). Further investigations are needed in order to increase the quality of management regarding associated interventions such as the treatment of superficial endometriosis or adhesions performed during a gynecologic surgical procedure and, thereby, bolster these recommendations.

Preoperative Prophylactic Balloon-Assisted Occlusion of the Internal Iliac Arteries in the Management of Placenta Increta/Percreta.

Background and Objectives: Preoperative prophylactic balloon-assisted occlusion (PBAO) of the internal iliac arteries minimizes blood loss and facilitates surgery performance, through reductions in the rate of uterine perfusion, which allow for better control in hysterectomy performance, with decreased rates of bleeding and surgical complications. We aimed to investigate the maternal and fetal outcomes associated with PBAO use in women with placenta increta or percreta. Material and Methods: The records of 42 consecutive patients with a diagnosis of placenta increta or percreta were retrospectively reviewed. Of 42 patients, 17 patients (40.5%) with placenta increta or percreta underwent cesarean delivery after prophylactic balloon catheter placement in the bilateral internal iliac artery (balloon group). The blood loss volume, transfusion volume, postoperative hemoglobin changes, rates of hysterectomy and hospitalization, and infant Apgar score in this group were compared to those of 25 similar women who underwent cesarean delivery without balloon placement (surgical group). Results: The mean intraoperative blood loss volume in the balloon group (2319 ± 1191 mL, range 1000-4500 mL) was significantly lower than that in the surgical group (4435 ± 1376 mL, range 1500-10,500 mL) (p = 0.037). The mean blood unit volume transfused in the balloon group (2060 ± 1154 mL, range 1200-8000 mL) was significantly lower than that in the surgical group (3840 ± 1464 mL, range 1800-15,200 mL) (p = 0.043). There was no significant difference in the postoperative hemoglobin change, hysterectomy rates, length of hospitalization, or infant Apgar score between the groups. Conclusion: PBAO of the internal iliac artery prior to cesarean delivery in patients with placenta increta or percreta is a safe and minimally invasive technique that reduces the rate of intraoperative blood loss and transfusion requirements.

Risk-reducing Bilateral Salpingo-oophorectomy: Assessing the Incidence of Occult Ovarian Cancer and Surgeon Adherence to Recommended Practices.

STUDY OBJECTIVE: This study aimed to determine the incidence of ovarian cancer diagnosed at the time of risk-reducing bilateral salpingo-oophorectomy in a large cohort of patients with a BRCA mutation. In addition, we aimed to determine the adherence to the recommended practices for performing a risk-reducing bilateral salpingo-oophorectomy as described by the American College of Obstetricians and Gynecologists and the Society of Gynecologic Oncology. We sought to determine if adherence differed by the type of training (i.e., gynecologic oncologists vs benign gynecologists). DESIGN: Descriptive, retrospective analysis. SETTING: Academic medical center. PATIENTS: Two hundred sixty-nine patients with a known BRCA mutation. INTERVENTIONS: Prophylactic risk-reducing bilateral salpingo-oophorectomy performed either by a gynecologic oncologist or a benign gynecologist between July 2007 and September 2018. MEASUREMENTS AND MAIN RESULTS: Among 269 patients who underwent risk-reducing bilateral salpingo-oophorectomies, 220 procedures were performed by gynecologic oncologists, and 49 were performed by benign gynecologists. Washings were not performed in 5% of the procedures performed by gynecologic oncologists and 37% of the procedures performed by benign gynecologists (p <.001). Complete serial sectioning of the adnexa was not performed in 12% of the procedures performed by oncologists, and 13% of the procedures performed by benign gynecologists (p = .714). There were 8 cases (2.9%) of tubal or ovarian cancer diagnosed within this cohort. Of these cases, only 3 (1.1%) were diagnosed at the time of surgery and met the criteria for conversion to a staging procedure. CONCLUSION: Because the incidence of ovarian cancer diagnosis at the time of risk-reducing bilateral salpingo-oophorectomy is low and is often not diagnosed at the time of surgery owing to the presence of only microscopic disease, it may not be necessary for gynecologic oncologists to exclusively perform these procedures. However, this study also revealed that when this procedure is performed by benign gynecologic surgeons, some of the recommended practices are not routinely followed. If general gynecologic surgeons are to routinely perform risk-reducing bilateral salpingo-oophorectomies, it is important to promote better adherence to these practices.

Value of ileus-prophylactic surgery for metastatic neuroendocrine midgut tumours.

Neuroendocrine tumours of the small intestine (SINET) are a rare disease. However, a rising incidence rate and excellent long-term survival, even in the setting of metastatic disease lead to a high prevalence of SINET of up to 11/100.000. At the time of diagnosis, most patients already suffer from metastatic disease. About one third of patients demonstrate localized or regional metastatic disease at time of presentation. For those patients the indication for curative surgery is not debated and 10-year cancer specific survival of almost 90% can be achieved. Due to major limitations of existing studies actually there is no sufficient evidence in favour of ileus-prophylactic palliative surgery for metastatic SINET. Until now the available evidence favouring an ileus-prophylactic palliative small bowel resection for stage IV SI-NET must be weighed against available high-level evidence from randomized trials that showed long-term survival under systemic therapy. Importantly, there is not a single study that indicates surgery for a symptomatic patient should be postponed. Because the majority of patients are symptomatic at the time of diagnosis, the rationale for an ileus-prophylactic palliative surgery is to operate before progression of mesenteric tumour mass and desmoplasia takes place and before intestinal obstruction and ischaemia occurs. To what extent a prophylactic palliative small bowel resection will provide a survival benefit in a situation where the mesenteric tumour mass cannot be resected radically is not clearly addressed by the current level of evidence.

Health Care Provider Adherence to Surgical Guidelines for Risk-Reducing Salpingo-Oophorectomy.

OBJECTIVE: To evaluate health care provider adherence to the surgical protocol endorsed by the National Comprehensive Cancer Network and the American College of Obstetricians and Gynecologists at the time of risk-reducing salpingo-oophorectomy and compare adherence between gynecologic oncologists and obstetrician-gynecologists (ob-gyns). METHODS: In this multicenter retrospective cohort study, women were included if they had a pathogenic BRCA mutation and underwent risk-reducing salpingo-oophorectomy between 2011 and 2017. Adherence was defined as completing all of the following: collection of washings, complete resection of the fallopian tube, and performing the Sectioning and Extensively Examining the Fimbriated End (SEE-FIM) pathologic protocol. RESULTS: Of 290 patients who met inclusion criteria, 160 patients were treated by 18 gynecologic oncologists and 130 patients by 75 ob-gyns. Surgery was performed at 10 different hospitals throughout a single metropolitan area. Demographic and clinical characteristics were similar between groups. Overall, 199 cases (69%) were adherent to the surgical protocol. Gynecologic oncologists were more than twice as likely to fully adhere to the full surgical protocol as ob-gyns (91% vs 41%, P<.01). Specifically, gynecologic oncologists were more likely to resect the entire tube (99% vs 95%, P=.03), to have followed the SEE-FIM protocol (98% vs 82%, P<.01), and collect washings (94% vs 49%, P<.01). Complication rates did not differ between groups. Occult neoplasia was diagnosed in 11 patients (3.8%). The incidence of occult neoplasia was 6.3% in gynecologic oncology patients and 0.8% in obstetrics and gynecology patients (P=.03). CONCLUSION: Despite clear surgical guidelines, only two thirds of all health care providers were fully adherent to guidelines. Gynecologic oncologists were more likely to follow surgical guidelines compared with general ob-gyns and more likely to diagnose occult neoplasia despite similar patient populations. Rates of risk-reducing surgery will likely continue to increase as genetic testing becomes more widespread, highlighting the importance of health care provider education for this procedure. Centralized care or referral to subspecialists for risk-reducing salpingo-oophorectomy may be warranted.

The role of endoscopy in the management of hereditary diffuse gastric cancer syndrome.

Hereditary diffuse gastric cancer (HDGC) syndrome is an inherited cancer risk syndrome associated with pathogenic germline CDH1 variants. Given the high risk for developing diffuse gastric cancer, CDH1 carriers are recommended to undergo prophylactic total gastrectomy for cancer risk reduction. Current guidelines recommend upper endoscopy in CDH1 carriers prior to surgery and then annually for individuals deferring prophylactic total gastrectomy. Management of individuals from HDGC families without CDH1 pathogenic variants remains less clear, and management of families with CDH1 pathogenic variants in the absence of a family history of gastric cancer is particularly problematic at present. Despite adherence to surveillance protocols, endoscopic detection of cancer foci in HDGC is suboptimal and imperfect for facilitating decision-making. Alternative endoscopic modalities, such as chromoendoscopy, endoscopic ultrasound, and other non-white light methods have been utilized, but are of limited utility to further improve cancer detection and risk stratification in HDGC. Herein, we review what is known and what remains unclear about endoscopic surveillance for HDGC, among individuals with and without germline CDH1 pathogenic variants. Ultimately, the use of endoscopy in the management of HDGC remains a challenging arena, but one in which further research to improve surveillance is crucial.

Web-Based Model for Predicting Time to Surgery in Young Patients with Familial Adenomatous Polyposis: An Internally Validated Study.

INTRODUCTION: The timing of prophylactic colorectal surgery in patients with familial adenomatous polyposis (FAP) is based on the immediacy of the colorectal cancer risk. The ability to predict the need for surgery may help patients and their families plan in the context of life events and CRC risk. We created a model to predict the likelihood of surgery within 2 and 5 years of first colonoscopy at our institution. METHODS: A single institution hereditary colorectal syndrome (Cologene™) database was interrogated for all patients with FAP having a deleterious APC mutation. Patients with first colonoscopy after age 30 and before year 2000 were excluded. Cox regression analysis was done to assess multiple factors associated with surgery, followed by stepwise Cox regression analysis to select an optimal model. Receiver operator curve (ROC) analysis was performed to assess the model. RESULTS: A total of 211 (53% female) patients were included. Forty-five percent underwent surgery after an average of 3.8 years of surveillance. The final model was created based on initial clinical characteristics (age, gender, BMI, family history of desmoids, genotype-phenotype correlation), initial colonoscopic characteristics (number of polyps, polyp size, presence of high-grade dysplasia); and on clinical events (chemoprevention and polypectomy). AUC was 0.87 and 0.84 to predict surgery within 2 and 5 years, respectively. The final model can be accessed at this website: http://app.calculoid.com/#/calculator/29638 . CONCLUSION: This web-based tool allows clinicians to stratify patients' likelihood of colorectal surgery within 2 and 5 years of their initial examination, based on clinical and endoscopic features, and using the philosophy of care guiding practice at this institution.

Life After Surgery: Surgeon Assessments of Quality of Life Among Patients With Familial Adenomatous Polyposis.

BACKGROUND: Without prophylactic surgery, patients with familial adenomatous polyposis are at high risk for colorectal cancer development. Various surgical options for prophylaxis are available. Patient decision-making for preventative treatments is often influenced by the preferences of healthcare providers. OBJECTIVE: We determined surgeon preferences for the surgical options available to patients with familial adenomatous polyposis. DESIGN: We obtained preference estimates for postoperative health states from colorectal surgeons who had treated ≥10 patients with familial adenomatous polyposis. SETTINGS: Assessments were made at an annual meeting of the American Society of Colon and Rectal Surgeons. MAIN OUTCOME MEASURES: Utilities were measured through the time trade-off method. We determined utilities for 3 procedures used for prophylaxis, including total proctocolectomy with permanent ileostomy, colectomy with ileorectal anastomosis, and total proctocolectomy with IPAA. We also assessed utilities for 2 short-term health states: 90 days with a temporary ileostomy and 2 years with a poorly functioning ileoanal pouch. RESULTS: Twenty-seven surgeons who had cared for >1700 patients with familial adenomatous polyposis participated in this study. The highest utility scores were provided for colectomy with ileorectal anastomosis (0.98). Lower utility scores were provided for total proctocolectomy with permanent ileostomy (0.87) and IPAA (0.89). The number of patients with familial adenomatous polyposis who were treated by participating surgeons did not influence these estimates; however, more-experienced surgeons gave lower utility scores for a poorly functioning ileoanal pouch than less-experienced surgeons (0.15, 0.50, and 0.25 for high-, medium-, and low-volume surgeons; p = 0.02). LIMITATIONS: This study was limited by the sample size. CONCLUSIONS: For patients with familial adenomatous polyposis and relative rectal sparing, surgeon preferences are greatest for colectomy with ileorectal anastomosis. Utility estimates provided by this study are important for understanding surgical decision-making and suggest a role for ileorectal anastomosis in appropriately selected patients. See Video Abstract at http://links.lww.com/DCR/A656.

p.Val804Met, the Most Frequent Pathogenic Mutation in RET, Confers a Very Low Lifetime Risk of Medullary Thyroid Cancer.

Context: To date, penetrance figures for medullary thyroid cancer (MTC) for variants in rearranged during transfection (RET) have been estimated from families ascertained because of the presence of MTC. Objective: To gain estimates of penetrance, unbiased by ascertainment, we analyzed 61 RET mutations assigned as disease causing by the American Thyroid Association (ATA) in population whole-exome sequencing data. Design: For the 61 RET mutations, we used analyses of the observed allele frequencies in ∼51,000 individuals from the Exome Aggregation Consortium (ExAC) database that were not contributed via The Cancer Genome Atlas (TCGA; non-TCGA ExAC), assuming lifetime penetrance for MTC of 90%, 50%, and unbounded. Setting: Population-based. Results: Ten of 61 ATA disease-causing RET mutations were present in the non-TCGA ExAC population with observed frequency consistent with penetrance for MTC of >90%. For p.Val804Met, the lifetime penetrance for MTC, estimated from the allele frequency observed, was 4% [95% confidence interval (CI), 0.9% to 8%]. Conclusions: Based on penetrance analysis in carrier relatives of p.Val804Met-positive cases of MTC, p.Val804Met is currently understood to have high-lifetime penetrance for MTC (87% by age 70), albeit of later onset of MTC than other RET mutations. Given our unbiased estimate of penetrance for RET p.Val804Met of 4% (95% CI, 0.9% to 8%), the current recommendation by the ATA of prophylactic thyroidectomy as standard for all RET mutation carriers is likely inappropriate.

The open abdomen in trauma and non-trauma patients: WSES guidelines.

Damage control resuscitation may lead to postoperative intra-abdominal hypertension or abdominal compartment syndrome. These conditions may result in a vicious, self-perpetuating cycle leading to severe physiologic derangements and multiorgan failure unless interrupted by abdominal (surgical or other) decompression. Further, in some clinical situations, the abdomen cannot be closed due to the visceral edema, the inability to control the compelling source of infection or the necessity to re-explore (as a "planned second-look" laparotomy) or complete previously initiated damage control procedures or in cases of abdominal wall disruption. The open abdomen in trauma and non-trauma patients has been proposed to be effective in preventing or treating deranged physiology in patients with severe injuries or critical illness when no other perceived options exist. Its use, however, remains controversial as it is resource consuming and represents a non-anatomic situation with the potential for severe adverse effects. Its use, therefore, should only be considered in patients who would most benefit from it. Abdominal fascia-to-fascia closure should be done as soon as the patient can physiologically tolerate it. All precautions to minimize complications should be implemented.

Quality of life following prophylactic gynecological surgery: experiences of female Lynch mutation carriers.

Lynch syndrome (LS) is a genetic condition conferring an elevated risk of gastrointestinal, gynecologic and other malignancies, often before the age of 50. Current guidelines recommend prophylactic gynecologic surgery to manage inherited cancers for female mutation carriers. Data is lacking on women's quality of life following surgery. In this pilot study, we explored how women described their quality of life post-prophylactic gynecologic surgery and the factors that affected post-surgery experiences. A qualitative interview study was the chosen design. Ten female Lynch syndrome mutation carriers were interviewed by phone. Interviews were transcribed and analysed for themes relating to quality of life post-surgery using content analysis and constant comparison. Women largely reported doing well since their surgeries, though all described deleterious impacts on quality of life. Positive impacts of surgery included a reduction in cancer worry and an increase in healthy lifestyle behaviors, while negative impacts due to the sudden onset of menopause and impact on sexual function were common. Pre-surgical knowledge, drug and topical therapies, and post-surgical support all contributed to a positive quality of life. This small pilot study revealed increased endocrine symptoms and a negative impact on sexual health following prophylactic gynecological surgery. Women who were informed of potential symptoms pre-surgery coped better with surgical outcomes, as did women using some form of HRT. All women experienced reduced cancer worry post-surgery. Findings highlight areas for discussion in pre-operative settings (e.g., sexual health), as well as the need for better follow-up support post-surgery.

Comparison of Nutritional and Clinical Outcomes in Patients with Head and Neck Cancer Undergoing Chemoradiotherapy Utilizing Prophylactic versus Reactive Nutrition Support Approaches.

BACKGROUND: The optimal method of tube feeding for patients with head and neck cancer remains unclear. A validated protocol is available that identifies high-nutritional-risk patients who would benefit from prophylactic gastrostomy tube placement. Adherence to this protocol is ultimately determined by clinical team discretion or patient decision. OBJECTIVE: The study aim was to compare outcomes after adherence and nonadherence to this validated protocol, thus comparing a prophylactic and reactive approach to nutrition support in this patient population. DESIGN: We conducted a prospective comparative cohort study. Patients were observed during routine clinical practice over 2 years. PARTICIPANTS/SETTING: Patients with head and neck cancer having curative-intent treatment between August 2012 and July 2014 at a tertiary hospital in Queensland, Australia, were included if assessed as high nutrition risk according to the validated protocol (n=130). Patients were grouped according to protocol adherence as to whether they received prophylactic gastrostomy (PEG) per protocol recommendation (prophylactic PEG group, n=69) or not (no PEG group, n=61). MAIN OUTCOME MEASURES: Primary outcome was percentage weight change during treatment. Secondary outcomes were feeding tube use and hospital admissions. STATISTICAL ANALYSIS PERFORMED: Fisher's exact, χ2, and two sample t tests were performed to determine differences between the groups. Linear and logistic regression were used to examine weight loss and unplanned admissions, respectively. RESULTS: Patients were 88% male, median age was 59 years, with predominantly stage IV oropharyngeal cancer receiving definitive chemoradiotherapy. Statistically significantly less weight loss in the prophylactic PEG group (7.0% vs 9.0%; P=0.048) and more unplanned admissions in the no PEG group (82% vs 75%; P=0.029). In the no PEG group, 26 patients (43%) required a feeding tube or had ≥10% weight loss. CONCLUSIONS: Prophylactic gastrostomy improved nutrition outcomes and reduced unplanned hospital admissions. Additional investigation of characteristics of patients with minimal weight loss or feeding tube use could help refine and improve the protocol.