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We described trends in pelvic pain characteristics over 2 years of follow-up among adolescents and adults with and without endometriosis participating in the longitudinal observational cohort of the Women's Health Study: From Adolescence to Adulthood, using data reported at baseline and at years 1 and 2 of follow-up. Participants completed a questionnaire at baseline (between November 2012 and May 2019) and annually thereafter that included validated measures of severity, frequency, and life interference of dysmenorrhea, acyclic pelvic pain, and dyspareunia. Our study population included 620 participants with surgically confirmed endometriosis (rASRM stage I/II = 95%) and 671 community-based and hospital-based controls, with median age = 19 and 24 years, respectively. The proportion reporting hormone use varied across the 3 years ranging from 88% to 92% for cases and 56% to 58% for controls. At baseline, endometriosis cases were more likely to report severe, frequent, and life-interfering dysmenorrhea, acyclic pelvic pain, and dyspareunia compared with controls. Among cases, frequency and severity of dysmenorrhea and dyspareunia were relatively static across 2 years. However, acyclic pelvic pain improved. Severe acyclic pain decreased from 69% at baseline to 46% at year 2. Daily pain decreased from 28% to 14%, and life interference from 68% to 38%. Trends among controls remained fairly stable across 2 years. Among endometriosis cases who completed the questionnaire at all 3 time points, 18% reported persistent, severe acyclic pelvic pain at all 3 time points. Over time, different trends were observed by pelvic pain type among endometriosis cases and controls, supporting the importance of assessing multidimensional features of pelvic pain.

Study Objective To study the feasibility of virtual visits for ambulatory encounters in pediatric and adolescent gynecology Design A retrospective review Setting Boston Children's Hospital Participants Patients who were seen virtually through the Division of Gynecology between January 1, 2020 and June 1, 2020 Main Outcome measure(s) Patient demographics, visit diagnoses, and operational characteristics of the completed visits Results There were a total of 654 virtual visits for 614 patients. Ninety-one percent (n=558) of patients were in-state, and the median age of patients was 17 years (range 0 – 37 years). The majority were return visits (n=502, 76.8%), 115 (17.6%) were new patient visits, and 32 (4.89%) were post-operative visits. The median virtual visit duration was 12 minutes and 39 seconds (range 5 minutes to over 1 hour). The most common gynecologic diagnoses were dysmenorrhea/endometriosis (n=485, 74.2%), abnormal uterine bleeding (n=225, 34.4%), and pelvic pain (n=82, 12.5%). The percentage of virtual visits which required an in-person follow-up visit within 90 days was low (n=14, 2.1%). Five of these were within 30 days from the initial virtual visit, 6 were within 60 days, and 3 were within 90 days. Conclusion Telemedicine is a feasible method for expanding access to, and healthcare delivery for, pediatric and adolescent gynecology, with low rates of short interval in-person follow-up required. Virtual visits can be conducted for a range of patients with a variety of gynecologic conditions, upon initial presentation and follow-up.

The field of Obstetrics and Gynecology is one that provides intersections between one's most private and intimate moments with scenarios that could potentially trigger significant emotional trauma. As providers, one must balance providing appropriate care with the respect and autonomy of the patient. The perioperative and operative space presents many ethical dilemmas in navigating these boundaries, particularly among individuals with a history of sexual trauma. In our commentary, we present one case of an adolescent patient, with a history of sexual trauma and pelvic pain, who underwent laparoscopic surgery. We explore the interplay of delegating autonomy during amnesia, chronic pelvic pain, post-traumatic stress disorder, and hyperarousal within this period. There is knowledge to be gained amongst Obstetrics and Gynecology providers in navigating perioperative services in individuals with complex history of social stress and trauma so that we can better understand the landscape of providing competent care.

Objective To estimate the efficacy and safety of a novel nonhormonal therapeutic agent, cabergoline, compared with that of the standard clinical therapy, norethindrone acetate (NETA), for the treatment of endometriosis-associated pain in young women with endometriosis. Design Randomized, double-blind, placebo-controlled pilot study. Setting Tertiary care center. Patient(s) Women (n = 9) with surgically confirmed endometriosis. Intervention(s) A random, double-blind assignment to either NETA (5 mg/day) + placebo twice weekly or cabergoline (0.5 mg) twice weekly + placebo daily for 6 months. Main Outcome Measure(s) We collected the measures of pelvic pain and laboratory parameters every 3 months. Result(s) We observed a decrease in pain scores and increase in pain relief in women randomized to receive cabergoline, who appeared to show similar or more improvements than women treated with NETA. The serum measures of vascular endothelial growth factor receptor 1 declined over 6 months in those who received cabergoline. Cabergoline was well tolerated, and no serious adverse events occurred. Conclusion(s) Safe, effective adjunct treatments are lacking for patients with endometriosis who do not respond to standard care. Because the growth of endometriosis requires angiogenesis, blood vessel growth is an attractive therapeutic target. This pilot study suggests that cabergoline, a vascular endothelial growth factor pathway inhibitor, is an effective therapeutic option for women with chronic pain due to endometriosis. Building upon this investigation, we will conduct larger, randomized trials of cabergoline, advancing research on the best treatments for endometriosis—particularly disease resistant to hormonal therapies. Clinical Trial Registration Number clinicaltrials.gov; registration number NCT02542410.

Background Vaginal bleeding in the prepubertal child has several etiologies, including structural anomalies, endocrinologic dysfunction, infection, trauma, foreign body, and hematologic disorders. Case An 8-year-old premenarcheal girl presented with vaginal bleeding. On ultrasound, she was found to have an intramural hyperechogenic mass in the left uterine fundus. This was biopsied and showed smooth muscle. She was subsequently started on a GnRH agonist to suppress bleeding and has since been followed with serial ultrasounds. We have 6 years of follow-up imaging showing a presumed adenomyoma versus fibroid that has been stable in size. Summary and Conclusion We present a novel case of adenomyosis presenting as prepubertal bleeding. Pelvic imaging is prudent to exclude structural etiologies as the cause of prepubertal vaginal bleeding.

OBJECTIVE: Inadequate communication about endometriosis symptom burden between women and healthcare providers is a barrier for optimal treatment. This study describes the development of the EndoWheel, a patient-reported assessment tool that visualizes the multi-dimensional burden of endometriosis to facilitate patient-provider communication. METHODS: Assessment questions for the tool were developed using an iterative Delphi consensus process. A consensus phase included additional practitioners and specialists to broaden perspectives and select revised statements. Semi-structured qualitative interviews were conducted with 13 women with endometriosis to assess the scoring and content of the measures. RESULTS: Symptoms included in the tool were pelvic pain, vaginal bleeding, bowel/bladder symptoms, energy levels, fertility, impact on activities, emotional and sexual well-being, and self-perceived global health. Additional life impact areas included relationships, social and occupational activity, and self-perception. The 13 interviewees completed the tool in approximately 5-6 minutes (range 4.0-7.5 minutes). Most participants (92%) perceived that the tool would enable better patient-provider communication, including addressing symptoms and areas of impact not normally discussed during office visits. CONCLUSION: Similar to visual circular tools used in burden assessment of other chronic diseases, the tool may facilitate improved patient dialogue with providers around endometriosis treatment goals and options.

Study Objective Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a female reproductive disorder characterized by the absence or underdevelopment of the uterus, cervix and vagina. Limited research has examined factors related to psychological adjustment in MRKH. This study aimed to explore associations among illness representations, self-concept, psychological distress, and self-esteem in MRKH. Design Cross-sectional. Setting Participants were recruited globally online and from patient meetings. Participants A total of 263 patients with MRKH (age 16.1-74.4 years; mean = 31.7 years) completed questionnaires. Intervention None. Main Outcome Measures Validated self-reported measures of psychological distress and self-esteem (outcomes) and illness representations, self-concept, social support-seeking, and positive affect (hypothesized correlates) were explored in correlation and hierarchical regression analyses, alongside demographic and clinical variables. Results Younger age and shorter time since diagnosis was associated with higher distress and lower self-esteem. Patients with MRKH reported significantly higher distress and lower self-esteem than the general population. Higher distress and lower self-esteem were associated with higher reported engulfment (defining one's identity or feeling consumed by MRKH) and beliefs about the serious consequences of MRKH, and lower reported MRKH coherence, enrichment (positive changes to self-identity because of MRKH) and positive affect. Conclusions Findings suggest that the impact of MRKH on identity plays an important role in adjustment. High perceived coherence and maintenance of positive affect may play a protective role in psychological adjustment. A 12-month follow-up study is planned to examine associations among these variables longitudinally. Baseline data suggest that early availability of psychological support would be beneficial, and interventions focused on identity and psychoeducation about MRKH would be valuable.

Study Objective To study the presentation of dysmenorrhea and endometriosis in transmasculine adolescents and review their treatment outcomes. Design A retrospective review. Setting Boston Children's Hospital. Participants Transmasculine persons younger than 26 years old who were diagnosed with dysmenorrhea and treated between January 1, 2000 and March 1, 2020. Interventions Not applicable. Main Outcome Measures An electronic medical record review of the clinical characteristics, transition-related care, and treatment outcomes. Results Dysmenorrhea was diagnosed in 35 transmasculine persons. Mean age was 14.9 years ± 1.9 years. Twenty-nine (82.9%) were diagnosed after social transition. Twenty-three of 35 (65.7%) were first treated with combined oral contraceptives, but 14/23 (61%) discontinued or transitioned to alternative therapy. Twelve patients with dysmenorrhea alone initiated testosterone treatment, and 4/12 (33.3%) experienced persistent symptoms. Seven of 35 patients with dysmenorrhea (20.0%) were laparoscopically evaluated for endometriosis, and it was confirmed in all seven. Six had stage I disease, and one had stage II. Three of the 7 (42.9%) were diagnosed after social transition, with one diagnosed 20 months after initiating testosterone treatment. Their endometriosis was treated with combined oral contraceptives, danazol, or progestins; four experienced suboptimal response during treatment with these therapies alone. Two of those with suboptimal response subsequently resolved their dysmenorrhea when using testosterone. Five patients with endometriosis initiated testosterone treatment, and of the 5 (40%) experienced persistent symptomatology with combined testosterone and progestin therapies. Conclusion To our knowledge, this is the first study to characterize endometriosis in transmasculine persons. Evaluation for endometriosis was underutilized in transmasculine persons with dysmenorrhea, despite those who underwent laparoscopic evaluation and had disease confirmation. Although testosterone treatment can resolve symptoms in some, others might require additional suppression. Endometriosis should be considered in transmasculine persons with symptoms even when they are using testosterone.

Endometriosis is a common gynecologic disorder often associated with severe pelvic pain and infertility with few known modifiable risk factors. We investigated whether in utero and early life exposures are associated with surgically diagnosed endometriosis among adolescents and young adults.This case-control study, including 295 laparoscopically-confirmed endometriosis cases and 309 population-based controls aged25 years, was conducted using data from The Women's Health Study: From Adolescence to Adulthood which enrolled participants from 2012 to 2018. Information on in utero and early life factors were collected using a modified WERF EPHect questionnaire at enrollment, including their mother's age at delivery, birthweight, gestation length, exposure to smoking in utero and secondhand smoke during childhood up to age 13, and if the participant was breastfed.Median age at enrollment was 17 years (range 12-24) in cases and 22 years (range 7-24) in controls, with 83 % and 68 % non-Hispanic whites, respectively. The majority of cases (95 %) were rASRM stage I or II at diagnostic surgery. Exposure to breastfeeding in early life was associated with lower odds of endometriosis diagnosis (OR = 0.39, 95 % CI = 0.21-0.74). Exposure to secondhand smoke during childhood due to maternal smoking was associated with increased odds of endometriosis diagnosis (OR = 2.70, 95 % CI = 1.11-6.60).Among adolescents and young adults, our data suggest exposures to breastfeeding in early life and secondhand smoke during childhood may be associated with endometriosis risk, providing insight into etiologic pathways to be explored in this young population.

Endometriosis is the leading pathologic cause of dysmenorrhea and chronic pelvic pain among adolescents. The appearance of endometriosis in adolescents may be different from that in female adults, resulting in delayed recognition and intervention. This article addresses the epidemiology, pathophysiology, clinical presentation, diagnosis, and management of endometriosis in the adolescent.

Background Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina. Case We present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.

Malignant ovarian neoplasms are uncommon in the pediatric and adolescent population. Imaging and tumor markers help to guide the preoperative risk/benefit analysis for planned surgical management, which is the mainstay of therapy. An interdisciplinary approach should be taken in the management of this vulnerable population from diagnosis through post-treatment surveillance. In this review, the initial evaluation, risk stratification, and management of various types of malignant ovarian masses will be addressed with a special focus on how to optimize an interdisciplinary approach to ovarian masses.

Introduction GNAS mutations have been reported in both McCune-Albright syndrome (MAS) and juvenile granulosa cell tumors (JGCT) but have never been reported simultaneously in the same patient. Case Presentation A 15-year-old girl developed secondary oligomenorrhea. Laboratory studies revealed suppressed gonadotropin levels with markedly elevated estradiol and inhibin B levels. Pelvic ultrasound showed a 12-cm heterogeneous right adnexal mass; pelvic magnetic resonance imaging to further characterize the mass displayed heterogeneous bilateral femoral bone lesions initially concerning for metastatic disease. Positron emission tomography/computed tomography showed minimal 18F-fluorodeoxyglucose (FDG) uptake in the pelvic mass but unexpectedly revealed FDG uptake throughout the skeleton, concerning for polyostotic fibrous dysplasia in the context of MAS. The adnexal mass was excised and pathology confirmed a JGCT. The patient’s affected bone and JGCT tissue revealed the same pathogenic GNAS p.R201C mutation, while her peripheral blood contained wild-type arginine at codon 201. Conclusion This mutation has been previously reported in cases of MAS and JGCT but never simultaneously in the same patient. This demonstration of a GNAS mutation underlying both JGCT and MAS in the same patient raises questions about appropriate surveillance for patients with these conditions.

PROBLEM Associations between immune dysfunction conditions (eg, systemic lupus erythematous, rheumatoid arthritis) and endometriosis have been observed in adult women, but not assessed among a younger population. We investigated the association between immune-mediated conditions and endometriosis among young women. METHOD OF STUDY This cross-sectional analysis in the Women's Health Study: From Adolescence to Adulthood included 551 participants with surgically diagnosed endometriosis (median age=19) and 652 controls without endometriosis (median age=24). Participants completed an expanded Endometriosis Phenome and Biobanking Harmonization Project questionnaire. We used logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (CIs) to investigate the associations between autoimmune/inflammatory, atopic, chronic pain/fatigue, and endocrine disorders with endometriosis, adjusting for confounders. RESULTS Participants with any autoimmune and/or inflammatory condition had an increased odds of co-occurring endometriosis (OR: 1.87; CI: 0.92-3.80), as did participants with allergies (OR: 1.76; CI: 1.32-2.36), asthma (OR: 1.35; CI: 0.97-1.88), chronic fatigue syndrome and/or fibromyalgia (OR: 5.81; CI: 1.89-17.9), or previous mononucleosis (OR: 1.75; CI: 1.14-2.68). Odds of endometriosis were lower among participants with eczema (OR: 0.68; CI: 0.44-1.04). We observed a positive trend between the number of immune-mediated conditions and the odds of endometriosis (p-trend=0.0002). Endocrine disorders were not associated with endometriosis. CONCLUSIONS Among this population of adolescents and adult women, endometriosis was more likely among participants with autoimmune and/or inflammatory diseases, allergies, asthma, previous mononucleosis infection, and chronic fatigue and/or fibromyalgia. We observed that an increasing number of immune-mediated conditions were positively associated with endometriosis risk. It is important for clinicians who care for adolescents and women with these conditions to consider endometriosis as a comorbidity.

Long-term survival following hematopoietic stem cell transplant (HSCT) in childhood continues to improve, and patients are thus increasingly faced with the late effects of treatment. Infertility is very common for both males and females following HSCT and is one of the most distressing sequelae. Adoption and surrogate egg or sperm donation are possibilities for some patients, but post-HSCT reversal of gonadal failure is not possible. We have recently initiated an oncofertility program with a dedicated practitioner with specific expertise in this area. Our practice is for her to meet with all families and age-appropriate patients during the pre-HSCT evaluation period. This allows patients and families to be accurately informed about the expected treatment-related infertility risk and the available options for fertility preservation. Sperm banking and egg or embryo cryopreservation are established approaches but are not achievable for many children and adolescents. Recently, the harvesting and cryopreservation of ovarian and testicular tissue represents a novel surgical option that allows for the possibility of fertility preservation to be extended to children of all ages. The purpose of this investigation is to evaluate the safety of these procedures proximal to conditioning therapy and HSCT. This is a retrospective report on a consecutive cohort of all patients aged 0 to 25 years who, after discussion with our oncofertility specialist, chose to undergo surgical fertility preservation (laparoscopic unilateral oophorectomy or testicular biopsy) at our institution between March 2018 and April 2020. These procedures occurred under general anesthesia at the time of central line placement prior to the initiation of HSCT conditioning. We assess the safety of the procedures in terms of postoperative complications and impact on HSCT course. Twenty-two patients underwent fertility preservation surgical procedures. Thirteen patients (59%) were female, median age 13 years (1 to 22 years), and 9 (41%) were male, median age 8 years (5 to 12 years). Fourteen (63%) were prepubertal and 8 (36%) pubertal. HSCT indications were hematologic malignancies/solid tumor (40%) and nonmalignant diseases (60%). Most received an allogenic graft (68%) and 81% had myeloablative conditioning. All patients became neutropenic at a median of 10 days (0 to 51 days) from the surgical procedure; 1 was neutropenic at the time of testicular tissue cryopreservation (TTC). The mean duration for the procedures performed, including ovarian tissue cryopreservation (OTC) or TTC, was 98 minutes (49 to 260 minutes) and 97 minutes (56 to 178 minutes), respectively. Estimated blood loss was minimal and no postoperative site infections occurred. One postprocedure, blood culture-negative fever was reported without an identifiable source; the patient completed 48 hours of antibiotics with resolution of fever. Sixty-two percent of females and 56% of males started conditioning within 24 hours of OTC/TTC (15 hours to 113 days; median, 1 day). The median time to engraftment was 22 days (9 to 33 days) in females and 17 days (11 to 67 days) in males, consistent with our institutional benchmarks. One patient with aplastic anemia had primary graft failure, attributed to low cell dose. This patient engrafted after a second transplant from an alternative donor but ultimately died of multiorgan failure. He was neutropenic for over 60 days and never experienced surgical site infection. There were no procedure-related delays to start of conditioning or to discharge. Children of all ages can now be offered the possibility of fertility preservation following HSCT for benign and malignant conditions. Our review suggests that these procedure for both females and males can be performed close to the start of conditioning, which allows for coupling with central access placement. These procedures appear to be safe and do not add to transplant-related morbidity.

Endometriosis may exert a profound negative influence on the lives of individuals with the disorder, adversely affecting quality of life, participation in daily and social activities, physical and sexual functioning, relationships, educational and work productivity, mental health, and well-being. Over the course of a lifetime, these daily challenges may translate into limitations in achieving life goals such as pursuing or completing educational opportunities; making career choices or advancing in a chosen career; forming stable, fulfilling relationships; or starting a family, all of which ultimately alter one’s life trajectory. The potential for endometriosis to impact the life course is considerable, as symptom onset generally occurs at a time of life (menarche through menopause, adolescence through middle age) when multiple life-changing and trajectory-defining decisions are made. Using a life-course approach, we examine how the known effects of endometriosis on life-domain satisfaction may impact health and well-being across the life course of affected individuals. We provide a quasi-systematic, narrative review of the literature as well as expert opinion on recommendations for clinical management and future research directions.

Endometriosis is generally histopathologically defined as the presence of at least 2 of the following: endometrial stroma, Mullerian epithelium, and/or hemosiderin-laden macrophages (HLM). Despite clinically evident endometriotic lesions, biopsies are frequently nondiagnostic. In this study, we conducted a large-scale review of biopsies of lesions clinically thought to represent endometriosis and correlate the histologic findings with clinical appearance to expand sensitivity of the pathologic definition of endometriosis, particularly in patients on hormonal therapy. In all, 112 biopsies from 78 patients (mean age=25, range 18-39 yr) were reviewed for histopathologic features suggestive of or diagnostic for endometriosis including the presence of endometrial stroma, Mullerian epithelium, dystrophic calcifications, HLM, chronic inflammation, adhesions, and vascular proliferation. Endometriosis was confirmed by pathologic criteria in 37 of 78 patients (47%). Biopsies from patients on hormonal therapy (n=62, 80%) were significantly less likely to meet pathologic criteria for endometriosis (P=0.01). Nondiagnostic biopsies (70/112; 63%) frequently displayed HLM (20%), chronic inflammation (29%), dystrophic calcifications (26%), vascular proliferation (20%), or adhesions (20%) and were significantly more likely to have a vascular clinical appearance (P=0.01). Diagnostic biopsies (42/112; 38%) were more likely to have a blue/black clinical appearance (P=0.03), demonstrate HLM (P=0.004), and display pseudodecidualization (P=0.05). Patients with a high clinical suspicion of endometriosis have a range of histologic findings, with less than half meeting the current histopathologic criteria for diagnosing endometriosis. Given the heterogeneous histopathologic appearance, revision of the histologic criteria may be warranted with further exploration, particularly for lesions with predominantly vascular features.

PROBLEM To compare inflammatory- and immune-associated peritoneal cytokines of adolescents and adults with and without endometriosis. METHODS OF STUDY In a nested case-control study in multiple university-affiliated scientific centers, ten adolescents and thirteen adults with visually and histologically confirmed endometriosis (cases), thirteen adolescents with visually suspected endometriosis but indeterminate (seven patients) or negative (six patients) histology, and fifteen adults undergoing surgery for non-malignant gynecologic disease without endometriosis (controls) underwent laparoscopic aspiration of peritoneal fluid (PF), from which PF and conditioned medium (CM) cytokine levels were assayed. RESULTS Compared to adults with endometriosis, MCP-3, IL-12p40, MIP-1β, and IL-15 were significantly higher among adolescents with endometriosis, while TNF-β and CTACK were lower among adolescents. These differences were similar comparing adolescents with endometriosis to adult controls except for MIP-1β, which was not statistically different. MIP-1β was, however, the only cytokine observed to differ between adult cases and controls. There were no significant differences in CM cytokines among the three groups. Results were similar when analyses were restricted to samples collected (a) during menstrual cycle days 1-10, (b) from patients unexposed to exogenous hormones, or (c) from all adolescents despite presence or absence of histologic endometriosis. CONCLUSION Biologically relevant and statistically significant differences in six PF cytokines were observed and suggest a more pro-invasion cytokine profile among adolescents with endometriosis. Adolescents with endometriosis have unique peritoneal cytokine profiles and molecular behavior when compared to adults with and without endometriosis.

Endometriosis is a painful gynecologic disease affecting one in ten reproductive aged women worldwide. Few studies have correlated this symptomatology with biomarker levels among women with and without endometriosis, and no studies correlating pain with biomarker levels have been performed in young patient populations. The purpose of this study was to examine whether CA125 correlates with different types and severity of pain among adolescents and young women with and without endometriosis and assess its performance as an endometriosis biomarker among those presenting with dysmenorrhea in this young population. Reproductive-aged women with laparoscopically-confirmed endometriosis (n = 282) and controls (n = 293) who participated in The Women's Health Study: From Adolescence to Adulthood (A2A), a cohort of adolescents and young women enrolled from 2012-2018, were included in this cross-sectional analysis. Plasma CA125 values were measured using WERF EPHect compliant blood samples collected at enrollment. Average CA125 were calculated by self-reported pain type (i.e. dysmenorrhea, non-cyclic/general pelvic pain, dyspareunia), severity, and frequency in endometriosis cases and controls. Median age at blood draw was 24 years in controls and 17 years in cases, with 68% and 89% non-Hispanic white, respectively. Most endometriosis cases (95%) were rASRM stage I/II. Average CA125 values were 12.5 U/mL in controls and 12.1 U/mL in cases adjusted for age. CA125 did not differ by pain type, its severity, or frequency in endometriosis cases or controls. Among participants who reported dysmenorrhea, CA125 did not discriminate endometriosis cases from controls using cutoff of 35 U/mL (AUC = 0.51, 95%CI = 0.50-0.53). Among adolescents and young adult women, CA125 did not correlate with pain type. CA125 did not efficiently discriminate endometriosis cases from controls even when accounting for pain symptomatology. Average CA125 values were low in adolescents and young women in both endometriosis cases and controls, suggesting cautious interpretation may be needed when measuring CA125 in this population.

Background Granular cell tumors (GCTs) are rare soft tissue lesions that can involve the female genital tract, including the vulva. Although malignant tumors are aggressive and uncommon, benign and atypical lesions still have associated risks, including recurrence and multisite development. Cases Four cases of pediatric vulvar GCT are presented, including 1 atypical and 3 benign tumors. The atypical lesion occurred in a 3-year-old girl, to our knowledge, the youngest reported patient with GCT of the vulva. Follow-up information is available for 3 patients, ranging 0-12 years. Summary and Conclusion GCTs are an important differential diagnosis in patients with vulvar lesions. In the pediatric population, the tumors' risk of recurrence and multisite development require gynecologists to advocate for complete resection, careful follow-up, and thorough examination for additional tumor sites.

Objective To determine the prevalence and experience of migraines in adolescents with surgically confirmed endometriosis compared with those without endometriosis. Design Cross-sectional study conducted within The Women's Health Study: From Adolescence to Adulthood—an ongoing longitudinal cohort. Setting Boston Center for Endometriosis. Patient(s) Adolescent females enrolled November 2012 through November 2016. The case group included adolescents surgically diagnosed with endometriosis. The control group included adolescents without endometriosis, recruited from the local community and clinics. Intervention(s) Not available. Main Outcome Measure(s) An extensive online health questionnaire regarding medical history, lifestyle, medication use, anthropometrics, and symptom experience and treatments. Migraine diagnosis was self-reported. Migraine pain and noncyclic pelvic pain severity were rated using an 11-point numerical rating scale. Cyclic pelvic pain was categorized. Result(s) Adolescents with endometriosis were more likely to experience migraines (69.3%) than those without endometriosis (30.7%) (multivariable odds ratio=4.77, 95% confidence interval 2.53, 9.02). For each 1-point increase in the migraine numerical rating scale, the odds of endometriosis increased by 22% (multivariable odds ratio=1.22, 95% confidence interval 1.03, 1.44; P trend = .02). Among those with endometriosis, age of menarche was associated inversely with the odds of migraines. Participants with endometriosis and migraines have more dysmenorrhea than those without migraines. Conclusion(s) Adolescents with endometriosis are more likely to experience migraines than adolescents without endometriosis. A linear relationship exists between migraine pain severity and the odds of endometriosis, suggesting heightened pain sensitivity for adolescents with endometriosis. Due to the strong correlation, patients who present with either condition should be screened for comorbidity to maximize the benefits of care.

Background Adolescent endometriosis typically presents as stage I with superficial peritoneal disease and less commonly as stage III or IV with deeply infiltrative disease. Endometriosis lesions can be destroyed (cautery or laser), cutting out the discrete lesion with excision and destroyed, or radically excised with removal of the lesion and surrounding tissue. It has been shown to be beneficial to excise deeply infiltrative disease to improve pain. Radical excision has been promoted by a subset of surgeons and involves removal of large areas of peritoneum with the promise/proposal of a cure and suggestion of no need for medical suppression of endometriosis. The best technique to manage superficial peritoneal disease has not yet been defined. Case A 15-year-old young woman with a history of 2 previous laparoscopies for pain and an ovarian cyst who underwent removal of a mucinous cystadenoma, presented to a local gynecologist with chronic pelvic pain. She underwent a third laparoscopy and was found to have superficial peritoneal endometriosis and filmy adhesions believed to be due to the previous ovarian surgery. The endometriosis was surgically destroyed with the use of cautery and the filmy adhesions were lysed. Months later she had a return of pain and was advised to have a fourth laparoscopy with radical excision by an “excisionalist” gynecologist. She was found to have superficial peritoneal disease with ASRM-defined stage I endometriosis and underwent radical excision of the peritoneum of the anterior cul de sac, posterior cul de sac, and both pelvic side walls. She was informed that she had been cured of her endometriosis and was thus not treated with postoperative hormonal suppression. Her pain did not improve and in fact worsened after the radical excisional surgery. She self-referred for care. She started menstrual suppression treatment with continuous estrogen/progestin therapy for medical treatment of endometriosis but after 6 months she was still having severe pain without bleeding. Eight months after the radical excisional surgery she elected to have a fifth laparoscopy to address potential adhesions. At that time she was found to have extensive pelvic adhesions with the uterus adherent to the anterior cul de sac, and adhesions in the posterior cul de sac. In addition, both ovaries were involved with adhesions and adherent to the pelvic side walls. She was found to have clear and red lesions of superficial peritoneal endometriosis. She underwent a lysis of adhesions, and excision of lesions, and destruction of endometriosis. Her pain improved postoperatively; menstrual suppression was continued and she has remained with a continued excellent quality of life with over 2 years of follow-up. Summary and Conclusion For this patient, radical excisional surgery resulted in increased pain and extensive adhesion formation. It was not curative because endometriosis was documented on follow-up surgery. In a previously published long-term follow-up report of adolescents with recurrent pain 2-10 years after destruction of superficial peritoneal disease, it was reported that there were no increased adhesions and no trend toward disease progression. Excisional gynecologists who perform this procedure should not suggest that radical excisional surgery is helpful and without increased risk, until studies have shown long-term benefit in the surgical management of superficial peritoneal endometriosis.

Background Menorrhagia is a common gynecologic complaint among adolescents, which rarely is secondary to malignancy. Burkitt lymphoma can mimic gynecologic malignancy, however it is rarely seen in adolescents. Burkitt lymphoma of the gynecologic tract requires early diagnosis and intervention for optimal outcomes. Case We report a case of a 15-year-old adolescent who had multiple admissions for menorrhagia that was thought to be secondary to anovulatory bleeding until pelvic ultrasound revealed a large 8-cm vaginal/cervical mass. Histologic examination of the biopsy specimen revealed Burkitt lymphoma, which was treated with chemotherapy leading to resolution of her menorrhagia. Summary and Conclusion Burkitt lymphoma presenting as a vaginal/cervical mass is exceedingly rare, especially in the adolescent patient. Burkitt lymphoma is generally highly responsive to chemotherapy, and symptoms rapidly improve after initiation of treatment.

Background Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital condition typified by underdevelopment of the female reproductive tract. Much of the MRKH literature addresses surgical options and psychosocial aspects, however little is known about the routine gynecologic care of individuals with MRKH. The purpose of this study was to assess the knowledge of and prior counseling on genital infection and prevention in a sample of people with MRKH. Methods The Beautiful You MRKH Foundation disseminated an IRB-approved, web-based survey through social media. Participants were eligible if they had previously been diagnosed with MRKH and were 18 years and older. Survey content included past gynecologic counseling, sexually transmitted genital infection knowledge, and sexual history. Questions assessing knowledge of human papillomavirus (HPV) and sexually transmitted infections (STI) were adapted from validated tools. For unanswered survey questions, an analysis was conducted according to the number of responses for each question. Results Eighty respondents consented to the study, mean age 30 ± 9 years. The majority (73%, 58/80) never received HPV vaccination counseling. Of the respondents, 38 out of 80 (48%) never received any HPV vaccine, 10 (13%) were unsure, 6 (8%) received at least one dose, and 26 (33%) completed the vaccination series. The most common reasons for not being vaccinated were lack of knowledge about the vaccine (22%, 8/37), did not think it was necessary (14%, 5/37), not sexually active (11%, 4/37), and did not want to receive the vaccine (11%, 4/37). The majority (61%, 49/80) also did not receive counseling on other STI. Sixty respondents completed the knowledge portion of the survey. HPV knowledge was low; 13 did not think they were a candidate for the HPV vaccine (22%) and 22 were unsure (37%). Less than half of respondents thought the HPV vaccine could help prevent anal cancer (15%), genital warts (30%), and vulvar/vaginal cancer (48%). Twenty-four respondents (40%) thought routine pap smear screening should be performed among MRKH patients, and 20 (33%) were unsure. The majority responded that sexually active persons with MRKH should be routinely tested for STI (87%) and use condoms (82%). Sixty-four percent of 56 respondents were undecided or unhappy with the gynecologic care they have received, and 75% strongly wished their providers knew more about MRKH. Conclusions The majority of individuals with MRKH did not receive adequate HPV, HPV vaccination, and STI counseling. As a result, the majority did not receive the vaccine. These data indicate that there is a lack of appropriate gynecological care for people with MRKH.

Cancer treatments may compromise the fertility of children, adolescents, and young adults, and treatment-related infertility represents an important survivorship issue that should be addressed at diagnosis and in follow-up to ensure optimal decision-making, including consideration of pursuing fertility preservation. Risk of infertility varies substantially with patient and treatment factors. The ability to accurately assess fertility risk for many patients is hampered by limitations of the current literature, including heterogeneity in patient populations, treatments, and outcome measures. In this article, we review and synthesize the available data to estimate fertility risks from modern cancer treatments for both children and adult cancer survivors to enable clinicians to counsel patients about future fertility.

Background Vaginal bleeding in the prepubertal child has several etiologies including structural anomalies, endocrinologic dysfunction, infection, trauma, foreign body, and hematologic disorders. While bleeding in this age group is often benign, it can be distressing to both the child and parent so a comprehensive evaluation is important. Case An 8-year-old premenarcheal girl presented with vaginal bleeding. On ultrasound, she was found to have an intramural hyperechogenic mass in the left uterine fundus. This was biopsied and showed smooth muscle. She was subsequently started on a GnRH agonist to suppress bleeding and has since been followed with serial ultrasounds. We have 6 years of follow up imaging showing a presumed adenomyoma versus fibroid stable in size. Comments We present a novel case of a benign uterine neoplasms presenting as prepubertal bleeding. Pelvic imaging is prudent to exclude structural etiologies as the cause of prepubertal vaginal bleeding.

Peritoneal fluid sampling has been recommended during surgery to resect an ovarian or fallopian tube mass, particularly for its staging relevance in ovarian carcinoma. Guidelines specifically for children are not well established, partly because of incomplete knowledge of the test characteristics in this age group. We sought to determine whether peritoneal fluid cytology sampling aids in diagnosis or staging of pediatric uterine adnexal masses. Children who underwent adnexal mass biopsy or excision from 1993 to 2014 were identified via archival review. Accompanying peritoneal fluid (washings or ascites) was assessed to determine whether it provided information beyond that in the accompanying surgical pathology specimen. Of 656 patients who underwent adnexal mass resection, 350 had concurrent cytologic sampling of peritoneal fluid. Median age at surgery was 14 years. Adnexal mass histologic examination showed tumors with malignant potential in 54 (8%) patients. Malignancy was identified in 3 cytology specimens (2 dysgerminomas and 1 mixed germ cell tumor). By today's guidelines, the malignant cytology specimens would have upstaged 1 of the 3 patients, and her therapy may not have changed. Cytology findings did not refine the histologic diagnosis in any benign or malignant case. Our findings demonstrated a low rate of malignancy (

Study Objective To describe the rates of use and effectiveness of gonadotropin-releasing hormone (GnRH) agonists and other forms of hormonal menstrual suppression in prevention of vaginal bleeding among young women who underwent hematopoietic stem cell transplantation (HCT). Design Retrospective descriptive study. Setting University-based pediatric HCT practice. Participants Fifty-five postmenarchal women who underwent HCT between 2004 and 2011. Interventions Administration of GnRH agonists or other forms of hormonal menstrual suppression. Main Outcome Measures Rates of use of GnRH agonists and other forms of hormonal menstrual suppression, and rates and descriptions of vaginal bleeding. Results Forty-six of the 55 patients had experienced regular or irregular vaginal bleeding before HCT and were considered to be at risk for thrombocytopenia-associated menorrhagia. Forty of the 46 (87%) received hormonal menstrual suppression. Thirty-three patients were treated with a GnRH agonist, 4 with combined hormonal contraceptive pills, 1 with a combined hormonal contraceptive patch, 1 with depot medroxyprogesterone, and 1 with oral norethindrone. Twenty-nine of the 33 patients (88%) who received a GnRH agonist had complete amenorrhea during HCT and 4 of 33 (12%) experienced some degree of vaginal bleeding. Conclusion GnRH agonists appear effective in prevention of vaginal bleeding complications in most postmenarchal women who underwent HCT. Some patients who might benefit do not receive a GnRH agonist and multiple barriers exist in identification and treatment of them.

The purpose of this study was to develop a pre-operative risk assessment tool for childhood and adolescent ovarian malignancy, in order to guide operative management of pediatric ovarian masses.We conducted a retrospective analysis of patients18 years old who underwent ovarian surgery at two quaternary care pediatric centers over 4 years (1/1/13-12/31/16). Probability of malignancy was estimated based on imaging characteristics (simple cyst, heterogeneous, or solid), maximal diameter, and tumor markers (α-fetoprotein, β-human chorionic gonadotropin).Among 188 children with ovarian masses, 11% had malignancies. For simple cysts, there were no malignancies (0/24, 95% CI = 0-17%). Among solid lesions, 44% (15/34, 95% CI = 28-62%) were malignant. Among marker-elevated heterogeneous masses, 40% (2/5, 95% CI = 12-77%) were malignant. Conversely, small (≤10 cm) and large (10 cm) marker-negative heterogeneous lesions had malignancy proportions of 0% (0/39, 95% CI = 0-11%) and 5% (2/40, 95% CI = 1-18%), respectively.Given the malignancy estimates identified from these multi-institutional data, we recommend an attempt at ovarian-sparing resection for simple cysts or tumor marker-negative heterogeneous lesions ≤10 cm. Oophorectomy is recommended for solid masses or heterogeneous lesions with elevated markers. Finally, large (10 cm) heterogeneous masses with non-elevated markers warrant a careful discussion of ovarian-sparing techniques. Complete surgical staging is mandatory regardless of operative procedure.Study of Diagnostic Test.Level I.

While endometriosis is recognized to have a high patient burden for adults, the level of morbidity it causes for adolescents has been understudied, and may be minimized by clinicians. The purpose of this study was to determine whether endometriosis has a significant impact on quality of life (QOL) for adolescents and young adults.Five hundred and sixty-seven participants (360 cases and 207 controls) aged25 years old who are enrolled in the Women's Health Study: From Adolescence to Adulthood longitudinal study were included in this analysis. Participants were enrolled from medical clinics and the communities surrounding Boston, MA. Participants completed an expanded World Endometriosis Research Foundation Endometriosis Phenome and Biobanking Harmonization Project standard clinical questionnaire that included the Short Form-36 (SF-36), a widely used measure of health-related QOL. We calculated SF-36 physical component summary and mental component summary scores, as well as eight subscale scores. On the SF-36, lower scores reflect greater impairment.Adolescents with endometriosis had significantly lower physical component summary (mean [standard deviation]: 43.4 [11.1] vs. 53.8 [7.6], p.0001) and mental component summary (43.3 [12.3] vs. 46.3 [10.8], p = .008) scores compared to controls, as well as lower scores on all eight subscales. More cases than controls reported mental health diagnoses, pain medication use, and avoidance of exercise during menstruation. Among cases, earlier age at menarche, more severe pelvic pain, and longer duration of diagnostic delay were associated with poorer QOL.Endometriosis is associated with significantly worse reports of QOL for adolescents and young women with endometriosis compared to unaffected peers. Earlier menarche was associated with poorer physical health-related QOL among cases, whereas severe pelvic pain was associated with both poorer physical and mental health-related QOL among cases. Impairment in QOL is not limited to adults with endometriosis, but affects younger patients as well.

Study Objective To explore the potential occurrence of long-term side effects and tolerability of gonadotropin-releasing hormone agonist (GnRHa) plus 2 different add-back regimens in adolescent patients with endometriosis. Design Follow-up questionnaire sent in 2016 to patients who participated in a drug trial between 2008 and 2012. Setting Tertiary care center in Boston, Massachusetts. Participants Female adolescents with surgically confirmed endometriosis (n = 51) who enrolled in a GnRHa plus add-back trial as adolescents. Interventions Leuprolide depot 11.25 mg intramuscular injection every 3 months, plus oral norethindrone acetate 5 mg daily or oral norethindrone acetate 5 mg daily and oral conjugated equine estrogens 0.625 mg daily. Main Outcome Measures Side effects during and after treatment, irreversible side effects, changes in pain, overall satisfaction. Results The response rate was 61% (25 of 41; 10 subjects could not be located). Almost all (24 of 25) reported side effects during treatment; 80% (16 of 21) reported side effects lasting longer than 6 months after stopping treatment. Almost half (9 of 20) reported side effects they considered irreversible, including memory loss, insomnia, and hot flashes. Despite side effects, participants rated GnRHa plus add-back as the most effective hormonal medication for treating endometriosis pain; two-thirds (16 of 25) would recommend it to others. More participants who received a modified 2-drug add-back regimen vs standard 1-drug add-back would recommend GnRHa and believed it was the most effective hormonal medication. Conclusion Subjects believed that GnRHa used with add-back was effective and would recommend it to others, despite significant side effects. Those who received 2-drug add-back reported more success than those who received standard add-back. A subset of patients reported side effects they consider to be irreversible.

The following review seeks to summarise the current data regarding reproductive outcomes associated with congenital uterine anomalies. Such malformations originate from adverse embryologic events ranging from agenesis to lateral and vertical fusion defects. Associated renal anomalies are common both for the symmetric and asymmetric malformations. While fertility is minimally impacted upon by müllerian anomalies in most cases, such malformations have historically been associated with poor obstetric outcomes such as recurrent miscarriage, second trimester loss, preterm delivery, malpresentation and intrauterine foetal demise (IUFD). The following review delineates the existing literature regarding such outcomes and indicates therapies, where applicable, to optimise the care of such patients.

Background Modeling early endometrial differentiation is a crucial step towards understanding the divergent pathways between normal and ectopic endometrial development as seen in endometriosis. Methods To investigate these pathways, mouse embryonic stem cells (mESCs) and embryoid bodies (EBs) were differentiated in standard EB medium (EBM). Immunofluorescence (IF) staining and reverse-transcription polymerase chain reaction (RT-PCR) were used to detect expression of human endometrial cell markers on differentiating cells, which were sorted into distinct populations using fluorescence-activated cell sorting (FACS). Results A subpopulation (50%) of early differentiating mESCs expressed both glandular (CD9) and stromal (CD13) markers of human endometrium, suggestive of a novel endometrial precursor cell population. We further isolated a small population of endometrial mesenchymal stem cells, CD45−/CD146+/PDGFR-β+, from differentiating EBs, representing 0.7% of total cells. Finally, quantitative PCR demonstrated significantly amplified expression of transcription factors Hoxa10 and Foxa2 in CD13+ EBs isolated by FACS (p = 0.03). Conclusions These findings demonstrate that mESCs have the capacity to express human endometrial cell markers and demonstrate potential differentiation pathways of endometrial precursor and mesenchymal stem cells, providing an in vitro system to model early endometrial tissue development. This model represents a key step in elucidating the mechanisms of ectopic endometrial tissue growth. Such a system could enable the development of strategies to prevent endometriosis and identify approaches for non-invasive monitoring of disease progression. Electronic supplementary material The online version of this article (doi:10.1186/s12958-017-0273-2) contains supplementary material, which is available to authorized users.

Background Endometriosis—the ectopic implantation of endometrial-like tissue—affects 10% of adolescent females and adults. Bladder involvement, causing dysuria and hematuria, occurs in a very small number of endometriosis patients. Case The patient presented at age 12 years with dysuria and pelvic pain. Laparoscopy revealed stage I endometriosis. Postoperatively, she reported persistent dysuria and passage of tissue in her urine. Cystoscopy showed diffuse erythema; urine cytology revealed glandular and spindle cells suggestive of endometriosis. She was transitioned from oral contraceptives to an intranasal gonadotropin-releasing hormone agonist, with symptom resolution. Summary and Conclusion Intravesicular endometriosis coinciding with stage I disease supports a mechanism of endometriosis dissemination other than direct bladder infiltration. Patients with endometriosis who complain of urinary symptoms warrant assessment, because intravesicular bladder involvement cannot be excluded using pelviscopy.