Large Pelvic Mass in a Female Adolescent: Atypical Presentation and Successful Treatment of Extraskeletal Ewing Sarcoma.

Extraskeletal Ewing sarcoma is a rare soft tissue tumor primarily affecting pediatric patients. The treatment is currently based on a multidisciplinary approach which allows, in cases of localized disease, good survival rates. We report the case of a 15-year-old female patient with a rapidly growing suspected pelvic mass misdiagnosed following the preliminary radiological exams, which assessed the findings as a mass of ovarian origin. The girl underwent surgery and, thanks to histopathological, immunohistochemical and real-time polymerase chain reaction (RT-PCR) examinations, it was possible to make the right diagnosis and to administer the best treatment in terms of surgery, chemotherapy and radiotherapy, obtaining a long disease-free interval and no recurrence to date.

Fibrin Glue Repair of a Traumatic Rectovaginal Fistula in a Pediatric Patient.

BACKGROUND: Rectovaginal fistulas are often associated with obstetric trauma and present with leakage of stool or flatus from the vagina. They are often repaired via fistulaectomy, but sometimes more complex repairs are needed. There are limited data regarding success using fibrin glue to close the tract. CASE: A developmentally delayed pediatric patient presented with right hip pain. Imaging studies identified a hairpin penetrating the rectovaginal space. The hairpin was removed during an exam under anesthesia, and the subsequent rectovaginal fistula was closed with fibrin glue. Closure of the tract has persisted for more than 1 year without need for further intervention. SUMMARY AND CONCLUSION: Fibrin glue may be a minimally invasive and safe approach for rectovaginal fistulas in the pediatric patient.

Vaginal Metastases of Wilms' Tumor in a Pediatric Patient: A Rare Case.

BACKGROUND: Wilms' tumor is the second most common pediatric abdominal cancer; however, it rarely involves the female reproductive tract. There are few cases reported in the literature describing uterine, ovarian, cervical, and vaginal involvement. CASE: We report the case of a 7-year-old girl presenting with a large renal mass with retroperitoneal nodal and lung metastases; she was diagnosed with stage 4 favorable histology Wilms' tumor. She was treated with surgery, chemotherapy, and radiation. She presented with vaginal bleeding 10 months after completing treatment; biopsy of a vaginal mass confirmed recurrence, and this was sent for molecular profiling, which did not identify an inherited cancer predisposition or targetable mutation. She was again treated with chemotherapy; examination redemonstrated a small vaginal mass, but re-biopsy of the lesion was negative for malignancy. Due to high risk of local relapse, ongoing chemotherapy and pelvic radiation ensued. End-of-treatment imaging and vaginoscopy showed no residual disease. SUMMARY AND CONCLUSION: Vaginal metastases of Wilms' tumor are very rare; this is the second reported case in the literature. Pediatric clinicians should have a strong suspicion for vaginal metastases in cancer patients presenting with vaginal bleeding, especially when their pubertal development does not suggest that bleeding would be secondary to menarche. Long-term gynecologic care for these patients is paramount to reduce morbidity from chemotherapy and pelvic radiation. Fertility preservation counselling should be made early, through referral to a specialist.

Interest in Continued Use After Participation in a Study of Over-the-Counter Progestin-Only Pills in the United States.

Objective: To assess interest in continued use of over-the-counter progestin-only pills among individuals who used them in a trial. Methods: From January 2020 to September 2021, we conducted a cross-sectional online survey with individuals who completed participation in a trial evaluating over-the-counter use of norgestrel 0.075 mg tablets in the United States. We calculated descriptive statistics, Pearson's chi-square and Fisher's exact tests, and logistic regression models to assess likelihood of future over-the-counter progestin-only pill use, reasons for interest/noninterest, situations for over-the-counter progestin-only pill use, willingness to pay for an over-the-counter progestin-only pill, likelihood of future preventive health screenings, prior difficulties getting prescription contraception, and background characteristics. Results: Among 550 adult and 115 adolescent participants (75% response rate), 83% reported likelihood of future over-the-counter progestin-only pill use. Hispanic/Latinx and Black participants and adults with public insurance, prior pregnancies, and some college reported higher likelihood of future use compared with their counterparts. Among likely users, 90% were interested in long-term use and 79 % ≥ 25 years of age reported they would get future preventive screenings; participants would pay up to $20/month on average. Primary reasons for interest included convenience (81%), ease of access (80%), and saving time (77%) and money (64%). The primary reason for noninterest was bleeding associated with progestin-only pill use (52%). Conclusion: There was high interest in continuing to use over-the-counter progestin-only pills among individuals who had used them in a study. These findings highlight the real-world acceptability of taking a progestin-only pill without a prescription, and contribute to evidence supporting over-the-counter access.

The Use of Telemedicine in Pediatric and Adolescent Gynecology.

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.

Vulvar Aphthous Ulcer in an Adolescent After Pfizer-BioNTech (BNT162b2) COVID-19 Vaccination.

BACKGROUND: Vulvar aphthous ulcers are a rare type of genital lesion most common in non-sexually active adolescents. Vulvar aphthous ulcers are typically associated with viral infections. To date, there have been several cases reported in patients infected with COVID-19. Vulvar aphthous ulcers following vaccination have not been previously reported in the literature. CASE: We present the case of a 16-year-old adolescent who developed vulvar aphthous ulceration following Pfizer-BioNTech (BNT162b2) vaccination. SUMMARY AND CONCLUSION: Through an extensive literature search, we found no previous reports of vulvar aphthous ulcer following vaccination. Our case highlights a potential novel side effect of Pfizer-BioNTech COVID-19 vaccination and a new etiology for vulvar aphthous ulcers. This case suggests that vulvar aphthous ulcers might be associated with COVID-19 vaccination through a yet undetermined mechanism that requires further investigation.

The Current State of Pediatric and Adolescent Gynecology Residency Training in Canada: A Needs Assessment From Program Directors.

OBJECTIVE: To perform a needs assessment to determine whether a mandatory Pediatric and Adolescent Gynecology (PAG) training experience in each Obstetrics and Gynecology (ObGyn) residency program in Canada is required and feasible. MATERIALS AND METHODS: This was a comparative descriptive design in which the 16 ObGyn Residency Program Directors (PD) in Canada were asked to undergo a 20-minute structured phone interview. These interviews were recorded, and explored how PAG and Reproductive Endocrinology (RE) objectives are met in each program, the PD's awareness of PAG opportunities in North America, and the feasibility of a mandatory training experience. This project is Research Ethics Board (REB) approved. RESULTS: Of 16 PDs, 12 gave consent and completed the phone interview. There is at least 1 PAG-trained ObGyn per institution, with a wide variety of clinical and academic experiences in PAG for residents between residency programs. There is much overlap among PAG and RE. All PDs interviewed believe that PAG training is important and should be mandatory; however, many feel that they lack the resources to implement a PAG mandatory training experience and that many barriers to such a curriculum exist. CONCLUSION: PAG training experiences should be mandatory in all ObGyn training programs, according to participating PDs. PAG providers were identified at all the participating residency programs, and efforts should be made to support these providers in delivering the educational PAG content to ObGyn residents so the residents can become competent in the care of young women and children. PDs should be provided with the available PAG resources and resident elective opportunities.

The Current State of Pediatric and Adolescent Gynecology Residency Training in Canada: Summary of Educational Resources to Enhance the Achievement of Pediatric and Adolescent Gynecology Objectives.

A previous study determined that Obstetrics and Gynecology (ObGyn) residency Program Directors (PD) endorse mandatory Pediatric and Adolescent Gynecology (PAG) training to achieve national PAG objectives. Although barriers exist that limit the achievement of PAG objectives, this paper presents existing PAG tools and curricula. These include the North American Society for Pediatric and Adolescent Gynecology (NASPAG) short and long curricula, a wide variety of PAG electives available across Canada, more than 25 online clinical cases, and a simulation curriculum. This paper details these resources and provides a 4-week PAG schedule to accommodate ObGyn residency training programs.

Gynecological Problems in Newborns and Infants.

Pediatric-adolescent or developmental gynecology has been separated from general gynecology because of the unique issues that affect the development and anatomy of growing girls and young women. It deals with patients from the neonatal period until maturity. There are not many gynecological problems that can be diagnosed in newborns; however, some are typical of the neonatal period. This paper aims to discuss the most frequent gynecological issues in the neonatal period.

Factors Associated with Torsion in Pediatric Patients with Ovarian Masses.

BACKGROUND: Management of ovarian torsion has evolved toward ovarian preservation regardless of ovarian appearance during surgery. However, patients with torsion and an ovarian neoplasm undergo a disproportionately high rate of oophorectomy. Our objectives were to identify factors associated with ovarian torsion among females with an ovarian mass and to determine if torsion is associated with malignancy. METHODS: A retrospective review of females aged 2-21 y who underwent an operation for an ovarian cyst or neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariate logistic regression was used to assess factors associated with torsion. Imaging data were assessed for sensitivity, specificity, and predictive value in identifying ovarian torsion. RESULTS: Of 814 girls with an ovarian neoplasm, 180 (22%) had torsion. In risk-adjusted analyses, patients with a younger age, mass size >5 cm, abdominal pain, and vomiting had an increased likelihood of torsion (P < 0.01 for all). Patients with a mass >5 cm had two times the odds of torsion (odds ratio: 2.1; confidence interval: 1.2, 3.6). Imaging was not reliable at identifying torsion (sensitivity 34%, positive predictive value 49%) or excluding torsion (specificity 72%, negative predictive value 87%). The rates of malignancy were lower in those with an ovarian mass and torsion than those without torsion (10% versus 17%, P = 0.01). Among the 180 girls with torsion and a mass, 48% underwent oophorectomy of which 14% (n = 12) had a malignancy. CONCLUSIONS: In females with an ovarian neoplasm, torsion is not associated with an increased risk of malignancy and ovarian preservation should be considered.

Neuroendocrine Tumors: Clinical, Histological and Immunohistochemical Perspectives and Case Report-Mature Teratoma in a 16-Year-Old Girl.

A mature teratoma is a germinal neoplasm that differentiates from embryonic multipotent cells into three germ layers. There may also be glandular tissue. The literature describes a total of 658 cases of ovarian neuroendocrine neoplasms, mainly in women over 40 years of age. The authors, together with a systemic review, present a case of a 16-year-old girl diagnosed with and treated for a neuroendocrine tumor. Case description: A 16-year-old girl visited the Paediatric Gynaecology Outpatient Clinic because of abdominal pains that intensified during menstruation. Standard painkillers and diastolic drugs were ineffective. An ultrasound examination revealed a large tumor with a heterogeneous structure in her right ovary. A sparing operation was carried out. During laparotomy, the lesion was enucleated, leaving healthy tissue. Histopathological examination revealed the typical features of teratoma, as well as the coexistence of a G1 neuroendocrine tumor. Immunohistochemical examination (IHC) showed the presence of markers characteristic for this type of tumor. The patient requires constant monitoring in the Endocrinology and Oncological Gynaecology Clinic. Conclusion: Tissue of neuroendocrine neoplasm within a teratoma is rare in this age group of patients; thus, there are currently no standards for long-term follow-up. This case adds to the body of evidence and demonstrates a possible good prognosis with non-aggressive behavior in G1 neuroendocrine tumors and teratomas in young patients.

Laparoscopic Management of an Ovarian Dermoid Cyst Containing a Mandibular Structure with Teeth: A Novel Surgical Approach.

BACKGROUND: The presence of ectodermal-derived tissue, including teeth, in an ovarian dermoid cyst is a common occurrence. The presence of a fully formed mandibular structure with teeth, however, is rare, and there are few case reports in the literature that discuss its surgical management. CASE: We report a case of an adolescent girl found to have a mandibular structure with teeth in her dermoid cyst at the time of her laparoscopic ovarian cystectomy and a novel surgical approach in the extraction of the cyst contents from the abdominal cavity. SUMMARY AND CONCLUSION: The use of an arthroscopic surgical blade to morcellate the mandibular-like bone allowed for completion of the procedure laparoscopically, without laparotomy for specimen extraction, allowing the patient to benefit from the advantages of minimally invasive surgery.

A Rare Case of Vulvar Superficial Angiomyxoma in a Pediatric Patient.

BACKGROUND: Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor. Originally described as a component of Carney complex, it is now recognized as a sporadic condition. CASE: A 7-year-old girl was referred for management of a 2.5-cm mass arising from the right labia majora. Key pathologic features included lobules of spindle-shaped cells in a myxoid matrix and prominent neutrophilic infiltrate. The cells were positive for CD34 and negative for desmin, progesterone receptor, and estrogen receptor staining. SUMMARY AND CONCLUSION: This case is that of the youngest described patient with vulvar SAM. Patients should be carefully examined for manifestations of Carney complex to avoid potentially life-threatening complications. It is critical to distinguish SAM from aggressive angiomyxoma. The patient was referred to genetics and will be followed for local recurrence.

The Prevalence of Children and Adolescents at Risk for Avoidant Restrictive Food Intake Disorder in a Pediatric and Adolescent Gynecology Clinic.

STUDY OBJECTIVE: The purpose of this study was to determine the prevalence of child and adolescent females at risk for Avoidant Restrictive Food Intake Disorder (ARFID) in a tertiary care pediatric and adolescent gynecology (PAG) clinic. DESIGN: Cross-sectional study design. SETTING: Tertiary care PAG clinic at the Hospital for Sick Children in Toronto, Ontario, Canada. PARTICIPANTS: Females between 8 and 18 years of age presenting to the tertiary care PAG clinic. INTERVENTION: Between October 2017 and April 2019, eligible patients completed a 3-part, self-administered questionnaire that included demographic and anthropometric information, reason(s) for referral, medical history, menstrual history and function, and the Eating Disorders in Youth-Questionnaire (EDY-Q). MAIN OUTCOME MEASURES: The main outcome measure was the prevalence of child and adolescent females who were identified to be at risk for ARFID in a tertiary care PAG clinic. RESULTS: Seven (3.7%) of 190 patients were identified to be at risk for ARFID based on the EDY-Q. All patients at risk for ARFID had a significantly lower body mass index (17.4 ± 1.6 vs 24.4 ± 6.7, P < .001) than patients not at risk for ARFID. CONCLUSIONS: This study demonstrated that 3.7% of patients seeking treatment in a tertiary care PAG clinic were identified to be at risk for ARFID. Clinicians in tertiary care PAG clinics can play a pivotal role in the identification and referral of children and adolescents at risk for ARFID. Referral to the patients' primary care physician or to an eating disorder program is important so as not to delay the diagnosis and treatment.

Evaluation of the usefulness of laparoscopy in the diagnosis and treatment of adnexal pathologies in the pediatric population.

INTRODUCTION: The current trend in adnexal surgery in children is to minimize the invasiveness of diagnostic and therapeutic procedures, reduce the number of complications and reduce the risk of infertility. AIM: Evaluation of the usefulness and effectiveness of laparoscopy in diagnostics and treatment of pathological lesions of adnexa in the pediatric population and evaluation of the correlation of imaging with the intraoperative diagnosis during laparoscopy. MATERIAL AND METHODS: The study included 89 patients aged 0-18 years (mean: 12.62) who underwent an emergency or elective laparoscopic procedure due to the suspicion of adnexa pathology in the imaging tests or in which intraoperative pathology of the adnexa was found without previous suspicion of these changes in the imaging tests. Patients were divided into 2 groups according to the procedure (emergency or elective) and into 4 age groups. RESULTS: The most frequent postoperative diagnosis was an adnexal cyst and ovarian tumor. The ratio of malignant to benign lesions was 0.087. In 32.58% of patients appendix pathology was found. Coexistence of adnexa and appendix pathology was statistically significantly more frequent in patients undergoing emergency surgery (p = 0.013). There was no correlation between the size of the tumor or ovarian cyst and the occurrence of adnexal torsion, and no correlation between the size of the tumor and the degree of torsion of the adnexa. Three conversions were performed. The average operation time was 63.7 min. CONCLUSIONS: Laparoscopic diagnosis and treatment of adnexal pathology seems to be safe due to the low percentage of perioperative complications. It shows high sensitivity in recognizing adnexal pathologies and other defects.

Is there a need for a formal gynecology curriculum in a pediatric surgery training program? A needs assessment.

PURPOSE: Fellows in Pediatric Surgery need to learn to manage a variety of gynecologic conditions. We completed a needs assessment of Pediatric Surgery training programs to inform development of a standardized gynecology curriculum. METHODS: A survey was sent to Program Directors of Canadian Pediatric Surgery training programs with 27 questions that focused on the fellowship program, surgical practice, and trainee exposure to pediatric gynecology, and how the envision a standardized gynecology curriculum. RESULTS: Six of eight Program Directors responded. All respondents had treated ovarian-related conditions and genital injuries in the past 5 years, and most felt trainees received adequate training in managing these conditions. Most respondents felt trainees had minimal or inadequate training in imperforate hymens, Müllerian anomalies, vulvar abscesses, vaginal foreign bodies, and labial adhesions. Program Directors currently allot an average of 3.5 h to delivering the gynecology objectives. All Program Directors expressed interest in a formal gynecology curriculum delivered through some combination of case-based teaching and/or simulation. CONCLUSION: There is a need for a standardized gynecology curriculum for Pediatric Surgery trainees. Most Pediatric Surgeons will manage gynecological conditions as part of their practice and current Program Directors feel that training is inadequate for a number of gynecological conditions. TYPE OF STUDY: Observational Cross-Sectional Study. LEVEL OF EVIDENCE: Level IV.

A Case of a Prolapsed Fibroid in a 12-Year-Old Girl.

BACKGROUND: Uterine fibroids are a common cause of abnormal uterine bleeding in adult women, but are extremely rare in the adolescent years. CASE: A 12-year-old nulliparous girl presented with abnormal uterine bleeding and was found to have a prolapsing submucosal fibroid. The fibroid and her symptoms were successfully removed using an Endosnare. SUMMARY AND CONCLUSION: The most common cause of abnormal uterine bleeding in adolescent bleeding is due to anovulation. Though rare, fibroids should remain on the differential as the cause of abnormal uterine bleeding in the adolescent population, particularly if symptoms persist despite initial therapy. Fibroids can be managed successfully with minimally invasive techniques.

Hormonal cytology.

The spectrum of hormonal disorders of children and adolescents is broad. It reflects some current features of the advancing lifestyle and health of the population: thelarche praecox and accelerated development in young girls - early menarche, dysfunctional juvenile metrorrhagia, eating disorders - anorexia-bulimia, obesity increase, extreme sports load, autoimmune disorders, central and peripheral endocrine disorders, and others. Many of these conditions can be favorably influenced by professional lifestyle adjustments and fine hormonal medication with simultaneous monitoring of the adjusting cytogram. Our experience is based on twelve years of close collaboration between clinical gynecologists and cytopathologists. We investigated more than 2500 patients, many of them repeatedly. Upon detection of the primary disorder, the results of treatment are monitored by repeated cytological examinations, which in most patients progressively normalize the hormone status and restore reproductive health. The article provides an overview of basic physiological and most common pathological images of hormonal cytology with illustrative case reports. It also describes the usual formulation of a cytological report. Cytological examinations of the fornix vaginal epithelium are noninvasive, economically feasible and easily repeatable, which still have a place in childhood gynecological practice. They contribute to reproductive health by identifying the need and type of hormonal treatment, monitoring the treatment of menstrual cycle disorders and eliminating or identifying the need for a more detailed examination of steroid metabolism.

Case Report of the Successful Use of Mirena Levonorgestrel Intrauterine System in a Patient with Didelphys Uterus and Obstructed Hemivagina.

BACKGROUND: The Mirena levonorgestrel intrauterine system (IUS; Bayer HealthCare Pharmaceuticals) is frequently used for menstrual suppression in adolescents with special needs. However, the presence of a uterine anomaly is generally considered a contraindication to IUS insertion, thereby excluding a potentially highly effective option for this patient population. CASE: A Mirena IUS was used in a medically and surgically complex special needs 14-year-old adolescent with a didelphys uterus and obstructed hemivagina. With the IUS inserted into the unobstructed uterus, she achieved amenorrhea and significant reduction in pain, with interval reduction in hematometra in the contralateral obstructed uterus. SUMMARY AND CONCLUSION: We report the successful use of the Mirena IUS in a patient with a Müllerian anomaly, supporting the use of this device in carefully selected patients.