Medical Management of Endometriosis in Adolescent and Young Adult Women: A Review of 91 Cases of Biopsy-Confirmed Endometriosis.

OBJECTIVE: To characterize contemporary trends in the hormonal management of endometriosis in adolescent and young adult patients with biopsy-proven endometriosis. METHODS: Retrospective chart review of women aged 14-25 who underwent laparoscopy for pelvic pain with biopsy-proven endometriosis between January 2011 and September 2020 at an academic tertiary hospital system. Final sample included 91 patients with biopsy-confirmed endometriosis. RESULTS: Combined oral contraceptives (COCs) were the most common initial treatment (64% of patients). Progestin-only formulations (low- and high-dose norethindrone acetate) were offered to younger patients (age 15.9 ± 2.7 years) than those offered COCs (19.9 ± 3.3 years) and levonorgestrel intrauterine devices (LNG-IUDs) (21.9 ± 1.7 years). Current treatments varied widely and included COCs (32%), LNG-IUDs (18%), oral progestins (low- and high-dose norethindrone, medroxyprogesterone) (14%), elagolix (9%), and leuprolide (8%). Oral adjuncts to LNG-IUD were common: usually low- or high-dose norethindrone (37% of patients with an LNG-IUD), but also included progesterone, COCs, and elagolix. CONCLUSION: Oral progestins, LNG-IUDs, and COCs were the mainstay of initial treatment. Subsequent treatments varied widely and included COCs, LNG-IUDs, oral progestins, elagolix, leuprolide, and combinations of these agents. We observed that most young women switched between therapies, suggesting that a personalized approach is often used to determine treatment plans among the wide range of options currently available. This study helps define the spectrum of treatment regimens for endometriosis in adolescent females.

Ovarian Leydig Cell Tumor Associated with Recurrent Torsion and Virilization in an Adolescent Patient.

Ovarian tumors are rare in children; however, their incidence increases with age. Of these ovarian tumors, Leydig cell tumors are some of the rarest, accounting for less than 0.1% of all ovarian tumors across all ages. Leydig cell tumors predominantly occur in postmenopausal women and are characterized by nodular proliferation of Leydig cells in the ovarian hilum with intracytoplasmic Reinke crystals. These tumors secrete androgens, which can disrupt ovarian function, clinically presenting with abnormal uterine bleeding and virilization. Although they are generally benign, current recommendations are for treatment with a unilateral salpingo-oophorectomy. In adolescents, hyperandrogenism is most commonly caused by polycystic ovarian syndrome (PCOS); however, the differential for hyperandrogenism is broad. We present a case of a 15-year-old girl with a history of primary amenorrhea who presented with a Leydig cell tumor associated with recurrent ovarian torsion and virilization. This case reviews the challenges with diagnosis, management, and future implications of a rare androgen-secreting tumor in young patients.

Diagnostic and surgical approaches to congenital uterine anomalies for the minimally invasive gynecologic surgeon.

PURPOSE OF REVIEW: Congenital uterine anomalies (CUAs) impact the physical and psychosocial wellbeing of affected patients. Managing these conditions depends on the clinical scenario, and in some cases, can involve the use of minimally invasive surgical techniques. The purpose of this review is to provide an update of the diagnosis, perioperative considerations, and treatment of CUAs. RECENT FINDINGS: The American Society for Reproductive Medicine (ASRM) updated the guidelines for classification of CUAs to provide practitioners with a standardized classification system and have created an interactive tool designed for provider use. SUMMARY: Gynecologic surgeons are likely to encounter CUAs during their career. This review provides updated guidance for the workup and treatment of CUAs.

Vulvovaginal Complaints as the First Manifestation of Crohn's Disease in Children: Case Report.

BACKGROUND: Crohn's disease, a chronic gastrointestinal inflammatory condition, can involve gynecological structures. Rectovaginal or rectovestibular involvement may be the first sign in the pediatric population and may result in delayed diagnosis and treatment. CASE: A 9-year-old premenarchal female with chronic constipation and poor growth presented to the pediatric gynecologist for evaluation of persistent vulvovaginal discharge and vulvar irritation. Examination under anesthesia revealed a rectolabial fistula; colonoscopy was diagnostic of Crohn's disease. Immunotherapy resulted in improvement of symptoms and anatomical changes. SUMMARY AND CONCLUSION: In cases of persistent vulvar complaints in a child without clear diagnosis, a high index of suspicion for a non-gynecologic diagnosis is necessary. Collaboration between pediatric gynecologists, gastroenterologists, and surgeons can result in prompt diagnosis and treatment of genital Crohn's disease.

Evaluation of the Association of Endometrial Thickness, Insulin Resistance, and Menstrual Patterns in Adolescent Females with Polycystic Ovarian Syndrome.

STUDY OBJECTIVE: To evaluate endometrial stripe (EMS) thickness and its association with menstrual pattern and insulin resistance in adolescent females with or at risk for polycystic ovarian syndrome (PCOS) METHODS: This was a retrospective case-control study of adolescent females ranging between 12 and 21 years old evaluated in the Adolescent Gynecology & Endocrinology Clinic (AGEC) at a tertiary children's hospital between 2017 and 2021. Transabdominal pelvic ultrasound (US) was obtained for evaluation of PCOS or acute pelvic pain. Unadjusted comparisons were performed between imaging measurements in the PCOS and control (girls without PCOS with acute pelvic pain) groups, as well as analysis of the PCOS group adjusted for age, body mass index, race, and biochemical values. This study was approved by the Institutional Review Board. RESULTS: In our study, 54 subjects met the inclusion criteria for the PCOS group and 42 for the control group. EMS thickness was thinner in the PCOS group than in the control (0.55 ± 0.31 cm vs 0.70 ± 0.23 cm; P < .001). There was no difference in EMS thickness in the PCOS group when stratified by intermenstrual interval, insulin resistance, and other biochemical factors. CONCLUSION: Our findings support recommendations by the 2018 International Guidelines to avoid use of US for the establishment of PCOS diagnosis in adolescents. These results highlight the unique pathophysiology of adolescent PCOS in contrast to PCOS in adult women. Further large-scale prospective studies are needed to understand the role of EMS thickness as a prognostic marker in adolescent PCOS.

Establishing an Association between Polycystic Ovarian Syndrome and Pilonidal Disease in Adolescent Females.

STUDY OBJECTIVE: We sought to determine whether pilonidal disease (PD) is associated with polycystic ovarian syndrome (PCOS) in adolescent females. DESIGN: Retrospective cohort study SETTING: Urban tertiary children's hospital PARTICIPANTS: All girls aged 12 to 21 who received a diagnosis of PD and/or PCOS from 2012 to 2019 INTERVENTIONS: Treatment for PCOS and PD MAIN OUTCOME MEASURES: The variables analyzed included age, race/ethnicity, body mass index, age at menarche, tobacco use, payer status, treatment of PCOS and PD, and serum markers of hyperandrogenism and metabolic syndrome. RESULTS: During the study period, 100,043 patients presented to an urban tertiary medical center. Of these patients, 966 were diagnosed with PD, and 219 were diagnosed with both PD and PCOS. Compared with patients with only PD, patients with both diagnoses had a higher body mass index (31.4 vs 27.4 kg/m2; P < .01) and were older (18.76 vs 18.30 years; P = .003). The prevalence ratio for patients with PD having PCOS per the original Rotterdam criteria was 26.1 (CI, 22.0-31.0) and 28.7 (CI, 24.3-33.9) for having PCOS per the modified Rotterdam criteria. Patients with both diagnoses were less likely to receive intervention for PD (OR = 0.22; CI, 0.13-0.37; P < .001). CONCLUSIONS: Adolescent females diagnosed with PD are likely to demonstrate features of PCOS. PCOS treatment could positively alter the disease course of PD. As such, surgical providers should consider referring adolescent females with PD to endocrinologists, gynecologists, and adolescent medicine specialists for evaluation of PCOS.

Laparoscopic ovarian tissue harvesting for cryopreservation from a child with galactosemia.

OBJECTIVE: To describe an approach to fertility preservation by a multidisciplinary team of reproductive endocrinology and infertility, pediatric gynecology and surgery, and genetics experts via ovarian tissue harvesting and cryopreservation for a toddler with galactosemia. Galactosemia is associated with progressive primary ovarian insufficiency (POI) and early intervention with ovarian tissue cryopreservation may help preserve fertility. DESIGN: Video description of a tissue harvesting and cryopreservation technique. SETTING: Academic institution. PATIENT(S): 16-month-old female with classic galactosemia. INTERVENTION(S): At 6 months of age, despite good metabolic control, the infant's antimüllerian hormone (AMH) level was <0.015 ng/ml; luteinizing hormone level was 3.1 mIU/ml; and follicle stimulating hormone level was 30.2 mIU/ml. She was referred by her geneticist to the reproductive endocrinology and infertility specialist for fertility preservation. The AMH levels and pelvic magnetic resonance imaging findings of the patient were monitored over the next 9 months. Although the magnetic resonance imaging exam showed the presence of a dominant follicle in the right ovary and multiple small antral follicles in both ovaries at the age of 8 months, her laboratory assessment at the age of 14 months suggested impending POI (estradiol level <11.80 pg/mL; LH, 3.3 mIU/ml; follicle stimulating hormone, 35.97 mIU/ml; AMH, 0.03 ng/mL). At 16 months of age, given the low AMH levels, right ovary was laparoscopically harvested, so that a sufficient reserve of primordial follicles may be cryopreserved for fertility preservation. We dissected the mesosalpinx initially to separate the ovary from the tube in a manner that minimized the effects of cauterization on the ovary and preserved the fallopian tube. MAIN OUTCOME MEASURE(S): Successful harvesting and cryopreservation of the ovarian tissue containing primordial follicles. RESULT(S): The right ovary, which measured 20 × 3 × 3mm, was bisected under a stereomicroscope along the hilum, trimmed to the cortical thickness of 1 mm and sliced into eight 4 × 4-mm pieces. These were then frozen with an established slow freezing protocol. The child was discharged the same day and had an uneventful postoperative course. A subsequent histological examination showed presence of primordial follicles, albeit at a reduced density for her age. CONCLUSION(S): Ovarian tissue cryopreservation is feasible in very young female children with rare genetic disorders associated with POI. We illustrated the unique aspects of performing these procedures in very young children.

Minimally Invasive Ovarian-Preserving Approach for the Management of a Sclerosing Stromal Tumor in an Adolescent: A Case Report.

BACKGROUND: Sclerosing stromal tumors (SSTs) are rare benign ovarian tumors that occur in adolescents and young adults. They are often treated with unilateral salpingo-oopherectomy due to concern for malignancy. CASE: A 13-year-old postpubertal female presented with sharp, constant abdominal pain with physical exam concerning for a lower abdominal mass. An ultrasound revealed a 9.7-cm solid, heterogenous left ovarian mass. The abdomen and pelvis CT confirmed the findings and showed a predominantly cystic mass arising from the left adnexa. During surgery, a smooth and distinct mass arising from the left ovary was encountered without abnormal findings in surrounding structures. A cystectomy was performed, and intraoperative findings showed no evidence of malignancy. Based on the lab, imaging, and surgical findings, staging and salpingo-oopherectomy were not pursued. SUMMARY AND CONCLUSION: Given the benign nature of SSTs, it is important to entertain the diagnosis in adolescents presenting with clinically congruent ovarian masses. The minimally invasive approach allows for sparing of the ovary in the adolescent population.

Progression of Cystadenoma to Mucinous Borderline Ovarian Tumor in Young Females: Case Series and Literature Review.

STUDY OBJECTIVE: To study the progression of benign ovarian lesions to mucinous borderline ovarian tumors (mBOTs); analyze the clinicopathologic features, diagnosis, and management of mBOTs in pediatric and adolescent girls; and provide a review of the literature on mBOTs in this population. DESIGN: Retrospective chart review of female adolescents younger than 18 years diagnosed with mBOTs between July 2017 and February 2021. SETTING: Yale New Haven Hospital, New Haven, Connecticut; and Yale New Haven Health Bridgeport Hospital, Bridgeport, Connecticut. PARTICIPANTS: Three female patients diagnosed with mBOTs between ages 12 and 17 years. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Clinical presentation, preoperative characteristics, surgical technique, histology, tumor stage, treatment, progression, outcome, and rate of recurrence. RESULTS: Three adolescent patients were identified to have mBOTs. All three patients presented with a chief complaint of abdominal pain. One of the 3 patients was premenarchal at presentation. Two of the 3 patients were initially diagnosed with a mucinous cystadenoma and had recurrences of an ovarian cyst in the same ovary within 5 and 17 months, respectively. Pathology of the recurrent cyst was consistent with mBOT. Two of the 3 patients initially underwent cystectomy, and all ultimately had a unilateral salpingo-oophorectomy. Subsequent surveillance over 2 to 4 years found no evidence of disease recurrence. CONCLUSION: mBOTs are rare in the pediatric and adolescent population and could arise from benign ovarian tumors.

CIDEA expression in SAT from adolescent girls with obesity and unfavorable patterns of abdominal fat distribution.

OBJECTIVE: This study investigated whether variations in cell death-inducing DNA fragmentation factor alpha subunit-like effector A (CIDEA) mRNA expression and protein levels are modulated by the pattern of abdominal fat distribution in adolescent girls with obesity. METHODS: This study recruited 35 adolescent girls with obesity and characterized their abdominal fat distribution by magnetic resonance imaging. Participants had only a periumbilical/abdominal (n = 14) or a paired abdominal and gluteal subcutaneous adipose tissue (SAT) biopsy (n = 21). CIDEA expression was determined by reverse transcription-polymerase chain reaction, CIDEA protein level by Western blot, and the turnover of adipose lipids and adipocytes by 2 H2 O labeling. In six girls, a second abdominal SAT biopsy was performed (after ~34.2 months) to explore the weight gain effect on CIDEA expression in abdominal SAT. RESULTS: CIDEA expression decreased in abdominal SAT from participants with high visceral adipose tissue (VAT)/(VAT+SAT); CIDEA inversely correlated with number of small adipocytes, with the increase in preadipocyte proliferation, and with adipogenesis. A strong inverse correlation was found between CIDEA protein level with the newly synthetized glycerol (r = -0.839, p = 0.0047). Following weight gain, an increase in adipocytes' cell diameter with a decrease in CIDEA expression and RNA-sequencing transcriptomic profile typical of adipocyte dysfunction was observed. CONCLUSIONS: Reduced expression of CIDEA in girls with high VAT/(VAT+SAT) is associated with adipocyte hypertrophy and insulin resistance.

Unindicated cervical cancer screening in adolescent females within a large healthcare system in the United States.

BACKGROUND: Current consensus recommendations are to not initiate cervical cancer screening for immunocompetent adolescent females before 21 years of age. This is in part because of the very low rate of 0.8 per 100,000 new cervical cancer cases diagnosed among women aged between 20 to 24 years. Timely human papillomavirus vaccination further decreases the incidence of cervical cancer to 4 cases per 100,000 persons by the age of 28 years. Screening before 21 years of age has demonstrated no clear benefit in cancer risk reduction or outcomes. In addition, unindicated screening among adolescents can lead to patient harm and increasing costs to the healthcare system. OBJECTIVE: It is important to assess the rates of overutilization of cervical cancer screening and to identify areas where improvements have occurred and where further opportunities exist. This study aimed to assess the trends over time and the practice and provider factors associated with unindicated cervical cancer screening tests in adolescent females within the largest healthcare system in the state. STUDY DESIGN: Cross-sectional data from patients aged 13 to 20 years who underwent cervical cancer screening between January 1, 2012, and December 31, 2018, across a large multihospital health system were reviewed. All cervical cancer screening results were included. The incidence rate of unindicated screening was analyzed over 6-month intervals using the Poisson regression analysis. RESULTS: The study included data from 118 providers and 794 women. Among the 900 screening results, most (90%) were unindicated: 87% with unindicated cytology testing alone and 14% with unindicated human papillomavirus testing. Screening tests were collected from patients aged 13 to 20 years, many of whom had multiple unindicated cytology tests, with 25 patients having ≥3 tests before the age of 21 years. Most results of cytology testing were negative for intraepithelial lesion or malignancy (77%). Moreover, 52 invasive diagnostic or therapeutic procedures (49 colposcopies and 3 conizations) were performed, of which 45 (87%) followed an unindicated screening test. Between 2012 and 2018, the incidence rate of unindicated cytology decreased by 33% (12.6 to 8.5 unindicated cytology per 1000 encounters). The incidence rate of unindicated screening was lower in the academic setting than in the community setting (incidence rate ratio, 0.43; P<.01). Even with decreases in the overall rates of unindicated screening throughout the study period, there were still 58 unindicated screening tests performed in the final year of this study. CONCLUSION: Despite substantial reductions in unindicated screening for women aged <21 years, there remained areas for improvement. Our data reflected practices of guideline nonadherence up to 7 years after the 2012 guideline. Now, with a new series of changes to the guidelines, which may be even more challenging for patients and providers, it is more important than ever to utilize evidence-based strategies to improve guideline dissemination and adherence.

National Trends in Cervical Cancer Screening in Adolescents.

STUDY OBJECTIVE: To characterize patterns of cervical cancer screening among adolescents ages 14-20 years before and after the 2009 American College of Obstetrics and Gynecology recommendations. DESIGN: Retrospective cohort study. SETTING: National Ambulatory Medical Care Survey 2005-2016 data. PARTICIPANTS: Female adolescents and young women ages 14-26 years. INTERVENTIONS: Multivariable logistic regression models identified independent predictors of unindicated cervical cancer screening at office-based visits among adolescents ages 14-20 years. MAIN OUTCOME MEASURES: Cervical cancer screening in women ages 14-20 and 21-26 years in 2 time periods: 2005-2008 and 2009-2016. RESULTS: Between 2005 and 2016, 11,768 office visits were among adolescents ages 14-20 years. Overall, the cervical cancer screening rate for young women ages 14-20 years was 4.0%, which decreased from 4.5% to 0.4% (P = .008) during the study period. Adolescents who received cervical cancer screening during an office visit were older (18-20 years: 24.1% vs 14-17 years: 8.2%; P < .001), had a preventive care visit (preventive care: 79.7% vs other visit types: 20.3%; P < .001), and saw an obstetrician/gynecologist (obstetrician/gynecologist: 74.81% vs other specialties: 25.1%; P < .001). After adjusting for age, year, period, insurance status, region, and provider type, screening for cervical cancer was associated with living in the Southern region of the United States (adjusted odds ratio, 1.88; 95% confidence interval, 1.09-3.25; P = .02) and public insurance (adjusted odds ratio, 0.47, 95% confidence interval, 0.34-0.64; P < .001). CONCLUSION: Despite recommendations, cervical cancer screening continued to occur in the adolescent population-especially older adolescents-creating unnecessary costs and potential harms. These findings show a slow uptake of guidelines nationally and the need for initiatives that encourage implementation of performance measures for providers, including ongoing provider and patient education.

Laparoscopic Colpo-Pneumo Occluder as a Vaginal Mold for Vaginal Reconstruction.

BACKGROUND: Currently, there is no commercially available soft vaginal mold designed for reconstructive surgeries for congenital vaginal anomalies. Stricter operating room regulations discourage the use of makeshift molds from foams and gloves. A colpo-pneumo-occluder balloon is designed to maintain pneumoperitoneum after colpotomy in laparoscopic hysterectomies and is approved for use in vaginal surgeries. CASE: A 17-year-old girl with a congenital transverse vaginal septum experienced recurrent obstruction and hematocolpos. We successfully used a colpo-pneumo-occluder balloon as a vaginal mold during postoperative care. Its size and design make this device ideal for use in vaginal reconstructive surgeries in adolescents. SUMMARY AND CONCLUSION: The laparoscopic colpo-pneumo-occluder, a sterile vaginal device, is appropriate to use as an adjustable, soft vaginal mold for correction of congenital and acquired vaginal anomalies.

The association between anti-Müllerian hormone and vitamin 25(OH)D serum levels and polycystic ovarian syndrome in adolescent females.

BACKGROUND: High anti-Müllerian hormone (AMH) levels and 25-hydroxyvitamin D [25(OH)D] deficiency have been associated with polycystic ovarian syndrome (PCOS) in adult women, and implicated in its pathogenesis. Herein we determined if the level of both AMH and 25(OH)D are altered in adolescent females with clinical features of PCOS. METHODS: This is a cross-sectional study utilizing a retrospective chart review of 128 patients aged 12-20 referred to an academic adolescent gynecology and endocrinology clinic for an evaluation of suspected PCOS. Unadjusted comparisons of AMH and 25(OH)D distributions between subjects with and without PCOS were performed using the Wilcoxon Rank Sum test. Quantile regression was used to compare the median AMH and 25(OH)D between subject groups; adjusting for race, ethnicity, BMI, insurance type, age, and season when bloodwork was performed. RESULTS: Seventy-four subjects were classified as having PCOS by meeting ≥2 of the three Rotterdam diagnostic criteria, and 47 subjects met only one Rotterdam diagnostic criteria, and were used as the comparative non-PCOS group. There were statistically significant unadjusted differences in median levels of AMH and 25(OH)D. In the adjusted analyses, median AMH was significantly higher in the PCOS group compared to the non-PCOS group (+ 2.39 ng/mL, 95% CI 0.43, 4.35, p = 0.018); 25(OH)D was significantly lower in the PCOS group (- 9.01 ng/mL, 95% CI -14.49, - 3.53 p = 0.001). In our sample, adolescents in both groups had insufficient 25(OH)D level (22 ng/mL) and elevated BMI (32.2 kg/m2). CONCLUSIONS: Adolescents with PCOS display high levels of AMH and low 25(OH)D levels. Since traditional clinical markers of PCOS may be physiologic in adolescents, AMH and 25(OH)D may be used as surrogate markers of PCOS risk in adolescents.

The omentum of obese girls harbors small adipocytes and browning transcripts.

Severe obesity (SO) affects about 6% of youth in the United States, augmenting the risks for cardiovascular disease and type 2 diabetes. Herein, we obtained paired omental adipose tissue (omVAT) and abdominal subcutaneous adipose tissue (SAT) biopsies from girls with SO undergoing sleeve gastrectomy (SG), to test whether differences in cellular and transcriptomic profiles between omVAT and SAT depots affect insulin sensitivity differently. Following weight loss, these analyses were repeated in a subgroup of subjects having a second SAT biopsy. We found that omVAT displayed smaller adipocytes compared with SAT, increased lipolysis through adipose triglyceride lipase phosphorylation, reduced inflammation, and increased expression of browning/beiging markers. Contrary to omVAT, SAT adipocyte diameter correlated with insulin resistance. Following SG, both weight and insulin sensitivity improved markedly in all subjects. SAT adipocytes' size became smaller, showing increased lipolysis through perilipin 1 phosphorylation, decreased inflammation, and increased expression in browning/beiging markers. In summary, in adolescent girls with SO, both omVAT and SAT depots showed distinct cellular and transcriptomic profiles. Following weight loss, the SAT depot changed its cellular morphology and transcriptomic profiles into more favorable ones. These changes in the SAT depot may play a fundamental role in the resolution of insulin resistance.

Establishing a Pediatric and Adolescent Gynecology Subinternship for Medical Students.

Within the pediatric and adolescent gynecology (PAG) community, an important issue deserving of our attention is expansion of formalized PAG training opportunities for medical students. In addition to those in obstetrics and gynecology, many physicians practicing family medicine, internal medicine, and surgery provide care to young female patients. However, residency programs provide inconsistent training in addressing specific PAG health care needs and communication skills required for delivery of care to pediatric and adolescent patients; opportunities for supervised clinical experience in these areas at the medical school level therefore have great importance because they might not be offered again during some residencies. At the undergraduate medical education level, training opportunities in PAG are currently limited, with only 3 PAG subinternships in North American medical schools. In contrast, other available subinternships include 113 in maternal fetal medicine and 82 in gynecologic oncology. To address this gap in opportunities for medical students, we developed a PAG subinternship and elective for medical students, which we describe in this report. Our 4-week PAG subinternship and 2-week elective expose students to a variety of ambulatory and surgical opportunities, with formal, informal, and self-guided learning activities. In this article, we explain the process of developing the curriculum, gaining alignment from key stakeholders, obtaining formal approval for the course, and advertising the opportunity to medical students. We also provide resources and guidance for medical educators who wish to establish a PAG subinternship or elective course at their institutions.

Adolescent Vulvar Angiokeratoma Associated with Lichen Sclerosus.

BACKGROUND: We present an adolescent with multiple vulvar angiokeratomas within a background of lichen sclerosus. CASE: A 13-year-old girl presented with vulvar pruritus and wart-like vulvar lesions. Four lesions were resected because of discomfort and uncertainty of the diagnosis. Pathology revealed angiokeratomas with chronic inflammation suggestive of lichen sclerosus. Postoperatively, pruritus continued in the largest excised lesion, which was associated with lichen sclerosus, and symptoms were treated successfully with topical steroids. SUMMARY AND CONCLUSION: Vulvar angiokeratomas are asymptomatic red papular lesions and are rare in the female adolescent population. In this case, the pathology revealed the rare co-occurrence of angiokeratomas and lichen sclerosus. Biopsies of vulvar vascular lesions in symptomatic adolescents are recommended. Vulvar angiokeratomas might manifest rare genetic disease in otherwise asymptomatic female patients and warrant further follow-up.

Endometrial Carcinoma in a 26-Year-Old Patient with Bardet-Biedl Syndrome.

BACKGROUND: Bardet-Biedl Syndrome (BBS) is a rare genetic condition characterized by cognitive impairment, dysmorphism, central obesity, and diabetes mellitus, among other abnormalities. Although some of these characteristics are known independent risk factors for endometrial cancer and its precursors, the association between BBS and endometrial cancer is underreported. CASE: We present the case of a 26-year-old patient with BBS and clinical signs of hyperestrogenism who presented with abnormal uterine bleeding and was diagnosed with endometrioid adenocarcinoma. She ultimately underwent definitive surgical treatment with hysterectomy and bilateral salpingectomy. CONCLUSIONS: This is one of only a few reports in the literature describing the association of BBS and endometrioid endometrial adenocarcinoma. Given the association of BBS with risk factors for hyperestrogenism such as truncal obesity, hyperinsulinemia, and ovulatory dysfunction, providers should have increased suspicion for endometrial cancer in young patients with BBS and abnormal uterine bleeding.