Urology

Mature Ovarian Cystic Teratoma Mimicking a Bladder Diverticulum in a 16-year-old Female

A 16-year-old female was incidentally found to have an abnormal pelvic ultrasound while undergoing evaluation for mild scoliosis. A large, thick-walled, lobular, fluid-filled structure was found at midline in the pelvis that initially resembled bladder diverticula due to its anatomical position and sonographic appearance. Voiding cystourethrogram and computed tomography later revealed a large cystic mass originating from the right ovary. This report outlines a case of a mature cystic teratoma convincingly mimicking bladder anomaly on sonography.

Variation of Gonadal Dysgenesis and Tumor Risk in Patients With 45,X/46,XY Mosaicism

To describe the gonadal features of patients with 45,X/46,XY mosaicism, and to evaluate the prevalence of gonadal tumor in different phenotypes. The medical records of consecutive patients with 45,X/46,XY karyotype or its variants who had undergone gonadal biopsy or gonadectomy at a single institute between 1996 and 2017 were retrospectively reviewed. Of 34 patients with 45,X/46,XY mosaicism, a unilateral dysgenetic testis and a contralateral streak gonad was detected in 20 patients (59%), bilateral streak gonads in 9 (26%), and bilateral dysgenetic testes in 5 (15%). A gonad composed of both streak and dysgenetic testicular portions was observed in 7 gonads of 6 patients. All streak gonads were removed, and bilateral gonadectomy was performed in 15 patients raised as girls. Pathologic examination revealed gonadal tumors in 6 of the 34 (18%) patients, including a gonadoblastoma in 7 gonads among 5 patients and an association of dysgerminoma with gonadoblastoma in 1 gonad. All 6 patients who developed gonadal tumor had female genitalia. Postoperative course was uneventful except 1 boy. A seminoma was developed in his soritaly scrotal testis at the age of 16 years. The prevalence of gonadal tumor in patients with 45,X/46,XY mosaicism may vary according to the phenotype, and high in patients with female phenotype. Considering the increased risk of gonadal tumors in such patients, early investigation and individual management, including prophylactic gonadectomy, are recommended. In male patients, a close follow-up of the preserved testes is mandatory until adulthood.

Urothelial Carcinoma of the Bladder With a Rare Solitary Metastasis to the Ovary

Management and Long-term Follow-up of Vaginal Rhabdomyosarcoma in a 3-Year-Old Female

We present the case of a 3-year-old female who presented with a vaginal mass and was subsequently diagnosed with intermediate risk, stage I, group III vaginal embryonal rhabdomyosarcoma. She was staged and treated with chemotherapy according to the Children's Oncology Group protocol. Surgical management included initial tumor debulking and subsequent partial vaginectomy with vaginal reconstruction following chemotherapy. There has been no evidence of disease on surveillance 7 years after diagnosis.

A Successful Therapeutic Strategy for a Young Girl With Vaginal Yolk Sac Tumor by buccal mucosa vaginoplasty

Gonadoblastoma in Turner Syndrome: A Surprise in a Streak

Urologic Manifestations of Uterine Leiomyomata in Women with Spina Bifida

To examine our experience with presentation and management of uterine leiomyomata (fibroids) in women with spina bifida (SB). Advances in health care have dramatically improved the life expectancy for individuals living with SB, but they face unique health challenges surrounding reproductive health. Uterine leiomyomata have not previously been described in this population. We hypothesize that their course is likely complicated by SB-specific disabilities and prior surgeries. We queried our institutional database for adults with SB and a diagnosis of uterine leiomyomata and/or history of hysterectomy. Clinical data were abstracted from patient records. Five individuals were identified who required management of leiomyomata, representing 2.4% of adult women in our SB clinic. All had lumbar myelomeningocele, hydrocephalus (managed by ventriculoperitoneal shunt), and neurogenic bladder. Three had histories of sexual activity, and 1 had given birth. Two presented with increased urinary frequency/incontinence, and 2 presented with abdominal/pelvic pain. Four underwent hysterectomy with the urology team present to prevent/repair injury to the urinary tract, which had been reconstructed in 2. Mean age of menarche, presentation, and hysterectomy were 10.8, 39.8, and 42.5 years, respectively. One developed upper tract dilation due to compression, which resolved after hysterectomy. Adult women living with SB face unique considerations and now live long enough to develop leiomyomata. When symptomatic, surgical management is often needed and may require urologic collaboration. Special care should be taken to protect the bladder and ureters, particularly with a history of prior bladder reconstructive surgery, as well as ventriculoperitoneal shunt, if present.

Successful Multimodal Treatment of Uterine Rhabdomyosarcoma

Uterine rhabdomyosarcoma is exceedingly rare. The treatment strategy has evolved from aggressive local control with upfront surgery followed by radiation to a more conservative approach with chemotherapy followed by additional treatment pending response, which is outlined in a recent consensus statement from the International Soft-Tissue Sarcoma Consortium. We present a case of a 2-year-old with intermediate risk uterine rhabdomyosarcoma. She was treated with a multimodal regimen including chemotherapy, radiation, and surgery with abdominal hysterectomy, bilateral salpingectomy, oophoropexy, partial proximal vaginectomy, and right extravesical nonrefluxing ureteral reimplant to achieve a complete resection.

Management of an Incidentally Discovered Ovarian Teratoma in an Adolescent Patient

Mature ovarian teratomas are a benign subset of germ cell tumors (GCT) often diagnosed during reproductive years and are the most common GCT among children and adolescents. Many are found incidentally on unrelated imaging. There are no definitive guidelines on the management of these teratomas, particularly in the pediatric population. This case presents the management of an adolescent patient with an incidentally found ovarian teratoma. A discussion of contemporary literature also identifies a significant gap in the literature for definitive guidelines in pediatric patients with mature teratomas.

A Case of Dysgerminoma in a Pediatric Patient With a PIK3CD Mutation

Dysgerminoma is a rare germ cell tumor, typically presenting with abdominal pain and distension in young patients with ovaries. Primary surgical management is first-line treatment, with adjuvant chemotherapy for late-stage cases. The presence of an SRY gene and dysgenic gonad is a risk factor for abnormal dysgerminoma presentation. Associations have been reported between dysgerminoma and other genetic mutations, though no definitive correlation has been shown. This article reviews a case of abnormal presentation of dysgerminoma in a pediatric patient with unique genetic abnormalities, and discusses epidemiology, diagnosis, and treatment of dysgerminoma, and associated genetic mutations related to this case.

Adult-Type Granulosa Cell Tumor of the Testis in a 16-Year-Old: A Case Report and Review of the Literature

Adult-type Granulosa cell tumor of the testis is a rare subtype of sex cord-stromal tumors, with fewer than 100 cases reported. The typical clinical presentation is an asymptomatic, painless testicular mass. We report a case of a 16-year-old male with adult-type testicular Granulosa cell tumor who presented with a palpable, painless right testicular mass, and subsequently underwent right inguinal radical orchiectomy. This report contributes to the growing body of literature regarding this rare diagnosis, furthering our understanding of clinical, imaging, and histological findings of its presentation.

Longitudinal Outcomes of Malignant Ureteral Obstruction Secondary to Ovarian Cancer: Predictors of Resolution and the Role of Surgical Management

To review the management of ovarian cancer (OCa) associated hydronephrosis (HN). Specifically, we aim to identify optimal management of HN in the acute setting, predictors of HN resolution, and the role of surgery (tumor debulking/(+/-)ureterolysis/hysterectomy). The study cohort included OCa patients managed at our institution from 2004-2019 that developed OCa-associated HN. Initial HN management was recorded as none, retrograde ureteral stent (RUS) or percutaneous nephrostomy tube (PCN). Primary outcomes included (1) HN management failure, (2) HN management complications, and (3) HN resolution. Patient, cancer, and treatment predictors of outcomes were assessed using logistic regression and fine-Gray competing risk models. Of 2580 OCa patients, 190 (7.4%) developed HN. HN was treated in 121; 90 (74.4%) with RUS, 31 (25.6%) with PCN. Complication rates were similar between PCN and RUS (83% vs 85.1%; P = .79; all Clavian Grade I/II). Initial HN treatment failure occurred in 28 patients, predicted by renal atrophy (hazard ratios (HR) 3.27, P <.01). HN resolution occurred in only 52 (27%) patients and was predicted by lower International Federation of Gynecology and Obstetrics (FIGO) stage (FIGO III/IV HR 0.42, P <.01) and surgical tumor debulking/ureterolysis (HR 2.83, P = .02). Resolution of HN associated with malignant obstruction from OCa is rare and is most closely associated with tumor debulking and International Federation of Gynecology and Obstetrics (FIGO) stage. Initial endoscopic treatment modality was not significantly associated with complications or resolution, though RUS failures were slightly more common. Ureteral reconstruction at time of debulking/ureterolysis is potentially underutilized.

Buccal Mucosa Vaginoplasty Through an Anterior Sagittal Transrectal Approach (ASTRA) for Management of Yolk Sac Tumor in a 3-Year-Old Girl

Vaginal yolk sac tumors are rare pediatric malignant tumors and the most common form of vaginal germ-cell tumors in children. They are almost exclusively found in females under 3 years of age. Treatment involves local excision either with or without chemotherapy. Herein, we describe a case of a 3-year-old girl with vaginal Yolk sac tumor, who underwent buccal mucosa vaginoplasty through an anterior sagittal transrectal approach , as an effective oncological procedure, with preservation of reproductive function.

Inflammatory Vulvar Dermatoses (Part I)

Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (part I) and benign and malignant vulvar neoplasms (part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.

Vulvar Neoplasms (Part II)

Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (Part I) and benign and malignant vulvar neoplasms (Part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.

Urologic Complications Requiring Intervention Following High-dose Pelvic Radiation for Cervical Cancer

To identify the incidence of radiation-induced urologic complication requiring procedural intervention following high-dose radiotherapy for cervical carcinoma, and to identify predictors of complication occurrence. We performed a retrospective chart review of cervical cancer patients undergoing radiotherapy with primary focus on procedural complications (Clavien-Dindo ≥ III). Clinical data were collected including radiation dose, procedure performed, timing of complication, and need for additional procedures. Univariate and multivariate logistic regression modeling was performed to assess predictive value of demographic and clinical variables. A total of 126 patients with FIGO stage 1A2-4B cervical cancer were included in study analysis, with 18 patients experiencing procedural complication (14.3%). A total of 22 complications were identified, representing an average of 1.2 complications per patient with complication. The most common complications were ureteral stricture and radiation cystitis. The most common nononcologic procedures performed in the treatment of these complications were ureteral stenting, percutaneous nephrostomy tube placement, and cystoscopy. Notably, a total of 259 procedures were performed in the treatment of urologic complications, representing 14.4 procedures per patient and 24.6 procedures per patient with ureteral stricture. Logistic regression demonstrated active smoking at the time of diagnosis to be a predictor of procedural complication. Radiotherapy in the treatment of cervical cancer is associated with a high rate of urologic procedural complication. These complications often require numerous procedures and long-term management given their complexity. These findings suggest a need for awareness and plans for multidisciplinary management of urologic complications in this patient population.