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Gonadal tumors in a contemporary cohort of patients with differences in sex development undergoing surgery - A multi-site study from the Pediatric Urologic Oncology Working Group of the societies for pediatric urology.
Peard, Leslie M; Morin, Jacqueline; Flores, Viktor; Graham, Kyle; Taylor, Abby S; Pope, John C; Halstead, Valeska; Cost, Nicholas G; Roberts, Evan M; Makari, John H; Cranford, Will; Saltzman, Amanda F.
Afiliação
  • Peard LM; Department of Urology, University of Kentucky, 800 Rose St., Lexington, KY 40536, USA; Division of Pediatric Urology, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address: Leslie.p.hackett@gmail.com.
  • Morin J; Department of Urology, University of Kentucky, 800 Rose St., Lexington, KY 40536, USA. Electronic address: jacqueline.morin@uky.edu.
  • Flores V; Division of Pediatric Urology, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address: viktor.flores@vumc.org.
  • Graham K; Division of Pediatric Urology, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address: mason.k.graham@vumc.org.
  • Taylor AS; Division of Pediatric Urology, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address: abby.s.taylor@vumc.org.
  • Pope JC; Division of Pediatric Urology, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address: john.pope@vumc.org.
  • Halstead V; Department of Surgery, Division of Urology, Surgical Oncology Program at Children's Hospital Colorado, University of Colorado School of Medicine, Children's Hospital of Colorado, 13123 E. 16th Ave., Aurora, CO 80045, USA. Electronic address: nvhalstead@gmail.com.
  • Cost NG; Department of Surgery, Division of Urology, Surgical Oncology Program at Children's Hospital Colorado, University of Colorado School of Medicine, Children's Hospital of Colorado, 13123 E. 16th Ave., Aurora, CO 80045, USA. Electronic address: Nicholas.Cost@childrenscolorado.org.
  • Roberts EM; Section of Pediatric Urology, Children's Hospital and Medical Center, Omaha, NE; Division of Urology, University of Nebraska Medical Center, 8200 Dodge St., Omaha, NE 68114, USA. Electronic address: Evan.Roberts@unmc.edu.
  • Makari JH; Section of Pediatric Urology, Children's Hospital and Medical Center, Omaha, NE; Division of Urology, University of Nebraska Medical Center, 8200 Dodge St., Omaha, NE 68114, USA. Electronic address: jmakari@childrensomaha.org.
  • Cranford W; Department of Biostatistics, College of Public Health, University of Kentucky, 800 Rose St., Lexington, KY 40536, USA. Electronic address: willcranford@uky.edu.
  • Saltzman AF; Department of Urology, University of Kentucky, 800 Rose St., Lexington, KY 40536, USA. Electronic address: afsaltzman@gmail.com.
J Pediatr Urol ; 19(4): 399.e1-399.e8, 2023 08.
Article em En | MEDLINE | ID: mdl-37117082
BACKGROUND: Disturbances in gonadal development lead to increased risk of gonadal malignancy in some but not all patients with differences in sex development (DSD). However, the natural history of these tumors is poorly described, and the literature remains sparse. OBJECTIVE: The objective of this study was to describe the incidence of germ cell neoplasia in situ (GCNIS) and germ cell tumor (GCT) in a contemporary cohort of patients with DSD undergoing surgery and to provide long-term oncologic outcomes for these patients. STUDY DESIGN: Patients with DSD who have undergone gonadectomy or gonadal biopsy were identified at four institutions. Clinical characteristics, pathology, and treatment details were obtained retrospectively. Patients were stratified into risk categories based on DSD diagnosis. Oncologic treatment and outcomes were recorded. Descriptive statistics are reported using parametric methods. RESULTS: 83 patients were identified. Distribution of diagnoses is summarized in the summary table. 14 (16.9%) patients underwent gonadal biopsy, and 71 (85.5%) patients underwent gonadectomy (50/71 gonadectomies were bilateral). 8/83 (9.6%) patients had GCNIS or GCT (7 GCNIS, 1 GCT). Median age at surgery was 2.95 years (y) (interquartile range [IQR] 0.6-12.2) and 14y (IQR 0.85-16.9) in patients without and with GCNIS/GCT, respectively. All 8 patients with GCNIS/GCT had high or intermediate risk DSD diagnoses (4 mixed gonadal dysgenesis, 3 Turner with Y, 1 partial gonadal dysgenesis). Of the patients with high-risk diagnoses, 8/54 (15%) had GCNIS/GCT. No patient received adjuvant therapy, no patient had a recurrence, and all patients were living with mean follow up 6.4y. DISCUSSION: The risk of gonadal malignancy is heterogeneous in the DSD population and can vary based on DSD diagnosis as well as maturation, testicularization, and location of the gonads. The most recent consensus recommendations on gonadal management emphasize risk stratification and consideration of gonadal surveillance based on gender of rearing, but supporting literature remains sparse. In this contemporary cohort of DSD patients who underwent gonadal surgery, most patients did not have evidence of adverse pathology, all patients with malignant or premalignant pathology had a high/intermediate risk DSD diagnosis, and all patients with GCNIS/GCT were treated with surgery alone without recurrence. CONCLUSIONS: The distribution of patients with premalignant and malignant gonadal pathology and DSD in this cohort aligns with prior literature, and oncologic outcomes were excellent. These data add valuable information to the current literature and highlight the necessity to develop appropriate screening regimens for retained gonads.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Urologia / Neoplasias Embrionárias de Células Germinativas / Disgenesia Gonadal Idioma: En Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Urologia / Neoplasias Embrionárias de Células Germinativas / Disgenesia Gonadal Idioma: En Ano de publicação: 2023 Tipo de documento: Article