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| ID | Type | Description | Link |
|---|---|---|---|
| 23-5235 | Registry Identifier | GDPR |
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Disorders of Sexual Development are rare and represent a spectrum of heterogeneous pathologies. The risk of developing a malignant gonadal germ cell tumor (MGCT) exists and varies according to the etiology.
The indication for prophylactic gonadectomy must be modulated and discussed for all patients. It is essential to assess each patient's individual risk.
The aim of our study is to assess gonadal tumor risk in patients with Disorders of Sexual Development according to etiology
The primary objective is to evaluate tumor risk and the indication for gonadectomy in cases of Disorders of Sexual Development according to etiology.
The secondary objectives are to evaluate the timing of gonadectomy, to monitor changes in practice over time, and to assess the contribution of imaging and/or biological tests to tumor detection.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Not applicable . Non-interventional retrospective study, only on datas. | Other | Not applicable . Non-interventional retrospective study, only on datas. |
| Measure | Description | Time Frame |
|---|---|---|
| Presence or absence of gonadal tumor lesion after gonadectomy, in case of Disorders of sexual development (DSD) according to anatomo-pathological analysis. | Datas about the gonadectomy surgical pieces performed between1990 and 2022 are reviewed for the study by Dr Frédérique Dijoud, anatomo-pathologist at the DEV-GEN CRMR in Lyon, to confirm the présence or absence of a gonadal tumor lesion, in order to avoid intra- and inter-reader interpretation bias. | The outcome measure will be assessed and the datas reported for each patient will be collected, from birth until the study completion date, or by default until the date of death from any cause. Datas will be collected during the 10 months of the study |
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Inclusion Criteria:
Patients who underwent gonadectomy for variation in genital development between 1990 and 2022.
Pathological anatomy examination in Lyon
With clinical data
With karyotype
3 categories:
Exclusion Criteria:
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Patients who underwent gonadectomy for Disorders of Sexual Development between 1990 and 2022, followed by Dr. Daniela Gorduza in the Pediatric Uro-Visceral Surgery Department of the hospital Femme-Mère-Enfant.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de Chirurgie uro-viscérale pédiatrique | Bron | 69500 | France |
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| ID | Term |
|---|---|
| D012734 | Disorders of Sex Development |
| D018238 | Gonadoblastoma |
| D004407 | Dysgerminoma |
| D009369 | Neoplasms |
| D006059 | Gonadal Dysgenesis |
| D013734 | Androgen-Insensitivity Syndrome |
| C537770 | Anorchia |
| ID | Term |
|---|---|
| D014564 | Urogenital Abnormalities |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
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| D052801 | Male Urogenital Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D006058 | Gonadal Disorders |
| D004700 | Endocrine System Diseases |
| D009373 | Neoplasms, Germ Cell and Embryonal |
| D009370 | Neoplasms by Histologic Type |
| D018309 | Neoplasms, Gonadal Tissue |
| D006061 | Gonadal Dysgenesis, 46,XY |
| D058490 | Disorder of Sex Development, 46,XY |
| D018237 | Germinoma |
| D040181 | Genetic Diseases, X-Linked |
| D030342 | Genetic Diseases, Inborn |