Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature
Disorder of sex development (DSD) is a rare condition with atypical development of chromosomal, gonadal, or anatomical sex. It is classified in different subgroups based on the patient’s karyotype, gonadal dysgenesis, and the appearance of the internal and external genitalia. Within the subgroups, t...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2021/5552305 |
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| author | Steffen Gretser Maria-Noemi Welte Frederik Roos Jens Köllermann |
| author_facet | Steffen Gretser Maria-Noemi Welte Frederik Roos Jens Köllermann |
| author_sort | Steffen Gretser |
| collection | DOAJ |
| description | Disorder of sex development (DSD) is a rare condition with atypical development of chromosomal, gonadal, or anatomical sex. It is classified in different subgroups based on the patient’s karyotype, gonadal dysgenesis, and the appearance of the internal and external genitalia. Within the subgroups, the risk for developing neoplasms varies a lot. Here, we report the case of a 41-year-old patient with disorder of sex development, showing a 46,XX karyotype with an ovotestis and the simultaneous manifestation of a Leydig cell tumor in the ovotestis. The patient initially presented with infertility, and a suspicious lesion of the left testicle was noted on MRI-Scan. Upon resection, a Leydig cell tumor and an ovotestis were diagnosed. Nongerm call tumors are rare in patients with DSD. We report a nongerm cell tumor in a patient with 46,XX DSD, ovotesticular. This shows that although 46,XX DSD, ovotesticular is known to have a low potential for germ cell neoplasia, nongerm cell tumors can develop and should be into account for the management of those patients. |
| format | Article |
| id | doaj-art-a1ac7fc23d43482eaa1d500629a3d656 |
| institution | Kabale University |
| issn | 2090-6781 2090-679X |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-a1ac7fc23d43482eaa1d500629a3d6562025-02-03T06:07:40ZengWileyCase Reports in Pathology2090-67812090-679X2021-01-01202110.1155/2021/55523055552305Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the LiteratureSteffen Gretser0Maria-Noemi Welte1Frederik Roos2Jens Köllermann3Dr. Senckenberg Institute of Pathology, University Hospital, Goethe University Frankfurt am Main, Frankfurt am Main, GermanyDepartment of Urology, University Hospital, Goethe University Frankfurt am Main, Frankfurt am Main, GermanyDepartment of Urology, University Hospital, Goethe University Frankfurt am Main, Frankfurt am Main, GermanyDr. Senckenberg Institute of Pathology, University Hospital, Goethe University Frankfurt am Main, Frankfurt am Main, GermanyDisorder of sex development (DSD) is a rare condition with atypical development of chromosomal, gonadal, or anatomical sex. It is classified in different subgroups based on the patient’s karyotype, gonadal dysgenesis, and the appearance of the internal and external genitalia. Within the subgroups, the risk for developing neoplasms varies a lot. Here, we report the case of a 41-year-old patient with disorder of sex development, showing a 46,XX karyotype with an ovotestis and the simultaneous manifestation of a Leydig cell tumor in the ovotestis. The patient initially presented with infertility, and a suspicious lesion of the left testicle was noted on MRI-Scan. Upon resection, a Leydig cell tumor and an ovotestis were diagnosed. Nongerm call tumors are rare in patients with DSD. We report a nongerm cell tumor in a patient with 46,XX DSD, ovotesticular. This shows that although 46,XX DSD, ovotesticular is known to have a low potential for germ cell neoplasia, nongerm cell tumors can develop and should be into account for the management of those patients.http://dx.doi.org/10.1155/2021/5552305 |
| spellingShingle | Steffen Gretser Maria-Noemi Welte Frederik Roos Jens Köllermann Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature Case Reports in Pathology |
| title | Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature |
| title_full | Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature |
| title_fullStr | Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature |
| title_full_unstemmed | Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature |
| title_short | Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature |
| title_sort | leydig cell tumor in a patient with 46 xx disorder of sex development dsd ovotesticular a case report and a review of the literature |
| url | http://dx.doi.org/10.1155/2021/5552305 |
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