Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature
Extramammary Paget’s disease (EMPD) is a rare cancer. Although EMPD is usually noninvasive and treated with local therapy, once metastatic the prognosis of EMPD is poor and treatment options are limited. We report a case of a complete response to single agent trastuzumab in a hemodialysis patient wi...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2015-01-01
|
| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/895151 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832548450971942912 |
|---|---|
| author | Peter Barth Essel Dulaimi Al-Saleem Kristin W. Edwards Sherri Z. Millis Yu-Ning Wong Daniel M. Geynisman |
| author_facet | Peter Barth Essel Dulaimi Al-Saleem Kristin W. Edwards Sherri Z. Millis Yu-Ning Wong Daniel M. Geynisman |
| author_sort | Peter Barth |
| collection | DOAJ |
| description | Extramammary Paget’s disease (EMPD) is a rare cancer. Although EMPD is usually noninvasive and treated with local therapy, once
metastatic the prognosis of EMPD is poor and treatment options are limited. We report a case of a complete response to single agent trastuzumab in a hemodialysis patient with metastatic Her2/neu overexpressed EMPD of the scrotum. Molecular profiling of his case as well as 12 other EMPD and 8 mammary Paget disease (MPD) cases was completed and revealed multiple biomarker aberrations. Overexpression of Her2 was frequently noted (30%–40%) in both EMPD and MPD patients and when present can be effectively treated with Her2 targeted agents. Trastuzumab therapy can be safely utilized in a hemodialysis patient. In addition, multiple protein overexpression and loss were seen in EMPD including PD-1, PD-L1, PTEN, and AR as well as PIK3CA mutation. These findings may lead to possible therapeutic interventions targeting these pathways in a disease with few effective treatment options. |
| format | Article |
| id | doaj-art-25f8cc45a92f45939061f37b72f6914a |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-25f8cc45a92f45939061f37b72f6914a2025-02-03T06:13:56ZengWileyCase Reports in Oncological Medicine2090-67062090-67142015-01-01201510.1155/2015/895151895151Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the LiteraturePeter Barth0Essel Dulaimi Al-Saleem1Kristin W. Edwards2Sherri Z. Millis3Yu-Ning Wong4Daniel M. Geynisman5Department of Hematology/Oncology Oncology, Fox Chase Cancer Center, Temple University Health System, Philadelphia, PA 19111, USADepartment of Pathology, Fox Chase Cancer Center, Temple University Health System, Philadelphia, PA 19111, USADepartment of Radiology, Fox Chase Cancer Center, Temple University Health System, Philadelphia, PA 19111, USACaris Life Sciences, Phoenix, AZ 85040, USADepartment of Hematology/Oncology Oncology, Fox Chase Cancer Center, Temple University Health System, Philadelphia, PA 19111, USADepartment of Hematology/Oncology Oncology, Fox Chase Cancer Center, Temple University Health System, Philadelphia, PA 19111, USAExtramammary Paget’s disease (EMPD) is a rare cancer. Although EMPD is usually noninvasive and treated with local therapy, once metastatic the prognosis of EMPD is poor and treatment options are limited. We report a case of a complete response to single agent trastuzumab in a hemodialysis patient with metastatic Her2/neu overexpressed EMPD of the scrotum. Molecular profiling of his case as well as 12 other EMPD and 8 mammary Paget disease (MPD) cases was completed and revealed multiple biomarker aberrations. Overexpression of Her2 was frequently noted (30%–40%) in both EMPD and MPD patients and when present can be effectively treated with Her2 targeted agents. Trastuzumab therapy can be safely utilized in a hemodialysis patient. In addition, multiple protein overexpression and loss were seen in EMPD including PD-1, PD-L1, PTEN, and AR as well as PIK3CA mutation. These findings may lead to possible therapeutic interventions targeting these pathways in a disease with few effective treatment options.http://dx.doi.org/10.1155/2015/895151 |
| spellingShingle | Peter Barth Essel Dulaimi Al-Saleem Kristin W. Edwards Sherri Z. Millis Yu-Ning Wong Daniel M. Geynisman Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature Case Reports in Oncological Medicine |
| title | Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature |
| title_full | Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature |
| title_fullStr | Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature |
| title_full_unstemmed | Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature |
| title_short | Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature |
| title_sort | metastatic extramammary paget s disease of scrotum responds completely to single agent trastuzumab in a hemodialysis patient case report molecular profiling and brief review of the literature |
| url | http://dx.doi.org/10.1155/2015/895151 |
| work_keys_str_mv | AT peterbarth metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature AT esseldulaimialsaleem metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature AT kristinwedwards metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature AT sherrizmillis metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature AT yuningwong metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature AT danielmgeynisman metastaticextramammarypagetsdiseaseofscrotumrespondscompletelytosingleagenttrastuzumabinahemodialysispatientcasereportmolecularprofilingandbriefreviewoftheliterature |