Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin
Extramammary Paget disease (EMPD) is a rare form of cutaneous, intraepithelial adenocarcinoma, which typically presents itself as an erythematous plaque originating from apocrine-gland rich regions, such as the vulva, the perianal region, the scrotum, the penis, or the axilla. EMPD patients typicall...
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| Format: | Article |
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Frontiers Media S.A.
2022-08-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2022.925551/full |
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| author | Emma Zattarin Federico Nichetti Francesca Ligorio Francesca Ligorio Laura Mazzeo Riccardo Lobefaro Giovanni Fucà Giorgia Peverelli Andrea Vingiani Andrea Vingiani Giulia V. Bianchi Giuseppe Capri Filippo de Braud Filippo de Braud Claudio Vernieri Claudio Vernieri |
| author_facet | Emma Zattarin Federico Nichetti Francesca Ligorio Francesca Ligorio Laura Mazzeo Riccardo Lobefaro Giovanni Fucà Giorgia Peverelli Andrea Vingiani Andrea Vingiani Giulia V. Bianchi Giuseppe Capri Filippo de Braud Filippo de Braud Claudio Vernieri Claudio Vernieri |
| author_sort | Emma Zattarin |
| collection | DOAJ |
| description | Extramammary Paget disease (EMPD) is a rare form of cutaneous, intraepithelial adenocarcinoma, which typically presents itself as an erythematous plaque originating from apocrine-gland rich regions, such as the vulva, the perianal region, the scrotum, the penis, or the axilla. EMPD patients typically have a good prognosis, with expected 5-year survival of 60%–92%, but it is estimated that about one-third of EMPD patients will develop lymph node or distant metastases. Treatment approaches for EMPD include locoregional therapies such as broad surgical resection, radiotherapy, or topical imiquimod, when the disease is localized, and chemotherapy and biological agents for advanced EMPD. We report the case of a 58-year-old man diagnosed with locally advanced, symptomatic HER2-overexpressing, AR-positive EMPD, who achieved long-term tumor control with a sequence of several trastuzumab-based treatments (more than 30 months with second-line carboplatin plus paclitaxel plus trastuzumab followed by trastuzumab maintenance; 9 months for third-line vinorelbine plus trastuzumab). Even if it is reported that AR expression occurs concomitantly with HER2 overexpression in more than half of the cases of EMPD, to the best of our knowledge, this is the first case report describing androgen receptor blockade therapy in combination with an anti-HER2 agent. Our patient did not benefit from androgen receptor blockade in combination with trastuzumab, thus suggesting that AR expression may simply reflect an intrinsic characteristic of the EMPD cell of origin, rather than tumor dependence upon AR signaling. Given the reported sensibility to anti-HER2 therapy, also new antibody drug conjugates targeting HER2 are worth exploring in the management of advanced EMPD. |
| first_indexed | 2024-04-12T06:09:02Z |
| format | Article |
| id | doaj.art-02ed4f0921e249db898877b9b34d54a9 |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-04-12T06:09:02Z |
| publishDate | 2022-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-02ed4f0921e249db898877b9b34d54a92022-12-22T03:44:46ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2022-08-011210.3389/fonc.2022.925551925551Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groinEmma Zattarin0Federico Nichetti1Francesca Ligorio2Francesca Ligorio3Laura Mazzeo4Riccardo Lobefaro5Giovanni Fucà6Giorgia Peverelli7Andrea Vingiani8Andrea Vingiani9Giulia V. Bianchi10Giuseppe Capri11Filippo de Braud12Filippo de Braud13Claudio Vernieri14Claudio Vernieri15Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyIstituto Fondazione di Oncologia Molecolare Ente del Terzo Settore, The AIRC Institute of Molecular Oncology, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, ItalyIstituto Fondazione di Oncologia Molecolare Ente del Terzo Settore, The AIRC Institute of Molecular Oncology, Milan, ItalyExtramammary Paget disease (EMPD) is a rare form of cutaneous, intraepithelial adenocarcinoma, which typically presents itself as an erythematous plaque originating from apocrine-gland rich regions, such as the vulva, the perianal region, the scrotum, the penis, or the axilla. EMPD patients typically have a good prognosis, with expected 5-year survival of 60%–92%, but it is estimated that about one-third of EMPD patients will develop lymph node or distant metastases. Treatment approaches for EMPD include locoregional therapies such as broad surgical resection, radiotherapy, or topical imiquimod, when the disease is localized, and chemotherapy and biological agents for advanced EMPD. We report the case of a 58-year-old man diagnosed with locally advanced, symptomatic HER2-overexpressing, AR-positive EMPD, who achieved long-term tumor control with a sequence of several trastuzumab-based treatments (more than 30 months with second-line carboplatin plus paclitaxel plus trastuzumab followed by trastuzumab maintenance; 9 months for third-line vinorelbine plus trastuzumab). Even if it is reported that AR expression occurs concomitantly with HER2 overexpression in more than half of the cases of EMPD, to the best of our knowledge, this is the first case report describing androgen receptor blockade therapy in combination with an anti-HER2 agent. Our patient did not benefit from androgen receptor blockade in combination with trastuzumab, thus suggesting that AR expression may simply reflect an intrinsic characteristic of the EMPD cell of origin, rather than tumor dependence upon AR signaling. Given the reported sensibility to anti-HER2 therapy, also new antibody drug conjugates targeting HER2 are worth exploring in the management of advanced EMPD.https://www.frontiersin.org/articles/10.3389/fonc.2022.925551/fullextramammary Paget disease (EMPD)HER2 overexpressiontrastuzumabprolonged benefitantiandrogen therapyrare cancer |
| spellingShingle | Emma Zattarin Federico Nichetti Francesca Ligorio Francesca Ligorio Laura Mazzeo Riccardo Lobefaro Giovanni Fucà Giorgia Peverelli Andrea Vingiani Andrea Vingiani Giulia V. Bianchi Giuseppe Capri Filippo de Braud Filippo de Braud Claudio Vernieri Claudio Vernieri Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin Frontiers in Oncology extramammary Paget disease (EMPD) HER2 overexpression trastuzumab prolonged benefit antiandrogen therapy rare cancer |
| title | Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin |
| title_full | Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin |
| title_fullStr | Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin |
| title_full_unstemmed | Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin |
| title_short | Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin |
| title_sort | case report prolonged clinical benefit with sequential trastuzumab containing treatments in a patient with advanced extramammary paget disease of the groin |
| topic | extramammary Paget disease (EMPD) HER2 overexpression trastuzumab prolonged benefit antiandrogen therapy rare cancer |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2022.925551/full |
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