Extramammary Paget Disease (Paget Disease, Extramammary)

A rare cutaneous neoplasm that occurs in the elderly. It develops more frequently in women and predominantly involves apocrine gland-bearing areas, especially the vulva, scrotum, and perianal areas. The lesions develop as erythematous scaly patches that progress to crusted, pruritic, erythematous plaques. The clinical differential diagnosis includes squamous cell carcinoma in situ and superficial fungal infection. It is generally thought to be an adenocarcinoma of the epidermis, from which it extends into the contiguous epithelium of hair follicles and eccrine sweat ducts. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1478)

Also Known As:

Networked: 493 relevant articles (37 outcomes, 42 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Basal Cell Carcinoma (Rodent Ulcer)
2.	Squamous Cell Carcinoma (Epidermoid Carcinoma)
3.	Warts (Wart)
4.	Neoplasms (Cancer)
5.	Carcinoma (Carcinomatosis)

Experts

1.	Ihn, Hironobu: 10 articles (01/2022 - 02/2015)
2.	Furue, Masutaka: 10 articles (02/2021 - 01/2007)
3.	Kajihara, Ikko: 9 articles (01/2022 - 02/2015)
4.	Fukushima, Satoshi: 8 articles (01/2022 - 02/2015)
5.	Moroi, Yoichi: 8 articles (10/2011 - 01/2007)
6.	Takeuchi, Satoshi: 8 articles (10/2011 - 01/2007)
7.	Uhara, Hisashi: 7 articles (08/2021 - 01/2002)
8.	Aoi, Jun: 6 articles (01/2022 - 02/2015)
9.	Sakamoto, Ryoko: 6 articles (01/2022 - 08/2005)
10.	Uchi, Hiroshi: 6 articles (06/2016 - 01/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Extramammary Paget Disease:

1.	Imiquimod (Aldara)FDA LinkGeneric 01/01/2012 - "[Extramammary Paget disease: successful therapy with imiquimod 5% cream]." 10/01/2012 - "Sustained remission of extramammary Paget disease following treatment with imiquimod 5% cream." 04/01/2010 - "Recently, topical Imiquimod has been shown as a safe and effective treatment option for extramammary Paget's disease. " 09/25/2018 - "Surgical excision is the standard treatment; however, previous studies have demonstrated good response with Imiquimod 5% cream in patients with vulval extramammary Paget disease (EMPD). " 01/01/2023 - "Patient's Experience With Imiquimod for Extramammary Paget Disease."
2.	Docetaxel (Taxotere)FDA Link 03/01/2012 - "Complete remission of advanced extramammary Paget's disease treated with docetaxel: a case report." 01/01/2021 - "Efficacy of S-1 plus docetaxel in the treatment of metastatic extramammary Paget's disease: a multicentre retrospective study." 04/01/2020 - "Monthly docetaxel (DTX) monotherapy is the first-line regimen that is preferably used for metastatic extramammary Paget's disease (EMPD). " 04/01/2020 - "Weekly docetaxel monotherapy for metastatic extramammary Paget's disease: Retrospective single-institute analysis." 01/01/2002 - "Docetaxel may be an effective drug for patients with advanced extramammary Paget's disease."
3.	1-phenyl-3,3-dimethyltriazene (PDT)IBA 06/15/2011 - "Following Cochrane guidelines, a comprehensive systematic review of all clinical studies and reports examining the use of PDT for mammary and extra-mammary Paget's disease was conducted. " 03/01/2022 - "Efficacy and safety of HpD-PDT for Extramammary Paget's Disease refractory to conventional therapy: A prospective, open-label and single arm pilot study." 11/01/2009 - "Our aim was to determine the usefulness of fluorescence examination and photodynamic therapy (PDT) for the management of perianal extramammary Paget's disease. " 06/01/2022 - "The purpose of this study was to illustrate the efficiency and safety of PDD-guided tumor excision combined with ALA-PDT in patients with extramammary Paget's disease (EMPD). " 09/01/2019 - "ALA-PDT combined with holmium laser therapy of postoperative recurrent extramammary Paget's disease."
4.	AcidsIBA 02/01/2012 - "Incomplete efficacy of 5-aminolevulinic acid (5 ALA) photodynamic therapy in the treatment of widespread extramammary Paget's disease." 11/20/2017 - "5-aminolevulinic acid (5-ALA) fluorescence-guided Mohs surgery resection of penile-scrotal extramammary Paget's disease." 02/01/2005 - "5-Aminolevulinic acid-based photodynamic therapy for the treatment of two patients with extramammary Paget's disease." 09/01/2021 - "The application of Wood's lamp combined with 5-aminolevulinic acid for defining tumor margins in patients with extramammary Paget's disease." 11/20/2017 - "This report aims to evaluate the usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-guided Mohs surgery resection of penile-scrotal extramammary Paget's disease for achieving maximum tumor resection. "
5.	Fluorouracil (Carac)FDA LinkGeneric 12/01/2000 - "Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget's disease: response to topical 5-fluorouracil." 05/01/2000 - "Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget's disease: response to topical 5-fluorouracil." 09/01/1994 - "Extramammary Paget's disease of the scrotum: treatment with topical 5-fluorouracil and plastic surgery." 09/01/1987 - "Topical 5-fluorouracil may have a role as an adjunct to surgery by highlighting subclinical areas of extramammary Paget's disease. " 09/01/2009 - "A promising palliative response was demonstrated following 5-fluorouracil chemotherapy.A review of the literature on the pathogenesis, diagnosis, treatment options, and outcomes for metastatic extramammary Paget disease is presented."
6.	Cisplatin (Platino)FDA LinkGeneric 01/01/2018 - "Docetaxel Combined With Cisplatin for Metastatic Extramammary Paget Disease." 05/01/2018 - "Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget's disease." 02/01/2004 - "Trial of low-dose 5-fluorouracil/cisplatin therapy for advanced extramammary Paget's disease." 02/01/2015 - "Combination chemotherapy of low-dose 5-fluorouracil and cisplatin for advanced extramammary Paget's disease." 04/01/2020 - "Low-dose 5-fluorouracil/cisplatin therapy as conversion chemotherapy for advanced extramammary Paget's disease."
7.	Oxygen (Dioxygen)IBA 01/01/2020 - "In conclusion, the interesting therapeutic efficacy of oxygen flow-assisted LP3-MTX3% for treating superficial basal cell carcinoma, extramammary Paget disease, and MF lesions prompts further studies on a larger scale." 01/01/2020 - "Patients with superficial basal cell carcinoma (n = 12), extramammary Paget disease (n = 5), classic mycosis fungoides (MF; n = 10), and folliculotropic MF (n = 6) were included in the study and were treated with four weekly applications of oxygen flow-assisted LP3-MTX3%. " 08/01/2020 - "Oxygen flow-assisted topical administration of methotrexate 5% (OFAMTX, 5% methotrexate in a carrier solution) has recently been proven to be an efficacious alternative treatment for extramammary Paget's disease, which is considered to be an in situ mammary adenocarcinoma of the epidermis. "
8.	Trastuzumab (Herceptin)FDA Link 01/01/2022 - "Efficacy of trastuzumab in HER-2-positive advanced extramammary Paget's disease." 01/01/2022 - "Case Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin." 01/01/2012 - "We report the first case of 68-year-old Japanese woman with metastatic HER2-positive extramammary Paget's disease that showed the validity of trastuzumab monotherapy. " 01/01/2012 - "Dramatic Clinical Response of Relapsed Metastatic Extramammary Paget's Disease to Trastuzumab Monotherapy." 01/01/2015 - "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."
9.	Keratin-19 (Keratin 19)IBA 03/01/2010 - "[A case of cytokeratin 19 positive extramammary Paget's disease with pulmonary metastases]." 06/01/2017 - "Serum cytokeratin 19 fragment 21-1 is a useful tumor marker for the assessment of extramammary Paget's disease." 03/01/2019 - "Monitoring serum cytokeratin 19 fragment 21-1 to determine the efficacy of docetaxel chemotherapy in advanced extramammary Paget's disease." 01/01/2019 - "Serum levels of carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA) are not elevated in most patients with extramammary Paget disease (EMPD) without metastasis. " 01/01/2019 - "Although carcinoembryonic antigen (CEA) and cytokeratin 19 fragment (CYFRA) are useful markers for extramammary Paget disease (EMPD), serum CEA and CYFRA levels are not elevated in most patients with EMPD without metastasis. "
10.	HematoporphyrinsIBA 06/01/2023 - "Efficacy of hematoporphyrin injection (HpD) photodynamic therapy in the treatment of widespread extramammary Paget's disease." 07/31/2023 - "We suggest that hematoporphyrin photodynamic therapy may be an effective alternative to conventional surgery for the treatment of extramammary Paget's disease." 07/31/2023 - "Case Report: Hematoporphyrin Injection (HpD) Photodynamic Therapy for the Treatment of Groin Extramammary Paget's Disease in an Elderly Male Patient."

Therapies and Procedures

1.	Radiotherapy 05/01/2015 - "Extramammary Paget's disease of the perianal region treated successfully with radiotherapy." 01/01/1991 - "Radiotherapy: an effective treatment for extramammary Paget's disease." 09/01/2007 - "Several reports have suggested the efficacy of radiotherapy for treating extramammary Paget's disease (EMPD); however, these reports comprised only clinical observations, without in-depth histopathological observations. " 01/01/2023 - "Extramammary Paget disease imaged by LC-OCT and treated with radiotherapy." 01/01/2022 - "Treatment-refractory extramammary Paget disease responsive to radiotherapy."
2.	Drug Therapy (Chemotherapy) 05/01/1999 - "We encountered a patient in whom extramammary Paget's disease had metastasized to the lymph nodes beyond the regional lymph nodes and systemic chemotherapy was partially effective. " 08/25/2023 - "Efficacy of chemotherapies for unresectable extramammary Paget disease: a single-centre retrospective study." 02/12/2021 - "The efficacy and survival impact of conventional chemotherapies for metastatic extramammary Paget's disease (EMPD) have not been fully elucidated. " 02/12/2021 - "The Outcome of Chemotherapy for Metastatic Extramammary Paget's Disease." 04/01/2015 - "Invasive cases of extramammary Paget's disease have a poor prognosis, owing to its rapid progression and resistance to irradiation and chemotherapy. "
3.	Therapeutics 07/01/2022 - "Published systemic therapy options for metastatic extramammary Paget's disease have largely been anecdotal due to the rarity of this disease, which has precluded the ability to conduct clinical trials. " 05/01/2021 - "Although topical therapies are used to treat extramammary Paget's disease (EMPD), reliable treatment outcomes and the effects of these therapies on subsequent surgical treatments are unclear. " 01/01/2021 - "Metastatic primary cutaneous extramammary Paget disease (EMPD) is a rare clinical entity with a 5-year survival <10% and no standard therapy. " 11/01/2020 - "Our observations suggest that HER2 should be checked in patients with advanced and/or metastatic extramammary Paget's disease, and that therapy with HER2 blockers should be considered as an option for treatment of HER2-positive extramammary Paget's disease, especially in cases positive for both HER2 gene amplification and overexpression." 01/01/2020 - "Although the prognosis of advanced extramammary Paget's disease (EMPD) is poor, there have been no preclinical research models for the development of novel therapeutics. "
4.	Mohs Surgery (Mohs' Micrographic Surgery) 07/01/2019 - "Surgical excision remains the mainstay of treatment of extramammary Paget disease, and Mohs micrographic surgery is the surgical treatment of choice. " 01/01/2021 - "Modified peripheral and central Mohs micrographic surgery for improved margin control in extramammary Paget disease." 06/01/2012 - "In this study, we discuss our experience with extramammary Paget's disease, along with a novel approach to treatment by using a modification of peripheral Mohs micrographic surgery that incorporates histologic analysis of the central specimen's depth." 01/01/2023 - "Local Recurrence Rates of Extramammary Paget Disease Are Lower After Mohs Micrographic Surgery Compared With Wide Local Excision: A Systematic Review and Meta-Analysis." 02/01/2022 - "The treatment of anogenital extramammary Paget's disease as part of a multidisciplinary approach: The use of Mohs surgery moat method with CK7."
5.	Photochemotherapy (Photodynamic Therapy) 12/03/2019 - "Photodynamic Therapy Using a New Painless Light-Emitting Fabrics Device in the Treatment of Extramammary Paget Disease of the Vulva (the PAGETEX Study): Protocol for an Interventional Efficacy and Safety Trial." 10/01/2023 - "Photodynamic Therapy for Primary Cutaneous Extramammary Paget Disease." 06/01/2023 - "Photodynamic therapy in a patient with perianal extramammary Paget's disease." 01/01/2023 - "Applications of photodynamic therapy in extramammary Paget's disease." 01/01/2023 - "Treatment Outcomes of Adjunct and Stand-alone Photodynamic Therapy in Patients With Extramammary Paget Disease: A Literature Review."