Neoplasms

All Conditions

Skin and Connective Tissue Diseases

Rare Diseases

Paget Disease, Extramammary

Paget's Disease, Mammary

Adenocarcinoma

Carcinoma

Neoplasms, Glandular and Epithelial

Neoplasms by Histologic Type

Breast Neoplasms

Carcinoma in Situ

Breast Carcinoma In Situ

Carcinoma, Intraductal, Noninfiltrating

Extramammary Paget Disease

Paget Disease of the Breast

Dermatologic Agents

All Drugs and Chemicals

Methyl 5-aminolevulinate

Photosensitizing Agents

PAGETEX medical device for photodynamic therapy (PDT). Composed of the association: "Laser source + optical fiber + diffuser support incorporating luminous textiles + drug photosensitizer (Metvixia®)"

2 to 4 sessions of PDT treatment during 2.5 hours after application of Metvixia and incubation under occlusive coat.

pagetex PDT

Laurent Mortier, MD,PhD

Serge Mordon, MD,PhD

Vulvar Paget's disease is a rare skin tumour which affect Caucasian post-menopausal women. The disease is revealed by erythematous, eczematous, pruritus and vulvar burns. The diagnosis is often late (from a few months to several years) because the symptoms are neglected by patients or misinterpreted by doctors. The reference treatment is based on surgical excision but unfortunately local recurrences are very frequent (17 to 38% of cases).

Photodynamic therapy (PDT) is already used in some dermatological pathologies and could therefore be an alternative treatment.

The objective of this study is to assess the efficacy and evaluate the safety of the new PDT device "PAGETEX" for the treatment of vulvar Paget's disease.

Patients diagnosed with Paget's Disease Extra-Mammary Vulvae confirmed by biopsy of less than one year.

PAGETEX® Photodynamic Therapy Device for the Treatment of Extra Mammary Paget's Disease of the Vulva (EMPV).

An Interventional, Phase II, Non Randomized, Mono-centric Study on the Clinical Efficacy and Safety of the Medical Device PAGETEX® as a Photodynamic Therapy Device in the Treatment of Extra-Mammary Paget's Disease of the Vulva (EMPV)

Clinical response will be assessed by vulvar examination by the investigator and independent medical committee. Measurement is defined as complete remission, partial remission (decrease by \>50% of total lesion size), stability ( decrease by \<50% of total lesion size )or progression of the disease

Clinical response will be assessed by vulvar examination by the investigator and independent medical committee. Measurement is defined as complete remission, partial remission (decrease by ≥50% of total lesion size), stability ( decrease by \<50% of total lesion size )or progression of the disease

Visual Analogic Scale Evaluation of pain (0 = no pain to 10= unbearable)

erythema 4 points scale (0 = no erythema, 3=severe erythema) and Chroma meter CR400 (Konica Minolta) measures

DLQI is a ten-question questionnaire used to measure the impact of skin disease on the quality of life of an affected person The scoring of each question is as follows:Very much/A lot/A little/Not at all/Not relevan

Each item on the questionnaire is scored from 0-3

The FSFI is a brief questionnaire measure of sexual functioning in women. 19 questions scoring as follow : 0 = Did not attempt intercourse ;1 = Almost always or always 2 = Most times (more than half the time);3 = Sometimes (about half the time);4 = A few times (less than half the time); 5 = Almost never or never

Incidence and severity of adverse device effects during the study period

Galderma R&D

Institut National de la Santé Et de la Recherche Médicale, France

University Hospital, Lille

Anti-Arrhythmia Agents

Channel Blockers

Central Nervous System Depressants

Antineoplastic Agents

Antipyretics

Analgesics

Anti-Infective Agents

Lidocaine

Imiquimod

Petrolatum

Acetaminophen

Anesthetics

Anesthetics, Local

Sodium Channel Blockers

Diuretics, Potassium Sparing

Analgesics, Non-Narcotic

Immunomodulating Agents

Immunologic Factors

Adjuvants, Immunologic

Interferons

Emollients

Topical 5% imiquimod cream will be applicated to the vulvar skin lesion 3 times a week during 16 weeks.

topical 5% imiquimod cream 3 times a week for 16 weeks.

paracetamol

lidocaine in Vaseline ointment

Joanne de Hullu, MD PhD

The purpose of this study is to evaluate the efficacy, safety and immunological response of topical 5% imiquimod cream for non-invasive vulvar Paget's disease.

Vulvar Paget's disease is a rare skin tumour and generally seen in postmenopausal, Caucasian women as an erythematous, eczematous, scaling or ulcerating plaque. Initial symptoms are irritation, burning, pain and a skin lesion. The origin of vulvar Paget's disease is not yet entirely understood. The treatment of choice is surgical excision, despite high recurrence rates. The mutilating consequences of surgery can lead to impressive morbidity to address this, several series report the use of topical 5% imiquimod cream for vulvar Paget's disease.

The objective of this study is to assess the efficacy, evaluate the safety, immunological effect of topical 5% imiquimod cream for vulvar Paget's disease and the quality of life during treatment.

Topical 5% Imiquimod Cream for Vulvar Paget's Disease

Topical 5% Imiquimod Cream for Vulvar Paget's Disease: Clinical Efficacy, Safety and Immunological Response

Clinical response will be assessed by vulvar examination and measurement and is defined as complete remission, partial remission (decrease by ≥50% of total lesion size) or no remission.

Tolerability will be assessed in a descriptive manner with a tolerability questionnaire with the following questions:

1. Have you used paracetamol during treatment?

   1. How many days?
   2. What dosage?
   3. Was the use sufficient?
2. Have you used lidocaine in Vaseline ointment during treatment?

   1. How many days?
   2. What dosage?
   3. Was the use sufficient?
3. Have you stopped the treatment with imiquimod because of side effects/pain?

Most common adverse events will be evaluated with the following questions:

1. Have you been nauseous? No / a little (less than 3 days per week) / severe (more than 3 days a week) / Yes, daily.
2. Have you had a head ache? No / a little (less than 3 days per week) / severe (more than 3 days a week) / Yes, daily.
3. Have you felt more tired than you normally are? No / a little (less than 3 days per week) / severe (more than 3 days a week) / Yes, daily.

Vulvar skin biopsies will be taken at baseline, 4 and 28 weeks after the start of

The questionnaire EQ5D (EuroQOL 5 Dimensions questionnaire) will be used.

The questionnaire DLQI (Dermatology Life Quality Index) will be used.

The questionnaire FSDS (Female Sexual Distress Scale) will be used.

Leiden University Medical Center

University Medical Center Groningen

Erasmus Medical Center

The Netherlands Cancer Institute

UMC Utrecht

Catharina Ziekenhuis Eindhoven

University Medical Center Nijmegen

MD PhD

Urinary Tract, Sexual Organs, and Pregnancy Conditions

Skin Diseases

Vaginal Diseases

Vulvar Diseases

All women presenting for evaluation of Extramammary Paget's Disease (EMPD) at Mayo Clinic in Rochester MN.

Maureen A. Lemens, R.N.

Karen P Ishitani, RN

Jamie N. Bakkum-Gamez, M.D.

In addition to evaluating treatment outcomes, tissue samples and swabs will be collected as part of this study to allow physicians to better understand Extramammary Paget's Disease (EMPD). The researchers also want to learn more about the microorganisms (microbiome) that live on or near the areas of skin affected by EMPD, in order to better understand this condition.

This is a prospective cohort study. Patients with Extramammary Paget's Disease (EMPD), specifically women with vulvar/perianal Paget's disease, will be identified during presentation to either the Dermatology Department or Gynecologic Surgery department at Mayo Clinic.

During physical exam, the patients will undergo skin/rectum/vagina cultures to evaluate the microbiome in these patients. The patients will be entered into the established care pathway for EMPD. They will be asked to complete sexual health questionnaires including: The Women's Health Clinical Questionnaires include the Menopause Rating Scale (MRS)1, Perceived Stress Scale2, Brief Resilience Scale (BRS)3, LASA Quality of Life Assessment4, Female Sexual Function Index (FSFI)5, Female Sexual Distress Scale-Revised6, Kansas Marital Satisfaction Scale7, and Pittsburgh Sleep Quality Index (PSQI)8.

The patients will be referred to Women's Health Clinic as part of the care pathway and will undergo preoperative genital sensory testing (GST) which measures sensation of the clitoris, vulva, and vagina. The patients will have a Dermatology consultation and Moh's micrographic procedure as indicated. If choosing to proceed with surgical intervention, the patients will then undergo an excisional procedure with the Department of Gynecologic surgery. Swabs, urine and tissue will be collected prior to procedures or surgery for microbiome analysis.

Tumor tissues will be obtained in order to perform tumor molecular profiling to identify targetable somatic mutations in EMPD.

The vulvar and perianal microbiome of women with extramammary Paget's disease will be evaluated.

Gynecologic Extramammary Paget's Disease

Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease

Prospective data including patient demographic information, surgical outcomes, surgical morbidity, recurrence, and long-term survival in extramammary Paget's disease (EMPD) will be collected as part of this registry..

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
PAGETEX® Photodynamic Therapy Device for the Treatment of Extra Mammary Paget's Disease of the Vulva (EMPV).	NCT03713203	Paget Disease o... Paget Disease, ...	pagetex PDT	18 Years -	University Hospital, Lille
Topical 5% Imiquimod Cream for Vulvar Paget's Disease	NCT02385188	Paget Disease, ...	Imiquimod paracetamol lidocaine in Va...	18 Years -	University Medical Center Nijmegen
PAGETEX® Photodynamic Therapy Device for the Treatment of Extra Mammary Paget's Disease of the Vulva (EMPV).	NCT03713203	Paget Disease o... Paget Disease, ...	pagetex PDT	18 Years -	University Hospital, Lille
Gynecologic Extramammary Paget's Disease	NCT03564483	Extramammary Pa... Extramammary Pa... Paget Disease, ... Extramammary Pa... Extramammary Pa... Extramammary Pa... Extramammary Pa... Extramammary Pa... Extramammary Pa...		18 Years -	Mayo Clinic

Spots Global Cancer Trial Database for paget disease, extramammary