Recommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic subtype - 17/01/25

Doi : 10.1016/j.jaad.2024.07.1531

Nour Kibbi, MD ^a, Joshua L. Owen, MD, PhD ^b, Brandon Worley, MD, MSc ^c, Murad Alam, MD, MSCI, MBA ^d,^⁎
Extramammary Paget's Disease Guideline Study Group
Jake X. Wang, MD ^e, Vishnu Harikumar, BS ^f, Malia B. Downing, MD ^g, Sumaira Z. Aasi, MD ^h, Phyu P. Aung, MD, PhD ⁱ, Christopher A. Barker, MD ^j, Diana Bolotin, MD, PhD ^k, Jeremy S. Bordeaux, MD, MPH ^h, Todd V. Cartee, MD ^l, Sunandana Chandra, MD ^m, Nancy L. Cho, MD ⁿ, Jennifer N. Choi, MD ^o, Kee Yang Chung, MD, PhD ^p, William A. Cliby, MD ^q,^r, Oliver Dorigo, MD, PhD ^s, Daniel B. Eisen, MD ^t, Yasuhiro Fujisawa, MD, PhD ^u, Nicholas Golda, MD ^v, Thorvardur R. Halfdanarson, MD ^w, Christos Iavazzo, MD, MSc, PhD ^x, Shang I. Brian Jiang, MD ^y, Esther M. John, PhD, MSPH ^z,^aa,^ab, Jean Kanitakis, MD, PhD ^ac, Emily L. Keimig, MD ^f, Ashraf Khan, MD, FRCPath ^ad, Eleni Linos, MD, MPH, DrPH ^h,^ab,^ae, John Y.S. Kim, MD ^af, Timothy M. Kuzel, MD ^ag, Naomi Lawrence, MD ^ah, Mario M. Leitao, MD ^ai,^aj, Allan B. MacLean, MD ^ak, Ian A. Maher, MD ^al, Bharat B. Mittal, MD ^am, Kishwer S. Nehal, MD ^an, David M. Ozog, MD ^ao, Curtis A. Pettaway, MD ^ap, Neil Rajan ^aq,^ar, Jeffrey S. Ross, MD ^as, Anthony M. Rossi, MD ^at, Sabah Servaes, MD ^at, Michael J. Solomon, DMedSc, Dmed ^au, Valencia D. Thomas, MD ^av, Maria Tolia, MD, MSc, PhD ^aw, Bryan B. Voelzke, MD, MS ^ax, Abigail Waldman, MD ^ay, Michael K. Wong, MD, PhD ^az, Youwen Zhou, MD, PhD ^ba, Alexandria Brackett, MA, MLIS ^bb, Emily Poon, PhD ^o, Areeba Ahmed, MD ^o
^e University Hospitals Cleveland Medical Center Department of Dermatology, Case Western Reserve University, Cleveland, Ohio
^f Department of Dermatology, Miami School of Medicine, University of Miami, Miami, Florida
^g Cahaba Dermatology, Birmingham, Alabama
^h Department of Dermatology, Stanford University School of Medicine, Palo Alto, California
ⁱ Department of Pathology (Dermatopathology), University of Texas M.D. Anderson Cancer Center, Houston, Texas
^j Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York
^k Section of Dermatology, University of Chicago, Chicago, Illinois
^l Department of Dermatology, Pennsylvania State College of Medicine, Hershey, Pennsylvania
^m Division of Hematology Oncology, Department of Medicine, Northwestern University Medical Center, Chicago, Illinois
ⁿ Department of Surgery, Brigham and Women's Hospital, Harvard University, Boston, Massachusetts
^o Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
^p Department of Dermatology, Yonsei University College of Medicine, Seoul, South Korea
^q Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota
^r Virgil S. Counseller MD Professor of Surgery
^s Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Stanford Women's Cancer Center, Cancer Institute, Stanford University School of Medicine, Stanford, California
^t Department of Dermatology, University of California Davis, Sacramento, California
^u Department of Dermatology, Ehime University, Toon, Japan
^v Department of Dermatology, University of Missouri School of Medicine, Columbia, Missouri
^w Division of Medical Oncology, Department of Oncology, Mayo Clinic, Rochester, Minnesota
^x Department of Gynecologic Oncology, Metaxa Cancer Hospital, Piraeus, Greece
^y Department of Dermatology, University of California San Diego, San Diego, California
^z Department of Epidemiology and Population Health, Stanford University School of Medicine, Stanford, California
^aa Department of Medicine (Oncology), Stanford University School of Medicine, Stanford, California
^ab Stanford Cancer Institute, Stanford University School of Medicine, Stanford, California
^ac Department of Dermatology, Ed. Herriot Hospital Group (Pav. R), Lyon, France
^ad Department of Pathology, University of Massachusetts Medical School-Baystate, Baystate Health, Springfield, Massachusetts
^ae Center for Digital Health (Department of Medicine), Stanford University School of Medicine, Stanford, California
^af Division of Plastic and Reconstructive Surgery, Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
^ag Division of Hematology, Oncology and Cell Therapy, Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois
^ah Division of Dermatology, Cooper Hospital, Rowan University, Camden, New Jersey
^ai Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York
^aj Department of OB/GYN, Weill Cornell Medical College, New York, New York
^ak Department of Gynaecology, University College, London, United Kingdom
^al Department of Dermatology, University of Minnesota, Minneapolis, Minnesota
^am Department of Radiation Oncology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
^an Dermatology Service, Memorial Sloan Kettering Cancer Center, New York, New York
^ao Department of Dermatology, Henry Ford Hospital, Detroit, Michigan
^ap Division of Surgery, Department of Urology, University of Texas M.D. Anderson Cancer Center, Houston, Texas
^aq Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom
^ar Department of Dermatology and NIHR Newcastle Biomedical Research Centre, Newcastle Hospitals NHS Foundation, Newcastle upon Tyne, United Kingdom
^as Departments of Pathology and Urology, Upstate Medical University, Syracuse, New York
^at Department of Radiology, West Virginia University Children's Hospital, Charleston, West Virginia
^au Department of Colorectal Surgery, Royal Prince Alfred Hospital, Sydney, Australia
^av Department of Dermatology, University of Texas M.D. Anderson Cancer Center, Houston, Texas
^aw Department of Radiotherapy, School of Medicine, University of Crete, Heraklion, Crete, Greece
^ax Spokane Urology, Spokane, Washington
^ay Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
^az Division of Cancer Medicine, Department of Melanoma Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, Texas
^ba Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada
^bb Harvey Cushing/John Hay Medical Library, Yale School of Medicine, Yale University, New Haven, Connecticut

^a Department of Dermatology, Stanford University School of Medicine, Palo Alto, California
^b Department of Medicine/Dermatology & Cutaneous Surgery, University of Texas Health Science Center at San Antonio, San Antonio, Texas
^c Florida Dermatology and Skin Cancer Centers, Lake Wales, Florida
^d Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois

^∗Correspondence to: Murad Alam, MD, MSCI, MBA, Department of Dermatology, Northwestern Memorial Hospital, Arkes Family Pavilion, Suite 1600, 676 N Saint Clair, Chicago, IL 60611.Department of DermatologyNorthwestern Memorial HospitalArkes Family PavilionSuite 1600676 N Saint ClairChicagoIL60611

Abstract

Introduction

Extramammary Paget's disease (EMPD) may be associated with an underlying internal adenocarcinoma, referred to as secondary EMPD. Differences in this association by EMPD anatomic subtype and implications for screening are not fully understood.

Objective

Define the rates of secondary EMPD and types of associated adenocarcinomas by EMPD anatomic subtype and propose a screening algorithm for underlying adenocarcinoma.

Methods

Systematic literature review of EMPD (January 1990-November 2022). One hundred twenty-two studies met the inclusion criteria. A multidisciplinary expert panel reviewed the recommendation statements on adenocarcinoma screening.

Results

Perianal EMPD was associated with a high rate of underlying adenocarcinoma (25%, primarily colorectal) compared with penoscrotal and vulvar EMPD (6% each, primarily of genitourinary origin). Thorough screening in perianal EMPD includes a colonoscopy, urine cytology, and computed tomography of the chest, abdomen, and pelvis. Cost-conscious screening tests in low-risk penoscrotal disease include urine cytology, heme-occult test, and prostate-specific antigen test (especially if under 70 years of age). For low-risk vulvar EMPD, urine cytology and mammography are recommended. EMPD with high-risk features may warrant more sensitive organ-specific testing.

Limitations

Selection bias; retrospective data without systematic follow-up.

Conclusions

Screening for underlying adenocarcinoma in EMPD should be guided by anatomic location.

Le texte complet de cet article est disponible en PDF.

Key words : adenocarcinoma, cancer screening, extramammary Paget's disease, secondary EMPD

Abbreviation used : CISTERN, CT, EMPD, GRADE, PSA, USPSTF

Plan

Introduction

Methods

Systematic review search strategy and selection criteria

Medical decision-making disclaimer

Discussion

	Funding sources: None.
	Patient consent: The patient provided consent for use of their clinical information for the purposes of publication.
	IRB approval status: Not applicable.
	Members of Extramammary Paget's Disease Guideline Study Group: Jake X. Wang, MD,^e Vishnu Harikumar, BS,^f Malia B. Downing, MD,^g Sumaira Z. Aasi, MD,^h Phyu P. Aung, MD, PhD,ⁱ Christopher A. Barker, MD,^j Diana Bolotin, MD, PhD,^k Jeremy S. Bordeaux, MD, MPH,^h Todd V. Cartee, MD,^l Sunandana Chandra, MD,^m Nancy L. Cho, MD,ⁿ Jennifer N. Choi, MD,^o Kee Yang Chung, MD, PhD,^p William A. Cliby, MD,^q,r Oliver Dorigo, MD, PhD,^s Daniel B. Eisen, MD,^t Yasuhiro Fujisawa, MD, PhD,^u Nicholas Golda, MD,^v Thorvardur R. Halfdanarson, MD,^w Christos Iavazzo, MD, MSc, PhD,^x Shang I. Brian Jiang, MD,^y Esther M. John, PhD, MSPH,^z,aa,ab Jean Kanitakis, MD, PhD,^ac Emily L. Keimig, MD,^f Ashraf Khan, MD, FRCPath,^ad Eleni Linos, MD, MPH, DrPH,^h,ab,ae John Y.S. Kim, MD,^af Timothy M. Kuzel, MD,^ag Naomi Lawrence, MD,^ah Mario M. Leitao Jr, MD,^ai,aj Allan B. MacLean, MD,^ak Ian A. Maher, MD,^al Bharat B. Mittal, MD,^am Kishwer S. Nehal, MD,^an David M. Ozog, MD,^ao Curtis A. Pettaway, MD,^ap Neil Rajan,^aq,ar Jeffrey S. Ross, MD,^as Anthony M. Rossi, MD,^at Sabah Servaes, MD,^at Michael J. Solomon, DMedSc, Dmed,^au Valencia D. Thomas, MD,^av Maria Tolia, MD, MSc, PhD,^aw Bryan B. Voelzke, MD, MS,^ax Abigail Waldman, MD,^ay Michael K. Wong, MD, PhD,^az Youwen Zhou, MD, PhD,^ba Alexandria Brackett, MA, MLIS,^bb Emily Poon, PhD^o Areeba Ahmed, MD.^o University Hospitals Cleveland Medical Center Department of Dermatology, Cleveland, Ohio^{^e}; Case Western Reserve University, Cleveland, Ohio^{^f}; Department of Dermatology, Miami School of Medicine, University of Miami, Miami, Florida^{^g}; Cahaba Dermatology, Birmingham, Alabama^{^h}; Department of Dermatology, Stanford University School of Medicine, Palo Alto, California^ⁱ; Department of Pathology (Dermatopathology), University of Texas M.D. Anderson Cancer Center, Houston, Texas^{^j}; Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York^{^k}; Section of Dermatology, University of Chicago, Chicago, Illinois^{^l}; Department of Dermatology, Pennsylvania State College of Medicine, Hershey, Pennsylvania^{^m}; Division of Hematology Oncology, Department of Medicine, Northwestern University Medical Center, Chicago, Illinois^ⁿ; Department of Surgery, Brigham and Women's Hospital, Harvard University, Boston, Massachusetts^{^o}; Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois^{^p}; Department of Dermatology, Yonsei University College of Medicine, Seoul, South Korea^{^q}; Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota^{^r}; Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Stanford Women's Cancer Center, Cancer Institute, Stanford University School of Medicine, Stanford, California^{^s}; Department of Dermatology, University of California Davis, Sacramento, California^{^t}; Department of Dermatology, Ehime University, Toon, Japan^{^u}; Department of Dermatology, University of Missouri School of Medicine, Columbia, Missouri^{^v}; Division of Medical Oncology, Department of Oncology, Mayo Clinic, Rochester, Minnesota^{^w}; Department of Gynecologic Oncology, Metaxa Cancer Hospital, Piraeus, Greece^{^x}; Department of Dermatology, University of California San Diego, San Diego, California^{^y}; Department of Epidemiology and Population Health, Stanford University School of Medicine, Stanford, California^{^z}; Department of Medicine (Oncology), Stanford University School of Medicine, Stanford, California^{^aa}; Stanford Cancer Institute, Stanford University School of Medicine, Stanford, California^{^ab}; Department of Dermatology, Ed. Herriot Hospital Group (Pav. R), Lyon, France^{^ac}; Department of Pathology, University of Massachusetts Medical School-Baystate, Baystate Health, Springfield, Massachusetts^{^ad}; Center for Digital Health (Department of Medicine), Stanford University School of Medicine, Stanford, California^{^ae}; Division of Plastic and Reconstructive Surgery, Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois^{^af}; Division of Hematology, Oncology and Cell Therapy, Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois^{^ag}; Division of Dermatology, Cooper Hospital, Rowan University, Camden, New Jersey^{^ah}; Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York^{^ai}; Department of OB/GYN, Weill Cornell Medical College, New York, New York^{^aj}; Department of Gynaecology, University College, London, United Kingdom^{^ak}; Department of Dermatology, University of Minnesota, Minneapolis, Minnesota^{^al}; Department of Radiation Oncology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois^{^am}; Dermatology Service, Memorial Sloan Kettering Cancer Center, New York, New York^{^an}; Department of Dermatology, Henry Ford Hospital, Detroit, Michigan^{^ao}; Division of Surgery, Department of Urology, University of Texas M.D. Anderson Cancer Center, Houston, Texas^{^ap}; Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom^{^aq}; Department of Dermatology and NIHR Newcastle Biomedical Research Centre, Newcastle Hospitals NHS Foundation, Newcastle upon Tyne, United Kingdom^{^ar}; Departments of Pathology and Urology, Upstate Medical University, Syracuse, New York^{^as}; Department of Radiology, West Virginia University Children's Hospital, Charleston, West Virginia^{^at}; Department of Colorectal Surgery, Royal Prince Alfred Hospital, Sydney, Australia^{^au}; Department of Dermatology, University of Texas M.D. Anderson Cancer Center, Houston, Texas^{^av}; Department of Radiotherapy, School of Medicine, University of Crete, Heraklion, Crete, Greece^{^aw}; Spokane Urology, Spokane, Washington^{^ax}; Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts^{^ay}; Division of Cancer Medicine, Department of Melanoma Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston, Texas^{^az}; Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada^{^ba}; and Harvey Cushing/John Hay Medical Library, Yale School of Medicine, Yale University, New Haven, Connecticut.^{^bb}

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Recommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic subtype - 17/01/25

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