Nour Kibbi1, Joshua L Owen2, Brandon Worley3, Jake X Wang4, Vishnu Harikumar5, Malia B Downing6, Sumaira Z Aasi1, Phyu P Aung7, Christopher A Barker8, Diana Bolotin9, Jeremy S Bordeaux10, Todd V Cartee11, Sunandana Chandra12, Nancy L Cho13, Jennifer N Choi5, Kee Yang Chung14, William A Cliby15, Oliver Dorigo16, Daniel B Eisen17, Yasuhiro Fujisawa18, Nicholas Golda19, Thorvardur R Halfdanarson20, Christos Iavazzo21, Shang I Brian Jiang22, Jean Kanitakis23, Ashraf Khan24, John Y S Kim25, Timothy M Kuzel26, Naomi Lawrence27, Mario M Leitao28,29, Allan B MacLean30, Ian A Maher31, Bharat B Mittal32, Kishwer S Nehal33, David M Ozog34, Curtis A Pettaway35, Jeffrey S Ross36,37, Anthony M Rossi33, Sabah Servaes38, Michael J Solomon39, Valencia D Thomas40, Maria Tolia41, Bryan B Voelzke42, Abigail Waldman43, Michael K Wong44, Youwen Zhou45, Nobuo Arai46, Alexandria Brackett47, Sarah A Ibrahim5, Bianca Y Kang5, Emily Poon5, Murad Alam5,48,49,50. 1. Department of Dermatology, Stanford University School of Medicine, Redwood City, California. 2. Dermatology Service, South Texas Veterans Health Care System, San Antonio. 3. Florida Dermatology and Skin Cancer Centers, Lake Wales. 4. Department of Dermatology, Yale School of Medicine, Yale University, New Haven, Connecticut. 5. Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 6. Division of Dermatology, University of Kansas, Kansas City. 7. Department of Pathology (Dermatopathology), The University of Texas MD Anderson Cancer Center, Houston. 8. Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York. 9. Section of Dermatology, University of Chicago, Chicago, Illinois. 10. University Hospitals Cleveland Medical Center, Department of Dermatology, Case Western Reserve University, Cleveland, Ohio. 11. Division of Dermatology, Pennsylvania State College of Medicine, Hershey. 12. Division of Oncology, Department of Medicine, Northwestern University Medical Center, Chicago, Illinois. 13. Department of Surgery, Brigham and Women's Hospital, Harvard University, Boston, Massachusetts. 14. Department of Dermatology, Yonsei University College of Medicine, Seoul, South Korea. 15. Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota. 16. Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Stanford Women's Cancer Center, Cancer Institute, Stanford University School of Medicine, Stanford, California. 17. Department of Dermatology, University of California Davis, Sacramento. 18. Department of Dermatology, University of Tsukuba, Tsukuba, Japan. 19. Department of Dermatology, University of Missouri School of Medicine, Columbia. 20. Division of Medical Oncology, Department of Oncology, Mayo Clinic, Rochester, Minnesota. 21. Department of Gynecologic Oncology, Metaxa Cancer Hospital, Piraeus, Greece. 22. Department of Dermatology, University of California San Diego, San Diego. 23. Department of Dermatology, Ed. Herriot Hospital Group (Pav. R), Lyon, France. 24. Department of Pathology, University of Massachusetts Medical School-Baystate, Baystate Health, Springfield. 25. Division of Plastic and Reconstructive Surgery, Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 26. Division of Hematology, Oncology and Cell Therapy, Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois. 27. Division of Dermatology, Cooper Hospital, Rowan University, Camden, New Jersey. 28. Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York. 29. Department of Obstetrics and Gynecology, Weill Cornell Medical College, New York, New York. 30. Department of Gynaecology, University College, London, United Kingdom. 31. Department of Dermatology, University of Minnesota, Minneapolis. 32. Department of Radiation Oncology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 33. Dermatology Service, Memorial Sloan Kettering Cancer Center, New York, New York. 34. Department of Dermatology, Henry Ford Hospital, Detroit, Michigan. 35. Division of Surgery, Department of Urology, The University of Texas MD Anderson Cancer Center, Houston. 36. Department of Pathology, Upstate Medical University, Syracuse, New York. 37. Department of Urology, Upstate Medical University, Syracuse, New York. 38. Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia. 39. Department of Colorectal Surgery, Royal Prince Alfred Hospital, Sydney, Australia. 40. Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston. 41. Department of Radiotherapy, School of Medicine, University of Crete, Heraklion, Crete, Greece. 42. Spokane Urology, Spokane, Washington. 43. Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. 44. Division of Cancer Medicine, Department of Melanoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston. 45. Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada. 46. Electrical Engineering, Kanagawa University, Kanagawa-ku, Yokohama, Japan. 47. Cushing/Whitney Medical Library, Yale School of Medicine, Yale University, New Haven, Connecticut. 48. Department of Otolaryngology-Head and Neck Surgery and Surgery (Organ Transplantation), Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 49. Department of Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. 50. Department of Medical Social Sciences, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.
Abstract
IMPORTANCE: Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. OBJECTIVE: To develop recommendations for the care of adults with EMPD. EVIDENCE REVIEW: A systematic review of the literature on EMPD from January 1990 to September 18, 2019, was conducted using MEDLINE, Embase, Web of Science Core Collection, and Cochrane Libraries. Analysis included 483 studies. A multidisciplinary expert panel evaluation of the findings led to the development of clinical care recommendations for EMPD. FINDINGS: The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years. CONCLUSIONS AND RELEVANCE: Clinical practice guidelines for EMPD provide guidance regarding recommended diagnostic approaches, differentiation between invasive and noninvasive disease, and use of surgical vs nonsurgical treatments. Prospective registries may further improve our understanding of the natural history of the disease in primary vs secondary EMPD, clarify features of high-risk tumors, and identify superior management approaches.
IMPORTANCE: Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. OBJECTIVE: To develop recommendations for the care of adults with EMPD. EVIDENCE REVIEW: A systematic review of the literature on EMPD from January 1990 to September 18, 2019, was conducted using MEDLINE, Embase, Web of Science Core Collection, and Cochrane Libraries. Analysis included 483 studies. A multidisciplinary expert panel evaluation of the findings led to the development of clinical care recommendations for EMPD. FINDINGS: The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years. CONCLUSIONS AND RELEVANCE: Clinical practice guidelines for EMPD provide guidance regarding recommended diagnostic approaches, differentiation between invasive and noninvasive disease, and use of surgical vs nonsurgical treatments. Prospective registries may further improve our understanding of the natural history of the disease in primary vs secondary EMPD, clarify features of high-risk tumors, and identify superior management approaches.
Authors: Emma Zattarin; Federico Nichetti; Francesca Ligorio; Laura Mazzeo; Riccardo Lobefaro; Giovanni Fucà; Giorgia Peverelli; Andrea Vingiani; Giulia V Bianchi; Giuseppe Capri; Filippo de Braud; Claudio Vernieri Journal: Front Oncol Date: 2022-08-18 Impact factor: 5.738