Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
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Kibbi Nour, Owen Joshua L, Worley Brandon, Wang Jake X, Harikumar Vishnu, Downing Malia B, Aasi Sumaira Z, Aung Phyu P, Barker Christopher A, Bolotin Diana, Bordeaux Jeremy S, Cartee Todd V, Chandra Sunandana, Cho Nancy L, Choi Jennifer N, Chung Kee Yang, Cliby William A, Dorigo Oliver, Eisen Daniel B, Fujisawa Yasuhiro, Golda Nicholas, Halfdanarson Thorvardur R, Iavazzo Christos, Jiang Shang I Brian, Kanitakis Jean, Khan Ashraf, Kim John Y S, Kuzel Timothy M, Lawrence Naomi, Leitao Mario M, MacLean Allan B, Maher Ian A, Mittal Bharat B, Nehal Kishwer S, Ozog David M, Pettaway Curtis A, Ross Jeffrey S, Rossi Anthony M, Servaes Sabah, Solomon Michael J, Thomas Valencia D, Tolia Maria, Voelzke Bryan B, Waldman Abigail, Wong Michael K, Zhou Youwen, Arai Nobuo, Brackett Alexandria, Ibrahim Sarah A, Kang Bianca Y, Poon Emily, Alam Murad
Аннотация
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.