Metastatic extramammary Paget's disease: Pathogenesis and novel therapeutic approach

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Abstract

Extramammary Paget's disease (EMPD) is a rare, slow-growing, cutaneous adenocarcinoma that usually originates in the anogenital area and axillae outside the mammary glands. EMPD mostly progresses slowly and is often diagnosed as carcinoma in situ; however, upon becoming invasive, it promptly and frequently metastasizes to regional lymph nodes, leading to subsequent distant metastasis. To date, several chemotherapy regimens have been used to treat metastatic EMPD; however, they present limited effect and patients with distant metastasis exhibit a poor prognosis. Recently, basic and translational investigative research has elucidated factors and molecular mechanisms underlying the promotion of metastasis, which can lead to targeted therapy-based emerging treatment strategies. Here, we aim to discuss current therapies and their limitations; advancements in illustrating mechanisms promoting invasion, migration, and proliferation of EMPD tumor cells; and future therapeutic approaches for metastatic EMPD that may enhance clinical outcomes.

Original languageEnglish
Article number38
JournalFrontiers in Oncology
Volume8
Issue numberFEB
DOIs
Publication statusPublished - 2018 Feb 16

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Keywords

  • Anti-PD-1 antibody
  • CD163M2 macrophage
  • CXCR4-stromal cell-derived factor-1 axis
  • HER2-PI3K/ERK signaling
  • Lymphangiogenesis
  • Metastatic extramammary Paget's disease
  • Mismatch-repair deficient
  • Receptor activator of nuclear factor kappa-B ligand-RANK signaling

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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