Digital Papillary Adenocarcinoma: An Updated Review of Epidemiology, Pathogenesis, and Management

BACKGROUND – Digital papillary adenocarcinoma (DPAc) is an uncommon sweat gland malignancy.OBJECTIVE – To review recent updates in epidemiology, pathogenesis, and treatment of DPAc.MATERIALS AND METHODS – A PubMed/MEDLINE search of DPAc, with an emphasis on articles published after 2010.RESULTS – DPAc has an incidence of approximately one in 10 million person-years. Males are more commonly affected than women, and the tumor is commonly diagnosed in the fifth and sixth decades of life. Recent studies have detected human papillomavirus 42 DNA in DPAc, leading to a paradigm shift in the authors' understanding of DPAc oncogenesis. Additional studies also suggest BRAF V600E polymorphisms and fibroblast growth factor receptor dysregulation to be potential drivers of DPAc pathogenesis. Regarding treatment, wide local excision or amputation both appear to afford similar rates of recurrence-free survival; Mohs micrographic surgery is an emerging but understudied modality for DPAc. At present, there are insufficient data to strongly recommend the utilization of sentinel lymph node biopsy.CONCLUSION – Recent molecular advances in DPAc have paved the way for enhanced diagnosis and treatment. Further research on the utility of Mohs micrographic surgery and sentinel lymph node biopsy would be helpful in clarifying the optimal treatment approach for DPAc.