Vigorous treatment of aggressive digital papillary adenocarcinoma (ADPA), including amputation, has been recommended by most authors, but the appropriateness and effectiveness of excision as an alternative to amputation has not been systematically evaluated. To evaluate the appropriateness and effectiveness of excision as an alternative to amputation in the treatment of ADPA, we reviewed the clinical presentations, treatments and patient outcomes presented in case reports on ADPA available on Ovid MEDLINE. We also assessed the results of immunohistochemical staining for proliferation markers in one patient in order to explain the nonaggressive nature of ADPA noted in that patient. Except for the duration of lesions, there was no significant difference in clinical outcome between the excision and amputation groups. We also found that p63 may be a useful marker for distinguishing primary ADPA from metastatic adenocarcinomas. In addition, the intensity of Ki67 expression in tumour cells may be a marker of aggressive behaviour and thus be helpful in therapeutic decision-making. Wide excision with or without sentinel lymph-node biopsy is a feasible alternative to amputation. It should be considered in patients who present with a long-standing history of ADPA without evidence of underlying bone invasion or distant metastasis and with low-intensity expression of proliferation markers.