Aggressive Digital Papillary Adenocarcinoma: A Meta-Analysis of the Presentation, Treatment, and Outcomes of a Rare Hand Tumor.

BACKGROUND

Aggressive digital papillary adenocarcinoma (ADPA) is a rare skin adnexal tumor with a predilection for the hand. The presentation, treatment, and outcomes of ADPA remain poorly defined due to the scarcity of reports and low-level evidence of published findings.

METHODS

We performed a meta-analysis following Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines with the intent to provide hand surgeons a better understanding of the diagnosis and treatment of the disease. Three additional cases of ADPA from our institution were combined with the results from the systematic review.

RESULTS

A total of 238 cases were included in the meta-analysis. Most ADPAs were painful, evolving, and less than 2 cm in size. Regional sentinel lymph node biopsies were performed in 55 patients, of which 25.5% were positive. Overall cohort recurrence and distant metastasis rates were 24.3% and 18.1%, respectively. Patients who obtained clear margins within 6 months of diagnosis had significantly lower rates of recurrence, metastasis, and mortality ( < .001, = .04, = .003, respectively). There were no differences in recurrence or metastasis rates in those who obtained clear margins by excision or amputation ( = .27 and = .07, respectively). In patients who died from disease (6.0%), 5.0 years was the average time to death.

CONCLUSIONS

Aggressive digital papillary adenocarcinoma should be included in the differential diagnosis of hand lesions with unusual presentations or lack of response to other therapies. Clear margins should be obtained within 6 months of diagnosis, and routine surveillance should be provided by an oncologic physician familiar with ADPA as these tumors have high recurrence rates.