Aggressive digital papillary adenocarcinomas (ADPA) are malignant tumors of sweat glands having recurrence/metastatic potential.

We sought to describe the clinical/histopathological characteristics of a series of ADPA.

This is a retrospective case series of 19 ADPA.

The tumors occurred in 17 men and 2 women (mean age: 47 years). They involved digits (15), big toe (3), and palm (1), and measured from 3 to 30 mm. They were mostly solid and cystic, with papillary projections and tubular structures. Atypia was mostly mild to moderate. Tumors tested positive for p63, keratin 7, keratin 77 (eccrine duct-specific), PHLDA1, and epithelial membrane antigen in most cases, and for carcinoembryonic antigen, smooth muscle actin, S100 protein, estrogen, progesterone, and androgen receptors in 50%. Mean Ki67 proliferation index was 15%. Local recurrence was observed in 4 cases. One patient had axillary lymph node metastasis. Histopathologic parameters were not predictive of evolution. Conservative surgical treatment, performed in 7 of 19 cases, did not result in more recurrences than amputation.

The study was retrospective and the number of cases is small.

ADPA are histologically variable, but papillary projections are always present. Keratin 77 expression suggests an eccrine origin. P63 is helpful to exclude metastasis. Conservative surgery may be sufficient in some cases.