Case Of Aggressive Digital Papillary Adenocarcinoma Research Articles

Aggressive Digital Papillary Adenocarcinoma: Population-Based Analysis of Incidence, Demographics, Treatment, and Outcomes

As a rare cutaneous malignancy, epidemiologic and outcomes data for aggressive digital papillary adenocarcinoma (ADPA) are limited and no treatment guidelines exist. To provide a population-based study of ADPA incidence and outcomes with a subgroup comparison of patients with localized versus regional disease. Data from 18 registries within the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program were examined for patients with ADPA (1995-2013) to provide demographic- and cancer-related information, and to calculate race- and age-specific rate ratios, incidence, and mortality. Patients were stratified by the stage for further comparison. Ninety-four cases of ADPA were identified. Overall, ADPA incidence was 0.08 per 1,000,000 person-years, 4 times higher in males than in females (0.13 vs 0.03, p < .001), and most common in Caucasians. Regional disease spread occurred in 22.3% of patients and disease-specific mortality in 2.1% of patients. Patients with regional versus localized disease at diagnosis did not differ significantly in sex, age, race, primary site, tumor size, or mortality. Aggressive digital papillary adenocarcinoma is a rare malignancy with increasing incidence. Regional disease spread is not infrequent, but mortality is rare. Identification of patients best suited for additional diagnostic procedures or more extensive surgical resection remains challenging.

Aggressive digital papillary adenocarcinoma: A report of two diseases and review of the literature

Aggressive digital papillary adenocarcinoma (ADPAca) is a rare, underreported, and often misdiagnosed malignant tumor of the sweat glands most commonly occurring in males in their fifties to seventies. We report two cases of ADPAca with important clinical implications. A 54-year-old man presented 3 years after digit amputation for ADPAca with new blue nodules on his arm, lymphadenopathy, and a lung nodule; he was diagnosed with and treated for metastatic ADPAca. He underwent chemotherapy, but died 4 months later. A 15-year-old boy presented with an enlarging tumor on his finger occurring after a trauma 3 years earlier. The tumor was suspected to be a deep fungal infection or pyogenic granuloma; however, results of excisional biopsy revealed an ADPAca. The patient underwent amputation and sentinel lymph node examination. No signs of metastases were found, and he is alive and well. These cases highlight both the importance of high clinical suspicion of digital tumors, even in children, enabling prompt diagnosis and treatment and also emphasize the metastatic potential of the tumor and the need for aggressive treatment and close long-term follow-up.