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Treatment of Aggressive Digital Papillary Adenocarcinoma—Amputation Versus Digit Salvage
Shaun D. Mendenhall, MD; Ryan W. Schmucker, MD; Jennifer L. Koechle, MD; Steven J. Verhulst, MD; Michael W. Neumeister, MD
Southern Illinois University School of Medicine, Springfield, IL

Introduction: Aggressive digital papillary adenocarcinoma (ADPA) is a rare tumor of eccrine sweat gland origin with predilection for the hand. Because the rarity of this tumor, published data include only case reports or small case series, making it exceptionally difficult to define an appropriate treatment algorithm. The purpose of this study was to pool the current world literature in order to compare outcomes between amputation and digit salvage.
Methods: A Medline database search was performed to locate all ADPA cases reported in the world literature. Translations were performed as necessary. We included reports of individual variables and excluded reports without case-specific information. The data was compiled and a meta-analysis of treatment methods and outcomes was performed. Descriptive statistics and intergroup comparisons were performed with a p value of <0.05 considered significant.
Results: There were 217 cases were reported in the literature, 174 of which were included in this aggregate data analysis (including 3 of our own cases). Mean patient age was 47 and 82% were male. Average tumor size was 2 cm and 82% were on the upper extremity. Duration of lesion until diagnosis was 4.6 years and 5.2% had a history of trauma to the affected area. The majority were treated by excision (88% vs. 11% with amputation). The overall recurrence rate was 34%, and overall metastasis rate was 33%. Recurrence rate was higher for excision compared to amputation (38% vs. 13%, p=0.087), however metastatic rates were similar (30% vs. 33%, p=0.79). A delay in treatment after the initial diagnosis for > 6 months led to a higher recurrence and metastatic rate (Figure 1).

Conclusion: To our knowledge, the current report is the only meta-analysis of published ADPA cases and the largest series comparing outcomes of amputation vs. digit salvage. Recurrence rate was lower in patients who had amputations, although this did not reach significance. There was no difference in metastatic rates. Due to implications of amputation on form and function, wide local excision may offer similar benefits without the detriments of losing a digit. Furthermore, a definitive surgical procedure within 6 months of diagnosis was shown to significantly reduce recurrence. Early diagnosis and complete excision of ADPA will likely lead to improved outcomes that further promote digit salvage.


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