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SearchTwo cases of a rare cancer of the sweat glands
By Rebecca Knackstedt, MD, PhD, and Thomas Knackstedt, MD
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Aggressive digital papillary adenocarcinoma (ADPA) is a rare malignancy of the sweat glands. It usually presents as a single mass on a finger, and excision and amputation treatments are often unsuccessful with local recurrence. Successful management of other sweat gland malignancies with Mohs micrographic surgery has been well established in the literature, and the cases described here demonstrate the viability of Mohs surgery as a successful treatment for ADPA.
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A verrucous tender plaque on the third right radial nail fold of a 53-year-old man. He attributed its 25-year presence to trauma, but radiographs showed no bony abnormalities. Biopsy revealed subungual large lobular aggregates of basaloid glandular cells with multiple papillary fronds and cystic areas, all consistent with ADPA. Most cases of ADPA present with a long history like this one.
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Because the tumor was aggressive, two-stage Mohs surgery was performed. The second stage included an additional lateral 2 to 3 mm. A deep bony margin was excised with a bone rongeur, and no tumor was identified upon frozen section analysis.
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At 34 months, there is no evidence of recurrence. Chest CT and sentinel lymph node biopsy showed no evidence of metastatic disease. In contrast, traditional treatments for ADPA have high rates of both local recurrence (28 percent) and distant metastases (14 percent).
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In the second case, a 65-year-old man presented with a 2.0 x 1.2 cm pearly telangiectatic fissured nodule that had been slowly growing on his thumb for six years. He underwent shave biopsy.
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The biopsy showed epithelial cells with overall lobulated and nodular architecture (inset) forming papillary structures with duct-like lumens. Slit-like spaces with focal squamous metaplasia and occasional atypical mitoses were present. All histologic findings were consistent with ADPA.
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Upon initiation of Mohs surgery, persistent disease was noted at the deep promixal margin of the first Mohs layer, leading to a second Mohs layer being performed as an amputation. Image shows the volar aspect of the digit. Margins are clear.
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Image shows the dorsal aspect of the amputated digit. Nine months after surgery, the patient is recovering well, and all scans were negative for metastatic disease.
Dr. Thomas Knackstedt’s additional work on a population based analysis of incidence, demographics, treatment and outcomes of aggressive digital papillary adenocarcinoma will be published in Dermatologic Surgery in the coming weeks.
Dr. R. Knackstedt is a resident physician and Dr. T. Knackstedt is associate staff in the Dermatology & Plastic Surgery Institute.
Images and text republished with permissions from John Wiley and Sons. Originally published in the International Journal of Dermatology.
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