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Dear Dr. Roach: Do you know anything about aggressive digital papillary adenocarcinoma? My 39-year-old nephew was just diagnosed, and his parents are searching for answers. He's getting scans to determine whether they should amputate his finger. As far as we know, it hasn't metastasized. -- A.M.
Answer: No, I'd never heard of this condition. It's extremely rare (1 in 10 million people). "Adenocarcinoma," by definition, is cancer of the glandular tissue, and there isn't really much glandular tissue in a finger. CANCER of any extremity is almost always of connective tissue (sarcoma). This includes cancers of the bone, muscles, fat, blood vessels or soft tissue.
Digital papillary adenocarcinoma starts in the sweat glands of the fingers or toes. It's slow-growing and frequently misdiagnosed. Recently, it's been discovered that these cancers are related to the human papillomavirus, specifically type 42, which is normally considered a low-risk type.
Both wide excision and amputation are effective treatments, and the five-year disease-specific survival rate is 98%. But the tumor can recur locally unless it's very carefully removed. Metastatic disease can show up very late (up to 20 years after the tumor's removed), so people require long-term surveillance after getting treatment.
Although this disease is very rare, there are a lot of very rare disorders (10,000 or so that are known). This means that as many as 6% of people have a rare disorder. Since they aren't as well-known or well-understood, the diagnosis and treatment can be problematic. These conditions don't get as much funding or recognition, so the optimal treatment isn't always understood.
Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.cornell.edu.