The X Files and Paget's Carcinoma:
the story behind the story

I was watching the latest episode of The X Files this past Sunday night. I was dismayed to hear Agent Scully, the pathologist, state that Agent Mulder's mother killed herself rather than die in pain from Paget's Carcinoma. Apparently she performed an autopsy on his mother at his request.

I was dismayed because a good pathologist would never make such an ambiguous statement without a qualifying comment.

Paget's name has been applied to at least three diseases: Paget's disease of the breast, Extramammary Paget's disease, and Paget's disease of the bone. The first two diseases are carcinomas while the latter is associated with a greatly increased risk of bone cancers.

Paget's disease of the breast often begins as scaly lesion on the nipple that doesn't respond to the usual topical treatments. This often leads to a nipple biopsy where the pathologist will find the characteristic cancer cells haphazardly spreading throughout the epidermis, creating a buckshot-like pattern. This pattern has also been termed pagetoid spread, because of its characteristic association with the disease. The importance of the diagnosis centers on the association of an underlying carcinoma in the same breast, which some investigators have found in up to 100% of cases.

Extramammary Paget's disease, as the name suggests, shows the characteristic histologic changes in skin overlying areas other than the breast. It is most commonly seen around the genitals in women and may require very disfiguring surgery.

Paget's disease of the bone is most likely caused by a paramyxovirus. It occurs in one or more bones and usually occurs in the skull, vertebral column, and femur although all bones can be affected. The hallmark is enlarged and thickened bone that is extremely brittle and subject to fractures. Fractures may compress the nerves leading to excruciating pain. In about 5-10% of patients, sarcomas may develop.

Agent Scully indicated that it was a horribly disfiguring disease. Again, this is an ambiguous statement because all three diseases may lead to disfigurement. Of the three, probably Paget's disease of the bone would cause the most obvious physical deformity since it affects most of the skeletal system. Agent Scully did state that it was a carcinoma, however, so we may have to assume it was one of the other diseases.

Before I get hate mail from all you X Files fans, let me state that I enjoy the show and am not trying to pick on it. As a pathologist, however, I cannot help but comment on these diagnoses. Picky? Yes, but you would demand nothing less from your pathologist! Incidentally, Sir James Paget was one of the founding fathers of modern pathology (1814-1899). His careful observations made over a hundred years ago laid the groundwork for understanding the diseases of the breast and bone that today bear his name.

The Doctor's Doctor

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