Dr. J. Hibler, Board-Certified Dermatologist at Skin Cancer & Dermatology Institute, Talks About Rare Skin Cancers

The recent loss of Jimmy Buffett has placed a spotlight on rare skin cancers that most of us are unaware of. The famous “Margaritaville” singer passed away from a very aggressive form of skin cancer called Merkel cell carcinoma (MCC). Dr. J. Hibler, a Board-Certified Dermatologist from Skin Cancer & Dermatology Institute, walks us through some of these rare skin cancers so that we may better understand what to look for.

Q: What are some different types of rare skin cancers?

There are several categories of the rarer types of skin cancer. Most of these are grouped and named for the component of the skin that turns into a cancerous process. In part one of this series, we will discuss the following three skin cancers:

  1. Merkel Cell Carcinoma is an extremely rare and very aggressive cancer that typically presents in patients 60+ in sun-exposed areas such as the head and neck.
  2. Adnexal Cancers: cancers that arise from sub-structures of the skin such as hair follicles, sweat glands, and oil glands.
  3. Sarcomas and other blood vessel malignancies that present on the skin. Some are very aggressive, some less so.

It is important to know about these very rare forms of skin cancer as they can be more aggressive than melanoma, a relatively more common type of skin cancer that can be deadly if not caught in time.

Q: Of these rare skin cancers, are there some in which you are seeing an increase?

There is a specific type of blood vessel (vascular) carcinoma called Kaposi sarcoma that can be associated with AIDS, as well as those taking immunosuppressants or immunocompromised. Due to the HIV epidemic of the 80s and 90s and the advance of immunosuppressant medications, we saw an uptick in these conditions.

Q: What do we need to know about these rare skin cancers?

Because there are so many different types of rare skin cancers, they can appear and behave very differently and have vastly different treatments. Also, there is some preference regarding age, skin type, genes, and associated medical conditions that predispose certain people to these rare cancers.

Merkel cell carcinoma – Merkel cell carcinoma (MCC) is the unchecked proliferation of skin cells that exhibit certain traits resembling those found in typical Merkel cells of the skin. According to Merkel, this type of cancer is on the rise, with about 2,000 new cases annually in the U.S. alone. They commonly present as a rapidly growing red or purple bump that is not painful. These bumps show mostly on areas of the body that are highly exposed to the sun, such as the face/head, neck, and arms, but they can be found anywhere. MCC is more frequently diagnosed in individuals aged 65 and older, with an elevated risk linked to fair skin and a history of prolonged sun exposure. Evidence of the Merkel cell polyomavirus can be found in most, but not all tumors. Weakened immune function, as seen in HIV patients or organ transplant recipients, is also associated with MCC. Treatment may include the removal of the tumor, as well as radiation, immunotherapy, and chemotherapy.

Adnexal cancers – are typically locally aggressive and mostly treated through surgery. An example of this type of cancer is Microcystic adnexal carcinoma (MAC,) a rare sweat gland cancer that often appears as a yellow spot or bump in the skin found on the neck or the face, though it can be found on the scalp, trunk, and upper extremities. Though the causes of MAC are unknown, some evidence shows that UV light, radiation, and immunosuppression may play a role. Patients ages 50 or older are more likely to develop MAC. These lesions are best treated using Mohs micrographic surgery.

Kaposi’s sarcoma – is an example of a cancer that develops from the cells that line the blood vessels or lymph vessels. It causes purple, red, or brown lesions on the skin, mucous membranes, and lymph nodes. The lesions can be flat or slightly raised, growing and spreading quickly. This cancer is caused by the human herpes virus 8, which turns healthy cells into cancerous cells. It is also associated with genetic factors, hormonal factors, and immunodeficiency. In the U.S., the more aggressive cancer affects people with weakened immune systems, including HIV-positive patients, cancer patients, and organ transplant recipients. Treatment will depend on various factors, including the type of Kaposi sarcoma and your overall health. For HIV-positive patients, it may be treated by using highly active antiretroviral therapy (HAART). For acquired Kaposi sarcoma, your immunosuppressant medications may be reduced or changed. Other treatments may include topical medications, targeted therapy, radiation therapy, and chemotherapy.

Q: Would an annual full skin exam with a Board-Certified Dermatologist help diagnose these rare skin cancers early?

Typically, yes, but some cancers, including Merkel cell, can appear and spread within a few months. So, if there is any new, rapidly enlarging lesion or rash that one is unsure of, it is best to have it checked as soon as possible with a skin expert.

Q: Any support or resources that you would recommend?

This depends on the type of disease or condition one is diagnosed with. Practically all rare medical conditions, including skin, have official support groups, resources, and additional information. You can check out the Skin Cancer Foundation for more information about rare skin cancers. The Cancer Support Community is a helpful resource, and if you want more details regarding Merkel cell carcinoma, check out

About Dr. John Hibler


Dr. Hibler practices at the Incline Village location of Skin Cancer & Dermatology Institute. He believes in treating the whole person, not just a specific symptom or condition. His specialties include medical, pediatric, surgical, and cosmetic dermatology. You can book an appointment with Dr. Hibler online.

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