Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.
Eyes front and ears open. Class is now in session.
The disease that we will be learning about today is:
Cutaneous Squamous Cell Carcinoma
What is Cutaneous Squamous Cell Carcinoma?
- CSCC accounts for the majority of nonmelanoma skin cancer deaths.
- Patients with CSCC have reported concerns about the spread of disease and the effects of surgeries that may be disfiguring.
- CSCC incidence is expected to increase 2% to 4% each year.
- Mortality above 70% has been reported for patients with regional or distant metastasis.
- They grow over weeks to months
- They may ulcerate
- They are often tender or painful
- Located on sun-exposed sites, particularly the face, lips, ears, hands, forearms and lower legs
- But squamous cell carcinoma of the skin can occur anywhere on your body, including inside your mouth, on your anus and on your genitals.
- Size varies from a few millimeters to several centimeters in diameter.
- Death from these cancers is uncommon. It’s thought that about 2,000 people in the US die each year from these cancers, and that this rate has been dropping in recent years. Most people who die from these cancers are elderly and may not have seen a doctor until the cancer had already grown quite large. Other people more likely to die of these cancers are those whose immune system is suppressed, such as those who have had organ transplants.
How Do You Get It?
- Particularly prevalent in elderly males. However, they also affect females and younger adults.
- Previous SCC or another form of skin cancer (basal cell carcinoma, melanoma) are a strong predictor for further skin cancers.
- Actinic keratoses
- Outdoor occupation or recreation
- Fair skin, blue eyes and blond or red hair
- Previous cutaneous injury, thermal burn, disease (eg cutaneous lupus, epidermolysis bullosa, leg ulcer)
- Inherited syndromes: SCC is a particular problem for families with xeroderma pigmentosum and albinism
- Other risk factors include ionizing radiation, exposure to arsenic, and immune suppression due to disease (eg chronic lymphocytic leukemia) or medicines. Organ transplant recipients have a massively increased risk of developing SCC.
- The number of these cancers has been increasing for many years. This is probably from a combination of better skin cancer detection, people getting more sun exposure, and people living longer.
What Are The Symptoms?
- A firm, red nodule
- A flat sore with a scaly crust
- A new sore or raised area on an old scar or ulcer
- A rough, scaly patch on your lip that may evolve to an open sore
- A red sore or rough patch inside your mouth
- A red, raised patch or wart-like sore on or in the anus or on your genitals
- Squamous cell carcinomas typically appear as persistent, thick, rough, scaly patches that may bleed.
- They often look like warts and sometimes have open sores with a raised border and crusted surface over an elevated pebbly base.
- The skin around them typically shows signs of sun damage such as wrinkling, pigment changes and loss of elasticity.
- In addition to the signs of SCC shown here, any change in a preexisting skin growth, such as an open sore that fails to heal, or the development of a new growth, should prompt an immediate visit to a physician.
- A persistent, scaly red patch with irregular borders that sometimes crusts or bleeds.
How Is It Treated?
- The goals of primary treatment of SCC are to ensure the complete removal of the primary tumor, prevent metastasis, and preserve cosmesis.
- In general, treatment of cSCC is most effectively accomplished by surgical therapy. There are relatively few exceptions to this guiding principle, especially for high-risk cSCC, because of the potential for recurrence and metastasis.
- Excision: Cutting out the tumor, along with a small margin of normal skin, is often used to treat squamous cell cancers.
- Curettage and electrodesiccation: This approach is sometimes useful in treating small (less than 1 cm across), thin squamous cell cancers, but it’s not recommended for larger tumors.
- Mohs surgery: Mohs surgery has the highest cure rate. It’s especially useful for squamous cell cancers larger than 2 cm (about 4/5 inch) across or with poorly defined edges, for cancers that have come back after other treatments, for cancers that are spreading along nerves under the skin, and for cancers on certain areas of the face or genital area. This approach is typically more complex and time-consuming than other types of surgery.
- Most squamous cell skin cancers are found and treated at an early stage, when they can be removed or destroyed with local treatment methods. Small squamous cell cancers can usually be cured with these treatments. Larger squamous cell cancers are harder to treat, and fast-growing cancers have a higher risk of coming back.
- In rare cases, squamous cell cancers can spread to lymph nodes or distant parts of the body. If this happens, treatments such as radiation therapy and/or chemotherapy may be needed.
- Lymph node dissection: Removing regional (nearby) lymph nodes might be recommended for some squamous cell cancers that are very large or have grown deeply into the skin, as well as if the lymph nodes feel enlarged and/or hard. The removed lymph nodes are looked at under a microscope to see if they contain cancer cells. Sometimes, radiation therapy might be recommended after surgery.
- Chemotherapy is an option for patients with squamous cell cancer that has spread to lymph nodes or distant organs. Sometimes it’s combined with surgery or radiation therapy.
- Immunotherapy: Another option for advanced squamous cell cancers that can’t be cured with surgery or radiation therapy might be using an immunotherapy drug such as cemiplimab (Libtayo).
- PD-1 is a checkpoint protein on immune cells called T cells. It normally acts as a type of “off switch” that helps keep the T cells from attacking other cells in the body. It does this when it attaches to PD-L1, a protein on some normal (and cancer) cells. When PD-1 binds to PD-L1, it basically tells the T cell to leave the other cell alone. Some cancer cells have large amounts of PD-L1, which helps them evade immune attack.
- Monoclonal antibodies that target either PD-1 or PD-L1 can block this binding and boost the immune response against cancer cells. These drugs have shown a great deal of promise in treating certain cancers.
Examples of drugs that target PD-1 include:
- Pembrolizumab (Keytruda)
- Nivolumab (Opdivo)
- Cemiplimab (Libtayo)