Terry Bradshaw’s Merkel Cell Carcinoma

With football back in action this fall, watchers of “Fox NFL Sunday” noticed that commentator and Hall of Fame quarterback Terry Bradshaw looked different. He appeared to have gained weight and seemed unsteady on his feet. Fans flooded social media with concerns about Bradshaw.

This past week, Bradshaw decided to address those concerns. In November 2021, Bradshaw was diagnosed with bladder cancer. He was treated at Yale University Medical Center in New Haven, Connecticut, and was considered “cancer free.” Fast forward to March 2022, when Bradshaw said he was feeling pretty good, but then began to have trouble with his neck. Although he originally attributed this to many years in the NFL, he later decided to have it checked out.

An MRI detected a mass in the left side of his neck, and biopsy determined that it was a rare skin tumor called Merkel cell carcinoma (MCC). He was treated at the University of Texas MD Anderson Cancer Center in Houston, where he underwent surgery, followed by radiation therapy. In a Facebook Live chat, Bradshaw explained that the swelling in his face is due to the radiation, which “maintains fluid in my face and jaw, and this side of my face is partially numb. It’s coming, it’s slow, it’s gonna take a while.”

“Folks, I may not look like my old self, but I feel like my old self,” he added. “I’m cancer free. I’m feeling great and over time I’m going to be back to where I normally am.”

Bradshaw told NBC News correspondent Harry Smith in a “Today” segment that this experience has changed his outlook on life. “We’re going to go to Europe, going to go to Paris, going to go to wherever we want to go. I’m going to get all this in because I feel like I don’t want to put this off anymore … I may have 25, 30 years left, but I’m going to act like I got one.”

Merkel Cell Carcinoma

MCC was first described in 1972 by Cyril Toker, MD. It has subsequently been known by a variety of names, including trabecular carcinoma of the skin, Toker tumor, primary small cell carcinoma of the skin, primary cutaneous neuroendocrine tumor, and malignant trichodiscoma. It is a rare and aggressive neuroendocrine carcinoma arising in the dermoepidermal junction. Although the exact origin and function of the Merkel cell remains under investigation, Merkel cells are normally found in the skin and in parts of mucosal surfaces derived from the ectoderm. They are thought to be of neuroectodermal origin and function as slow-acting mechanoreceptors in the basal layer of the epidermis to provide information about touch and hair movement.

Estimates show there are fewer than 5,000 people with this disease in the U.S. It is most common in adults, especially those ages 60 and up. About half of patients are diagnosed with localized disease. Surveillance, Epidemiology, and End Results registry data have shown an excess risk of MCC as a first or second cancer in patients with several primary cancers.

Presentation

MCC usually presents as a painless, indurated, solitary dermal nodule with a slightly erythematous to deeply violaceous color, and can infiltrate locally via dermal lymphatics, resulting in multiple satellite lesions. Because of its nonspecific clinical appearance, MCC is rarely suspected before a biopsy is performed.

A mnemonic summarizing typical clinical characteristics of MCC has been proposed by Heath et al.:

• A = Asymptomatic
• E = Expanding rapidly
• I = Immune suppressed
• O = Older than 50 years
• U = UV-exposed skin

Not all patients have every element in this mnemonic, but in Heath’s study, 89% of patients met three or more criteria, 52% met four or more criteria, and 7% met all five criteria.

Risk factors

Ultraviolet radiation is believed to play a significant role in the development of MCC. It is much more common in white patients than in Black patients (93.6% vs 0.2%). It occurs most frequently in sun-exposed areas of the skin, especially the head and neck, followed by the extremities.

A weakened immune system is also a risk factor for MCC. The risk of developing MCC is eight times greater for patients with HIV, 10 times greater in organ transplant patients, and 40 times greater in patients with chronic lymphocytic leukemia. That being said, 90% of patients who develop MCC do not have an immune deficiency.

In 2008, the Merkel cell polyomavirus (MCPyV) — found to be present in MCC tumor cells — was discovered at the University of Pittsburgh. High levels of viral DNA and clonal integration of the virus in MCC tumors have also been reported, along with expression of certain viral antigens in Merkel cells and the presence of antiviral antibodies. Approximately 70% to 80% of cases of MCC are associated with MCPyV.

Treatment

Being a rare tumor, management recommendations are based upon small studies or case reports. MCC is often treated like other nonmelanoma skin cancers, with an emphasis on treating local-regional disease with surgery and radiation therapy, as appropriate. MCC can also be treated according to its biological features. In recent years, the FDA approved the immunotherapies avelumab (Bavencio) and pembrolizumab (Keytruda) for MCC.

Michele R. Berman, MD, is a pediatrician-turned-medical journalist. She trained at Johns Hopkins, Washington University in St. Louis, and St. Louis Children’s Hospital. Her mission is both journalistic and educational: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.