Researchers Find Immunotherapy Drug May Treat Pheochromocytoma

Those diagnosed with paraganglioma-pheochromocytoma, squamous cell carcinoma of the skin (cSCC), adrenocortical carcinoma (ACC), and carcinoma of unknown primary (CUP) may soon have an additional option for treatment. According to recent findings in the Journal for ImmunoTherapy of Cancer, the immunotherapy drug pembrolizumab has displayed efficacy in preventing the growth and development of tumors. You can learn more about this in a release by the University of Texas MD Anderson Center.

About Pheochromocytoma, cSCC, ACC, and CUP

Adrenal Diagnoses

A pheochromocytoma, an often benign but rare tumor, usually occurs in the adrenal glands or, more specifically, the adrenal medulla. Less than 1% of people with high blood pressure develop pheochromocytomas, and many of these tumors develop randomly. Mutated genes are a cause. Pheochromocytomas are generally curable but may cause fatigue, dizziness, vomiting, anxiety, or heart palpitations.

Adrenocortical carcinoma (ACC) also affects the adrenal glands. According to the NIH, adrenocortical carcinoma negatively affects the parts of the adrenal glands which regulate hormones and blood pressure. Much like with a pheochromocytoma, ACC may cause or relate to high blood pressure. This type of cancer most often affects children and people with ovaries, and are often the cause of genomic mutations. Symptoms can include abdominal pain, excessive sweating, and an increased cortisol level.

cSCC and CUP

Squamous cell carcinoma (cSCC) often affects areas of skin most exposed to the sun. Chemotherapy can be a treatment in cases where cancer has spread beyond the skin. cSCC is also treatable with surgery or cryosurgery if discovered early enough. It presents with sores on the skin or scaly patches on the lips.

Carcinoma of unknown primary (CUP) is also known as occult primary cancer. This accounts for cancers where doctors are unable to determine the initial tumor occurrence. As a primary tumor helps doctors to determine a course of treatment, those with CUP receive treatment in the form of chemotherapy, radiation, or surgery. Some doctors may choose to explore the features of each unique tumor to determine what form of treatment to utilize. Those with CUP may experience a loss of appetite, changes in bowel movements, or night sweats. However, some may experience no symptoms.

The Study

Researchers from the University of Texas MD Anderson Cancer Center studied the non-progression rate of patients with one of the cancers listed above. 127 patients were given 200 mg of pembrolizumab, an immunotherapy treatment, every three weeks.

Out of all patients, there was a 36% non-progression rate for cSCC, 43% for pheochromocytoma, 31% for ACC, and 33% for CUP. This means that this portion of patients experienced no progression (growth, metastasis) of their tumors over the 27-week period. However, 52% of patients also experienced adverse treatment effects such as rash or increased tiredness.

Though approximately half of the patients in this study experienced adverse effects, the results are still promising. With additional research, clinicians will be better suited to address potential advances in this realm.

Looking Forward

Pheochromocytoma, CUP, sSCC, and ACC are becoming a significant part of new cancer and tumor diagnoses. Those with these cancers and tumor growths also experience aggressive development. Thus it is extremely important for researchers to focus on areas of treatment and improvement. Future research may consider how pembrolizumab and other immunotherapy drugs can impact and shape cancer treatments. 


Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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