It was actually just a routine appointment with her family doctor. There she told of a slight burning sensation in her lower abdomen that had been bothering her for some time. “I thought it might be appendicitis,” she said. But a subsequent ultrasound immediately raised suspicions of a tumor, which was confirmed by another doctor the same day.
Claudia recalls today that she was dazed by the shock. Further investigations did not dispel the suspicion. After a CT scan and biopsy, she received the final diagnosis: she had cancer.
Cancer but no tumor
But Claudia’s cancer was no ordinary disease. When tissue from her abdominal wall was biopsied, it was clear that there had been metastases there. “Usually, cancer arises from a primary tumor that forms in an organ,” says Alwin Krämer, senior oncologist at Heidelberg University Hospital and a specialist in CUP syndrome.
CUP – stands for “Cancer of Unknown Primary” which means that the primary tumor was not found despite extensive testing. Claudia also underwent this test. However, the original tumor was still undetectable.
How exactly this happens can only be explained by hypotheses. “The most common is that the immune system is able to destroy the primary tumor. However, metastases are no longer recognized by the immune system and can therefore form,” explains Krämer. Another explanation is that metastases grow faster than the tumor and are therefore not detected. However, the fact that no primary tumors have been found in long-term CUP patients argues against this, says Krämer.
“Can I survive this?”
A time of uncertainty and fear now began for Claudia. She knew that her cancer was considered advanced because of its metastases. Shortly after her diagnosis, she heard on a podcast that the average life expectancy for someone with CUP was only nine months. “Thoughts came into my mind like: What am I going to do with my life now, how limited am I physically and: Can I survive this?”
In the absence of a primary tumor, specific treatment is not possible. Instead of therapies that are effective against a specific type of cancer, broad, non-specific chemotherapy is usually used. This is broadly effective and covers a lot, says Krämer. “But the results are poor. The median survival of these patients is definitely less than a year.”
Claudia also initially received chemotherapy. Previously, tissue in the abdominal wall and ovaries were removed in several operations. A precaution, doctors said. The financial accountant tolerated the first chemotherapy well, a little tired, but no nausea. “But the feeling of her body was completely different.” She explained that she felt like she was under full pressure, and she was “very ticklish.”
New therapy is “six in the lottery”
A year and a half later, doctors found another abnormality and Claudia had to undergo chemotherapy again. She struggled with herself and was unsure whether she should undergo the therapy. Shortly before the third chemotherapy treatment, Claudia was finally accepted into a study that also involved Krämer. It was “six in the lottery,” Claudia said.
In this study, CUP patients will be treated with immunotherapy. With the help of immune checkpoint inhibitors, the affected person’s immune system is activated against the tumor cells. “If the immune system then finds the tumor cells, it can kill them,” says Krämer. A treatment that has shown promise in some patients. For some, it has even led to a cure.
Thanks to this new therapy, Claudia now lives a normal life. She also continues her job as a financial accountant. Normality helps her. “I have learned that if my daily life is okay, if I can go to work, then the disease will not be so present in my life.” She is convinced: If she had to quit her job, she would “fall into a hole”.
Self-help groups for support
She has now been suffering from the rare cancer for nine years and is sure there will be more. When she feels worried about her future, her friends and family support her. Claudia is also involved in a self-help group, Forum CUP, where those affected can exchange ideas.
Here she found support in knowing that she was not alone with her diagnosis. She did not have to explain her cancer first; she could share her concerns and experiences with people who were going through the same thing. “In a forum like this you feel understood.”
Talking about her illness, cancer has become less scary for Claudia. There are many sufferers on the forum who have lived a long time with their diagnosis. These positive examples are important. “My personal advice to everyone affected: Please don’t give up! Don’t always read only negative news, don’t do too much research on the internet, sometimes there are things there that you don’t care about at all, but still stick in your mind.” Claudia wants to show with her story that it’s not over after nine months. She’s still the same.
Oncologist Alwin Krämer is also convinced. They are still far from solving the problem. “But now we can help patients better with immunotherapy than we could a while ago.”
