When a tiny tumour wreaks havoc on the stomach

Candace* was sick and tired of being sick! She called me on a Sunday, early in the morning at 5 o’clock. She had been woken up by stomach pain and had ended up in the toilet vomiting blood.

I was well acquainted with Sophie*, Candace’s mother, through a project we had worked on together, but I first met Candace when she was 15. She was brought in to the clinic after she developed severe period pain in school and was unable to stay in class. The school nurse gave her some pain medication and when Candace did not improve, it was prudent to send her for a medical consultation.

On reviewing Candace, she noted that her period pain had been present since she was 12, when she began menstruation. Some cycles were worse than others but the pain had never been serious enough to send her home from school like this.

On examining Candace, it was evident that her abdomen was quite sore, unrelated to the period pain. When I enquired about this, Candace noted that she had been treated for peptic ulcers and severe heartburn multiple times in the past year to no avail.

This set us back with regard to her pain relief at the time. She would not be able to use a range of pain medication that is recommended for period pain, because this would worsen the existing peptic ulcers. We managed to relieve Candace of her immediate pain and sent her home on medication, but she was also scheduled to see a gastroenterologist.

I saw Candace on and off for her period pain but thankfully this got better with age and by the time she was in college, she hardly needed my services. Her ulcers though, were another story. She had been treated for ulcers repeatedly throughout high school but she only seemed to get worse with time.

Despite undergoing endoscopy twice, the ulcers kept recurring soon after the completion of her prescription. She was on a highly restricted diet because of the same. She had constant heartburn and unending diarrhoea. At 21, she could not even leave home to go away to college, but instead had to commute daily. She did not enjoy college life like her peers did. She sought a second and third opinion and was always back at the same spot.

On the morning she was calling, she had tried to reach out to her current gastroenterologist to no avail. I was heading into surgery and asked her to come to the emergency department first for the necessary immediate care as we located a gastroenterologist to manage the immediate danger. 

By the time I finished surgery and headed out to check on her, she was already hooked onto intravenous fluids and the vomiting was under control. Her family was worried sick. The gastroenterologist on call arrived, one that had never met Candace before, and took his time with her. This time, I was there as a friend, not her doctor.

The doctor paid attention to her long history and even as he took her to surgery to stop the bleeding ulcer, he indicated that Candace would be undergoing multiple tests after she stabilised. A few days later, Candace had a diagnosis. She had Zollinger-Ellison Syndrome (ZES), condition where she had a tumour in her pancreas that was producing excessive amounts of gastrin.

Gastrin is a hormone produced by special cells in the stomach and the duodenum (the first part of the small intestine, immediately after the stomach), that is responsible for the growth and thickening of the lining of the stomach, ensuring food in the stomach keeps moving along, and triggers secretion of hydrochloric acid into the stomach.

In ZES, the special cells are found growing as a tumour in the pancreas or in the duodenum, causing excessive gastrin production. This leads to abnormal thickening of the stomach lining; with excessive production of hydrochloric acid, resulting in stomach and duodenal ulcers, severe acid reflux into the oesophagus (food pipe) which causes erosion of the walls of the oesophagus; and constant diarrhoea as the effect of moving food along the gut is excessively stimulated.

Candace had a plum-sized gastrinoma, as the tumour is known, in her pancreas. This was seen on an MRI of the abdomen. The tumour needed surgery to remove it but there was need to ensure that it had not spread beyond the pancreas. Though slow to grow, the tumour has the potential to spread to the liver, the lymph nodes and other body organs.

The tumour is more common in females, occurs at any age and is also commonly associated with a genetic condition known as multiple endocrine neoplasia-1 (MEN1), where the patient has multiple tumours in other endocrine organs such as the parathyroid glands, the pituitary gland and other hormone-producing tissues around the body.

At the time Candace was being diagnosed, there was no PET scan in Kenya to guide on how far the tumour had spread. It was necessary to send Candace to India for a complete evaluation before treatment. The family made a quick decision and she travelled with her mother.

Thankfully Candace’s tumour was restricted to the pancreas and she did not have MEN1. The doctors were able to remove a portion of Candace’s pancreas that was lodging the tumour. She recovered well from surgery and thankfully, her remaining portion of her pancreas was sufficient to ensure adequate insulin production and avoid a new complication of diabetes.

It has been eight years and Candace cannot believe it! She truly bid Zollinger-Ellison Syndrome farewell. She has not needed ulcer medicine since. Her diarrhoea ceased and she is back to a normal diet. She is grateful she has no more heartburn and can occasionally gleefully indulge in her favourite sinful pleasure, chili Indian samosa!

Dr Bosire is an obstetrician/ gynaecologist