Doctors ID rare pancreatic neuroendocrine tumor in girl, 17

Doctors should consider the possibility of a pancreatic neuroendocrine tumor (pNET) when patients with a history of abdominal and back pain seek treatment.

That’s the recommendation made by a team of scientists in Syria in a case report detailing the diagnosis of a teenage girl with this rare type of pancreatic tumor.

The 17-year-old patient, who ultimately was diagnosed with pancreatic cancer at the researchers’ hospital, had first been misdiagnosed with more common gastrointestinal illnesses before doctors identified a pancreatic NET that had already spread to her liver. This delay meant that potentially curative surgery was no longer feasible for the teen’s rare cancer.

“The rarity of these tumors makes their diagnosis difficult, but they should not be omitted and must be considered when there are long-lasting symptoms that are not compatible with common illnesses,” the researchers wrote, noting that “these tumors are curable in their early stages.”

The report, titled “Liver metastasized pancreatic neuroendocrine tumor in a 17-year-old female: A case report,” was published in the journal Clinical Case Reports.

Delay in diagnosing pancreatic tumor made surgery no longer feasible

Neuroendocrine tumors, or NETs, grow in the network of cells responsible for producing and releasing hormones in the body — collectively known as the neuroendocrine system. NETs are a rare type of tumor, especially among children, but their incidence has been on the rise in recent years.

Because neuroendocrine cells can be found almost anywhere in the body, NETs can grow in many organs. However, such tumors are not commonly found in the pancreas, a gland that produces hormones needed for digestion and blood sugar control.

Given that pancreatic NETs are so uncommon — accounting for approximately 1% of all NETs — they can be difficult for clinicians to accurately diagnose. Moreover, these tumors often grow slowly and are accompanied by nonspecific symptoms for which it isn’t easy to pinpoint a cause.

By the time these tumors are identified, they’ve often spread to other parts of the body.

The teen in this case sought treatment at a hospital in Damascus for severe upper abdominal pain that was radiating up her shoulder, and which had started about two months before. She’d also experienced similar episodes of abdominal and back pain in prior years.

Doctors had previously diagnosed her with gastroenteritis — inflammation in the gastrointestinal tract — and renal colic, or pain related to kidney stones. However, the problems persisted.

Routine lab testing was done at the hospital, and most results were normal. Imaging tests, however, showed the presence of multiple masses in the liver and a lesion on the pancreas.

A biopsy then confirmed the presence of a neuroendocrine tumor on the liver that was deemed to have spread, or metastasized, from the pancreas.

In cases like ours, we encourage physicians to follow up on the symptoms in order to identify these tumors in their early stages and provide a chance of optimal recovery for our patients, evading the remarkable effect of these slowly progressing diseases on patient life expectancy.

According to the researchers, the rarity of this type of tumor in a patient so young was the likely reason the teen’s previous doctors hadn’t considered it.

“In our case, the pain was repeatedly attributed to common and nonspecific illnesses and treated as such. That led to a major delay in diagnosis as a result of the low index of suspicion surrounding these tumors,” the researchers wrote.

The girl was treated with long-acting octreotide, given once every month. This medication works by mimicking the activity of somatostatin, a naturally occurring molecule that regulates the release of hormones. It’s used to slow the growth of tumors when surgery isn’t possible.

When discovered early on, surgery to remove a NET can be curative. However, because the girl’s disease had already spread to the liver by the time it was caught, that approach was no longer feasible, the team noted.

“In cases like ours, we encourage physicians to follow up on the symptoms in order to identify these tumors in their early stages and provide a chance of optimal recovery for our patients, evading the remarkable effect of these slowly progressing diseases on patient life expectancy,” the researchers wrote.