Remembering Aretha Franklin and a Rare Pancreatic Cancer

The one detail her family released

Aretha Franklin died on August 16, 2018, at the age of 76. Her family said she died of advanced pancreatic cancer of the neuroendocrine type, and her oncologist confirmed the cause of death.

That phrase is the whole public record, and it is enough to build on, because the word "neuroendocrine" changes what the diagnosis means. Nothing about her care was disclosed, and this page does not guess at it.

One organ, two different cancers

The pancreas sits deep in the upper belly and does two jobs. Exocrine cells make digestive enzymes, while endocrine cells, gathered in clusters called islets, make hormones that control blood sugar.

Cancer can start in either group of cells, and the two kinds behave nothing alike.

Exocrine cancer is what most people mean by pancreatic cancer. SEER, the federal statistics program, lists 67,530 new cases and 52,740 deaths in the United States for 2026 — both American Cancer Society projections rather than counts — and its own five-year relative survival figure is 13.7 percent for people diagnosed from 2016 to 2022.

Islet cell tumors are different, and doctors call them pancreatic neuroendocrine tumors. NCI puts them at under 2 percent of pancreatic cancers, or roughly 1,000 new cases a year, and says they carry a better outlook than exocrine tumors. The Pancreatic Cancer Action Network describes them as slower growing.

Tumors that send hormone signals

NCI splits these tumors into two groups. Most are functioning, which means they pour a hormone into the blood, and about 15 percent are not.

A functioning tumor is named for its hormone. Its symptoms follow straight from that chemistry:

• A gastrinoma drives up stomach acid, so ulcers keep coming back. There is belly pain that may reach the back, plus reflux and diarrhea. Doctors call this picture Zollinger-Ellison syndrome.
• An insulinoma releases insulin, so blood sugar drops. NCI describes fasting sugar under 40 mg/dL with a high insulin level. People feel shaky, sweaty, hungry, or confused, and the heart races.
• A glucagonoma raises blood sugar, and it also brings a rash, blood clots, weight loss, and a sore tongue.
• A VIPoma causes heavy watery diarrhea, dehydration, and low potassium.

Tumors that make no hormone give none of these clues, and NCI says they look much like exocrine cancers. Signs include belly or back pain, indigestion, diarrhea, a lump in the abdomen, or yellow skin and eyes.

When to get checked

Take these to a doctor rather than waiting them out:

• Yellow skin, or yellow whites of the eyes, above all without pain. Ask for an appointment within days.
• Stomach ulcers that return despite treatment, or heartburn that will not settle on standard drugs.
• A blood sugar under 55 mg/dL in someone taking no diabetes drugs, above all if eating fixes the symptoms.
• Watery diarrhea in large amounts for more than two weeks, above all with a low potassium result.
• Weight loss you cannot explain, or upper belly pain boring through to the back for several weeks.

There is no screening test for pancreatic cancer in people without symptoms, and NCI has no evidence-based screening or prevention summary for it. Symptoms are the only route in.

Finding and staging the tumor

The workup mixes pictures with chemistry. NCI lists CT of the abdomen, MRI, endoscopic ultrasound, ERCP, angiography, and bone scans. Blood work includes a chromogranin A test, a marker many of these tumors release. A scan called somatostatin receptor scintigraphy can also light up tumor sites.

Hormone levels can pin down the type before a scan finds the lump, and NCI gives numbers. Serum glucagon above 1,000 pg/mL points to a glucagonoma, while serum VIP above 200 pg/mL points to a VIPoma. For a gastrinoma, a secretin test counts as positive when gastrin doubles within ten minutes.

Staging follows the AJCC TNM system. Stage I is a tumor under 2 cm, with no nodes and no spread. Stage II covers larger tumors, or growth into the duodenum or bile duct. Stage III means growth into nearby organs or major vessels, or spread to nodes. Stage IV means distant spread.

What treatment involves

NCI is direct about the main point, which is that surgery is the only approach that can cure, and only when the tumor can be removed.

The operation depends on where the tumor sits. Enucleation shells out the tumor alone. A distal pancreatectomy takes the tail. A larger operation on the head of the gland is called a pancreatoduodenectomy.

When the disease reaches the liver, teams have several tools. They may cut out the deposits, block the blood supply feeding them, or destroy them with heat or cold.

Drug options include chemotherapy, hormone therapy, and targeted therapy. NCI names streptozocin, doxorubicin, fluorouracil, dacarbazine, and temozolomide among the chemotherapy drugs.

One point needs care. NCI reports that trials of the targeted drugs everolimus and sunitinib showed longer progression-free survival, which measures time without the cancer growing. It is not the same as living longer, and NCI does not say it is.

Supportive care runs alongside all of this. Proton pump inhibitors control stomach acid, and octreotide, a somatostatin-type drug, controls diarrhea and low blood sugar.

Reading the outlook with care

NCI flags one limit openly, which is that most evidence for these tumors comes from case series rather than randomized trials.

Outcomes also swing widely by type. NCI reports that only about 10 percent of insulinomas are malignant, while around 75 percent of glucagonomas are. Most somatostatinomas have already spread by the time they are found.

None of that forecasts one person's course. These are group figures, gathered over years, from people whose tumors and health differed from each other and from any reader.

Sources

• https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq
• https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq
• https://seer.cancer.gov/statfacts/html/pancreas.html
• https://www.cancer.org/research/cancer-facts-statistics.html
• https://www.cbsnews.com/news/aretha-franklin-queen-of-soul-respect-singer-dead-at-76-today-2018-08-16/
• https://pancan.org/news/aretha-franklin-the-queen-of-soul-dies-of-pancreatic-cancer/