Everything You Need To Know About Some Rare Types Of Pancreatic Cancers

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“The most common symptoms include nausea, abdominal pain, alarming weight loss, changes in bowel movements, jaundice, vomiting, ulcers and diarrhoea,” says Dr Ajay Mehta, director and head of surgical oncology, HCG Cancer Centre, Nagpur.

Pancreatic cancer is known to be aggressive in nature with a high mortality rate. It affects the pancreas, which is a vital organ responsible for regulating blood sugar levels and helping with digestion. “While the majority of pancreatic cancer cases are of the more common adenocarcinoma type, there exist rare subtypes that pose unique challenges,” says Dr Ajay Mehta, director and head of surgical oncology, HCG Cancer Centre, Nagpur.

What are the causes of pancreatic cancers?

Genetic: Genetic mutations in certain genes lead to the development of pancreatic cancer. For example, mutations in genes such as DAXX, ATRX, MEN1, and others associated with pancreatic neuroendocrine tumours (PNET’s).

Environmental and lifestyle: Smoking, obesity, a diet rich in processed and red meat with less consumption of fruits and vegetables, excessive alcohol consumption, exposure to certain toxins and chemicals in the workplace.

Other medical vulnerabilities: Certain rare types of pancreatic cancer may be associated with specific medical conditions. For instance, intraductal papillary mucinous neoplasms (IPMNs) are often linked to chronic pancreatitis, which can result from various causes including heavy alcohol use.

Hormonal factors: Pancreatic neuroendocrine tumors (PNETs) are hormone-producing tumors. Hormonal factors and imbalances can play a role in their development.

What are the symptoms of rare pancreatic cancer?

Dr Mehta says the symptoms can vary depending on the type and location of the tumor, and they may overlap with symptoms of more common forms of pancreatic cancer. “It’s important to note that many rare types of pancreatic cancer, such as pancreatic neuroendocrine tumors (PNETs), may be non-functional, meaning they do not produce noticeable hormonal symptoms.”

“The most common types of symptoms include nausea, abdominal pain, alarming weight loss, changes in bowel movements, jaundice, vomiting, ulcers and diarrhoea. It’s important to remember these symptoms can also be caused by various other medical conditions. If, however, you experience these symptoms persistently — especially if you are at risk of pancreatic cancer due to family history — it is essential to seek medical attention,” he adds.

Acinar Cell Carcinoma

Acinar cell carcinoma is a rare subtype of pancreatic cancer that originates in the exocrine glands of the pancreas. Unlike the more common ductal adenocarcinoma, acinar cell carcinoma often presents with different clinical and histological features. It tends to affect a slightly younger patient population and has a better prognosis when diagnosed in the early stages, states the doctor, adding that treatment options typically include surgery, chemotherapy, and radiation therapy.

Pancreatic Neuroendocrine Tumors (PNETs)

PNETs originate from the endocrine cells in the pancreas and can be categorised into functional or non-functional types. Functional PNETs produce hormones that cause specific symptoms, while non-functional PNETs do not exhibit any hormonal symptoms. “The approach to treating PNETs depends on several factors, including the tumor’s size, grade, and whether it is functional or non-functional. Treatment options may include surgery, chemotherapy, targeted therapy, and somatostatin analogs to manage hormonal symptoms,” says Dr Mehta.

Pancreatic Adenosquamous Carcinoma

It is a rare and aggressive subtype of pancreatic cancer that combines elements of adenocarcinoma and squamous cell carcinoma. It is known for its rapid growth and tendency to spread to nearby tissues. The treatment typically involves surgery to remove the tumor when possible. Chemotherapy and radiation therapy may be used as adjuvant treatments, but the prognosis for this subtype remains challenging, the expert says.

Solid Pseudopapillary Neoplasm (SPN)

SPN is a rare, low-grade malignant tumor that primarily affects young women. It is characterised by solid and cystic components and generally exhibits a less aggressive clinical course compared to other pancreatic cancer subtypes. The primary treatment for SPN is surgical resection, which can provide excellent long-term outcomes. In most cases, patients do not require adjuvant therapy after surgery, the doctor concludes.