Pancreatic Neuroendocrine Tumor (PNET)

Causes:

1. Genetic factors: Inherited genetic syndromes such as multiple endocrine
neoplasia type 1 (MEN1) or von Hippel-Lindau (VHL) syndrome can
increase the risk of developing PNETs.
2. Sporadic mutations: Mutations in certain genes may occur randomly,
leading to the development of PNETs.
3. Exposure to certain toxins or environmental factors may also play a role,
although the exact causes are not well understood.

Signs and Symptoms:

Symptoms of PNETs may vary depending on the hormones produced by
the tumor and whether the tumor is functioning (producing hormones) or
non-functioning (not producing hormones). Common signs and symptoms
may include:

1. Abdominal pain or discomfort.
2. Jaundice (yellowing of the skin and eyes).
3. Changes in bowel habits.
4. Unexplained weight loss.
5. Flushing or redness of the skin (particularly with functioning
tumors).
6. Diarrhea or other gastrointestinal symptoms.
7. Hypoglycemia (low blood sugar levels) with insulinomas (insulin-
producing tumors).
8. Symptoms related to excess hormone production (e.g., excessive
sweating, palpitations, high blood pressure).

Prevention:

1. Due to the rarity of PNETs and the unclear understanding of their causes,
specific preventive measures for these tumors are not well-established.
However, managing risk factors such as genetic syndromes and avoiding
exposure to toxins or environmental carcinogens may help reduce the risk.

Treatment:

1. Medical Treatment:

1. Medications: Depending on the type of PNET and its hormone
production, medications may be prescribed to control symptoms or
hormone levels.
2. Targeted therapy: Drugs that target specific molecular pathways
involved in tumor growth and progression may be used in certain
cases.
3. Chemotherapy: Systemic chemotherapy drugs may be used to
shrink tumors and slow down cancer progression.
4. Somatostatin analogs: These drugs may be used to control
hormone production and alleviate symptoms in functioning tumors.

2. Surgical Treatment:

1. Surgery is often the primary treatment for PNETs, especially for
localized or resectable tumors.

2. Surgical options may include:

1. Tumor resection: Surgical removal of the tumor and
surrounding tissue.
2. Whipple procedure (pancreaticoduodenectomy): Removal of
the head of the pancreas, part of the small intestine,
gallbladder, and bile ducts for tumors located in the head of
the pancreas.
3. Distal pancreatectomy: Removal of the tail or body of the
pancreas for tumors located in the body or tail of the
pancreas.
4. Total pancreatectomy: Removal of the entire pancreas in
select cases of extensive PNETs.