pancreatic endocrine carcinoma

Pancreatic Endocrine Carcinoma: A Rare but Aggressive Form of Pancreatic Cancer

Pancreatic endocrine carcinoma, also known as pancreatic neuroendocrine tumor (PNET), is a rare and aggressive form of pancreatic cancer that originates from the hormone-producing cells of the pancreas. This type of cancer makes up less than 5% of all pancreatic cancers [14].

Characteristics and Symptoms

• Pancreatic endocrine carcinomas are typically slow-growing tumors that can cause symptoms similar to those of common medical issues, such as:
  • Chest and/or tummy pain
  • Acid reflux and heartburn
  • Diarrhea and tiredness (Zollinger-Ellison syndrome) [9]
• These tumors can also produce excess hormones, leading to various systemic effects [11]

Types and Classification

• Pancreatic endocrine carcinomas are classified by the hormones they produce:
  • Gastrinoma (gastrin)
  • Glucaganoma (glucagon)
  • Insulinoma (insulin) [11]
• Each type of PNET has distinct symptoms, behavior, and treatment responses [12]

Treatment Options

• Treatment for pancreatic endocrine carcinoma typically involves:
  • Surgery to remove the tumor
  • Chemotherapy to kill cancer cells [15]
  • Targeted therapy to specifically target the tumor's hormone production [not mentioned in context but a common practice]
• In cases where the cancer has spread to the liver, additional treatments such as removing part of the liver may be necessary [15]

Prognosis and Survival Rates

• Early diagnosis and treatment are crucial for improving survival rates, with more than 90% of people with this condition being alive five years after diagnosis [4]
• However, pancreatic endocrine carcinomas can still be aggressive and challenging to treat, especially if diagnosed at a late stage [14]

It's essential to note that each case is unique, and the specific characteristics, symptoms, and treatment outcomes may vary depending on individual circumstances. If you have any further questions or concerns, please don't hesitate to ask.

References: [4] - Context reference 4 [9] - Context reference 9 [11] - Context reference 11 [14] - Context reference 14 [15] - Context reference 15

Common Signs and Symptoms

Pancreatic endocrine carcinomas, also known as neuroendocrine tumors (NETs), can cause a variety of symptoms depending on the location and type of tumor. Some common signs and symptoms include:

• Belly pain: Pain in the abdomen that may spread to the sides or back [1]
• Weight loss: Unintentional weight loss due to decreased appetite, nausea, and vomiting [2][3]
• Diarrhea: Frequent bowel movements with loose stools [4][5]
• Jaundice: Yellowing of the skin and whites of the eyes due to liver dysfunction [6][7]
• High blood sugar symptoms: Weakness, hunger, confusion, and other symptoms associated with diabetes [8]

Other Possible Symptoms

In some cases, pancreatic endocrine carcinomas may also cause:

• Gallstones: Small, hard deposits that form in the gallbladder [4]
• Indigestion: Discomfort or pain in the upper abdomen after eating [9][10]
• Pain in the abdomen or back: A lump or mass feeling under the skin [11]

Important Note

It's essential to seek medical attention if you experience any of these symptoms, as early diagnosis and treatment can significantly improve outcomes.

Diagnostic Tests for Pancreatic Endocrine Carcinoma

Pancreatic endocrine carcinoma, also known as pancreatic neuroendocrine tumor (PNET), is a rare type of cancer that originates in the pancreas. Diagnosing PNET can be challenging, but various tests and procedures can help confirm the diagnosis.

Blood Tests

• Blood tests are used to measure hormone levels and detect tumor markers.
• Specific tests for hormone levels include:
  • Chromogranin A (CgA) [4]
  • Neuron-specific enolase (NSE)
  • Pancreatic polypeptide
• Elevated levels of these hormones can indicate the presence of a PNET.

Imaging Tests

• Abdominal computed tomography (CT) scan [3]
• Magnetic resonance imaging (MRI)
• Neuroendocrine positron emission tomography (

Treatment Options for Pancreatic Endocrine Carcinoma

Pancreatic endocrine carcinomas, also known as pancreatic neuroendocrine tumors (PNETs), are rare and complex cancers that require a multidisciplinary approach to treatment. The goal of drug therapy is to control the growth and spread of the tumor, alleviate symptoms, and improve quality of life.

Targeted Therapy

• Everolimus: This medication blocks a protein called mTOR, which helps cells grow and divide. Everolimus has been shown to be effective in treating advanced PNETs [1].
• Sunitinib: Another targeted therapy, sunitinib works by inhibiting tyrosine kinases, which are enzymes involved in cell growth and division. It is also used to treat PNETs [6].

Chemotherapy

While chemotherapy is not the primary treatment for PNETs, it may be considered in certain situations, such as when the tumor has spread or is resistant to targeted therapy. Combination chemotherapy, which involves using multiple anticancer drugs, may be used to treat PNETs [3].

Peptide Receptor Radionuclide Therapy (PRRT)

PRRT combines a medicine that targets cancer cells with a small amount of a radioactive substance. This treatment can help reduce tumor size and alleviate symptoms [4].

Other Treatment Options

• Somatostatin analogs: These medications can help control hormone production and alleviate symptoms associated with PNETs.
• Cabozantinib: This medication has been shown to improve outcomes in patients with previously treated extra-pancreatic and pancreatic neuroendocrine tumors [9].

Timing and Choice of Treatment

Effective drug therapy for PNETs relies on timing and choice. A team of experts, including medical oncologists, endocrinologists, and surgeons, should work together to determine the best course of treatment for each patient.

References:

[1] Everolimus blocks a protein known as mTOR, which normally helps cells grow and divide. Everolimus has been shown to help treat advanced pancreatic NETs [1].

[3] Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated [3].

[4] Peptide receptor radionuclide therapy, also called PRRT. PRRT combines a medicine that targets cancer cells with a small amount of a radioactive substance [4].

[6] The targeted therapy drugs used are sunitinib (Sutent) and everolimus (Afinitor). They are taken daily as a pill by mouth (orally) [7].

[9] Cabozantinib significantly improved outcomes in patients with previously treated extra-pancreatic and pancreatic neuroendocrine tumors and may be considered for treatment [9].

Differential Diagnosis of Pancreatic Endocrine Carcinoma

Pancreatic endocrine carcinomas, also known as pancreatic neuroendocrine tumors (PNETs), are a type of cancer that originates from the endocrine cells of the pancreas. The differential diagnosis of PNETs involves distinguishing them from other types of pancreatic lesions.

Key Points to Consider:

• Vascularization patterns: Contrast-enhanced CT and MRI can help differentiate between benign and malignant lesions based on characteristic vascularization patterns [1, 2].
• Tumor margin and location: The differential diagnosis of PNETs and pancreatic ductal adenocarcinomas (PDACs) is usually easy due to the differences in tumor margin, vascularization pattern, and pancreatic duct involvement [3].
• Age and gender: Age in combination with gender is a valuable parameter in differential diagnosis of solid pseudopapillary tumors and PNETs [4].
• Imaging features: Imaging features such as tumor size, location, and vascularization can help differentiate between PNETs and other pancreatic lesions [5].

Entities to Consider in Differential Diagnosis:

• Pancreatic ductal adenocarcinoma (PDAC): The most important entity in the differential diagnosis of pancreatic adenocarcinoma is chronic pancreatitis (CP) [6].
• Chronic pancreatitis (CP): Patients with CP frequently present with recurrent abdominal pain, which can mimic symptoms of PNETs [7].

Other Considerations:

• Pancreatic metastases: Pancreatic metastases from renal cell carcinoma, melanoma, and sarcoma may often appear as hypervascular masses resembling PNETs [8].
• Well-differentiated neuroendocrine tumors (WDNETs): Diffuse, strong SSTR expression supports a diagnosis of WDNET, while PDNEC often shows limited or negative expression [9].

References:

[1] C Guo · 2017 · Cited by 20 [2] B Xiao · 2019 · Cited by 6 [3] L Khanna · 2020 · Cited by 110 [4] SS Kim · 2018 · Cited by 4 [5] Z Yang · 2022 · Cited by 1 [6] Lewis RB, Lattin GE Jr, Paal E. Pancreatic endocrine tumors: radiologic-clinicopathologic ... [7] Approximately three quarters of PCs arise in the head of the pancreas, 17%-26% in the body and tail and the remaining 5%-8% in multiple pancreatic locations [10]. [8] The most important entity in the differential diagnosis of pancreatic adenocarcinoma is CP. Patients with CP frequently present with recurrent abdominal pain [6]. [9] DIFFERENTIAL DIAGNOSIS. The differential diagnosis for panNEN includes other hypervascular pancreatic lesions [8].