Intraductal Papillary Mucinous Neoplasms (IPMN)

Intraductal Papillary Mucinous Neoplasms (IPMN)

Overview

Intraductal Papillary Mucinous Neoplasms (IPMNs) are cystic neoplasm (tumors) that grow within the pancreatic ducts (intraductal) and are composed of mucin-producing cells (mucinous).

IPMNs are tumor in the pancreas which, if left untreated, can progress to an invasive cancer. Presenting as lesions, IPMNs form cysts within the pancreatic ducts and produce thick fluid (mucin). IPMNs often cause symptoms similar to other pancreatic conditions, such as nausea, vomiting, abdominal pain and weight loss.

IPMNs may involve the main pancreatic duct, branch ducts, or both. While IPMNs in the branch duct are at lower risk for developing malignancy, patients with IPMNs in the main duct are at higher risk. Consequently, proper and accurate diagnosis is recommended so that precise treatment can be delivered.

IPMNs that may lead to highly invasive cancer, can be divided into high-grade dysplasia (carcinoma, in situ), intermediate-grade dysplasia (borderline), and low-grade dysplasia (adenoma).

Our approach

Northwell Health’s Pancreas Disease Center is a nationwide leader in treating IPMNs. Our skilled staff includes surgeons, gastroenterologists, and other specialists, all dedicated to setting you on a path of health and wellness through innovative treatments and customized care.

We understand that diseases of the pancreas can be difficult to diagnose and treat. The wide range of symptoms and risk factors require careful testing and examination. We’ll guide you through the process, step-by-step -- and once we make a thorough diagnosis, we’ll work with you to create a care plan with the best treatment options for you.

Learn more about our approach and what the Pancreas Disease Center how we can help create a better life for you.

Symptoms

  • Abdominal or back pain
  • Nausea/Vomiting
  • Jaundice (a yellowing of the skin or eyes)
  • Weight loss
  • Pancreatitis
  • Fever
  • Steatorrhea (abnormal amounts of fat in the stools)

IPMNs are increasingly being found incidentally during imaging studies for other reasons, as they often produce no symptoms.

Risk factors for malignancy

  • More common in men
  • Main duct IPMN
  • Elderly patients
  • Symptoms presence
  • Tumor size > 2cm
  • Solid mural nodules
  • Enlarged lymph nodules
  • Distension of biliary and pancreatic main duct
  • Positive cyst cytology (CEA>200 in the content of the cyst)

Diagnosis

There are often no specific clinical signs or symptoms for the presence of IPMNs. However, the medical history, clinical examination, plus additional tests are used for the diagnostic evaluation.

IPMNs are mostly diagnosed through imaging techniques, including:

  • CT Scan
  • Abdominal ultrasound
  • Endoscopic ultrasound
  • MRI (Magnetic Resonance Imaging)
  • Magnetic resonance cholangiopancreatography (MRCP)
  • Fine Needle Aspiration Biopsy
  • Blood Tests: Blood count and Glucose Levels; Bilirubin; Pancreatic and Liver enzymes (examples: Serum Lipase, ALT, AST); Tumor markers (CA 19-9) 

Treatment

Resection is generally the treatment of choice for main-duct IPMNs since the chance of malignancy is about 50%. High survival rates should be expected at 5 years when there is no malignancy.

The survival rate after 5 years following treatment is about 80%-85%, if there is malignancy, but no lymph node spread. The rate decreases if malignancy has spread to the lymph nodes.

Surgery for IPMNs includes the removal of the head of the pancreas (pancreaticoduodenectomy), or the body and tail of the pancreas (distal pancreatectomy) - or in rare cases, the removal of the entire pancreas (a total pancreatectomy).

In select cases surgery can be performed using minimally invasive techniques such as laparoscopy or robotic surgery.

Side-branch IPMNs that are not resected, should be monitored by regular CT scans or MRIs; however, most are ultimately resected, with about a 30% rate of malignancy in the resected tumors.

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