Intraductal papillary mucinous neoplasm | |
---|---|
Other names | IPMN |
Intraductal papillary mucinous neoplasm in magnetic resonance imaging. | |
Specialty | Gastroenterology |
Symptoms | Usually asymptomatic |
Usual onset | 50-70 years of age |
Types | Main duct, branch duct or mixed |
Risk factors | Male gender |
Differential diagnosis | Mucinous cystic neoplasm |
Treatment | Imaging surveillance, surgical resection |
Intraductal papillary mucinous neoplasm (IPMN) is a type of tumor that can occur within the cells of the pancreatic duct. IPMN tumors produce mucus, [1] and this mucus can form pancreatic cysts. [2] Although intraductal papillary mucinous neoplasms are benign tumors, they can progress to pancreatic cancer. [1] As such IPMN is viewed as a precancerous condition. [3] Once an intraductal papillary mucinous neoplasm has been found, the management options include close monitoring and pre-emptive surgery.[ medical citation needed ]
IPMNs are lined with mucin-secreting columnar epithelium.
In most cases, IPMNs are diagnosed based on clinical and radiographic criteria. [5] If fluid from the cyst is aspirated, the CEA level is typically elevated. [5] Confirmation of the diagnosis with tissue is rarely necessary. [5]
By histopathology, IPMN is characterized on light microscopy by Mucinous epithelial cells, [6] and growth within the pancreatic ducts. [7] Mucin 5AC is a useful immunohistochemistry marker. [8] Characteristic genetic alterations are those of KRAS and GNAS. [8]
Further subtyping of IPMN can be done as either: [9]
The treatment of choice for main-duct IPMNs is resection due to approximately 50% chance of malignancy. Side-branch IPMNs are occasionally monitored with regular CT or MRIs, but most are eventually resected, with a 30% rate of malignancy in these resected tumors.[ citation needed ] Indications for surgical resection include obstructive jaundice, an enhancing mural nodule >5 mm, and pancreatic duct dilation (>10 mm). [12]
Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). [13] In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy [14] or robotic surgery. A study using Surveillance, Epidemiology, and End Results Registry (SEER) data suggested that increased lymph node counts harvested during the surgery were associated with better survival in invasive IPMN patients. [15]
Survival 5 years after resection of an IPMN without malignancy is approximately 80%, 85% with malignancy but no lymph node spread and 0% with malignancy spreading to lymph nodes. [16]
Side branch IPMNs are the most common pancreatic cysts. [5] IPMNs occur more often in men than women, and often occur in the 6th and 7th decade of life.
In 1982, IPMN was reported as a "mucin-producing tumor" by Kazuhiko Ohashi of the Japanese Foundation for Cancer Research.
Pancreatic cancer arises when cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a mass. These cancerous cells have the ability to invade other parts of the body. A number of types of pancreatic cancer are known.
Keratin 20, often abbreviated CK20, is a protein that in humans is encoded by the KRT20 gene.
Pseudomyxoma peritonei (PMP) is a clinical condition caused by cancerous cells that produce abundant mucin or gelatinous ascites. The tumors cause fibrosis of tissues and impede digestion or organ function, and if left untreated, the tumors and mucin they produce will fill the abdominal cavity. This will result in compression of organs and will destroy the function of the colon, small intestine, stomach, or other organs. Prognosis with treatment in many cases is optimistic, but the disease is lethal if untreated, with death occurring via cachexia, bowel obstruction, or other types of complications.
In medicine, a pancreatectomy is the surgical removal of all or part of the pancreas. Several types of pancreatectomy exist, including pancreaticoduodenectomy, distal pancreatectomy, segmental pancreatectomy, and total pancreatectomy. In recent years, the TP-IAT has also gained respectable traction within the medical community. These procedures are used in the management of several conditions involving the pancreas, such as benign pancreatic tumors, pancreatic cancer, and pancreatitis.
The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.
Cystadenoma is a type of cystic adenoma. When malignant, it is called cystadenocarcinoma.
Pancreatic diseases are diseases that affect the pancreas, an organ in most vertebrates and in humans and other mammals located in the abdomen. The pancreas plays a role in the digestive and endocrine system, producing enzymes which aid the digestion process and the hormone insulin, which regulates blood sugar levels. The most common pancreatic disease is pancreatitis, an inflammation of the pancreas which could come in acute or chronic form. Other pancreatic diseases include diabetes mellitus, exocrine pancreatic insufficiency, cystic fibrosis, pseudocysts, cysts, congenital malformations, tumors including pancreatic cancer, and hemosuccus pancreaticus.
Acinic cell carcinoma is a malignant tumor representing 2% of all salivary tumors. 90% of the time found in the parotid gland, 10% intraorally on buccal mucosa or palate. The disease presents as a slow growing mass, associated with pain or tenderness in 50% of the cases. Often appears pseudoencapsulated.
Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).
Eccrine carcinoma is a rare skin condition characterized by a plaque or nodule on the scalp, trunk, or extremities. It originates from the eccrine sweat glands of the skin, accounting for less than 0.01% of diagnosed cutaneous malignancies. Eccrine carcinoma tumors are locally aggressive, with a high rate of recurrence. Lack of reliable immunohistochemical markers and similarity to other common tumors has made identification of eccrine carcinoma difficult.
Mucinous cystadenocarcinoma of the lung (MCACL) is a very rare malignant mucus-producing neoplasm arising from the uncontrolled growth of transformed epithelial cells originating in lung tissue.
Pancreatic serous cystadenoma is a benign tumour of the pancreas. It is usually solitary and found in the body or tail of the pancreas, and may be associated with von Hippel–Lindau syndrome.
A solid pseudopapillary tumour is a low-grade malignant neoplasm of the pancreas of papillary architecture that typically afflicts young women.
Ductal cells refer to the epithelial cell lining of the pancreatic duct that deliver enzymes from the acinar cells to the duodenum. They have the essential function of producing bicarbonate-rich (HCO3-) secretion to neutralize stomach acidity. The hormone secretin stimulates ductal cells and is responsible for maintaining the duodenal pH and preventing duodenal injury from acidic chyme. Ductal cells mix their production with acinar cells to make up the pancreatic juice.
A pancreatic tumor is an abnormal growth in the pancreas. In adults, almost 90% are pancreatic cancer and a few are benign. Pancreatic tumors are rare in children.
Cystic lesions of the pancreas are a group of pancreatic lesions characterized by a cystic appearance. They can be benign or malignant.
A pancreatic cyst is a fluid filled sac within the pancreas.
A mucinous cystic neoplasm is an abnormal and excessive growth of tissue (neoplasm) that typically has elements of mucin and one or more cysts. By location, they include:
Pancreatic mucinous cystic neoplasm (MCN) is a type of cystic lesion that occurs in the pancreas. Amongst individuals undergoing surgical resection of a pancreatic cyst, about 23 percent were mucinous cystic neoplasms. These lesions are benign, though there is a high rate of progression to cancer. As such, surgery should be pursued when feasible. The rate of malignancy present in MCN is about 10 percent. If resection is performed before invasive malignancy develops, prognosis is excellent. The extent of invasion is the single most important prognostic factor in predicting survival.
Papillary carcinomas of the breast (PCB), also termed malignant papillary carcinomas of the breast, are rare forms of the breast cancers. The World Health Organization (2019) classified papillary neoplasms of the breast into 5 types: intraductal papilloma, papillary ductal carcinoma in situ (PDCIS), encapsulated papillary carcinoma (EPC), solid-papillary carcinoma (SPC), and invasive papillary carcinoma (IPC). The latter four carcinomas are considered here; intraductal papilloma is a benign neoplasm. The World Health Organization regarded solid papillary carcinoma as having two subtypes: in situ and invasive SPC.
{{cite journal}}
: CS1 maint: multiple names: authors list (link)