Pancreatic cyst

Last updated
Pancreatic cyst
Specialty Gastroenterology
Symptoms Usually asymptomatic. In some cases abdominal pain, jaundice, weight loss [1]
Complications Pancreatitis, pancreatic cancer
TreatmentSurgical resection for high risk cysts
Relative incidences of various pancreatic neoplasms, with cysts annotated at center right. Also, non-neoplastic cysts include pseudocyst, retention cyst, benign epithelial cysts, lymphoepithelial cysts, squamous lined cysts (dermoid cyst and epidermal cyst in intrapancreatic accessory spleen), mucinous nonneoplastic cysts, and lymphangiomas. Relative incidence of pancreatic neoplasms.png
Relative incidences of various pancreatic neoplasms, with cysts annotated at center right. Also, non-neoplastic cysts include pseudocyst, retention cyst, benign epithelial cysts, lymphoepithelial cysts, squamous lined cysts (dermoid cyst and epidermal cyst in intrapancreatic accessory spleen), mucinous nonneoplastic cysts, and lymphangiomas.

A pancreatic cyst is a fluid filled sac within the pancreas. The prevalence of pancreatic cysts is 2-15% based on imaging studies, but the prevalence may be as high as 50% based on autopsy series. [1] Most pancreatic cysts are benign and the risk of malignancy (pancreatic cancer) is 0.5-1.5%. Pancreatic pseudocysts and serous cystadenomas (which collectively account for 15-25% of all pancreatic cysts) are considered benign pancreatic cysts with a risk of malignancy of 0%. [1]

Contents

Causes range from benign to malignant. Pancreatic cysts can occur in the setting of pancreatitis, though they are only reliably diagnosed 6 weeks after the episode of acute pancreatitis.

Main branch intraductal papillary mucinous neoplasms (IPMNs) are associated with dilatation of the main pancreatic duct, while side branch IPMNs are not associated with dilatation. MRCP can help distinguish the position of the cysts relative to the pancreatic duct, and direct appropriate treatment and follow-up. The most common malignancy that can present as a pancreatic cyst is a mucinous cystic neoplasm.

Diagnosis

Pancreatic cysts are usually seen incidentally when medical imaging is obtained for other purposes and they are usually asymptomatic. [1] Pancreatic cysts may sometimes be definitively diagnosed based on imaging findings from an MRI or CT scan with contrast. However, sometimes additional imaging is required, such as an endoscopic ultrasound with or without fine needle aspiration or magnetic resonance cholangiopancreatography (MRCP). [1] EUS has a higher accuracy in diagnosing high risk radiographic features of pancreatic cysts compared to MRI, especially if contrast enhancement is also used. [1] Based on imaging, cysts that cause biliary obstruction, dilation of the main pancreatic duct greater than 10 mm, have a mass in their walls greater than 5 mm are considered high risk features and are associated with a 56-89% risk of cancer. [1] Cyst size greater than 3 cm, main pancreatic duct dilation of 5-10 mm, or a change in caliber or a narrowing of the main pancreatic duct with atrophy of the duct distally, presence of lymph node swelling, thickened or enhancing cyst walls, or an increase in cyst size over a year are considered intermediate risk imaging findings for cancer. [1]

Lab workup and other clinical findings can also be used to assess malignant risk of pancreatic cysts. An elevation in the biomarker CA19-9, new onset diabetes, pancreatitis, abdominal pain or weight loss are all considered high risk features, with the presence of jaundice being a very high risk feature. [1]

Cytologic analysis of the cystic fluid can help distinguish what type of pancreatic cyst is present, but it is not helpful in grading. However, cytologic fluid analysis by fine needle aspiration has low specificity as most samples contain only fluid without specific cell types. [1] Elevated levels of amylase in the fluid suggest communication with the pancreatic duct, which is indicative of a pseudocyst or IPMN. [1] Increased levels of carcinoembryonic antigen (CEA) are indicative of mucinous cysts in 75% of cases, and very low levels of CEA effectively rule out mucinous cysts. [1] And reduced glucose levels in the cyst fluid is useful in differentiating (with an approximate sensitivity of 90% and specificity of 85% at a cutoff of 50 mg/dL) between mucinous and non-mucinous cysts, with mucinous cysts having a low glucose level and non-mucinous cysts having a high glucose level. [4]

DNA analysis of the cystic fluid may aid in the diagnosis of pancreatic cysts, but yields are variable, between 25-50%. [1] VHL tumor suppressor gene mutations (associated with Von Hippel-Lindau disease) are associated with simple cysts, serous cystadenomas and less commonly pancreatic neuroendocrine tumors. [5] KRAS mutations are associated with mucinous cysts, GNAS mutations are associated with IPMNs, CTNNB1 mutations are associated with solid pseudopapillary tumours. [1]

Types

Pancreatic pseudocysts are benign, with a risk of malignant progression of 0%. [1] Pseudocysts are associated with acute or chronic pancreatitis and the cysts usually commnicate with the main pancreatic duct. They usually resolve spontaneously and are unilocular (not septated; ie. do not have walls separating parts of the cyst) and may be solitary or multiple.

Serous cystadenomas are benign as well, with a risk of malignant potential of 0% and they usually present in the 5-7th decade of life with 60% of instances being in women. They do not communicate with the main pancreatic duct. [1] Their imaging characteristics vary and have been described as muti-cystic with a honeycomb appearance, with less common variants being solid, macrocystic or unilocular. [1] Serous systadenomas may have a central scar in the cyst seen on CT or MRI, and this is characteristic of the type, but is only seen in 30% of cases. [1]

Intraductal papillary mucinous neoplasms (IPMN) involve the main pancreatic duct (main ductal IPMN) or its branches (branch duct IPMN) or both (mixed type IPMN). IPMNs are pre-malignant with main duct IPMNs having a 33-85% malignant potential and branch duct IPMNs having a 15% risk of malignancy at 15 years. [1] [6] IPMNs usually occur during the 5-th decade of life and have an equal male and female incidence. They may present as solitary or multiple lesions, and they may cause pancreatitis as the pancreatic duct is blocked by mucin. [1]

Pancreatic mucinous cystic neoplasm usually involve the tail of the pancreas and 90% of cases involve women and they usually present in the 4-6th decade of life. They have a malignant potential of 10-34%. [1] They do not communicate with the pancreatic ducts and they usually present as single, thick walled, non-loculated (having a single chamber) lesions. They characteristically contain ovarian type stromal cells. [1]

Pancreatic neuroendocrine tumors may sometimes undergo cystic degeneration forming cysts. These types of tumors arise from pancreatic endocrine cells, and 10% are functional, being able to secrete hormones. [1] They are characterized on imaging by their thick walls. 80% of these tumors express somatostatin receptors thus allowing them to be visualized on Gallium DOTA scans. [1]

Follow up guidelines

Cysts from 1–5 mm on CT or ultrasound are typically too small to characterize and considered benign. No further imaging follow-up is recommended for these lesions. Cysts from 6–9 mm require a single follow-up in 2–3 years, preferably with magnetic resonance cholangiopancreatography (MRCP) to better evaluate the pancreatic duct. If stable at follow-up, no further imaging follow-up is recommended. For cysts from 1–1.9 cm follow-up is suggested with MRCP or multiphasic CT in 1–2 years. If stable at follow-up, the interval of imaging follow-up is increased to 2–3 years. Cysts from 2–2.9 cm have more malignant potential, and a baseline endoscopic ultrasound is suggested, followed by MRCP or multiphasic CT in 6–12 months. If patients are young, surgery may be considered to avoid the need for prolonged surveillance. If these cysts are stable at follow-up, interval imaging follow-up can be done in 1–2 years. [7]

Related Research Articles

<span class="mw-page-title-main">Pancreatic cancer</span> Type of endocrine gland cancer

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.

<span class="mw-page-title-main">Chronic pancreatitis</span> Medical condition

Chronic pancreatitis is a long-standing inflammation of the pancreas that alters the organ's normal structure and functions. It can present as episodes of acute inflammation in a previously injured pancreas, or as chronic damage with persistent pain or malabsorption. It is a disease process characterized by irreversible damage to the pancreas as distinct from reversible changes in acute pancreatitis. Tobacco smoke and alcohol misuse are two of the most frequently implicated causes, and the two risk factors are thought to have a synergistic effect with regards to the development of chronic pancreatitis. Chronic pancreatitis is a risk factor for the development of pancreatic cancer.

<span class="mw-page-title-main">Serous tumour</span> Medical condition

A serous tumour is a neoplasm that typically has papillary to solid formations of tumor cells with crowded nuclei, and which typically arises on the modified Müllerian-derived serous membranes that surround the ovaries in females. Such ovarian tumors are part of the surface epithelial-stromal tumour group of ovarian tumors. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.

<span class="mw-page-title-main">Surface epithelial-stromal tumor</span> Medical condition

Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium or from ectopic endometrial or fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer; however is mainly only found in postmenopausal women with the exception of the United States where 7% of cases occur in women under the age of 40. Serum CA-125 is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment. 75% of women with epithelial ovarian cancer are found within the advanced-stages; however younger patients are more likely to have better prognoses than older patients.

<span class="mw-page-title-main">Pancreatectomy</span> Surgical removal of the pancreas

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 total pancreatectomy, the gallbladder, distal stomach, a portion of the small intestine, associated lymph nodes and in certain cases the spleen are removed in addition to the entire pancreas. 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.

<span class="mw-page-title-main">Pancreatic pseudocyst</span> Medical condition

A pancreatic pseudocyst is a circumscribed collection of fluid rich in pancreatic enzymes, blood, and non-necrotic tissue, typically located in the lesser sac of the abdomen. Pancreatic pseudocysts are usually complications of pancreatitis, although in children they frequently occur following abdominal trauma. Pancreatic pseudocysts account for approximately 75% of all pancreatic masses.

<span class="mw-page-title-main">Pseudocyst</span> Cyst-like sac growth lacking an epithelial or endothelial cell lining

Pseudocysts are like cysts, but lack epithelial or endothelial cells. Initial management consists of general supportive care. Symptoms and complications caused by pseudocysts require surgery. Computed tomography (CT) scans are used for initial imaging of cysts, and endoscopic ultrasounds are used in differentiating between cysts and pseudocysts. Endoscopic drainage is a popular and effective method of treating pseudocysts.

<span class="mw-page-title-main">Cystadenoma</span> Medical condition

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.

<span class="mw-page-title-main">Cystadenocarcinoma</span> Medical condition

Cystadenocarcinoma is a malignant form of a cystadenoma and is a cancer derived from glandular epithelium, in which cystic accumulations of retained secretions are formed. The neoplastic cells manifest varying degrees of anaplasia and invasiveness, and local extension and metastases occur. Cystadenocarcinomas develop frequently in the ovaries, where pseudomucinous and serous types are recognized. Similar tumor histology has also been reported in the pancreas, although it is a considerably rarer entity representing 1–1.5% of all Pancreatic cancer.

<span class="mw-page-title-main">Mucinous cystadenoma</span> Medical condition

Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).

<span class="mw-page-title-main">Intraductal papillary mucinous neoplasm</span> Medical condition

Intraductal papillary mucinous neoplasm (IPMN) is a type of tumor that can occur within the cells of the pancreatic duct. IPMN tumors produce mucus, and this mucus can form pancreatic cysts. Although intraductal papillary mucinous neoplasms are benign tumors, they can progress to pancreatic cancer. As such IPMN is viewed as a precancerous condition. Once an intraductal papillary mucinous neoplasm has been found, the management options include close monitoring and pre-emptive surgery.

<span class="mw-page-title-main">Pancreatic serous cystadenoma</span> Medical condition

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.

<span class="mw-page-title-main">Ovarian serous cystadenoma</span> Medical condition

Ovarian serous cystadenoma, also known as serous cystadenoma, is the most common ovarian neoplasm, representing 20% of ovarian neoplasms, and is benign.

<span class="mw-page-title-main">Solid pseudopapillary tumour</span> Medical condition

A solid pseudopapillary tumour is a low-grade malignant neoplasm of the pancreas of papillary architecture that typically afflicts young women.

<span class="mw-page-title-main">Pancreatic tumor</span> Medical condition

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.

Pancreatic mucinous cystadenoma is a tumour of pancreas. It may be benign or be associated with an invasive carcinoma component.

<span class="mw-page-title-main">Cystic lesions of the pancreas</span> Medical condition

Cystic lesions of the pancreas are a group of pancreatic lesions characterized by a cystic appearance. They can be benign or malignant.

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.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 Gonda, Tamas A.; Cahen, Djuna L.; Farrell, James J. (5 September 2024). "Pancreatic Cysts". New England Journal of Medicine. 391 (9): 832–843. doi:10.1056/NEJMra2309041. PMID   39231345.
  2. ↑ Wang Y, Miller FH, Chen ZE, Merrick L, Mortele KJ, Hoff FL; et al. (2011). "Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas". Radiographics. 31 (3): E47-64. doi:10.1148/rg.313105174. PMID   21721197.{{cite journal}}: CS1 maint: multiple names: authors list (link)
    Diagram by Mikael Häggström, M.D.
  3. ↑ Kim YS, Cho JH (2015). "Rare nonneoplastic cysts of pancreas". Clin Endosc. 48 (1): 31–8. doi:10.5946/ce.2015.48.1.31. PMC   4323429 . PMID   25674524.
  4. ↑ Mohan, Babu P.; Madhu, Deepak; Khan, Shahab R.; Kassab, Lena L.; Ponnada, Suresh; Chandan, Saurabh; Facciorusso, Antonio; Crino, Stefano F.; Barresi, Luca; McDonough, Stephanie; Adler, Douglas G. (1 February 2022). "Intracystic Glucose Levels in Differentiating Mucinous From Nonmucinous Pancreatic Cysts: A Systematic Review and Meta-analysis". Journal of Clinical Gastroenterology. 56 (2): e131–e136. doi:10.1097/MCG.0000000000001507. PMID   33731599.
  5. ↑ van Asselt, Sophie J; de Vries, Elisabeth GE; van Dullemen, Hendrik M; Brouwers, Adrienne H; Walenkamp, Annemiek ME; Giles, Rachel H; Links, Thera P (December 2013). "Pancreatic cyst development: insights from von Hippel-Lindau disease". Cilia. 2 (1): 3. doi: 10.1186/2046-2530-2-3 . PMC   3579754 . PMID   23384121.
  6. ↑ Oyama, Hiroki; Tada, Minoru; Takagi, Kaoru; Tateishi, Keisuke; Hamada, Tsuyoshi; Nakai, Yousuke; Hakuta, Ryunosuke; Ijichi, Hideaki; Ishigaki, Kazunaga; Kanai, Sachiko; Kogure, Hirofumi; Mizuno, Suguru; Saito, Kei; Saito, Tomotaka; Sato, Tatsuya; Suzuki, Tatsunori; Takahara, Naminatsu; Morishita, Yasuyuki; Arita, Junichi; Hasegawa, Kiyoshi; Tanaka, Mariko; Fukayama, Masashi; Koike, Kazuhiko (January 2020). "Long-term Risk of Malignancy in Branch-Duct Intraductal Papillary Mucinous Neoplasms". Gastroenterology. 158 (1): 226–237.e5. doi:10.1053/j.gastro.2019.08.032. PMID   31473224.
  7. ↑ Campbell, NM; Katz, SS; Escalon, JG; Do, RK (March 2015). "Imaging patterns of intraductal papillary mucinous neoplasms of the pancreas: an illustrated discussion of the International Consensus Guidelines for the Management of IPMN". Abdominal Imaging. 40 (3): 663–77. doi:10.1007/s00261-014-0236-4. PMID   25219664. S2CID   10097983.
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