Pathology of exocrine pancreatic neoplasms
- Daniel S Longnecker, MD
Daniel S Longnecker, MD
- Professor Emeritus
- Dartmouth Medical School
The pancreas gives rise to several malignant and benign neoplasms. At the histologic level, neoplasms of the pancreas can resemble normal ductal cells, acinar cells, or islet cells. In addition, some pancreatic neoplasms appear to arise from primitive cells that have the potential to differentiate along several lines, giving rise to complex tumors with mixed cell types (eg, pancreatoblastoma).
Pancreatic cancer ranks fourth among cancers as a cause of death (both sexes) in the United States, surpassed only by lung, colon, and breast cancers . The commonly used terms "carcinoma of the pancreas" or "pancreatic cancer" usually refer to ductal adenocarcinoma (including its subtypes), which represents approximately 85 percent of all pancreatic neoplasms. Of the several subtypes of ductal adenocarcinoma, most share a similar poor long-term prognosis, with the exception of colloid carcinomas, which have a somewhat better prognosis.
The more inclusive term "exocrine pancreatic neoplasms" includes all tumors that are related to the pancreatic ductal and acinar cells and their stem cells (including pancreatoblastoma). More than 95 percent of malignant neoplasms of the pancreas arise from the exocrine elements. Neoplasms arising from the endocrine pancreas (ie, islet cell tumors) comprise no more than 5 percent of pancreatic neoplasms .
Although the incidence of pancreatic cancer has been relatively stable over time, the increasing use of imaging techniques such as endoscopic ultrasound and helical (spiral) abdominal computed tomography (CT) scans has revealed an increasing number of incidentally found cystic lesions in the pancreas, many of which are neoplasms. This has focused attention on the diagnosis and management of cystic neoplasms of the pancreas.
Of the cystic neoplasms that arise in the pancreas, some (eg, intraductal papillary mucinous neoplasms [IPMN]) have a significant malignant potential (30 to 40 percent in reported series) while others (eg, serous cystadenomas) almost always remain benign. The distinction on clinical grounds between these two types of neoplasms, other cystic neoplasms, and other nonneoplastic cystic pancreatic masses (pseudocysts and developmental cysts) can be difficult. The exclusion of malignancy in a cystic pancreatic lesion often requires surgical resection and histopathological evaluation . (See "Classification of pancreatic cysts" and "Intraductal papillary mucinous neoplasm of the pancreas (IPMN): Pathophysiology and clinical manifestations".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Siegel RL, Miller KD, Jemal A. Cancer Statistics, 2017. CA Cancer J Clin 2017; 67:7.
- Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol 2007; 20 Suppl 1:S94.
- Crippa S, Salvia R, Warshaw AL, et al. Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Ann Surg 2008; 247:571.
- Hruban RH, Pitman MB, Klimstra DS. Tumors of the pancreas. In: Atlas of tumor pathology, Fascicle 6, Armed Forces Institute of Pathology, Washington, DC 2007. Vol 4th series.
- Chaper 12 - Tumours of the Pancreas. In: WHO Classification of Tumours of the Digestive System, Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds), IARC Press, Lyon 2010.
- Basturk O, Hong SM, Wood LD, et al. A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas. Am J Surg Pathol 2015; 39:1730.
- Klöppel G. Chronic pancreatitis, pseudotumors and other tumor-like lesions. Mod Pathol 2007; 20 Suppl 1:S113.
- Gil Z, Cavel O, Kelly K, et al. Paracrine regulation of pancreatic cancer cell invasion by peripheral nerves. J Natl Cancer Inst 2010; 102:107.
- Kamarajah SK, Burns WR, Frankel TL, et al. Validation of the American Joint Commission on Cancer (AJCC) 8th Edition Staging System for Patients with Pancreatic Adenocarcinoma: A Surveillance, Epidemiology and End Results (SEER) Analysis. Ann Surg Oncol 2017; 24:2023.
- Hruban RH, Adsay NV, Albores-Saavedra J, et al. Pancreatic intraepithelial neoplasia: a new nomenclature and classification system for pancreatic duct lesions. Am J Surg Pathol 2001; 25:579.
- Hruban RH, Takaori K, Klimstra DS, et al. An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms. Am J Surg Pathol 2004; 28:977.
- Terhune PG, Phifer DM, Tosteson TD, Longnecker DS. K-ras mutation in focal proliferative lesions of human pancreas. Cancer Epidemiol Biomarkers Prev 1998; 7:515.
- Stelow EB, Adams RB, Moskaluk CA. The prevalence of pancreatic intraepithelial neoplasia in pancreata with uncommon types of primary neoplasms. Am J Surg Pathol 2006; 30:36.
- Shi C, Klein AP, Goggins M, et al. Increased Prevalence of Precursor Lesions in Familial Pancreatic Cancer Patients. Clin Cancer Res 2009; 15:7737.
- Brat DJ, Lillemoe KD, Yeo CJ, et al. Progression of pancreatic intraductal neoplasias to infiltrating adenocarcinoma of the pancreas. Am J Surg Pathol 1998; 22:163.
- Brockie E, Anand A, Albores-Saavedra J. Progression of atypical ductal hyperplasia/carcinoma in situ of the pancreas to invasive adenocarcinoma. Ann Diagn Pathol 1998; 2:286.
- Takaori K, Matsusue S, Fujikawa T, et al. Carcinoma in situ of the pancreas associated with localized fibrosis: a clue to early detection of neoplastic lesions arising from pancreatic ducts. Pancreas 1998; 17:102.
- Konstantinidis IT, Vinuela EF, Tang LH, et al. Incidentally discovered pancreatic intraepithelial neoplasia: what is its clinical significance? Ann Surg Oncol 2013; 20:3643.
- Tanaka M, Chari S, Adsay V, et al. International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology 2006; 6:17.
- Histologic Typing of Tumours of the Exocrine Pancreas, Klöppel G, Solcia E, Longnecker DS, et al (Eds), Springer-Verlag, New York 1996.
- Klibansky DA, Reid-Lombardo KM, Gordon SR, Gardner TB. The clinical relevance of the increasing incidence of intraductal papillary mucinous neoplasm. Clin Gastroenterol Hepatol 2012; 10:555.
- Furukawa T, Klöppel G, Volkan Adsay N, et al. Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study. Virchows Arch 2005; 447:794.
- Takasu N, Kimura W, Moriya T, et al. Intraductal papillary-mucinous neoplasms of the gastric and intestinal types may have less malignant potential than the pancreatobiliary type. Pancreas 2010; 39:604.
- Grützmann R, Niedergethmann M, Pilarsky C, et al. Intraductal papillary mucinous tumors of the pancreas: biology, diagnosis, and treatment. Oncologist 2010; 15:1294.
- Adsay NV, Pierson C, Sarkar F, et al. Colloid (mucinous noncystic) carcinoma of the pancreas. Am J Surg Pathol 2001; 25:26.
- Klimstra DS, Pitman MB, Hruban RH. An algorithmic approach to the diagnosis of pancreatic neoplasms. Arch Pathol Lab Med 2009; 133:454.
- Masia R, Mino-Kenudson M, Warshaw AL, et al. Pancreatic mucinous cystic neoplasm of the main pancreatic duct. Arch Pathol Lab Med 2011; 135:264.
- Goh BK, Tan YM, Chung YF, et al. A review of mucinous cystic neoplasms of the pancreas defined by ovarian-type stroma: clinicopathological features of 344 patients. World J Surg 2006; 30:2236.
- Baker ML, Seeley ES, Pai R, et al. Invasive mucinous cystic neoplasms of the pancreas. Exp Mol Pathol 2012; 93:345.
- Tseng JF, Warshaw AL, Sahani DV, et al. Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment. Ann Surg 2005; 242:413.
- Goh BK, Tan YM, Yap WM, et al. Pancreatic serous oligocystic adenomas: clinicopathologic features and a comparison with serous microcystic adenomas and mucinous cystic neoplasms. World J Surg 2006; 30:1553.
- Bassi C, Salvia R, Molinari E, et al. Management of 100 consecutive cases of pancreatic serous cystadenoma: wait for symptoms and see at imaging or vice versa? World J Surg 2003; 27:319.
- Reese SA, Traverso LW, Jacobs TW, Longnecker DS. Solid serous adenoma of the pancreas: a rare variant within the family of pancreatic serous cystic neoplasms. Pancreas 2006; 33:96.
- Strobel O, Z'graggen K, Schmitz-Winnenthal FH, et al. Risk of malignancy in serous cystic neoplasms of the pancreas. Digestion 2003; 68:24.
- Law JK, Ahmed A, Singh VK, et al. A systematic review of solid-pseudopapillary neoplasms: are these rare lesions? Pancreas 2014; 43:331.
- Marchegiani G, Andrianello S, Massignani M, et al. Solid pseudopapillary tumors of the pancreas: Specific pathological features predict the likelihood of postoperative recurrence. J Surg Oncol 2016; 114:597.
- Surgery 2016; 159:1023.
- Martin SK, Agarwal G, Lynch GR. Subcutaneous fat necrosis as the presenting feature of a pancreatic carcinoma: the challenge of differentiating endocrine and acinar pancreatic neoplasms. Pancreas 2009; 38:219.
- Wisnoski NC, Townsend CM Jr, Nealon WH, et al. 672 patients with acinar cell carcinoma of the pancreas: a population-based comparison to pancreatic adenocarcinoma. Surgery 2008; 144:141.
- Schmidt CM, Matos JM, Bentrem DJ, et al. Acinar cell carcinoma of the pancreas in the United States: prognostic factors and comparison to ductal adenocarcinoma. J Gastrointest Surg 2008; 12:2078.
- Holen KD, Klimstra DS, Hummer A, et al. Clinical characteristics and outcomes from an institutional series of acinar cell carcinoma of the pancreas and related tumors. J Clin Oncol 2002; 20:4673.
- Bien E, Godzinski J, Dall'igna P, et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer 2011; 47:2347.
- Glick RD, Pashankar FD, Pappo A, Laquaglia MP. Management of pancreatoblastoma in children and young adults. J Pediatr Hematol Oncol 2012; 34 Suppl 2:S47.
- Perez EA, Gutierrez JC, Koniaris LG, et al. Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg 2009; 44:197.
- Dhebri AR, Connor S, Campbell F, et al. Diagnosis, treatment and outcome of pancreatoblastoma. Pancreatology 2004; 4:441.
- Cavallini A, Falconi M, Bortesi L, et al. Pancreatoblastoma in adults: a review of the literature. Pancreatology 2009; 9:73.
- Premalignant lesions
- PANCREATIC DUCTAL ADENOCARCINOMA
- Gross pathology
- Histology and grading
- Patterns of local spread
- PANCREATIC INTRAEPITHELIAL NEOPLASIA
- OTHER NEOPLASMS
- Intraductal papillary-mucinous neoplasms (IPMN)
- - Histology and grading
- Mucinous cystic neoplasms (MCN)
- - Histology and grading
- Serous cystadenoma
- Solid pseudopapillary neoplasms (SPN)
- Acinar cell carcinoma
- Other rare types