Abstract
Pancreatic neoplasms are a wide group of solid and cystic lesions with different and often characteristic imaging features, clinical presentations, and management. Among solid tumors, ductal adenocarcinoma is the most common: it arises from exocrine pancreas, comprises about 90% of all pancreatic neoplasms, and generally has a bad prognosis; its therapeutic management must be multidisciplinary, involving surgeons, oncologists, gastroenterologists, radiologists, and radiotherapists. The second most common solid pancreatic neoplasms are neuroendocrine tumors: they can be divided into functioning or non-functioning and present different degrees of malignancy. Cystic pancreatic neoplasms comprise serous neoplasms, which are almost always benign, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms, which can vary from benign to frankly malignant lesions, and solid pseudopapillary tumors. Other pancreatic neoplasms, such as lymphoma, metastases, or pancreatoblastoma, are rarely seen in clinical practice and have different and sometimes controversial managements. Rare clinical presentations and imaging appearance of the most common pancreatic neoplasms, both solid and cystic, are more frequently seen and clinically relevant than rare pancreatic tumors; their pathologic and radiologic appearances must be known to improve their management. The purpose of this paper is to present some rare or uncommon clinical and radiological presentations of common pancreatic neoplasms providing examples of multi-modality imaging approach with pathologic correlations, thus describing the histopathological bases that can explain the peculiar imaging features, in order to avoid relevant misdiagnosis and to improve lesion management.
Similar content being viewed by others
References
Bosman FT, Carneiro F, Hruban RH, et al. (2010) WHO classification of tumours of the digestive system, 4th edn. WHO classification of tumours, vol 3. IARC WHO Classification of Tumours, no. 3
Cameron JL (2001) Pancreatic Cancer (American Cancer Society Atlas of Clinical Oncology). Norwalk: PMPH USA
Schima W, Ba-Ssalamah A, Kölblinger C, et al. (2007) Pancreatic adenocarcinoma. Eur Radiol 17(3):638–649
Sahani DV, Shah ZK, Catalano OA, et al. (2008) Radiology of pancreatic adenocarcinoma: current status of imaging. J Gastroenterol Hepatol 23(1):23–33
D’Onofrio M, Zamboni G, Faccioli N, et al. (2007) Ultrasonography of the pancreas. 4. Contrast-enhanced imaging. Abdom Imaging 32(2):171–181
D’Onofrio M, Zamboni GA, Malagò R, et al. (2009) Resectable pancreatic adenocarcinoma: is the enhancement pattern at contrast-enhanced ultrasonography a pre-operative prognostic factor? Ultrasound Med Biol 35(12):1929–1937
Prokesch RW, Chow LC, Beaulieu CF, et al. (2002) Isoattenuating pancreatic adenocarcinoma at multi-detector row CT: secondary signs. Radiology 224:764–768
Yoon SH, Lee JM, Cho JY, et al. (2011) Small (≤20 mm) pancreatic adenocarcinomas: analysis of enhancement pat- terns and secondary signs with multiphasic multidetector CT. Radiology 259:442–452
Kim JH, Park SH, Yu ES, et al. (2010) Visually isoattenuating pancreatic adenocarcinoma at dynamic-enhanced CT: frequency, clinical and pathologic characteristics, and diagnosis at imaging examinations. Radiology 257:87–96
Barugola G, Partelli S, Marcucci S, et al. (2009) Resectable pancreatic cancer: who really benefits from resection? Ann Surg Oncol 16(12):3316–3322
Minniti S, Bruno C, Biasiutti C, et al. (2003) Sonography versus helical CT in identification and staging of pancreatic ductal adenocarcinoma. J Clin Ultrasound 31(4):175–182
Kosmahl M, Pauser U, Anlauf M, et al. (2005) Pancreatic ductal adenocarcinomas with cystic features: neither rare nor uniform. Mod Pathol 18(9):1157–1164
Capelli P, Parisi A (2012) Pancreatic lesions: pathologic correlation. In: D’Onofrio M (ed) Ultrasonography of the pancreas. Imaging and pathologic correlations. New York: Spinger-Verlag, pp 165–186
Balci NC, Semelka RC (2001) Radiologic diagnosis and staging of pancreatic ductal adenocarcinoma. Eur J Radiol 38(2):105–112
Lee LY, Hsu HL, Chen HM, et al. (2003) Ductal adenocarcinoma of the pancreas with huge cystic degeneration: a lesion to be distinguished from pseudocyst and mucinous cystadenocarcinoma. Int J Surg Pathol 11(3):235–239
Ranson JH (1982) Etiological and prognostic factors in human acute pancreatitis: a review. Am J Gastroenterol 77(9):633–638
Neff CC, Simeone JF, Wittenberg J, et al. (1984) Inflammatory pancreatic masses. Problems in differentiating focal pancreatitis from carcinoma. Radiology 150(1):35–38
Grendell JH (1990) Idiopathic acute pancreatitis. Gastroenterol Clin North Am 19(4):843–848
Kim T, Murakami T, Takamura M, et al. (2001) Pancreatic mass due to chronic pancreatitis: correlation of CT and MR imaging features with pathologic findings. AJR Am J Roentgenol 177(2):367–371
D’Onofrio M (2009) The role of ultrasound. In: Balthazar EJ, Megibow AJ, Pozzi Mucelli R (eds) Imaging of the pancreas. Acute and chronic pancreatitis. New York: Springer-Verlag
Kim DH, Pickhardt PJ (2007) Radiologic assessment of acute and chronic pancreatitis. Surg Clin North Am 87(6):1341–1358, viii
Fulcher AS, Turner MA (1999) MR pancreatography: a useful tool for evaluating pancreatic disorders. Radiographics 19(1):5–24; discussion 41–44; quiz 148–149
Cereser L, Bali MA, Delhaye M, et al. (2009) The role of MR imaging in chronic pancreatitis. In: Balthazar EJ, Megibow AJ, Pozzi Mucelli R (eds) Imaging of the pancreas. Acute and chronic pancreatitis. New York: Springer-Verlag
Ichikawa T, Sou H, Araki T, et al. (2001) Duct-penetrating sign at MRCP: usefulness for differentiating inflammatory pancreatic mass from pancreatic carcinomas. Radiology 221(1):107–116
Klimstra DS, Modlin IR, Coppola D, et al. (2010) The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas 39(6):707–712
O’Grady HL, Conlon KC (2008) Pancreatic neuroendocrine tumours. Eur J Surg Oncol 34(3):324–332
Lewis RB, Lattin GE Jr, Paal E (2010) Pancreatic endocrine tumors: radiologic-clinicopathologic correlation. Radiographics 30(6):1445–1464
D’Onofrio M, Mansueto G, Falconi M, et al. (2004) Neuroendocrine pancreatic tumor: value of contrast enhanced ultrasonography. Abdom Imaging 29(2):246–258
Wang Y, Chen ZE, Yaghmai V, et al. (2011) Diffusion-weighted MR imaging in pancreatic endocrine tumors correlated with histopathologic characteristics. J Magn Reson Imaging 33(5):1071–1079
Jang KM, Kim SH, Lee SJ, et al. (2013) The value of gadoxetic acid-enhanced and diffusion-weighted MRI for prediction of grading of pancreatic neuroendocrine tumors. Acta Radiol 55(2):140–148
D’Onofrio M, Gallotti A, Pozzi Mucelli R (2010) Imaging techniques in pancreatic tumors. Expert Rev Med Devices 7(2):257–273
Ligneau B, Lombard-Bohas C, Partensky C, et al. (2001) Cystic endocrine tumors of the pancreas: clinical, radiologic, and histopathologic features in 13 cases. Am J Surg Pathol 25(6):752–760
Lesniak RJ, Hoehenwalter MD, Taylor AJ (2002) Spectrum of causes of pancreatic calcifications. AJR 178(1):79–86
Poultsides GA, Huang LC, Chen Y, et al. (2012) Pancreatic neuroendocrine tumors: radiographic calcifications correlate with grade and metastasis. Ann Surg Oncol 19(7):2295–2303
La Rosa S, Franzi F, Albarello L, et al. (2011) Serotonin-producing enterochromaffin cell tumors of the pancreas: clinicopathologic study of 15 cases and comparison with intestinal enterochromaffin cell tumors. Pancreas 40(6):883–895
Osamura RY, Oberg K, Speel EJM, et al. (2004) Serotonin-secreting tumor. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C (eds) WHO Classification of tumours: pathology and genetics-tumours of endocrine organs. Lyon: IARC Press
Modlin IM, Sandor A (1997) An analysis of 8305 cases of carcinoid tumours. Cancer 79(4):813–829
Mao C, el Attar A, Domenico DR, et al. (1998) Carcinoid tumors of the pancreas: status report based on two cases and review of the world’s literature. Int J Pancreatol 23(2):153–164
Kawamoto S, Shi C, Hruban RH, et al. (2011) Small serotonin-producing neuroendocrine tumor of the pancreas associated with pancreatic duct obstruction. AJR Am J Roentgenol 197(3):W482–W488
Shi C, Siegelman SS, Kawamoto S, et al. (2010) Pancreatic duct stenosis secondary to small endocrine neoplasms: a manifestation of serotonin production? Radiology 257(1):107–114
Villanueva A, Pérez C, Llauger J, et al. (1994) Carcinoid tumors of the pancreas: CT findings. Abdom Imaging 19(3):221–224
Pelage JP, Soyer P, Boudiaf M, et al. (1999) Carcinoid tumors of the abdomen: CT features. Abdom Imaging 24(3):240–245
Hiller N, Berlowitz D, Fisher D, et al. (1998) Primary carcinoid tumor of the pancreas. Abdom Imaging 23(2):188–190
Sahani DV, Kadavigere R, Saokar A, et al. (2005) Cystic pancreatic lesions: a simple imaging-based classification system for guiding management. Radiographics 25(6):1471–1484
Megibow AJ, Lombardo FP, Guarise A, et al. (2001) Cystic pancreatic masses: cross-sectional imaging observations and serial follow-up. Abdom Imaging 26(6):640–647
Morana G, Guarise A (2006) Cystic tumors of the pancreas. Cancer Imaging 6:60–71
Lewin M, Hoeffel C, Azizi L, et al. (2008) Imaging of incidental cystic lesions of the pancreas. J Radiol 89(2):197–207
Sanaka MR, Kowalski TE, Brotz C, et al. (2007) Solid serous adenoma of the pancreas: a rare form of serous cystadenoma. Dig Dis Sci 52(11):3154–3156
Reese SA, Traverso LW, Jacobs TW, et al. (2006) Solid serous adenoma of the pancreas: a rare variant within the family of pancreatic serous cystic neoplasms. Pancreas 33(1):96–99
Yamamoto T, Takahashi N, Yamaguchi T, et al. (2004) A case of solid variant type of pancreatic serous cystadenoma mimicking islet cell tumor. Clin Imaging 28(1):49–51
Gabata T, Terayama N, Yamashiro M, et al. (2005) Solid serous cystadenoma of the pancreas: MR imaging with pathologic correlation. Abdom Imaging 30(5):605–609
Tseng JF, Warshaw AL, Sahani DV, et al. (2005) Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment. Ann Surg 242(3):413–419; discussion 419–421
Zamboni G, Scarpa A, Bogina G, et al. (1999) Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors. Am J Surg Pathol 23(4):410–422
Crippa S, Salvia R, Warshaw AL, et al. (2008) Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Ann Surg 247(4):571–579
Zamboni G, Bonetti F, Scarpa A, et al. (1993) Expression of progesterone receptors in solid-cystic tumour of the pancreas: a clinicopathological and immunohistochemical study of ten cases. Virchows Arch A Pathol Anat Histopathol 423(6):425–431
Suzuki M, Fujita N, Onodera H, et al. (2005) Mucinous cystic neoplasm in a young male patient. J Gastroenterol 40(11):1070–1074
Regi P, Salvia R, Cena C, et al. (2013) Cystic “feminine” pancreatic neoplasms in men. Do any alterations correlate with these uncommon entities? Int J Surg 11:157–160
Wilentz RE, Albores-Saavedra J, Zahurak M, et al. (1999) Pathologic examination accurately predicts prognosis in mucinous cystic neoplasms of the pancreas. Am J Surg Pathol 23:1320–1327
Borgne JL, Calan L, Partensky C (1999) Cystadenomas and cystadenocarcinomas of the pancreas. A multiinstitutional retrospective study of 398 cases. Ann Surg 230:152–161
Fukukura Y, Fujiyoshi F, Sasaki M, et al. (2000) Intraductal papillary mucinous tumors of the pancreas: thin-section helical CT findings. AJR Am J Roentgenol 174(2):441–447
Wang Y, Miller FH, Chen ZE, et al. (2011) Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas. Radiographics 31(3):E47–E64
Tanaka M, Fernandez-del Castillo C, Adsay V, et al. (2012) International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology 12(3):183–197
Yamada Y, Mori H, Hijiya N, et al. (2012) Intraductal papillary mucinous neoplasms of the pancreas complicated with intraductal hemorrhage, perforation, and fistula formation: CT and MR imaging findings with pathologic correlation. Abdom Imaging 37(1):100–109
Martin RC, Klimstra DS, Brennan MF, et al. (2002) Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 9(1):35–40
Buetow PC, Buck JL, Pantongrag-Brown L, et al. (1996) Solid and papillary epithelial neoplasm of the pancreas: imaging-pathologic correlation on 56 cases. Radiology 199(3):707–711
Cantisani V, Mortele KJ, Levy A, et al. (2003) MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR Am J Roentgenol 181(2):395–401
Yao X, Ji Y, Zeng M, et al. (2010) Solid pseudopapillary tumor of the pancreas: cross-sectional imaging and pathologic correlation. Pancreas 39(4):486–491
Baek JH, Lee JM, Kim SH, et al. (2010) Small (< or =3 cm) solid pseudopapillary tumors of the pancreas at multiphasic multidetector CT. Radiology 257(1):97–106
Yu MH, Lee JY, Kim MA, et al. (2010) MR imaging features of small solid pseudopapillary tumors: retrospective differentiation from other small solid pancreatic tumors. AJR Am J Roentgenol 195(6):1324–1332
Choi JY, Kim MJ, Kim JH, et al. (2006) Solid pseudopapillary tumor of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol 187(2):W178–W186
D’Onofrio M, Malagò R, Vecchiato F, et al. (2005) Contrast-enhanced ultrasonography of small solid pseudopapillary tumors of the pancreas: enhancement pattern and pathologic correlation of 2 cases. J Ultrasound Med 24(6):849–854
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
D’Onofrio, M., De Robertis, R., Capelli, P. et al. Uncommon presentations of common pancreatic neoplasms: a pictorial essay. Abdom Imaging 40, 1629–1644 (2015). https://doi.org/10.1007/s00261-015-0388-x
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00261-015-0388-x