Intrapancreatic accessory spleen.

Rare Solid Tumors of the Pancreas as Differential Diagnosis of Pancreatic Adenocarcinoma

Sabine Kersting, Monika S Janot, Johanna Munding, Dominique Suelberg, Andrea Tannapfel, Ansgar M Chromik, Waldemar Uhl, Uwe Bergmann


Context Rare solid tumors of the pancreas can be misinterpreted as primary pancreatic cancer. Objective The aim of this study was to report our experience in the treatment of patients with rare tumor lesions of the pancreas and to discuss clinical and pathological characteristics in the context of the role of surgery. Design Data from patients of our prospective data-base with rare benign and malignant tumors of the pancreas, treated in our division from January 2004 to August 2010, were analyzed retrospectively. Results One-thousand and ninety-eight patients with solid tumors of the pancreas underwent pancreatic surgery. In 19 patients (10 women, 9 men) with a mean age of 57 years (range: 20-74 years) rare pancreatic tumors (metastasis, solid pseudopapillary tumor, teratoma, hemangioma, accessory spleen, lymphoepithelial cyst, hamartoma, sarcoidosis, yolk sac tumor) were the reason for surgical intervention. Conclusion If rare benign and malignant pancreatic tumors, intrapancreatic metastasis, as well as pancreatic malformations or other abnormalities, present themselves as solid masses of the pancreas, they constitute an important differential diagnosis to primary pancreatic neoplasia, e.g. pancreatic ductal adenocarcinoma. Clinical imaging techniques cannot always rule out malignancy, thus operative exploration often remains the treatment of choice to provide the correct diagnosis and initiate adequate surgical therapy.

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Peters K, Kloppel G. [Undifferentiated pancreatic carcinomas. Leap into chaos]. Pathologe 2005;26:18-21. [PMID: 15592673].

Carpelan-Holmstrom M, Nordling S, Pukkala E, Sankila R, Luttges J, Kloppel G et al. Does anyone survive pancreatic ductal adenocarcinoma? A nationwide study re-evaluating the data of the Finnish Cancer Registry. Gut 2005;54:385-387. [PMID: 15710987].

Adsay NV, Basturk O, Klimstra DS, Kloppel G. Pancreatic pseudotumors: non-neoplastic solid lesions of the pancreas that clinically mimic pancreas cancer. Semin Diagn Pathol 2004;21:260-267. [PMID: 16273945].

Mortenson MM, Katz MH, Tamm EP. Current diagnosis and management of unusual pancreatic tumors. Am J Surg 2008;196:100-113. [PMID: 18466869].

Roland CF, van Heerden JA. Nonpancreatic primary tumors with metastasis to the pancreas. Surg Gynecol Obstet 1989;168:345-347. [PMID: 2928909].

Z'Graggen K, Fernandez-del Castillo C, Rattner DW, Sigala H, Warshaw AL. Metastases to the pancreas and their surgical extirpation. Arch Surg 1998;133:413-417; discussion 418-419. [PMID: 9565122].

Pratt WB, Maithel SK, Vanounou T, Huang ZS, Callery MP, Vollmer CM. Clinical and economic validation of the International Study Group of Pancreatic Fistula (ISGPF) classification scheme. Ann Surg 2007;245:443-451. [PMID: 17435552].

Baroni RH, Pedrosa I, Tavernaraki E, Goldsmith J, Rofsky NM. Pancreatic sarcoidosis: MRI features. J Magn Reson Imaging 2004;20:889-893. [PMID: 15503350].

Fong ZV, Wong J, Maley WR, Sandorfi N, Winter J M, Koniaris LG et al. Sarcoid-Reaction Mimicking Metastatic Malignant Hepatopancreatobiliary Tumors: Report of Two Cases and Review of the Literature. J Gastrointest Surg 2012. [PMID: 22258875].

Wijkstrom M, Bechara RI, Sarmiento JM. A rare nonmalignant mass of the pancreas: case report and review of pancreatic sarcoidosis. Am Surg 2010;76:79-84. [PMID: 20135945].

Caceres M, Sabbaghian MS, Braud R, Wilks S, Boyle M. Pancreatic sarcoidosis: unusual presentation resembling a periampullary malignancy. Curr Surg 2006;63:179-185. [PMID: 16757369].

Kerr AA. Cysts and pseudocysts of the pancreas. Surg Gynecol Obstet 1918;27:40.

Koomalsingh KJ, Fazylov R, Chorost MI, Horovitz J. Cystic teratoma of the pancreas: presentation, evaluation and management. Jop 2006;7:643-646. [PMID: 17095845].

Degrate L, Misani M, Mauri G, Garancini M, Maternini M, Moltrasio F et al. Mature cystic teratoma of the pancreas. Case report and review of the literature of a rare pancreatic cystic lesion. Jop 2012;13:66-72. [PMID: 22233950].

Iacono C, Zamboni G, Di Marcello R, Zicari M, Maran M, Montresor E et al. Dermoid cyst of the head of the pancreas area. Int J Pancreatol 1993;14:269-273. [PMID: 7906703].

Scheele J, Barth TF, Wittau M, Juchems M, Henne-Bruns D, Kornmann M. Cystic teratoma of the pancreas. Anticancer research 2012;32:1075-1080. [PMID: 2399635].

Adsay NV, Hasteh F, Cheng JD, Klimstra DS. Squamous-lined cysts of the pancreas: lymphoepithelial cysts, dermoid cysts (teratomas), and accessory-splenic epidermoid cysts. Semin Diagn Pathol 2000;17:56-65. [PMID: 10721807].

Truong LD, Rangdaeng S, Jordan PH, Jr. Lymphoepithelial cyst of the pancreas. Am J Surg Pathol 1987;11:899-903. [PMID: 3674287].

Matrone A, Russo M, Mollica C, Lombardi D, Lombardi G, Maurea S et al. Lymphoepithelial pancreatic cyst: an atypical benign pancreatic mass presenting with a "cheerios-like" appearance. Jop 2010;11:170-172. [PMID: 20208329].

Jian B, Kimbrell HZ, Sepulveda A, Yu G. Lymphoepithelial cysts of the pancreas: endosonography-guided fine needle aspiration. Diagn Cytopathol 2008;36:662-665. [PMID: 18677749].

Tucci G, Muzi MG, Nigro C, Cadeddu F, Amabile D, Servadei Fet al. Dermoid cyst of the pancreas: presentation and management. World J Surg Oncol 2007;5:85. [PMID: 17683548].

England RJ, Woodley H, Cullinane C, McClean P, Walker J, Stringer M D. Pediatric pancreatic hemangioma: a case report and literature review. Jop 2006;7:496-501. [PMID: 16998249].

Tebboune N, Lazure T, Fabre M, Pariente D. Pancreatic haemangioma in infancy: the place of radiology. Pediatr Radiol 2003;33:621-623. [PMID: 12879316].

Mundinger GS, Gust S, Micchelli ST, Fishman EK, Hruban RH, Wolfgang CL. Adult pancreatic hemangioma: case report and literature review. Gastroenterol Res Pract 2009;2009:839730. [PMID: 19421421].

Halpert B, Gyorkey F. Lesions observed in accessory spleens of 311 patients. Am J Clin Pathol 1959;32:165-168. [PMID: 13670140].

Rodriguez E, Netto G, Li QK. Intrapancreatic accessory spleen: A case report and review of literature. Diagnostic cytopathology 2012. [PMID: 22241738].

Guo W, Han W, Liu J, Jin L, Li JS, Zhang ZT et al. Intrapancreatic accessory spleen: a case report and review of the literature. World J Gastroenterol 2009;15:1141-1143. [PMID: 19266611].

Spencer LA, Spizarny DL, Williams TR. Imaging features of intrapancreatic accessory spleen. Br J Radiol 2010;83:668-673. [PMID: 19690077].

Meiler R, Dietl KH, Novak K, Patzel C. Intrapancreatic accessory spleen. Int Surg 2010;95:183-187. [PMID: 20718328].

Longnecker DS. Endocrine Tumors of the Pancreas in Transgenic Mice. Comp Med 2004;54:21-22. [PMID: 15382338].

McFaul CD, Vitone LJ, Campbell F, Azadeh B, Hughes ML, Garvey CJ et al. Pancreatic hamartoma. Pancreatology 2004;4:533-537; discussion 537-538. [PMID: 15340246].

Matos JM, Grutzmann R, Agaram NP, Saeger HD, Kumar HR, Lillemoe KD et al. Solid pseudopapillary neoplasms of the pancreas: a multi-institutional study of 21 patients. J Surg Res 2009;157:e137-142. [PMID: 19818965].

Campanile M, Nicolas A, LeBel S, Delarue A, Guys JM, de Lagausie P. Frantz's tumor: is mutilating surgery always justified in young patients? Surg Oncol 2011;20:121-125. [PMID: 20106656].

Chakhachiro ZI, Zaatari G. Solid-pseudopapillary neoplasm: a pancreatic enigma. Arch Pathol Lab Med 2009;133:1989-1993. [PMID: 19961258].

Zerbi A, Ortolano E, Balzano G, Borri A, Beneduce AA, Di Carlo V. Pancreatic metastasis from renal cell carcinoma: which patients benefit from surgical resection? Ann Surg Oncol 2008;15:1161-1168. [PMID: 18196343].

Butte JM, Brennan MF, Gonen M, Tang LH, D'Angelica MI, Fong Y et al. Solid pseudopapillary tumors of the pancreas. Clinical features, surgical outcomes, and long-term survival in 45 consecutive patients from a single center. J Gastrointest Surg 2011;15:350-357. [PMID: 20824369].

Martin RC, Klimstra DS, Brennan MF, Conlon KC. Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 2002;9:35-40. [PMID: 11833495].

Tien YW, Ser KH, Hu RH, LeeCY, Jeng YM, Lee PH et al. Solid pseudopapillary neoplasms of the pancreas: is there a pathologic basis for the observed gender differences in incidence? Surgery 2005;137:591-596. [PMID: 15933625].

Tang LH, Aydin H, Brennan MF, Klimstra DS. Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases with components of undifferentiated carcinoma and a comparative clinicopathologic analysis of 34 conventional cases. Am J Surg Pathol 2005;29:512-519. [PMID: 15767807].

Lee JS, Han HJ, Choi SB, Jung CW, Song TJ, Choi SY. Surgical outcomes of solid pseudopapillary neoplasm of the pancreas: a single institution's experience for the last ten years. The American surgeon 2012;78:216-219. [PMID: 22369832].

Nakagohri T, Kinoshita T, Konishi M, Takahashi S, Gotohda N. Surgical outcome of solid pseudopapillary tumor of the pancreas. J Hepatobiliary Pancreat Surg 2008;15:318-321. [PMID: 18535771].

Reddy S, Cameron JL, Scudiere J, Hruban RH, Fishman EK, Ahuja N et al. Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series. J Am Coll Surg 2009;208:950-957; discussion 957-959. [PMID: 19476869].

Reddy S, Wolfgang CL. Solid pseudopapillary neoplasms of the pancreas. Adv Surg 2009;43:269-282. [PMID: 19476869].

Razzi S, Luisi S, Gabbanini M, Lazzeri L, Mazzini M, Petraglia F. Yolk sac tumor in a young girl: a case report. Gynecol Endocrinol 2005;20:334-335. [PMID: 16019383].

Zhang B, Gao S, Chen Y, Wu Y. Primary yolk sac tumor arising in the pancreas with hepatic metastasis: a case report. Korean J Radiol 2010;11:472-475. [PMID: 20592932].

Guzel A, Tatli M, Belen D, Seckin H. Spinal cord compression of primary extragonadal giant yolk sac tumor. Spinal Cord 2007;45:254-257. [PMID: 16733519].

Kumar Y, Bhatia A, Kumar V, Vaiphei K. Intrarenal pure yolk sac tumor: an extremely rare entity. Int J Surg Pathol 2007;15:204-206. [PMID: 17478784].

Park NH, Ryu SY, Park IA, Kang SB, Lee HP. Primary endodermal sinus tumor of the omentum. Gynecol Oncol 1999;72:427-430. [PMID: 10053119].

Mourra N, Arrive L, Balladur P, Flejou JF, Tiret E, Paye F. . Isolated metastatic tumors to the pancreas: Hopital St-Antoine experience. Pancreas 2010;39:5 77-580. [PMID: 20173671].

Thompson LD, Heffess CS. Renal cell carcinoma to the pancreas in surgical pathology material. Cancer 2000;89:1076-1088. [PMID: 10964338].

Reddy S, Wolfgang CL. The role of surgery in the management of isolated metastases to the pancreas. Lancet Oncol 2009;10:287-293. [PMID: 19845185].

Sperti C, Pasquali C, Liessi G, Pinciroli L, Decet G, Pedrazzoli S. Pancreatic resection for metastatic tumors to the pancreas. J Surg Oncol 2003;83:161-166; discussion 166. [PMID: 12827684].

Hiotis SP, Klimstra DS, Conlon KC, Brennan MF. Results after pancreatic resection for metastatic lesions. Ann Surg Oncol 2002;9:675-679. [PMID: 12167582].

Law CH, Wei AC, Hanna SS, Al-Zahrani M, Taylor BR, Greig PD. Pancreatic resection for metastatic renal cell carcinoma: presentation, treatment, and outcome. Ann Surg Oncol 2003;10:922-926. [PMID: 14527912].

Ballarin R, Spaggiari M, Cautero N, De Ruvo N, Montalti R, Longo C et al. Pancreatic metastases from renal cell carcinoma: the state of the art. World J Gastroenterol 2011;17:4747-4756. [PMID: 22147975].

Nikfarjam M, Evans P, Christophi C. Pancreatic resection for metastatic melanoma. HPB (Oxford) 2003;5:174-179. [PMID: 18332980].

Reddy S, Edil BH, Cameron JL, Pawlik TM, Herman J M, Gilson MM et al. Pancreatic resection of isolated metastases from nonpancreatic primary cancers. Ann Surg Oncol 2008;15:3199-3206. [PMID: 18784960].

Sweeney AD, Wu MF, Hilsenbeck SG, Brunicardi FC, Fisher WE. Value of pancreatic resection for cancer metastatic to the pancreas. J Surg Res 2009;156:189-198. [PMID: 19375718].

Layfield LJ, Hirschowitz SL, Adler DG. Metastatic disease to the pancreas documented by endoscopic ultrasound guided fine-needle aspiration: a seven-year experience. Diagnostic cytopathology 2012;40:228-233. [PMID: 22334524].

Pettinato G, Di Vizio D, Manivel JC, Pambuccian SE, Somma P, Insabato L et al. Solid-pseudopapillary tumor of the pancreas: a neoplasm with distinct and highly characteristic cytological features. Diagn Cytopathol 2002;27:325-334. [PMID: 12451561].

Bassi C, Dervenis C, Butturini G, Fingerhut A, Yeo C, Izbicki J et al. Postoperative pancreatic fistula: an international study group (ISGPF) definition. Surgery 2005;138:8-13. [PMID: 16003309].



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