Ali COŞKUN, Hakan BULUŞ, Alper YAVUZ, Muzaffer AKKOCA

Kistik pankreas neoplazmlarına yaklaşım

Pankreasın kistik lezyonları klinik olarak sık izlenmesine rağmen tanı ve tedavisinde sıkıntılar yaşanmaktadır. Kistik lezyonların çoğunu psödokist ve retansiyon kistleri oluşturmaktadır. Neoplazmlar ise kistik lezyonların % 10- 15’lik kısmını oluşturur. Temel olarak pankreasın kistik neoplazmları; seröz kistik neoplazm (SCN), müsinöz kistik neoplazm (MCN), intraduktal papiller müsinöz neoplazmdır (IPMN) ve Solid psödopapiller neoplaziler ( SPN) olmak üzere dört gruba ayrılabilir. Pankreasın kistik neoplazmlarında klinik bulgular ve belirtiler özgün olmadığı için tanısı zordur. Tedavi tiplere hatta subtiplere göre belirgin ölçüde değişiklik gösterir. Bu yüzden pankreas kistlerinin ayırıcı tanısının yapılması önem arz etmektedir.

Approach to pancreatıc cystıc neoplazms

Although pancreatic cystic lesions are monitored frequently, there still occure diffuculties in the diagnosis and treatment. Most of the cystic lesions are pseudocysts and retention cysts. Basically, pancreatic cystic neoplasms can be divided into four groups: serous cystic neoplasm (SCN), mucinous cystic neoplasms (MCN), intaduktal papillary mucinous neoplasm (IPMN) and solid pseudopapiller neoplasy (SPN). The diagnosis of the pancreatic cystic neoplasms is challenging, since clinical signs and symptoms are not typical. The treatment varies across types or even sub-type of the disease. For this reason, making the differential diagnosis of pancreatic cysts is crucially important.

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1.Cullen PK Jr, Remine WH, Dahlin DC. A Clinicopathological study of cystadenocarsinoma of the pancreas. Surg Gynecol Obstet. 1963; 117: 189–195.
2.Campagno J, Oertel JE. Mucinous cystic neoplasms of the pancreas with overt and latent malignancy (cystadenocarcinoma and cystadenoma). A clinicopathologic study of 41 cases. Am J Clin Pathol.1978; 69: 573–580.
3.Warshaw AL, Compton CC, Lewandrowski K, Cardenosa G, Mueller PR. Cystic tumors of the pancreas: New clinical, radiologic and pathologic observations in 67 patients. Ann Surg. 1990; 212:432-445.
4.Klöppel G, Solcia E, Longnecker DS, Capella C, So-bin LH (1996) Histological typing of tumours of the exocrine pancreas. Second edition. WHO interna-tional histological classification of tumours. Springer, Berlin Heidelberg New York Tokio
5.Longnecker DS, Adler G. Intraductal papillary-mucinous neoplasms. In: Hamilton SR, Aaltonen LA, eds. Pathology and Genetics of Tumours of the Digestive System. Lyon, France: IARC Press; 2000:237-240.
6.Goldsmith JD. Cystic Neoplasms of the Pancreas. Am J Clin Pathol 2003;119: 3-16.
7.Cunningham SC, Hruban RH, Schulick RD. Differentiating intraductal papillary mucinous neoplasms from other pancreatic cystic lesions. World Journal of Gastrointestinal Surgery. 2010;2(10):331–336
8.Sarr MG, Kendrick ML, Nagorney DM, Thompson GB, Farley DR, Farnell MB. Cystic neoplasms of the pancreas. Benign to malignant epithelial neoplasms, Surg Clin North Am . 2001; 81: 497-509.
9.Le Borgne J. Cystic tumors of the pancreas. Br J Surg. 1998; 85: 577- 579.
10.Solcia E, Capella C, Kloppel G. Tumors of the Pancreas. Washington, DC: Armed Forces Institute of Pathology; 1997.Atlas of Tumor Pathology; Third Series, Fascicle 20.
11.Adsay NV, Klimstra DS, Compton CC. Cystic lesions of the pancreas: introduction. Semin Diagn Pathol. 2000;17:1-6.
12.Sarr MG, Carpenter HA, Prabhakar LP, Orchard TF, Hughes S, van Heerden JA, DiMagno EP. Clinical and pathologic correlation of 84 mucinous cystic neoplasms of the pancreas – can one reliably differentiate benign from malignant (or premalignant) neoplasms?. Ann Surg. 2000; 231: 205–212.
13.Wilentz RE, Albores-Saavedra J, Hruban RH. Mucinous cystic neoplasms of the pancreas. Semin Diagn Pathol..2000;17:31-42
14.Sarr MG, Murr M, Smyrk TC, Yeo CJ, Fernandez-del-Castillo C, Hawes RH, Freeny PC: Primary cystic neoplasms of the pancreas. Neoplastic disorders of emerging importance-current state of the art and unanswered questions. J Gastrointest Surg. 2003; 7:417-428.
15.Klöppel G, Kosmahl M. Cystic lesions and neoplasms of the pancreas. The features are becoming clearer. Pancreatology. 2001; 1:648-655.
16.Paal E, Thompson LD, Przygodzki RM, et al. A clinicopathologic and immunohistochemical study of 22 intraductal papillary mucinous neoplasms of the pancreas, with a review of the literature. Mod Pathol. 1999;12:518-528.
17.Sohn TA, Yeo CJ, Cameron JL, et al. Intraductal papillary mucinous neoplasms of the pancreas: an increasingly recognized clinicopathologic entity. Ann Surg. 2001;234:313-322.
18.Tibayan F, Vierra M, Mindelzun B, et al. Clinical presentation of mucin-secreting tumors of the pancreas. Am J Surg..2000;179:349-351.
19.Guo N, Zhou QB, Chen RF, Zou SQ, Li ZH, Lin Q, Wang J, Chen JS.Diagnosis and surgical treatment of solid pseudopapillary neoplasm of the pancreas: analysis of 24 cases. Can J Surg. 2011; 54: 011810- 11810.,
20.Lieber MR, Lack EE, Roberts JR Jr, et al. Solid and papillary epithelial neoplasm of the pancreas: an ultrastructural and immunocytochemical study of six cases. Am J Surg Pathol. 1987;11:85-93.
21.Kloppel G. Pancreatic non-endocrine tumors. In: Kloppel G, Heitz PU. Pancreatic Pathology. Edinburgh, Scotland: Churchill Livingstone; 1984.
22.Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol. 2000;17:66-80.
23.Hutchins GF, Draganov PV. Cystic neoplasms of the pancreas: a diagnostic challenge. World J Gastroenterol. 2009; 15: 48-54
24.Bassi C, Salvia R, Molinari E, Biasutti C, Falconi M, Pederzoli P. “Management of 100 consecutive cases of pancreatic serous cystadenoma: wait for symptoms and see at imaging or vice versa?” World Journal of Surgery,.2003; 27: 319-323,
25.Lim SJ, Alasadi R, Wayne JD, Rao S, Rademaker A, Bell R, Talamonti MS. Preoperative evaluation of pancreatic cystic lesions:Cost-benefit analysis and proposed management algorithm. J. surg. 2005.07.033.
26.Kerlin DL, Frey CF, Bodai BI, Twomey PL, Ruebner B. “Cystic neoplasms of the pancreas,” Surgery Gynecology and Obstetrics. 1987; 165: 475–478
27.Scheiman JM. Management of cystic lesions of the pancreas. J Gastrointest Surg. 2008;12:405-7.
28.Johnson CD, Stephens DH, Charboneau JW, et al. Cystic pancreatic tumors: CT and sonographic assessment, Am J Radiol .1988; 151: 1133–1138.
29.Khalid A, William B. ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts. Am J Gastroenterol 2007;102:2339-49.
30.Gress F, Gottlieb K, Cummings O, et al. Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas. Am J Gastroenterol 2000; 95:961–5.
31.Zamboni G, Scarpa A, Bogina G, et al. Mucinous cystic tumors of the pancreas: Clinicopathological features, prognosis, and relationship to other mucinous cystic tumors. Am J Surg Pathol 1999;23:410–22.
32.Obara T, Maguchi H, Saitoh Y, et all.Mucin-producing tumor of the pancreas. Am J Gastroenterol. 1993 ;88:564-9.
33.Cantisani V, Mortele KJ, Levy A, Glickman JN, Ricci P, Passariello R, et al. MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR Am J Roentgenol. 2003; 181: 395-401.
34. Brugge WR, Lewandrowski K, Lee-Lewandrowski E, Centeno BA, Szydlo T, Regan S, et al. Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study. Gastroenterology 2004;126:1330–6.
35.Feranandez-del Castillo C, Targarona J, Thayer SP, RattnerDW, Brugge WR,Warshaw Al. Incidental pancreatic cysts: clinicopathologic characteristics and comparison with symptomatic patients. Arch Surg2003;138:427–33.
36. Petrone MC , Arcidiacono PG. Role of endosocopic ultrasound in the diagnosis of cystic tumours of the pancreas Digestive and Liver Disease 40 (2008) 847–853
37.Allen, P. J. et al. Pancreatic cyst fluid protein expression profiling for discriminating between serous cystadenoma and intraductal papillary mucinous neoplasm. Ann. Surg. 2009; 250: 754–760
38.Pelaez-Luna M, Chari ST. Cyst fluid analysis to diagnose pancreatic cystic lesions: an as yet unfulfilled promise. Gastroenterology. 2006; 130: 1007–1009 .
39.Yong WH, Southern JF, Pins MR, Warshaw AL, Compton CC, Lewandrowski KB. Cyst fluid NB/70K concentration and leukocyte esterase: two new markers for differentiating pancreatic serous tumors from pseudocysts. Pancreas. 1995; 10:342-6.
40.Sakorafas GH, Sarr MG: Cystic pancreas neoplasm of the pancreas. In Bland KI, Sarr MG (eds): The Practice of General Surgery. Philedelphia, WB Saunders, 2002, pp 771-776.
41.Grogan JR, Saeian K, Taylor AJ, Quiroz F, Demeure MJ, Komorowski RA. Making sense of mucin-producing pancreatic tumours. AJR An J Roentgenol 2001;176:921–9.
42.Lima JE, Javitt MC, Mathur SC. Mucinous cystic neoplasm of the pancreas. Radiographics 1999;19:807–11.
43.Tanaka M, Chari S, Adsay V, Fernandez-del Castillo C, Falconi M, Shimizu M, et al. International Consensus Guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology 2006;6:17–32.
44.Kubo H, Chijiiwa Y, Akahoshi K, Hamada S, Harada N, Sumii T, et al. Intraductal papillary-mucinous tumours of the pancreas: differential diagnosis between benign and malignant tumours by endoscopic ultrasonography. Am J Gastroenterol 2001;96:1429–34.
45.Federle MP, McGrath KM. Cystic neoplasms of the pancreas. Gastroenterol Clin N Am 2007;36:365–76
46.Kurana B, Mortele KJ, Glickmam J, Silverman SG, Ros PR. Macrocystic serous adenoma of the pancreas: radiologyc-pathologic correlation. AJR Am J Roentgenol 2003;118:119–23.
47.Cohen-Scali F, Vilgrain V, Brancatelli G, Hammel P, Vullierme MP, Sauvanet A, et al. Discrimination of unilocular macrocystic serous cystadenoma from pancreatic pseudocyst and mucinous cystoadenoma with CT. Radiology 2003;228:727–33.
48.Dietrich CF, Jenssen C. Benign and Malignant (cystic) tumours of the pancreas. In: Dietrich CF, edt. Endoscopic ultrasound. An introductory manual and atlas. Thieme; 2006. p. 208–29.
49.Arakawa A, Yamashita Y, Namimoto T, et al. Intraductal papillary tumors of the pancreas. Histopathologic correlation of MR cholangiopancreatography findings. Acta Radiol. 2000 Jul;41(4):343-7.
50.Hara T, Yamaguchi T, Ishihara T, Tsuyuguchi T, Kondo F, Kato K, et al. Diagnosis and patient management of intraductal papillarymucinous tumours of the pancreas by using peroral pancreatoscopy and intraductal ultrasonography. Gastroenterology 2002;122:34–43.
51.Furukawa T, Tsukamoto Y, Naitoh Y, Hirooka Y, Hayakawa T. Differential diagnosis between benign and malignant localized stenosis of the main pancreatic duct by intraductal ultrasound of the pancreas. Am JGastroenterol 1994;89:2038–41.
52.Rogart JN, Loren DE, Singu BS, Kowalski TE. “Cyst wall puncture and aspiration during EUS-guided fine needle aspiration may increase the diagnostic yield of mucinous cysts of the pancreas,” Journal of Clinical Gastroenterology, 2011; 45: 164–169.
53.Zamboni G, Hruban RH, Longnecker DS, et al. Mucinous cystic neoplasms of the pancreas. In: Hamilton SR, Aaltonen LA, eds. Pathology and Genetics of Tumours of the Digestive System. Lyon, France: IARC Press; 2000:234-236.
54.Albores-Saavedra J, Angeles-Angeles A, Nadji M, et al. Mucinous cystadenocarcinoma of the pancreas: morphologic and immunocytochemical observations. Am J Surg Pathol. 1987;11:11-20.
55.Adsay NV, Longnecker DS, Klimstra DS. Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms. Semin Diagn Pathol. 2000;17:16-30..
56.Luttges J, Zamboni G, Longnecker D, et al. The immunohistochemical mucin expression pattern distinguishes different types of intraductal papillary mucinous neoplasms of the pancreas and determines their relationship to mucinous noncystic carcinoma and ductal adenocarcinoma. Am J Surg Pathol. 2001;25:942-948.
57.Yonezawa S, Sueyoshi K, Nomoto M, et al. MUC2 gene expression is found in noninvasive tumors but not in invasive tumors of the pancreas and liver: its close relationship with prognosis of the patients. Hum Pathol. 1997;28:344-352..
58.Nakamura A, Horinouchi M, Goto M, et al. New classification of pancreatic intraductal papillary-mucinous tumour by mucin expression: its relationship with potential for malignancy. J Pathol. 2002;197:201- 210
59.Iacobuzio-Donahue CA, Klimstra DS, Adsay NV, et al. Dpc-4 protein is expressed in virtually all human intraductal papillary mucinous neoplasms of the pancreas: comparison with conventional ductal adenocarcinomas. Am J Pathol. 2000;157:755-761.
60.Pyke CM, van Heerden JA, Colby TV, Sarr MG, Weaver AL. The spectrum of serous cystadenoma of the pancreas. Clinical, pathologic, and surgical aspe Ann Surg. 1992 Feb;215(2):132-9.
61.Chott A, Kloppel G, Buxbaum P, et al. Neuron specific enolase demonstration in the diagnosis of a solid-cystic (papillary cystic) tumour of the pancreas. Virchows Arch A Pathol Anat Histopathol. 1987;410:397-402.
62.Miettinen M, Partanen S, Fraki O, et al. Papillary cystic tumor of the pancreas: an analysis of cellular differentiation by electron microscopy and immunohistochemistry. Am J Surg Pathol. 1987;11:855-865
63.Zamboni G, Bonetti F, Scarpa A, et al. 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. 1993;423:425- 431.
64.Notohara K, Hamazaki S, Tsukayama C, et al. Solidpseudopapillary tumor of the pancreas: immunohistochemical localization of neuroendocrine markers and CD10. Am J Surg Pathol. 2000;24:1361-1371.
65.Abraham SC, Klimstra DS, Wilentz RE, et al. Solidpseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations. Am J Pathol. 2002;160:1361-1369.
66.Tanaka Y, Kato K, Notohara K, et al. Frequent beta-catenin mutation and cytoplasmic/nuclear accumulation in pancreatic solidpseudopapillary neoplasm. Cancer Res. 2001;61:8401-4
67.Jong K, Fockens P. Epidemiology, Diagnosis, andManagement of Cystic Lesions of the Pancreas. Gastroenterology Research and Practice. 2012, Article ID 147465, 8 pages doi:10.1155/2012/147465.
68.Talamini MA, Pitt HA, Hruban RH, et al., Spectrum of cystic tumors of the pancreas, Am J Surg. 1992; 163: 117–124.
69.Borgne JL, Calan L, Partensky C, et al.. Cystadenomas and cystadenocarcinomas of the pancreas. A multi-institutional retrospective study of 398 cases. Ann Surg. 1999; 230: 152–161.
70.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;247:571-9.
71.Sakorafas GH, Smyrniotis V, Reid-Lombardo KM, Sarr MG: Primary pancreatic cystic neoplasms revisited. Part I. Serous cystic neoplasms. Surg Oncol Surg Oncol 2011;20:e84–92.
72.Sakorafas GH, Smyrniotis V, Reid-Lombardo KM, Sarr MG: Primary pancreatic cystic neoplasms revisited. Part II. Mucinous cystic neoplasms. Surg Oncol Surg Oncol 2011;20:93–101.
73.Carbognin G. Lombardo F, Scholes JV, et al. Intraductal papillarymucinous tumors of the pancreas:Correlation of patholgy,imaging and clinical behevior.Radiology.1998; 209:332.
74.Sakorafas GH, Sarr MG, van de Velde CJ, Peros G: Intraductal papillary mucinous neoplasms of the pancreas: a surgical perspective. Surg Oncol 2005;14:155–178.
75.Sheehan MK, Beck K, Pickleman J, Aranha GV. Spectrum of cystic neoplasms of the pancreas and their surgical management. Arch Surg. 2003; 38 :657-60.
76.Salvia R, Bassi C, Festa L, et al. Clinical and biological behavior of pancreatic solid pseudopapillary tumors: report on 31 consecutive patients. J Surg Oncol 2007;95:304-10.
77.Alexandrescu DT, O’Boyle K, Feliz A, et al. Metastatic solidpseudopapillary tumour of the pancreas: clinico-biological correlates and management. Clin Oncol (R Coll Radiol) 2005;17:358-63.
78.Gedaly R, Toledano A, Millan G, et al. Treatment of liver metastases from a solid pseudopapillary tumor of the pancreas. J Hepatobiliary Pancreat Surg 2006;13:587-90.
79.Vollmer CM, Dixon E, Grant D. Management of a solid pseudopapillary tumor of the pancreas with liver metastases. HPB (Oxford) 2003;5:264-7.
80.Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 2005;200:965-72
81.Sumida W, Kaneko K, Tainaka T, et al. Liver transplantation for multiple liver metastases from solid pseudopapillary tumor of the pancreas. J Pediatr Surg 2007;42:e27-31.
82.Terris B, Ponsot P , Paye F, et al.. Intraductal papillary mucinous tumors of the pancreas confined to secondary ducts show less aggressive pathologic features as compared with those involving the main pancreatic duct, Am J Surg Pathol. 2000; 24: 1372–1377.
83.Mao C, Guvendi M, Domenico DR, et al. Papillary cystic and solid tumors of the pancreas: A pancreatic embryonic tumor? Studies of three cases and cumulative review of the world’s literature. Surgery 1995;118:82.