Isolated Desmoid Tumor of Pancreatic Tail with Cyst Formation Diagnosed by Beta-Catenin Immunostaining : A Rare Case Report with Review of Literature
Context Isolated pancreatic desmoid tumors with cyst formation are uncommon benign mesenchymal soft tissue tumors, characterized by the dense fibroblastic proliferations with abundant extra-cellular collagen matrix. Intraabdominal desmoid tumor usually involve the mesentery and retroperitoneum and mostly occur in association of familial adenomatous polyposis or Gardner’s syndrome.While desmoid tumors do not metastasize, their advancement can be life threatening due to aggressive local invasion, such as mesentery involvement. Isolated, sporadic pancreatic desmoid tumors have been considered anecdotal, with only 10 cases (cystic area in three cases) described in the literature. To our best of knowledge, this patient is fourth case report displaying cyst formatin in desmoid tumor of pancreatic tail. Case report We herein report a very unusual location of sporadic desmoid tumor involving the pancreatic tail with cystic area diagnosed by Beta-Catenin immunostaining. A 11year-old male presented with painless lump in left hypochondrium of abdomen.The diagnosis of pancreatic adenocarcinoma was suspected preoperatively and the patient underwent a splenopancreatectomy. Histopathologic examination revealed dense fibroblastic proliferation with occasional mitosis suggestive of mesenchymal tumor. The diagnosis of Desmoid tumor was confirmed by positivity of Beta-Catenin immunohistochemical analysis. Conservative treatment was given postoperatively. After ten month follow-up, no recurrence was observed. Conclusion Desmoid tumors are very rare in the tail of pancreas with cystic area and their diagnosis can be difficult, such as in our case where it presented as a solid-cystic lesion.
Image: Non-encapsulated tumor with cyst formation.
Micke O, Seegenschmiedt MH;German Cooperative Group on Radiotherapy for Benign Diseases.Radiation therapy for aggressive fibromatosis (desmoid tumors): Results of a national Patterns of Care Study. Int J Radiat Oncol Biol Phys 2005;61:882– 891.
Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg 1996; 83:1494-1504.[PMID: 9014661]
Roggli VL, Kim HS, Hawkins E. Congenital generalized fibromatosis with visceral involvement. A case report. Cancer 1980; 45:954-60. [PMID: 7260846]
Ure BM, Holschneider AM, Gharib M, Halsband H, Hinselmann D. Clinical aspects, classification and prognosis of 7 cases of pediatric fibromatosis. Z Kinderchir 1988; 43:27-30. [PMID: 3376585]
Bruce JM, Bradley EL 3rd, Satchidanand SK. Adesmoid tumor of the pancreas. Sporadic intraabdominal desmoids revisited. Int J Pancreatol 1996; 19:197-203. [PMID: 8807365]
Sedivy R, Ba-Ssalamah A, Gnant M, Hammer J,Klöppel G. Intraductal papillary-mucinous adenoma associated with unusual focal fibromatosis: a"postoperative" stromal nodule. Virchows Arch 2002; 441:308-11. [PMID: 12242530]
Nursal TZ, Abbasoglu O. Sporadic hereditary pancreatic desmoid tumor: a new entity? J Clin Gastroenterol 2003; 37:186-8. [PMID: 12869894]
Pho LN, Coffin CM, Burt RW. Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion. Fam Cancer 2005; 4:135-8.[PMID:15951964]
Weiss ES, Burkart AL, Yeo CJ. Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy. J Gastrointest Surg 2006; 10:679-88. [PMID: 16773761]
Aurélien Amiot, Safi Dokmak and Alain Sauvanet et al . Sporadic Desmoid Tumor.An Exceptional Cause of Cystic Pancreatic Lesion : a Case Report. JOP. J Pancreas (Online)2008; 9(3):339-345. [ISSN 1590-8577]
F. Polistina, G. Costantin, E. D’Amore, and G. Ambrosino. Sporadic, Nontrauma-Related, Desmoid Tumor of the Pancreas:A Rare Disease—Case Report and Literature Review. Case Reports in Medicine. 2010, 1155: 2-4. Article [PMID 272760]
Clark SK, Neale KF, Landgrebe JC, Phillips RK.Desmoid tumours complicating familial adenomatous polyposis. Br J Surg 1999; 86:1185-9. [PMID 10504375]
Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, et al. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol 2008; 6:215-9. [PMID: 18237870]
Hartley JE, Church JM, Gupta S, McGannon E, Fazio VW. Significance of incidental desmoids identified during surgery for familial adenomatous polyposis. Dis Colon Rectum 2004; 47:334-8. [PMID: 14991495]
Goellner JR, Soule EH. Desmoid tumors, an ultrastructural study of eight cases. Hum Pathol 1980;11:43-50. [PMID: 7364438]
16.Rampone B, Pedrazzani C, Marrelli D, Pinto E, Roviello F. Updates on abdominal desmoid tumors. World J Gastroenterol 2007;13:5985-8. [PMID:18023087]
Cohen S, Ad-El D, Benjaminov O, Gutman H.Post-traumatic soft tissue tumors: case report and review of the literature a propos a post-traumatic paraspinal desmoid tumor. World J Surg Oncol 2008; 6:28. [PMID: 18312655]
Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999; 17:158-67. [PMID: 10458229]
Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus Sr JL. Desmoid tumour: a pleomorphic lesion. Eur J Surg Oncol 1999; 25:487- 97. [PMID: 10527597]
Weiss S, Goldblum JR. Liposarcoma. In: Enzinger and Weiss’s Soft Tissue Tumors, 4th ed. St. Louis:Mosby, 2001: 641-93. [ISBN: 0323012000]
G. H. Sakorafas, C. Nissotakis, and G. Peros, “Abdominal desmoids tumors,” Surgical Oncology 2007, 16;(2):131–142.
K. Tanaka, R. Yoshikawa, H. Yanagi, et al.“Regression of sporadic intra-abdominal desmoid tumour following administration of non-steroidal anti-inflammatory drug,” World Journal of Surgical Oncology, vol. 6, article 17, 2008.
Carlson JW, Fletcher CD. Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: Analysis of a series and review of the literature. Histopathology 2007;51:509 –514.
Shimada S, Ishizawa T, Ishizawa K, et al. The value of MDM2 and CDK4 amplification levels using real-time polymerase chain reaction for the differential diagnosis of liposarcomas and their histologic mimickers. Hum Pathol 2006; 37:1123-9. [PMID: 16938516]
Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 2006; 130:1466-78. [PMID: 17090188]
Lasota J, Miettinen M. c-KIT and PDGFRA mutations in gastrointestinal stromal tumors (GISTs).Semin Diagn Pathol 2006; 23:91-102. [PMID: 17193822]
Lucas DR, al-Abbadi M, Tabaczka P, et al. c-Kit expression in desmoidfibromatosis. Comparative immunohistochemical evaluation of two commercial antibodies. Am J Clin Pathol 2003; 119:339-45. [PMID: 12645334]
Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, et al. Nuclear betacatenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol 2005;29:653-9. [PMID: 15832090]
Carlson JW, Fletcher CD.Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: Analysis of a series and review of the literature. Histopathology 2007;51:509 –514.
Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: Comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 2007;31:509 –520.
Swartz RD. Idiopathic retroperitoneal fibrosis: A review of the pathogenesis and approaches to treatment. Am J Kidney Dis 2009;54:546 –553.
Reitamo JJ.The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg 1983; 118:1318-22. [PMID: 6639341]
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