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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 445-446
CT appearance of pancreatoblastoma: A case report


Department of Radiodiagnosis Kasturba Medical College Manipal Academy of Higher Education Manipal 576 104, Karnataka, India

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Date of Submission13-Dec-2005
Date of Acceptance10-Aug-2006
 

Keywords: Pancreatoblastoma, CT

How to cite this article:
Rajagopal K V, Kulakarni N M, Lakhkar B N. CT appearance of pancreatoblastoma: A case report. Indian J Radiol Imaging 2006;16:445-6

How to cite this URL:
Rajagopal K V, Kulakarni N M, Lakhkar B N. CT appearance of pancreatoblastoma: A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:445-6. Available from: http://www.ijri.org/text.asp?2006/16/4/445/32241
Pancreatoblastoma is a rare primary neoplasm of childhood, first reported in 1957 [1]. Approximately 100 cases of pancreatoblastoma have been reported in the medical literature. Although it is rare, its pathological features are well described.

Microscopicaly pancreatoblastoma resembles the incompletely differentiated acini of fetal origin at approximately 7 week of gestation [2],[3]. Pancreatoblastoma usually affects children between 1-8 years, although it may affect neonates as well as elderly individuals [4],[5],[6],[7]. Males are more commonly affected. The tumor is often clinically occult and is usually large at the time of initial presentation [8]. The symptoms are usually due to mass effect and include satiety, vomiting, constipation and pain [9].


   Case report Top


A 5 year old female child presented with pain abdomen. Clinical examination revealed mass in the left hypochondrium extending to umbilical region, epigastrium and left lumbar region. Laboratory values revealed elevated levels of alfa-fetoproteins. Other lab investigations were unremarkable.

CT revealed a large well defined heterogenously enhancing soft tissue mass with few non enhancing areas and enhancing septa noted in the region of body and tail of pancreas. Displacement of the pancreatic duct anteriorly and splenic vein posteriorly were noted confirming the pancreatic origin of the mass [Figure - 1]A-C. Invasion into the splenoportal junction with a thrombus within it was documented [Figure - 1]C. Based of CT imaging findings diagnosis of pancreatoblastoma or sarcoma was given.

Patient was subsequently operated and histopathology confirmed the nature of lesion to be pancreatoblastoma. Surgery revealed large bosselated encapsulated pancreatic mass arising from the body and tail of pancreas. Gross specimen showed encapsulated heterogenous mass with multiple septae and areas of necrosis and hemorrhage [Figure - 2]. No calcifications were evident within the mass. Few mucinous and cystic areas were also noted. Histological examination revealed solid sheet and nests of epithelial cells showing acinar differentiation [Figure 3]A and squamoid corpuscles [Figure 3]B. Large areas of myxoid and necrotic changes were evident. Large cells with moderate amount of cytoplasm with coarse chromatin were seen and few show nucleoli.


   Discussion Top


Pancreatic tumors in children other than pancreatoblastoma are exceedingly rare. Hemangioendothelioma, cystadenoma, and sarcoma arising in the pancreas have been reported [10],[11]. Other lesions to be considered in the differential diagnosis include neoplasms which are classically found in adults, such as ductal adenocarcinoma, acinar cell carcinoma, solid and papillary epithelial neoplasms and endocrine neoplasms [12].

The most common ultrasound finding of pancreatoblastoma is a mixed echogenic, solid mass inseparable from pancreas and the CT finding is of a relatively well defined lobulated, large mass with multiloculated appearance by septae in or near the lesser sac [13]. It was earlier thought to arise from only in the pancreatic head which derived from the ventral pancreatic anlage [2]. However few cases of pancreatoblastoma arising from the pancreatic tail have been reported [14]. The presence of squamoid corpuscles, loose aggregate of larger epitheloid cells with acinar formation are characteristic morphological features [15].

It has been mentioned that encapsulation in the pancreatoblastoma is one of the characteristic feature of tumor derived from ventral pancreatic anlage. In the present case, the tumor derived from the dorsal pancreatic anlage and contained tumor capsule [Figure 3]. Horie et al [2] also documented similar experience. Thus the presence of encapsulation should not exclude the diagnosis of pancreatoblastoma.

In our patient there was invasion of portal vein by the mass. To our knowledge invasion into the portal vein has not been earlier reported in the literature.

 
   References Top

1.Becker WF. Pancreatoduodenectomy for carcinoma of the pancreas in an infant. Ann Surg 1957; 145:864-872.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Horie A, Yano Y, Kotoo Y. Morphogenesis of pancreatoblastoma, infantile carcinoma of the pancreas: report of two cases. Cancer 1977; 39:247-254.  Back to cited text no. 2    
3.Cubilla AL, Fitzgerald PJ. Classification of pancreatic cancer (nonendocrine). Mayo Clin Proc 1979; 54:449-458.  Back to cited text no. 3  [PUBMED]  
4.Kloppel G, Maillet B. Classification and staging of pancreatic nonendocrine tumors. Radiol Clin North Am 1989; 27:105-119.   Back to cited text no. 4  [PUBMED]  
5.Friedman AC, Edmonds PR. Rare pancreatic malignancies. Radiol Clin North Am 1989; 27:177-190.  Back to cited text no. 5  [PUBMED]  
6.Klimstra DS, Adair CF, Hefess CS. Pancreatoblastoma: a clinicopathologic study and review of the literature. Am J Surg Pathol 1987; 11:855-865.  Back to cited text no. 6    
7.Levey JM, Banner BF. Adult pancreatoblastoma: a case report and review of the literature. Am J Gastroenterol 1996; 91:1841-1844.  Back to cited text no. 7  [PUBMED]  
8.Mergo PJ, Helmberger TK, Buetow PC, et al. Pancreatic neoplasms: MR imaging and pathologic correlation. RadioGraphics 1997; 17:281-301.  Back to cited text no. 8  [PUBMED]  
9.Passmore SJ, Berry PJ, Oakhill A. Recurrent pancreatoblastoma with inappropriate adrenocorticotropic hormone secretion. Arch Dis Child 1988; 63:1494-1496.  Back to cited text no. 9  [PUBMED]  
10.Chang CH, Perrin EV, Hertzler J, et al. Cystadenoma of the pancreas with cytomegalovirus infection in a female infant. Arch Pathol Lab Med 1980; 104:7-8.  Back to cited text no. 10  [PUBMED]  
11.Tunell WP. Hemangioendothelioma of the pancreas obstructing the common bile duct and duodenum. J Pediatr Surg 1976; 11:827-830.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: Imaging Findings in 10 Patients and Review of the Literature. Radiology. 2000;214:476-482.  Back to cited text no. 12    
13.Lee JY, Kim I, Kim WS, Kim CW, Yeon KM. CT and US findings of pancreatoblastoma. Journal of computer Assisted Tomography 1996;20(3):370-374.  Back to cited text no. 13    
14.Thomas E, Herman, Marilyn J, Siegel, Dehner LP. CT of pancreatoblastoma derived from the dorsal pancreatic anlage. J Comput Assist Tomogr 1994;18:648-650.  Back to cited text no. 14    
15.Klimstra DS, Wenig BM, Adair CF, Hefess CS. Pancreatoblastoma: a clinicopathological review of the literature. Am J Pathol 1995;12:1371-1389.  Back to cited text no. 15    

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Correspondence Address:
K V Rajagopal
Department of Radiodiagnosis, Kasturba Medical College and Hospital , Manipal 576104, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32241

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