Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2004  |  Volume : 10  |  Issue : 1  |  Page : 29-31
Solid and cystic papillary epithelial neoplasm of the pancreas


1 Department of Histopathology, Royal Hospital, Sultanate of Oman
2 Department of Surgery, Nizwa Hospital, Sultanate of Oman

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Date of Submission14-Jul-2002
Date of Acceptance15-Jul-2003
 

How to cite this article:
Kuruvila S, Samarasinghe D, Musa MM, Khalaf SM. Solid and cystic papillary epithelial neoplasm of the pancreas. Saudi J Gastroenterol 2004;10:29-31

How to cite this URL:
Kuruvila S, Samarasinghe D, Musa MM, Khalaf SM. Solid and cystic papillary epithelial neoplasm of the pancreas. Saudi J Gastroenterol [serial online] 2004 [cited 2020 Nov 29];10:29-31. Available from: https://www.saudijgastro.com/text.asp?2004/10/1/29/33349


Solid and cystic papillary epithelial neoplasm of the pancreas is a large tumour with favourable prognosis that occurs almost exclusively in adolescent girls and young women. It is also known as papillary and cystic or solid and papillary epithelial neoplasm of the pancreas. Till recently, this tumour was incorrectly classified amongst tumors like adenocarcinoma, islet cell tumour, cystadenoma and cyst adenocarcinoma [1] . Due to the slow growth of the tumour it often remains asymptomatic till it manifests as an abdominal mass, occasionally with acute abdominal pain and features of peritonitis, suggesting rupture of the tumour. Absence of symptoms related to functional islet cell tumours of the pancreas, absence of weight loss or anorexia are quite characteristic of this tumour, although these could be associated with a higher grade of malignancy [1] . Due to its slow growth, obstructive jaundice is not a usual feature. We report one such case due to its rarity and unusual clinical presentation.


   Case Report Top


A healthy 19-year-old lady presented with spontaneous upper abdominal pain, nausea and vomiting. On examination, there was tenderness and guarding in the right hypochondrium. Routine investigations were normal. Ultrasound examination of the abdomen revealed a paraduodenal mass measuring about 7.5 cm, the remaining evaluation being normal. C.T.Scan of the abdomen confirmed a solid mass arising from the lateral aspect of the head of the pancreas measuring 7x6.5 cms without demonstrable capsule and showing both solid and cystic pattern.

Laparotomy and pancreaticoduodenectomy were done and the specimen was sent for histopathological examination. The segment of small bowel measured 33 cms in length. Eight cms from one end, a large circumscribed mass was seen measuring 9x9x7 cms. The tumour was clearly seen outside the bowel wall, but pressing into the lumen [Figure - 1]. On cutting the bowel open, the mucosa was unremarkable except for focal obliteration of the mucosal folds, corresponding to the area showing the external mass. On slicing the tumour, there were greyish white and cream coloured areas with necrosis. Cystic spaces were also seen. Compressed pancreatic tissue was seen outside the tumour in one area. Histological examination showed extensive necrosis. Focal viable areas were seen, which showed a neoplasm composed of cells arranged in small groups lining cystic spaces and also thrown into papillary folds. The cells were small with round to oval, well defined hyperchromatic nuclei, indistinct nucleoli and irregular nuclear margins. Mitotic activity was minimal [Figure - 2]. A compressed rim of normal pancreatic tissue was seen at the periphery. Immuno histochemical stains showed positivity for alpha-1 antichymotrypsin, alpha-1 antitrypsin, vimentin neuron specific enolase. Carcinoembryonic antigen, keratin, insulin, glucogon, gastrin, chromogranin and somatostatin, estrogen and progesterone receptors were all negative. A diagnosis of solid and cystic papillary epithelial neoplasm of the pancreas was made and pathological TNM staging of the tumour corresponded to pT 1b N X .


   Discussion Top


This tumour was first described by Frantz in 1959, who then called it `Papillary tumour of the pancreas [2] It has been diagnosed incorrectly as adenocarcinoma, islet cell tumour, cystadenoma or cystadenocarcinoma. This tumour however, is a unique tumour with any of the above mentioned tumour types inappropriate designations, since all of them have different functional, epidemiologic, morphologic and prognostic

Characteristics [1] This is a slow growing tumour with good prognosis. About 10­20% undergo malignant transformation into pseudopapillary carcinoma [3] . The malignant transformation is characterised by vascular invasion and metastasis to lymphnodes and liver. The histogenesis of this tumour is still controversial, but it is thought to be arising from primitive epithelial cells [4] . The diagnosis is often implied by radiological examination. Choi et al reported six cases of this tumour with C.T.scan showing well circumscribed round or lobulated mass with cystic and solid areas [5] . The cystic portion suggests hemorrhagic necrosis. They concluded that solid and cystic papillary epithelial neoplasm of the pancreas should be considered as the primary diagnosis when characteristic C.T. findings are detected in young female patients. Angiography would reveal avascular or hypovascular lesion that tends to encase and even displace them due to their slow growth [1] . Grossly it is a circumscribed tumour demarcated from the surrounding pancreas by a fibrous layer.

The classical histological pattern is that of a solid and papillary epithelial pattern. Papillary, microcystic and solid areas may be seen with extensive hemorrhage and necrosis. The cells are small, uniform and polygonal with dark nuclei, and moderate amount of eosinophilic or clear cytoplasm. The nuclei show typical indentation or grooving. Mitosis are few. The central cystic zone may contain papillae. These are considered by some to represent pseudopapillae resulting from degenerative changes [6] . All these features were identified in our case. Electron microscopy shows cells with large nucleoli in the centre of the nucleus and prominent nuclear membrane. The cytoplasm is rich in endoplasmic reticulum and. badly preserved lamellated mitochondria. Membrane bound granules are also seen [6] . This tumour must be differentiated from pancreatic neuro­endocrine or islet cell tumours. Solid and cystic papillary epithelial neoplasm shows cells positive for alpha 1 antichymotrypsin, alpha-1 antitrypsin, vimentin and neuron specific enolase. Cytokeratin may be positive. Insulin, glucagon, gastrin, chromogranin and somatostatin are negative. In our case the immunohistochemical stains favoured this diagnosis rather than endocrine or islet cell tumours since alpha antichymotrypsin, alpha-1 antitrypsin, vimentin and neuron specific enolase were positive.

The age and sex distribution, suggests hormonal factors in the pathogenesis of solid and cystic papillary epithelial neoplasm. Pancreaticoduodenectomy is the the treatment of choice [7] , but estrogen and progesterone receptors have been found to be positive in some cases [8] , thereby raising the possibility of hormonal therapy. Estrogen and progesterone receptors were negative in our case. The prognosis is generally good, despite its size. The majority of tumours seem to have borderline malignant potential, but most patients are symptom free many years after surgery [9] . In a study done by Yoon et al, curative resection and follow up of nine patients for 4-5 years revealed that all of them were doing very well [7] . This study supports the fact that prompt diagnosis and surgical resection of this tumour would favour excellent prognosis for the patient. In our case, the patient is doing well and she is on regular follow up.

 
   References Top

1.Zinner MJ. Solid and papillary neoplasm of the pancreas. In: Cameron JL, editor. Pancreatic neoplasms. Surgical clinics of North America. W B Saunders, 1995; 75:1017-23.  Back to cited text no. 1    
2.Frantz VK. Tumours of pancreas. In: Atlas of Tumour Pathology, Fascicle, U.S. Armed Forces Institute of Pathology. 1959; 1: 27-8.  Back to cited text no. 2    
3.Warren RB. Papillary cystic tumour of the pancreas. Arch Pathol Lab Med 1985; 109: 706­-07.  Back to cited text no. 3  [PUBMED]  
4.Longnecker DS. Pancreas. In: Damjanov I, Linder J, editors. Andersons Pathology. Mosby. 1996; 2 :1891-1915.  Back to cited text no. 4    
5.Choi BI. Kim KW. Han MC, et al. Solid and papillary epithelial neoplasm of the pancreas: CT findings. Radiology 1988; 166: 413-16.  Back to cited text no. 5    
6.Bogomoletz WV. Cystic tumours of the exocrine pancreas. In: Antony PP, MacSween RN, editors. Recent Advances in Histopathology. Churchill Livingstone, 1993: 150-2.  Back to cited text no. 6    
7.Yoon DY, Hines OJ, Bichik AJ Lowin K. Solid and papillary epithelial neoplasm of the pancreas, aggressive resection for cure. Am Surg 2001; 67:1195-9.  Back to cited text no. 7    
8.Wrba F, Chott A, Ludwik B et al. Solid and cystic tumours of pancreas: a hormonal dependent neoplasm? Histopathology 1988; 12: 338-40.  Back to cited text no. 8    
9.Kloppel G, Bogmoletz WV. Tumours of exocrine pancreas.In :Fletcher CD, editor. Diagnostic Histopathology of Tumours. Churchill Livingstone, 2000: 470.  Back to cited text no. 9    

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Correspondence Address:
Sarah Kuruvila
Department of Histopathology, Royal Hospital, P 0 Box 1331, Postal Code 111-Seeb, Sultanate of Oman

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Source of Support: None, Conflict of Interest: None


PMID: 19861825

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