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PAPILLARY CYSTIC NEOPLASM OF THE PANCREAS (Case Report and Review of Literature)
KETAN R VAGHOLKAR*, MANISH P GANATRA**, PRITI SRINIWASAN**
*Associate Professor; **Lecturer, Department of Surgery, Padmashree Dr. DY Patil Medical College and Rajawadi Municipal General Hospital, Ghatkopar, Mumbai 77, India.
Papillary cystic neoplasm of the pancreas is a rare neoplasm principally affecting young women. It has a favourable prognosis and has a low grade malignant potential. Preoperative diagnosis is difficult to make due to similarity with other cystic pancreatic lesions. We report here a case of 14 year old girl who presented with an abdominal mass. Definitive diagnosis could not be made preoperatively. The patient underwent exploratory laparotomy. Total surgical excision of the pancreatic neoplasm was done. The postoperative recovery was uneventful. The diagnosis of papillary cystic neoplasm was made after histological examination of the surgical specimen. The patient has had no evidence of recurrence for the last six months. This case-report highlights the need for greater awareness of the pathology, diagnostic and surgical options for the treatment of this rare pancreatic neoplasm, which has a good prognosis if completely excised. A review of literature is presented.
INTRODUCTION
Papillary cystic neoplasm of the pancreas is an interesting but a rare neoplasm of the pancreas. It was first described in 1959 and since then been referred to by different names such as ‘Frantz’s Tumour’, ‘Solid and Cystic Tumour’ and ‘Solid and epithelial neoplasm’. [1] Although quite a few number of cases have been reported in literature yet there has been an increased recognition of this lesion in the recent years. Till 1997 the total number of cases reported in world literature was 294. [2] A review of medline search reveals that approximately 80 more patients have been reported after 1997, taking the total number of reported cases to 374.
CASE REPORT
A 14 year old girl presented with an abdominal mass of 1 year duration. The mass had gradually increased in size. There were no other symptoms. Clinical examination of the patient revealed a large intraabdominal mass. It was not possible to ascertain its origin from the retro peritoneum. There was no hepatosplenomegaly or free fluid in the abdomen. Complete haemogram, liver function tests and renal function test were normal. Percutaneous fine needle aspiration cytology was inconclusive. Contrast enhanced CT scan of the abdomen revealed a large mass in the close proximity of the pancreas causing compression of the head of pancreas (Fig. 1). However the exact organ of origin could not be ascertained authentically. The patient underwent exploratory laparotomy through a roof top incision. There was no hepatosplenomegaly, free fluid or any other evidence of metastasis. The lesser sac was opened, duodenum Kocherized and the right colon mobilized. The mass was well encapsulated and was originating from the head of the pancreas. The duodenum was stretched over its surface, while the pancreatic tissue was compressed underneath it (Fig. 2). A plane of dissection was developed between the compressed pancreatic head and the tumour. The entire neoplasm was separated from the adjacent compressed pancreatic tissue and excised. There was no communication with the pancreatic duct. Adequate haemostasis was achieved. There was no involvement of the major vessels. A tube drain was kept in the lesser sac prior to closure of the peritoneal cavity. Postoperative recovery of the patient was uneventful. The lesser sac drain was removed on the fifth postoperative day and patient discharged on the fourteenth postoperative day after complete suture removed. Macroscopically the tumour was well encapsulated, brownish in colour measuring 11 x 9 x 8 cms and weighing 500 gms (Fig. 3). The cut surface had a spongy haemorrhagic appearance with solid and cystic areas (Fig. 3). Microscopically the tumour was characterized by the cellularity and the presence of several papillae covered by several layers of epithelial cells. Focally hyaline lobules and collection of foamy cells were seen. These features are typical of a papillary and solid epithelial neoplasm of the pancreas. Patient has been following up for the last 6 months regularly with no symptoms, signs or radiological evidence of recurrence.
Fig 1 : Contrast enhanced CT scan of the abdomen reveals a large mass causing compression of the head of the pancreas
Fig 2 : Intra operative finding of a well encapsulated mass originating from the head of the pancreas
Fig 3 : Cut section of the specimen reveals a spongy haemorrhagic appearance with both solid and cystic areas
DISCUSSION
Cystic neoplasms of the pancreas are an interesting heterogeneous group of pathologic entities. Papillary cystic neoplasm is the rarest of cystic neoplasm of the pancreas. It has an indolent nature. There is a strong female preponderance with ages ranging from 16 to 90 years. [2,3]
Papillary cystic neoplasms of the pancreas possibly originate from the primordial primitive cells and lack definite endocrine or exocrine differentiation. [4] These are well circumscribed, encapsulated neoplasms arising from the head or body of the pancreas. Grossly they may be solid, cystic or haemorrhagic. There has been an isolated case report of a multicentric synchronous papillary cystic tumour. [5] Cytological studies show uniform centrally formed solid sheets and loss of cohesion producing pseudopapillae, foam cells, cholesterol granules and entrapped rests of pancreatic parenchyma. [4]
Immunohistochemical studies reveal that these neoplasms are reactive for vimentin, alpha-1-antitrypsin, neuron specific enolase, cytokeratin and S-100 protein. None of these are reactive for pancreatic hormones, opioid peptides, hormonal receptors or neuro endocrine markers. [4] Flowcytometry study shows aneuploidy (DNA index 1.34). [6] C-Ha-ras oncogene presumably is linked tothe development of this tumour. [6]
The commonest presentation is some degree of abdominal discomfort or pain and then follows a variety of presentations either related to the upper digestive system or the presence of mass. [7,8] Upto 50% may be discovered incidentally. In the case presented, the only symptom was an abdominal mass which is rare and surprising. The diagnosis of papillary cystic neoplasm cannot be made based on a single investigation. Blood investigations are inconclusive.
Despite the availability and active utilization of modern imaging techniques, it is not always possible to exclude the diagnosis of a pseudocyst. Clinical and radiological information should be assessed critically and a value judgement made. Once the diagnosis of pseudocyst is ruled out, then the nature of the true cyst can be determined. Amongst all the available diagnostic modalities CT scan still remains the most sensitive diagnostic tool. [9,10] A combination of CT scan with CT guided fine needle aspiration cytology can provide valuable information regarding the cystic lesion. [11] Although CT and other diagnostic modalities can identify cystic neoplasms of the pancreas, it is often difficult to make a definitive preoperative diagnosis. [7]
Surgery remains the mainstay of treatment. [13,14] There is no role of non-operative management. [12] There has been a isolated report which claims the efficacy of chemotherapy in the treatment of papillary cystic neoplasm of the pancreas. [15] There is another report which shows the efficacy of radiotherapy. [16] However there is a need for a prospective randomized trial to authentically advocate the role of chemotherapy or radiotherapy. Drainage is to be condemned as the patient is left with a permanent fistula.
Excellent outcomes have been achieved by an aggressive surgical approach to patients diagnosed as a papillary cystic neoplasm of the pancreas. The diagnosis can be confirmed intraoperatively by frozen section. An aggressive surgical approach is justifiable for various reasons namely (a) Preoperative differentiation of a benign versus malignant tumour is unreliable and routine testing for this purpose is of doubtful utility (b) Potential adverse sequelae of non-operative treatment are significant (c) There is significant decrease in perioperative morbidity and mortality associated with pancreatic surgery and (d) prognosis with curative resection is good. [17] When surgery is performed, benign lesions should be treated with pancreatic parenchymal sparing procedures if anatomy permits. The principles of oncological surgery should be followed while dealing with indeterminate or malignant lesions. [18] The various surgical resection procedures advocated are Whipple’s operation, pylorus preserving pancreaticoduodenectomy, distal pancreatectomy and splenectomy, enucleation or excision. [19] Complete surgical excision alone results in an excellent clinical outcome and disease free survival irrespective of the histology or radiological parameters in over 90% cases. [20-22]
In conclusion papillary cystic neoplasms of the pancreas pose both a diagnostic and therapeutic dilemma. CT along with CT guided FNAC is helpful in the preoperative diagnosis of this condition. However aggressive surgery remains the mainstay of treatment of this condition which has potential to develop a malignant change.
ACKNOWLEDGEMENT
I thank the Medical Superintendent of Rajawadi Municipal General Hospital, Ghatkopar, Mumbai 77, India for allowing me to publish this case.
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