Solid Pseudopapillary Tumor / Neoplasm: Pitfalls in Management of Cystic tumor of Pancreas

A 13 year girl presented with history of mild upper abdominal pain of 6 years duration. The pain had increased in intensity for 1 month. However there was no history of use of intravenous analgesics or of hospitalization for pain relief. She was evaluated and diagnosed to have pancreatic pseudocyst and an open cystoenterostomy was attempted elsewhere. However operative findings at exploration precluded internal drainage. She had an uneventful recovery and was subsequently referred to higher center for further management.

On examination at our centre, she was found to be thin built with stable vitals. Abdominal examination was unremarkable except for well healed midline laparotomy scar.

All routine hematologic and biochemical investigations were within the prescribed normal range. A contrast enhanced computed tomography (CECT) scan of the abdomen was performed (Figure 1 & 2).

Figure 1.

Figure 1 of abdominal CECT shows a 6 cm tumour of the body and tail of the pancreas with solid and cystic components. 

Figure 2.

Figure 2 reveals an air pocket in the tumour at the site of previous biopsy.

 In view of young female patient with an indolent history and aforementioned image features, a diagnosis of solid pseudopapillary tumour (SPT) was made and distal pancreatectomy with splenectomy performed (Figure 3). Histopathology was confirmatory of SPT.

Our report reiterates that when managing cystic lesions of pancreas, a careful evaluation of history, biochemical investigations and imaging is essential to differentiate pseudocyst from cystic tumors. In most instances, the latter can be easily identified by an absence of documented episode of acute pancreatitis and cross sectional imaging depicting thick walled multilocular lesion with septae or solid components. Cyst fluid analysis (preferably endoscopic ultrasound guided) may be helpful when doubt exists in diagnosis. The features indicative of PC include high amylase content, inflammatory cytology and tumor markers (CEA, CA19-9, CA 72-4) within the specified normal range.¹

First described by Frantz in 1959, SPT of the pancreas is a rare neoplasm comprising of 1-2% of all pancreatic tumors.² This entity is encountered almost exclusively in young females in their second or third decade with male to female ratio of 1 is to 9. The tumor may be asymptomatic or associated with vague symptoms such as abdominal pain/discomfort, nausea or loss of appetite. Consequently SPTs often attain large size (3-16cms) by the time a diagnosis is made. At imaging, the characteristic CECT features include a large heterogeneous mass with solid and cystic components and areas of hyperattenuation representing hemorrhage.

With an increased understanding of the biological behavior [low malignant potential (15%) and excellent long-term prognosis in patients with metastatic disease the surgical management of SPTs has evolved over the last decade. The essence of management of primary tumor is on organ preservation. For the tumors in the pancreatic head, pylorus preserving pancreaticoduodenectomy and for the body and tail tumors into a spleen preserving distal pancreatectomy are acceptable procedures. However in patients with large tumors, spleen preservation may not always be feasible. Central resections for body tumors should only be performed at high volume centers. Due to a low incidence of lymph node involvement (<2%) a formal lymphadenectomy is not indicated. The currently available evidence supports metastectomy with 1 cm margin for liver metastases and debulking for peritoneal deposits. Overall 5-year survival is as high as 100% in patients undergoing surgical resection.³ Extended survival is still possible even with extensive disease with debulking surgery.

References

1.       Brugge WR, Lauwers GY, Sahani D, Fernandezdel,Castillo C, Warshaw AL. Cystic neoplasms of the pancreas. N Engl J Med 2004, 351:1218-26

2.       Frantz VK. Papillary tumors of the pancreas: Benign or malignant? Tumors of the pancreas. In: Atlas of Tumor Pathology, Section 7, Fascicles 27 and 28.Washington, DC, USA: Armed Forces Institute of Pathology, 1959:32-3.

3.       de Castro S. M. M, Singhal D, Aronson DC, Busch OR,TM van Gulik, Obertop H, D Gouma et al. Management of Solid-pseudopapillary Neoplasms of the Pancreas: a Comparison with Standard Pancreatic Neoplasms. World J Surg. 2007; 31(5): 1130–1135

Authors:

Dr Nitin Vashistha, MS, FIAGES, FACS

Dr Dinesh Singhal, MS, FACS, DNB (Surg Gastro)

Department of Surgical Gastroenterology,
Max Super Speciality Hospital, Saket, New Delhi, India
E mail: gi.cancer.india@gmail.com

www.gicancerindia.com