Table of Contents  

Kamal, Packirirsamy, Badri, Al Mazrouei, Khaleeq, and Alaqqad: Pancreatic solid pseudo-papillary tumours

Introduction

A solid pseudo-papillary tumour (SPT) of the pancreas is a rare primary neoplasm with low malignant potential which was first reported by Frantz in 1959.1 This type of tumour accounts for 1% of all pancreatic neoplasms, but its diagnosis is usually incidental in otherwise asymptomatic patients. Preoperative confirmation of diagnosis is difficult, but abdominal imaging can assist: abdominal computerized tomography (CT), endoscopic ultrasonography (EUS) with fine-needle aspiration cytology and magnetic resonance imaging (MRI) all help in establishing the diagnosis. Despite their large size, attachment of adjacent structures or even metastasis, these tumours, after resection, are associated with a very good prognosis. Adjuvant chemotherapy may be planned for malignant lesions.

Background

In this report, we present three cases of patients with SPTs of the pancreas and discuss the histopathological findings with a review of literature. In our case series, three female patients presented in the clinic and emergency settings for non-specified abdominal complaints and were investigated. Initially, abdominal imaging was performed and showed a pancreatic mass. Exploratory laparotomy with pancreatic resections was performed in all three women and reconstruction of the portal vein was also performed in one patient (case 2). After surgical resection, the histological diagnosis confirmed the presence of a SPT of the pancreas in all three women. All of the patients were followed up every 6 months. The investigations performed during follow-up included routine blood investigations, chest radiography, cancer antigen (CA) 19–9 level and either ultrasound or CT of the abdomen. In these subsequent follow-up visits, no complications or recurrences were found.

Case report

Case 1

A 59-year-old woman was incidentally diagnosed with a pancreatic cyst during ultrasound ordered for a clinically suspected gallstone. MRI of the abdomen confirmed a multiloculated cystic lesion in the body and tail of the pancreas (Figure 1).

FIGURE 1

Preoperative abdominal magnetic resonance image showing a pancreatic mass.

HMJ-636-fig1.jpg

Distal pancreatectomy with splenectomy was performed and histopathology of the resected specimen showed serous cystadenoma of the pancreas without any lymph node involvement (Figure 2). Her postoperative period was uneventful.

FIGURE 2

Histopathology of the resected specimens from (a) case 1; (b) case 2; and (c) case 3.

HMJ-636-fig2a.jpgHMJ-636-fig2b.jpgHMJ-636-fig2c.jpg

Case 2

A 23-year-old woman presented with complaints of off and on abdominal discomfort, bloating and a feeling of a swelling in the upper abdomen. CT of the abdomen showed a large, heterogeneous mass of retroperitoneal origin (Figure 3). The mass was compressing the inferior vena cava and, as a result of this, conventional angiography with selective embolization of the superior mesenteric artery and gastroduodenal artery was performed before the abdominal operation.

FIGURE 3

Preoperative abdominal CT image showing a mass arising from the pancreas.

HMJ-636-fig3.jpg

During laparotomy, total pancreatectomy with reconstruction of the portal vein and superior mesenteric vein was carried out (Figure 4). Despite the large size of the tumour, complete excision was possible. Histopathology of the resected specimen showed an SPT of the pancreas without any evidence of involvement of the peri-pancreatic tissue (Figures 5 and 6). Postoperative hospital recovery was smooth and follow-up for 2 years did not show any recurrence.

FIGURE 4

Intraoperative gross appearance of the tumour arising from the pancreas. Red arrow indicates the region where the tumour arises from the pancreas.

HMJ-636-fig4.jpg
FIGURE 5

Histopathology of the resected specimen.

HMJ-636-fig5.jpg
FIGURE 6

Histopathology of the resected specimen showing an SPT of the pancreas.

HMJ-636-fig6.jpg

Case 3

A 36-year-old woman presented with an 8-month history of epigastric pain. Abdominal examination showed mild tenderness over the epigastric area without any swelling or gross distension. CT of the abdomen showed a mass in the body of the pancreas (Figures 710).

FIGURE 7

Preoperative CT image of the abdomen showing a pancreatic mass in sagittal view.

HMJ-636-fig7.jpg
FIGURE 8

Follow-up CT image of the abdomen showing no recurrence after 2 years.

HMJ-636-fig8.jpg
FIGURE 9

Preoperative CT image of the abdomen showing a mass in the body of the pancreas.

HMJ-636-fig9.jpg
FIGURE 10

Follow-up CT image of the abdomen showing no recurrence after 1 year.

HMJ-636-fig10.jpg

During the operation, a frozen section of the tumour showed a solid cystic papillary neoplasm of pancreatic origin, so distal pancreatectomy was performed. Her postoperative stay was uneventful and follow-up over 1 year was unremarkable.

Discussion

An SPT of the pancreas is a rare tumour arising from the exocrine pancreas and represents only 1% of all tumours of the pancreas. It was first described by Frantz in 1959.1 The origin of SPTs is unclear, and several researchers favour the theory that they originate from multipotent primordial cells, whereas others suggest an extrapancreatic origin such as genital ridge cells.2,3

Pathology

Despite their large size, the majority of SPTs exhibit benign behaviour. Only 15% of these tumours show features of malignancy either on histology or as local invasion to adjacent structures or metastases to the liver, regional lymph nodes, omentum, mesentery and peritoneum.3,4 The majority of such tumours are located in the pancreatic body and tail.4 The morphological appearance of SPTs varies from solid to cystic components with cellular degenerative changes.5

Pancreatic SPTs, on gross appearance, are usually encapsulated and less likely to be cystic with variable fibrosis. Larger tumours appear capsulated owing to a fibrous pseudocapsule separated from the pancreas. There can be combinations of solid haemorrhagic and cystic necrotic areas of the tumour.6

Microscopically, true papillary stalks are not seen. However, a combination of solid, pseudo-papillary or haemorrhagic pseudo-cystic structures in various proportions are present.7 The solid areas consist of sheets and cords of cells arranged around fibrovascular or microvascular septa. As a result of degenerative changes, such as swelling and formation of mitotic spaces between cells farthest from the tiny blood vessels, a pseudo-papillary pattern is formed.7 The histology of metastasizing tumours shows a higher nuclear grade and aggregates of cells with pyknotic nuclei and eosinophilic cytoplasm.7

Clinical features

These pancreatic tumours are more commonly seen in females (male to female ratio 1 : 10), in particular those of Asian or African ethnicity, with a mean age at presentation of 22 years. They are commonly asymptomatic or cause a slowly enlarging abdominal mass with or without vague abdominal pain. These tumours may be found accidentally during diagnostic imaging procedures or may be suspected in the presence of a symptomatic palpable mass.8

Investigations

Tumour markers

Preoperative tumour markers such as alpha-fetoprotein, carcinoembryonic antigen (CA) 19–9 and CA 125 are all within normal limits in all patients with SPT.9

Radiology

Like other tumours of the exocrine pancreas, SPT can be diagnosed using CT, ultrasound, endoluminal ultrasound and MRI.10,11 The presence of a capsule, calcification, haemorrhagic necrosis, and solid and cystic components can be seen on both abdominal ultrasound and CT.12 However, cystic and haemorrhagic components inside the tumour itself and the presence of a capsule are better demonstrated by MRI than by CT.5

Endoscopic ultrasonography with fine-needle aspiration can be more accurate in establishing the diagnosis; however, this is not an accepted means of diagnosis because of the risk of spread of a malignant tumour.13 On EUS, several features can be suggestive of malignancy. These features include septations, calcifications, communication with the main pancreatic duct or dilatation of the main pancreatic duct.13

The American Society of Gastrointestinal Endoscopy guidelines suggest that cystic lesions of the pancreas are potentially malignant and EUS alone is not accurate enough to define the type of lesion and its malignant nature. Additional investigations such as endoscopic retrograde cholangiopancreatography can show duct obstruction, mural nodules or communication without defining the lesion.14

Management

In patients with SPT, the treatment of choice is conservative resection (saving as much of the pancreas as possible). The type of procedure is determined by the appearance of the tumour, as benign or malignant, the age of the patient and the anatomical location of the tumour in the head, body or tail of the pancreas. The Whipple procedure is performed for tumours of the pancreatic head,1517 and distal pancreatectomy, with or without splenic preservation, can be performed for tumours in the body or tail of the pancreas. In Japan, 35% of SPTs originating in the pancreatic head have been treated with enucleation and > 60% have been resected by a classical or pylorus-preserving pancreatoduodenectomy.18 As a result of their low malignant potential, in paediatric patients simple enucleation of smaller tumours is recommended.1921

Surgical management of SPT differs from that of other tumours of the exocrine pancreas in many ways: (1) for localised tumours of the neck and body of the pancreas, resection of the mid-portion of the pancreas, including the mass with preservation of head and tail portions, is possible;22 (2) invasion to the portal vein or superior mesenteric artery does not indicate that pancreatic neoplasms are non-resectable;22 (3) extensive lymphatic dissections or more radical local resections are not indicated;15 and (4) in cases of metastatic tumours, surgical debulking can be performed.23 Specifically, when liver metastases are present (and the liver is the most common site of metastasis), surgical treatment in the form of operative excisions (lobectomies or enucleations) is appropriate. Resection of liver metastases is possible if liver involvement is limited.4

Chemotherapy is successful in some patients,23 although intra-arterial chemoembolization in patients with multiple hepatic metastases seems to be of value.24 Radiotherapy has been suggested in cases of non-resectable SPTs because they are radiosensitive.23 Although not yet applied, laparoscopic surgery seems to be an alternative approach, especially where only enucleation of a smaller SPT is indicated.

Prognosis

Solid pseudo-papillary tumours, after resection, are associated with excellent prognosis. In the case of patients with tumours confined to the pancreas, overall survival is > 95%. If metastasis is seen, long-term survival from years to decades has been described in the literature for asymptomatic liver metastasis.25 Recurrence after a complete excision of a SPT can occur in 10–15% of cases.21 In our case reports, no recurrences were noted during follow-up ranging from 1 to 4 years.

Conclusion

In conclusion, malignant SPT is a low-grade tumour associated with a good prognosis. Adequate surgical intervention is necessary for the long-term survival of patients and, whenever possible, aggressive and repeated surgery is justified for locally invasive or metastatic SPT. Characteristic CT and MRI findings, combined with age and sex profile, are important to decide the extent of resection required. Regular follow-up is mandatory for all patients who have undergone potentially curative resection. Particular attention should be paid to patients with tumours > 5 cm in diameter and/or with histology suggesting possible malignancy. The role of chemotherapy, radiotherapy, transarterial chemoembolization and radiofrequency is still under scrutiny. Careful follow-up may prevent tumour progression.

References

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Frantz VK. Tumors of pancreas. In: Bumberg CW (ed.) Atlas of Tumors Pathology, VII. Fascicles 27 and 28. Washington, DC: Armed Forces Institute of Pathology; 1959. pp. 32–3.

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Zuriarrain A, Nir I, Bocklage T, et al. Pseudopapillary tumor of the pancreas in a 17 year-old girl. J Clin Oncol 2011; 29:e395–6. http://dx.doi.org/10.1200/JCO.2010.33.5364

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Mao C, Guvendi M, Domenico DR, et al. Papillary cystic and solid tumors of the pancreas: a pancreatic embryonic tumor? Studies of three cases and cumulative review of the world’s literature. Surgery 1995; 118:821–8. http://dx.doi.org/10.1016/S0039-6060(05)80271-5

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Bardales RH, Centeno B, Mallery JS, et al. Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid – pseudopapillary tumor of the pancreas: a rare neoplasm of elusive origin but characteristic cytomorphologic features. Am J Clin Pathol 2004; 121:654–62. http://dx.doi.org/10.1309/DKK2B9V4N0W26A8Q

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Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000; 17:66–80.

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Kosmahl M, Seada LS, Dieter-Harms UJ, Klöppel G. Solid pseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch 2000; 436:473–80. http://dx.doi.org/10.1007/s004280050475

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Chen X, Zhou GW, Zhou HJ, et al. Diagnosis and treatment of solid pseudopapillary tumors of the pancreas. Hepatobiliary Pancreas Dis Int 2005; 75:684–9.

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Yu PF, Hu ZH, Wang XB, et al. Solid pseudopapillary tumor of the pancreas: a review of 553 cases in Chinese literature. World J Gastroenterol 2010; 16:1209–14. http://dx.doi.org/10.3748/wjg.v16.i10.1209

10. 

Trivedi N, Sharma U, Das PM, Mittal MK, Talib VH. FNAC of papillary and solid epithelial neoplasm of pancreas. A case report. Indian J Pathol Microbiol 1999; 42:369–72.

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Cantisani V, Mortele KJ, Levy A, et al. MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. Am J Roentgenol 2003; 181:395–401. http://dx.doi.org/10.2214/ajr.181.2.1810395

13. 

Raffel A, Cupisti K, Krausch M, et al. Therapeutic strategy of papillary cystic and solid neoplasm (PCSN): a rare non-endocrine tumor of the pancreas in children. Surg Oncol 2004; 13:1–6. http://dx.doi.org/10.1016/j.suronc.2003.09.003

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Jacobson BC, Baron TH, Adler DG, et al. ASGE guideline: the role of endoscopy in the diagnosis and management of cystic lesions and inflammatory fluid collection of the pancreas. Gastrointest Endosc 2005; 61:363–70. http://dx.doi.org/10.1016/S0016-5107(04)02779-8

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Ferlan-Marolt V, Pleskovic L, Pegan V. Solid papillary-cystic tumor of the pancreas. Hepatogastroenterology 1999; 46:2978–82.

18. 

Akiyama H, Ono K, Takano M, et al. Solid pseudopapillary tumor of the pancreatic head causing marked distal atrophy. Int J Pancreatol 2002; 32:47–52. http://dx.doi.org/10.1385/IJGC:32:1:47

19. 

Sun CD, Lee WJ, Choi JS, et al. Solid-pseudopapillary tumors of the pancreas: 14 years’ experience. Aust NZ J Surg 2005; 75:684–9. http://dx.doi.org/10.1111/j.1445-2197.2005.03488.x

20. 

Jaksic T, Yaman M, Thorner P, et al. A 20-year review of pediatric pancreatic tumors. J Pediatr Surg 1992; 27:1315–17. http://dx.doi.org/10.1016/0022-3468(92)90284-E

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Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas: a clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer 1990; 65:283–91. http://dx.doi.org/10.1002/1097-0142(19900115)65:2%3C283::AID-CNCR2820650217%3E3.0.CO;2-X

22. 

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Rebhandl W, Felderbauer FX, Puig S, et al. Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature. J Surg Oncol 2001; 20:221–4. http://dx.doi.org/10.1002/jso.1048

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