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Year : 2016  |  Volume : 21  |  Issue : 2  |  Page : 154-157

Solid pseudopapillary tumor of pancreas: Arare case

Department of General Surgery,Government Medical College, Nagpur, Maharashtra, India

Date of Web Publication31-Aug-2016

Correspondence Address:
Chandratej Kadam
B-2 Anuradha Co-operative Housing Society Opp., Golf Club Rest House, Golf Club Road, Near Chandak Circle, Nashik - 422 002, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9903.189534

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Solid pseudopapillary neoplasms (SPNs) accounts for only 1–2% of pancreatic neoplasm. It occurs predominantly in young females. These are slow growing tumor having low-grade malignant potential with good long-term survival rate after complete resection. Due to its rarity, there are no large published series in a single institution and multicenter studies being necessary for its deeper understanding. We report one such rare case of SPN. A 19 year old female presenting with vague abdominal pain and no other significant Symptoms and after all investigations was found to have mass in the head of pancreas. Pancreaticoduodenectomy with complete resection of tumor mass was done. Histopathological features were suggestive of SPN with tumor-free margins. There were no signs of local or distant metastasis and is under 2 monthly follow-up for last 8 months

Keywords: Complete resection, pancreaticoduodenectomy, solid pseudopapillary

How to cite this article:
Gupta B B, Kadam C, Changole S S, Nandagawali V. Solid pseudopapillary tumor of pancreas: Arare case. J Mahatma Gandhi Inst Med Sci 2016;21:154-7

How to cite this URL:
Gupta B B, Kadam C, Changole S S, Nandagawali V. Solid pseudopapillary tumor of pancreas: Arare case. J Mahatma Gandhi Inst Med Sci [serial online] 2016 [cited 2020 Sep 19];21:154-7. Available from: http://www.jmgims.co.in/text.asp?2016/21/2/154/189534

  Introduction Top

Solid pseudopapillary neoplasms (SPNs) accounts for only 1–2% of pancreatic neoplasm.[1],[2] It occurs predominantly in young females with peak incidence between 20 and 30 years of age. It is frequently asymptomatic or minimally symptomatic. It is characterized by well capsulated, slow growing tumor having low-grade malignant potential. Most patients show good long-term survival rate after complete resection. It is also known as Hamoudi tumor, Frantz tumor, solid and cystic tumor, papillary cystic neoplasm, and papillary epithelial neoplasm. In 1996, the World Health Organization (WHO) classified these tumors as solid pseudopapillary tumor (SPT) of the pancreas.[3] Due to its rarity, there are no large published series in a single institution and multicenter studies being necessary for its deeper understanding. We report one such rare case of SPN being managed successfully at our institute.

  Case Report Top

A 19-year-old female resident of Amravati, Maharashtra, came to our institute with chief complaints of vague pain in abdomen on and off since 6 months. It was not associated with any aggravating or relieving factors. She had no other specific complaints or significant family history. On examination, she had palpable lump of size 4 cm × 4 cm mainly in the epigastric region which was well felt on standing than in supine position. There was no other significant finding. All routine blood investigations were within normal limit. Ultrasonography of abdomen showed an ill-defined heterogeneous solid cystic lesion of size 5.7 cm × 6.4 cm in relation to the head and uncinate process of pancreas, showing mild vascularity and few tiny calcification within. The lesion was surrounded by an ill-defined wall of thickness 2 mm of neoplastic etiology. However, it maintained fat planes with surrounding structure. Body and tail of pancreas were appeared normal. Main pancreatic duct was not dilated. Computed tomography (CT) scan of the abdomen was done which showed similar findings as shown in [Figure 1]. Postcontrast films pointed the lesion to be in close proximity of superior mesenteric vein and superior mesenteric artery (SMA). Moreover, the likely differentials were serious cyst adenoma or pancreatic adenoma.
Figure1: Computed tomography scan findings(red arrow)

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On the basis of the above findings, pancreaticoduodenectomy was planned for this patient. Intraoperatively as shown in [Figure 2], there was a mass of about 6 cm × 6 cm located at the head and uncinate process of pancreas which was abutting the inferior vena cava and SMA. After careful dissection, pancreaticoduodenectomy along with cholecystectomy was done followed by gastrojejunostomy, pancreaticojejunostomy, and choledocojejunostomy. Specimen, as shown in [Figure 3], was sent for histopathological examination. Postoperative course was uneventful and the patient was discharged on the 10th postoperative day.
Figure2: Intraoperative photographs showing 6cm×6cm mass in the head of pancreas

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Figure3: Specimen photographs of pancreatic mass(red arrow) with c loop of duodenum(blue arrow)

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Histopathological report as shown in [Figure 4] was suggestive of SPN with all margins of the specimen free from tumor infiltration and no lymph nodal metastasis.
Figure4: Histopathological features of solid pseudopapillary pancreatic tumor(typical papillary process with solid and cystic area)

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  Discussion Top

SPT of the pancreas is a rare exocrine pancreatic tumor, which represents only about 1% of all tumors of the pancreas. Lichtenstein gave the first description of SPN when he reported a large pancreatic tail mass in a young woman with peritoneal carcinomatosis. However, the first unequivocal account was a series of three cases with detailed pathologic descriptions by Frantz in 1959.[4] Hamoudi et al. were the first to characterize the pathognomonic electron microscopic features of pancreatic SPNs.[5] For this reason, some refer to these lesions as Frantz tumors or Hamoudi-Frantz tumors. Different names for this tumor were reported until it was defined by the WHO in 1996 as a “SPT” of the pancreas.[2] There are very few case reports and small series of pancreatic SPN in the world literature. The largest series comprised 718 cases and was published by Papavramidis and Papavramidis in 2005.[1],[2] They noted that the majority of the reports originated from Europe, Japan, and North America. There are very few cases reported in India.

The origin of SPTs is unclear. Many investigators favor the theory that SPTs originate from multipotent primordial cells, whereas others suggest an extrapancreatic origin, from genital ridge angle-related cells.[6] These tumors generally occur in young female with mean age of presentation is 22 years. It is not symptomatic most of the times; however, sometimes patient may present with symptoms from mass effect such as vomiting and early satiety due to gastric outlet obstruction. Jaundice is not a common feature. In Papavramidis' series, only 1% of patients with pancreatic head tumors were jaundiced.[1] Our patient presented with vague abdominal pain without any such symptoms.

These tumors can be visualized in many imaging modalities, such as ultrasonography, CT, and magnetic resonance imaging, which can be used to differentiate it from other pancreatic lesions. Encapsulated, well-defined mass with central areas of calcification, necrosis, hemorrhage, and/or cystic degenerations are the characteristic findings. In both the arterial and venous phases, there is usually peripheral enhancement with similar Hounsfield unit density as the nearby pancreatic parenchyma.[7] This differs from adenocarcinomas that are usually hypoattenuated on venous phase CT and from pancreatic neuroendocrine tumors that enhance on the arterial phase CT. Preoperative tissue diagnosis is not indicated.[8] Blood investigations or markers have no specific role in the diagnosis.

Patients with resectable lesions who are candidates for operation should be treated by en bloc resection with clear margins, since this provides the best chance for cure and good long-term survival rate.

Even in the presence of poor prognosticators (lymphovascular invasion, capsular invasion, local extension, nodal disease, and liver metastases) that traditionally predict malignant behavior, there are still relatively good outcomes compared to adenocarcinomas.[9],[10] Hence, resection is advisable even in those cases. In our case, we achieved a complete resection of tumor with clear margins. Role of chemotherapy in advance stages is still under trial. Our patient had no local or region metastasis, hence no further treatment was required. Our patient is being followed every two monthly and has no significant complaints even after 8 months.

  Conclusion Top

Pancreatic pseudopapillary tumors are rare neoplasms with malignant potential found in young females. Formal surgical resection may be performed safely and is associated with long-term survival. Because long-term survival can be achieved, patients with SPN should be treated aggressively with complete resection.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: Review of 718 patients reported in English literature. J Am Coll Surg 2005;200:965-72.  Back to cited text no. 1
de Castro SM, Singhal D, Aronson DC, Busch OR, van Gulik TM, Obertop H, et al. Management of solid-pseudopapillary neoplasms of the pancreas: A comparison with standard pancreatic neoplasms. World J Surg 2007;31:1130-5.  Back to cited text no. 2
Coleman KM, Doherty MC, Bigler SA. Solid-pseudopapillary tumor of the pancreas. Radiographics 2003;23:1644-8.  Back to cited text no. 3
Frantz VK. Atlas of Tumour Pathology. Tumours of the pancreas 1959;1:32-3.  Back to cited text no. 4
Hamoudi AB, Misugi K, Grosfeld JL, Reiner CB. Papillary epithelial neoplasm of pancreas in a child. Report of a case with electron microscopy. Cancer 1970;26:1126-34.  Back to cited text no. 5
Zuriarrain A, Nir I, Bocklage T, Rajput A. Pseudopapillary tumor of the pancreas in a 17-year-old girl. J Clin Oncol 2011;29:e395-6.  Back to cited text no. 6
Wang Y, Miller FH, Chen ZE, Merrick L, Mortele KJ, Hoff FL, et al. Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas. Radiographics 2011;31:E47-64.  Back to cited text no. 7
Procacci C, Graziani R, Bicego E, Zicari M, Bergamo Andreis IA, Zamboni G, et al. Papillary cystic neoplasm of the pancreas: Radiological findings. Abdom Imaging 1996;21:554-8.  Back to cited text no. 8
Reddy S, Cameron JL, Scudiere J, Hruban RH, Fishman EK, Ahuja N, et al. Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): A large single-institutional series. J Am Coll Surg 2009;208:950-7.  Back to cited text no. 9
Lam KY, Lo CY, Fan ST. Pancreatic solid-cystic-papillary tumor: Clinicopathologic features in eight patients from Hong Kong and review of the literature. World J Surg 1999;23:1045-50.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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