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Table of Contents
CASE REPORT
Year : 2017  |  Volume : 14  |  Issue : 4  |  Page : 221-223

A rare case report of solid pseudopapillary tumor of the pancreas


Department of Laboratory Services, Apollo Hospitals, Bilaspur, Chhattisgarh, India

Date of Web Publication5-Feb-2018

Correspondence Address:
Vandana Gite
Department of Laboratory Services, Apollo Hospitals, Bilaspur, Chhattisgarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_25_17

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  Abstract 

A solid pseudopapillary tumor is a low-grade malignant cystic exocrine neoplasm of the pancreas of papillary architecture that typically afflicts young women with excellent postsurgical curative rates and rare metastasis. It may be locally aggressive. Pathological evaluation remains the gold standard in reaching a definitive diagnosis. We report a case of solid pseudopapillary neoplasm of the pancreas that was suspected on radiology and diagnosed preoperatively on biopsy guiding definitive surgery.

Keywords: Cystic exocrine neoplasm, distal pancreatico – splenectomy, papillary architecture, solid pseudo papillary tumour, young women


How to cite this article:
Gite V, Dhakane M. A rare case report of solid pseudopapillary tumor of the pancreas. Apollo Med 2017;14:221-3

How to cite this URL:
Gite V, Dhakane M. A rare case report of solid pseudopapillary tumor of the pancreas. Apollo Med [serial online] 2017 [cited 2019 Mar 21];14:221-3. Available from: http://www.apollomedicine.org/text.asp?2017/14/4/221/224730


  Introduction Top


A solid pseudopapillary tumor is a rare low-grade malignant cystic exocrine neoplasm of the pancreas of papillary architecture with a reported incidence of 0.13%–2.7% of all pancreatic tumors [1] and is pathologically distinctive from other types of pancreatic tumors. This tumor generally is asymptomatic or minimally symptomatic. As a low-grade malignancy, the tumor seldom recurs and metastasizes. Complete resection is curative in most cases, but prognostic factors are not well established, and clinical evolution cannot be predicted.


  Case Report Top


An 18-year-old young female presented with a history of abdominal distension, pain and generalized weakness. On examination, epigastric fullness was noticed. Abdominal ultrasound detected a pancreatic mass measuring 15 cm × 10 cm. Computed tomography (CT) scan showed evidence of a solid cystic lesion in the body and tail of the pancreas with well-defined outline. The mass was compressing and displacing the splenoportal axis. CT guided preoperative biopsy was suggestive of papillary neoplasm of pancreas.

A specimen of distal pancreatico – splenectomy was received for histopathology and showed large encapsulated, white to yellow, soft, hemorrhagic, friable tumor with cystic degeneration, and well-defined margins [Figure 1] measuring 16 cm × 11 cm × 7 cm. Microscopic examination [Figure 2]a and [Figure 2]b revealed a cellular tumor composed of pseudo papillae with hyalinized, delicate fibrovascular cores lined by bland uniform poorly cohesive epithelial cells with clear to eosinophilic cytoplasm, round to oval nuclei, occasional nuclear grooves, and indistinct nucleoli. Hyaline globules were seen. Immunohistochemistry revealed CD10, progesterone receptor and vimentin positivity. Ki-67 was 2%. Final diagnosis of solid pseudopapillary tumor (SPT) of pancreas was made.
Figure 1: Large encapsulated, white to yellow, soft, hemorrhagic, friable tumour with cystic degeneration and well defined margins

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Figure 2: (a and b) Microscopic examination revealed a cellular tumour composed of pseudo papillae with hyalinized, delicate fibrovascular cores lined by bland uniform poorly cohesive epithelial cells with clear to eosinophilic cytoplasm, round to oval nuclei, occasional nuclear grooves and indistinct nucleoli

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  Discussion Top


A solid pseudopapillary neoplasm (SPN), a lesion of uncertain cellular differentiation, is an unusual tumor of the pancreas with an indolent clinical course that typically arises in young females. It is a rare low-grade malignant neoplasm of the pancreas of papillary architecture with a reported incidence of 0.13%–2.7% of all pancreatic tumors.[1] The tumor has been referred to with multiple different names, including SPT of the pancreas, SPN, solid pseudopapillary epithelial neoplasm (SPEN), papillary cystic neoplasm of the pancreas, Hamoudi tumor, and Gruber-Frantz tumor (or just Frantz tumor). It occurs predominantly in young women around second and third decades of age. Although this tumor may occur anywhere within the pancreas, the pancreatic tail is the most common site of origin. The entity was first described in 1959 by pathologist Virginia Kneeland Frantz and in 1996 reclassified by the World Health Organization.[2],[3],[4]

In general, the diagnosis of SPT depends on the clinical and imaging features. Clinically, a slowly growing mass with or without vague abdominal pain may be the only patient's complaint and the blood tests are usually normal. These tumors may also be incidental findings during complementary studies, such as ultrasound and/or CT done for various reasons. The sonographic features of septations in the cystic portion are possibly due to prominent papillae projecting into the space of cystic degeneration.[5] CT shows a well-demarcated large encapsulated pancreatic mass that are frequently in tail. The architecture of the mass varies from solid, homogenous muscle density, through mixed solid and cystic, to thick walled cyst, and depending on the degree of hemorrhage and/or necrosis.[6] Calcifications and enhanced solid areas may be present at the periphery of the mass. Magnetic resonance imaging typically shows a well-defined lesion with a mix of high- and low-signal intensity on T1- and T2-weighted images. Since this type of cancer is so rare, the preoperative diagnosis of SPT is often missed.

With the application of new techniques, such as endoscopic ultrasound-guided fine-needle aspiration, the diagnosis of SPT has become easier and more accurate. Characteristic cytological features of SPTs include branching papillary fronds with sheets and cords of cells arranged around fibrovascular septa. Grossly, SPEN is a large well-encapsulated mass that usually demonstrates variable degrees of internal hemorrhage and cystic degeneration. These changes are secondary to the delicate, easily disrupted vascular network that traverses this tumor. Areas of hemorrhagic degeneration vary from solid friable areas to frankly cystic cavities. Microscopically, there is limited, if any, evidence of aggressive behavior consisting of capsular invasion, extension into adjacent parenchyma or vascular invasion. The neoplastic cells are small to medium in size, polygonal to somewhat elongated and slightly eosinophilic with small to medium sized ovoid nuclei. Mitotic figures are rare. Stains for mucin are negative. The cells contain eosinophilic granules rich in alpha-1-antitrypsin, and the nuclei are typically grooved. The immunophenotype is nonspecific with positive staining for vimentin, alpha-1-antitrypsin, neuron-specific enolase, CD10, and CD56. Keratins, chromogranin, synaptophysin, and endocrine and pancreatic enzymes are generally not expressed. SPTs often stain positive for progesterone receptors while estrogen receptor positivity is more variable. The genetic profile associated with SPT is different from pancreatic adenocarcinoma, most notably for an absence of KRAS and DPC4 mutations. SPTs are characterised by the presence of an activating B-catenin gene mutation that interferes with protein phosphorylation.

Differential considerations including nonfunctioning islet cell tumor, pancreatoblastoma, and complicated pseudocyst are less likely possibilities. Nonfunctioning islet cell tumor may also be incidentally identified as a large mass with local mass effect on adjacent structures. However, nonfunctioning islet cell tumors typically occur in an older patient population and are most commonly located in the head, neck, or uncinate process. Pancreatoblastoma is a rare pediatric carcinoma of the pancreas in which the majority arises in the head of the pancreas. Elevated alpha-fetoprotein may also be an indicator of pancreatoblastoma. This diagnosis is unlikely given the location and unremarkable laboratory findings. Finally, the diagnosis of complicated pseudocyst is also less likely in this young patient without a history of pancreatitis.

SPEN of the pancreas is treated surgically and complete resection of the mass is usually curative. Pancreaticoduodenectomy or distal pancreatectomy is performed most commonly, with en bloc resection of resection of involved adjacent organs when indicated. Complete margin-negative resection (R0) is associated with a long-term disease-free survival. About 10%–15% patients have metastases at the time of diagnosis or develop metastases at some point in the future.[7],[8],[9] The most common sites of metastases include liver, mesentry, and peritoneum. Five-year survival rate for patients with resectable SPT is 95%. Tumor size, as well as vascular, lymphatic, or perineural invasion, have not been useful in predicting recurrence. The presence of nuclear atypia and high-mitotic rate may suggest an aggressive subtype. Adjuvant systemic therapy is not routinely utilized due to excellent survival rates following surgical resection alone.


  Conclusion Top


A cystic pancreatic mass in a young adult female should raise suspicion for an SPT. The characteristic imaging features can help to make the correct diagnosis of SPT and differentiate from other pancreatic tumors. Although typically a nonaggressive neoplasm with surgery curative in most cases, SPT may exhibit more aggressive features such as local invasion, metastases or recurrence in up to 10%–15% of cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Crawford BE 2nd. Solid and papillary epithelial neoplasm of the pancreas, diagnosis by cytology. South Med J 1998;91:973-7.  Back to cited text no. 1
    
2.
Frantz VK. Tumors of the pancreas. In: Bumberg CW, editor. Atlas of Tumor Pathology. VII. Fascicles 27 and 28. Washington: Armed Forced Institute of Pathology; 1959. p. 32-3.  Back to cited text no. 2
    
3.
Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH; World Health Organization. Histological typing of tumours of the exocrine pancreas. In: World Health Organization International Histological Classification of Tumours. 2nd ed. Berlin: Springer; 1996. p. 8452-1.  Back to cited text no. 3
    
4.
Klöppel G, Hruban RH, Klimstra DS, Maitra A, Morohoshi T, Notohara K, et al. Solid-pseudopapillary tumor of pancreas. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. World Health Organization Classification of Tumours of the Digestive System. Lyon: IARC; 2010. p. 327-30.  Back to cited text no. 4
    
5.
Lin JT, Wang TH, Wei TC, Sheu JC, Sung JL, How SW, et al. Sonographic features of solid and papillary neoplasm of the pancreas. J Clin Ultrasound 1985;13:339-42.  Back to cited text no. 5
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6.
Friedman AC, Lichtenstein JE, Fishman EK, Oertel JE, Dachman AH, Siegelman SS, et al. Solid and papillary epithelial neoplasm of the pancreas. Radiology 1985;154:333-7.  Back to cited text no. 6
    
7.
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. 7
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8.
Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayashi I. Papillary cystic tumors of the pancreas. Assessment of their malignant potential. Cancer 1993;71:82-92.  Back to cited text no. 8
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9.
Pasquiou C, Scoazec JY, Gentil-Perret A, Taniere P, Ranchere-Vince D, Partensky C, et al. Solid pseudopapillary tumors of the pancreas. Pathology report of 13 cases. Gastroenterol Clin Biol 1999;23:207-14.  Back to cited text no. 9
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  [Figure 1], [Figure 2]



 

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