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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 152-155
Malignant solid pseudopapillary tumor of pancreas causing sinistral portal hypertension


1 Department of Radiodiagnosis and Imaging, Sher-I- Kashmir institute of medical sciences (SKIMS), Srinagar, Jammu and Kashmir, India
2 Department of Gastroenterology, Sher-I- Kashmir institute of medical sciences (SKIMS), Srinagar, Jammu and Kashmir, India
3 Department of General Surgery, Sher-I- Kashmir institute of medical sciences (SKIMS), Srinagar, Jammu and Kashmir, India
4 Department of Pathology, Sher-I- Kashmir institute of medical sciences (SKIMS), Srinagar, Jammu and Kashmir, India

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Date of Web Publication7-Mar-2011
 

   Abstract 

Solid pseudopapillary tumor (SPT) of the pancreas is a rare benign or low-grade malignant epithelial tumor that occurs mainly in young females in second to fourth decades of life. Pathologic and imaging findings include a well-defined, encapsulated pancreatic mass with cystic and solid components with evidence of hemorrhage. We report a 23-year-old female who presented with upper abdominal pain of long duration and epigastric mass on palpation. Multidetector-row CT (MDCT) demonstrated a large well-defined heterogeneous attenuation mass, containing hyperdense areas of hemorrhage mixed with solid enhancing and cystic non-enhancing areas, arising from the pancreatic body and tail. Splenic vein thrombosis was present with dilated splenoportal collateral vessels between splenic hilum and portal/superior mesenteric veins, with dilated vessels seen in the gastric wall, with patent portal vein, compatible with sinistral portal hypertension. Typical imaging features and age and sex of the patient suggested a diagnosis of SPT of pancreas complicated by segmental portal hypertension due to splenic vein thrombosis. Histopathology of the biopsy material was confirmatory.

Keywords: CT, malignant, pancreas, portal hypertension, psueodopapillary tumor

How to cite this article:
Wani NA, Lone TK, Shah AI, Khan AQ, Malik RA. Malignant solid pseudopapillary tumor of pancreas causing sinistral portal hypertension. Indian J Pathol Microbiol 2011;54:152-5

How to cite this URL:
Wani NA, Lone TK, Shah AI, Khan AQ, Malik RA. Malignant solid pseudopapillary tumor of pancreas causing sinistral portal hypertension. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Dec 14];54:152-5. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/152/77382



   Introduction Top


Solid pseudopapillary tumor (SPT) is an uncommon neoplasm that mainly occurs in women in the second to fourth decades of life. It is characterized by low potential for malignancy and a favorable prognosis. Since Franz [1] described this tumor in 1959 as a "papillary tumor of the pancreas, benign or malignant," the number of reported cases has increased. Synonyms include solid and cystic tumor, solid and papillary epithelial neoplasm, papillary-cystic neoplasm, papillary cystic epithelial neoplasm, papillary-cystic tumor, and Franz tumor. [1] In 1996, the World Health Organization renamed this tumor SPT for the international histologic classification of tumors of the exocrine pancreas. [1] Pathologic examination reveals that SPT is usually a large, encapsulated mass composed of a mixture of cystic, solid, and hemorrhagic components. Both a capsule and intratumoral hemorrhage are important clues to the diagnosis because these features are rarely found in other pancreatic neoplasms. [1] Deep pancreatic parenchymal invasion and angioinvasion are features of malignant SPT also called as solid pseudopapillary carcinoma. [2] Isolated splenic vein thrombosis due to adjacent pancreatic tail mass may result in sinistral portal hypertension and is reported with pancreatic adenocarcinoma, cystadenoma, and islet cell tumors. [3] Ours is the first reported case of SPT of pancreas with sinistral portal hypertension.


   Case Report Top


A 23-year-old woman presented to the emergency department because of increasing upper abdominal pain and swelling for the last 1 month. She had a long history of vague upper abdominal discomfort and dyspepsia for the last 3 years. On examination there was a large epigastric mass on palpation. All laboratory parameters including serum amylase and liver funtion tests were normal. Carcinoembryogenic antigen (CEA), alpha-fetoprotein (AFP), and carbohydrate antigen (CA) 19-9 were all in the normal range. Ultrasound of the abdomen showed a large well-defined mixed solid-cystic mass. A multidetector CT (MDCT) study of abdomen was performed next to characterize the mass. A large well-defined mass measuring 20 cm × 15 cm in size was seen closely abutting pancreatic body and tail [Figure 1]. Non-contrast CT showed multiple areas of hyperdense (blood) attenuation within the mass, which showed mixed soft tissue and fluid attenuation. A dynamic contrast enhanced scan showed enhancing solid areas in the periphery of the mass and non-enhancing central cystic component. The venous phase of the study showed hypodense thrombus within the mid portion of the splenic vein with non-visualization of the vein beyond this level [Figure 2]. There was no plane of separation between the mass and the splenic vein compatible with splenic vein invasion. Spleen was mildly enlarged, there were no liver metastasis or enlarged lymph nodes. Superior mesenteric vein (SMV) and portal vein (PV) were normally outlined by the contrast. Multiple collateral vessels were seen extending from the splenic hilum and extending along gastric and colonic walls and communicating with patent SMV and PV. Prominent vessels were also seen in the wall of the stomach [Figure 3]. Esophagogastroduodenoscopy showed varices in the fundus of stomach, there were no esophageal varices. CT imaging findings suggested a possible diagnosis of SPT of pancreas with isolated splenic vein thrombosis resulting in sinistral portal hypertension.
Figure 1: Axial non-contrast CT image showing a large well-defined mass in the region of pancreatic tail, with solid (upward arrow) and cystic areas (downward arrow). More hyperdense areas (hemorrhages) are seen in the center of the solid-cystic mass (leftward arrow)

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Figure 2: Axial postcontrast CT image showing a large well-defined pancreatic body and tail mass having enhancing solid and nonenhancing cystic areas. Splenic vein posterior to the mass shows thrombus (upward arrow), PV is normal. Enlarged collateral vessels are seen in the splenic hilum and in the mesentry (downward arrows)

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Figure 3: Axial post contrast CT image at the level of stomach shows enlarged vessels in the wall of the fundus of stomach compatible with gastric varices (downward arrows)

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Histopathology

A percutaneous biopsy of the mass was performed under ultrasound guidance for histopathologic confirmation. Microscopic examination revealed solid areas alternating with pseudopapillary growth pattern and cystic spaces with cords of monomorphous small to medium-sized cells forming the solid areas [Figure 4]. Cells contained eosinophilic and vacuolated cytoplasm around ovoid nucleus. Some mitotic figures were seen but no significant nuclear atypia was present. Imaging and histopathologic findings were diagnostic of malignant SPT of pancreas with splenic vein invasion causing sinistral portal hypertension. At exploratory laparotomy pancreatic mass was seen invading splenic vein; mass was resected and splenectomy was done. Gross examination showed a huge largely encapsulated mass measuring 20 × 15 cm which on cut section revealed solid areas along with hemorrhages and necrotic zones.
Figure 4: Microscopic picture of the tumor showing solid and pseudopapillary pattern formed by uniform small epithelial cells (H and E, ×200)

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   Discussion Top


SPT of the pancreas is a rare exocrine pancreatic tumor, which comprises only 1-2% of all tumors of the pancreas, first described by Frantz in 1959. [1] This uncommon, typically benign tumor seems to have a predilection for Asian and African-American women, although rare cases have been reported in children and men. Patients with SPT of the pancreas are often clinically asymptomatic or present with a gradually enlarging abdominal mass. [2],[4] Although most SPTs exhibit benign behavior, malignant degeneration does occur in about 15% of cases evidencing as metastases or invasion of adjacent structures; malignant tumors have a male predilection. According to the WHO classification scheme, SPTs with clear criteria of malignancy (vascular and nerve sheath invasion or lymph node and liver metastases) are designated as solid-pseudopapillary carcinomas. [2],[4]

The splenic vein running along the posterosuperior aspect of pancreas is vulnerable to compression or infiltration by adjacent pancreatic lesions. This results in isolated splenic vein thrombosis and an unusual form of extrahepatic portal hypertension confined to the gastrosplenic side of the portal venous circulation. [3] Esophageal varices may result when short gastric veins cannot cope with shunted splenic circulation or when the coronary vein drains into the splenic vein rather than the PV. Thus an extensive collateral circulation develops involving the segment of stomach drained by the short gastric vein. This is called as left-sided, sinistral and segmental portal hypertension which is characterized by gastric (fundal) varices, normal liver function and patent PV. [5] Sinistral portal hypertension presenting as gastrointestinal bleeding has been reported with pancreatic adenocarcinoma, cystadenoma and islet cell tumor of pancreas. [3],[5] Though our patient had documented sinistral portal hypertension, she did not report any incident of hematemesis or melena.

Because of the low-grade malignant potential and good prognosis after complete resection of the tumor, it is important to make a correct diagnosis before operation. Up till now, imaging studies or an imaging-guided fine needle aspiration biopsy is the cardinal choice for preoperative diagnosis. Ultrasonography, CT and MRI reflect the gross morphology and internal structure of the tumor as a well-encapsulated, homogeneous or heterogeneous mass composed of solid cystic components. [6],[7],[8] Splenic vein thrombosis and or nonvisualization of splenic vein with splenoportal and gastrosplenic collaterals help in making the diagnosis of sinistral portal hypertension on CT in the presence of patent PV and normal liver function. Esophagogastroduodenoscopy helps in confirming the presence of gastric varices due to left-sided portal hypertension. [3]

The pathologic diagnosis of SPT is mainly based on the well-defined solid and cystic structure and characteristic pseudopapillary features under the microscope. [4],[9] On the cut surface, a variegated appearance is evident with variable combinations of solid hemorrhagic and cystic-necrotic areas. The microscopic features of SPT are solid areas which alternate with a pseudopapillary pattern composed of a fibrovascular stalk surrounded by several layers of epithelial cells. Immunohistochemical studies are frequently performed to confirm the diagnosis. SPT is typically positive for vimentin and antitrypsin and is negative for trypsin and chymotrypsin. They may also show focal immunoreactivity for neuron-specific enolase (NSE) and cytokeratin. [4]

Differential diagnosis on imaging is with microcystic adenoma, mucinous cystic neoplasm, nonfunctioning islet cell tumor, pancreatic carcinoma, pancreatoblastoma, and calcified hemorrhagic pseudocyst. [4] In the appropriate clinical settings imaging features can be highly suggestive for the pathologic diagnosis of SPT. This tumor should be considered when a well-marginated, large, encapsulated, solid and cystic mass with areas of hemorrhagic degeneration is detected in the pancreas of a young woman. [8] However, biopsy helps in confirming the diagnosis as the microscopy and immunohistochemical patterns are distinct and characteristic in the case of SPT.

Because the tumor is usually surrounded by a pseudo-capsule and exhibits benign or low-grade malignancy, conservative resection with preservation of as much pancreatic tissue as possible is the treatment of choice. [9],[10] Malignant SPT designated as a solid-pseudopapillary carcinoma occurs in 15% of adults and 13% of children with a significant increase in the elderly and male patients. According to the WHO classification system, the criteria for distinguishing malignant form of the tumor "SPT carcinoma" comprise angioinvasion, perineural invasion, and deep invasion of the surrounding pancreatic parenchyma and metastasis. As for metastases, the most common site is the liver; lymph node and peritoneal spread are rarely reported. [4],[9],[10] As for the treatment, splenectomy with distal pancreatectomy may be preferred in the presence of splenic vein thrombosis with sinistral portal hypertension to eliminate the risk of bleeding from the gastric varices. [3] Adjuvant therapy is used only in a small number of patients because of the high resectability of SPT. [4],[9],[10]

In conclusion, imaging findings of SPT of the pancreas are characteristic, and suggest the histopathologic diagnosis which can be confirmed by a biopsy. CT also helps in the diagnosis of sinistral portal hypertension, which may be a rare occurrence with malignant SPT.

 
   References Top

1.Coleman KM, Doherty MC, Bigler SA. Solid pseudopapillary tumor of the pancreas. Radiographics 2003;23:1644-8.  Back to cited text no. 1
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2.Sun CD, Lee WJ, Choi JS, Oh JT, Choi SH. Solid-pseudopapillary tumours of the pancreas: 14 years experience. ANZ J Surg 2005;75:684-9.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Thompson RJ, Taylor MA, McKie LD, Diamond T. Sinistral portal hypertension. Ulster Med J 2006;75:175-7.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Casadei R, Santini D, Calculli L, Pezzilli R, Zanini N, Minni F. Pancreatic Solid-Cystic Papillary Tumor: Clinical Features, Imaging Findings and Operative Management. JOP 2006;7:137-44.  Back to cited text no. 4
    
5.Singhal D, Kakodkar R, Soin AS, Gupta S, Nundy S. Sinistral portal hypertension. A case report JOP 2006;7:670-3.  Back to cited text no. 5
    
6.Lee DH, Yi BH, Lim JW, Ko YT. Sonographic findings of solid and papillary epithelial neoplasm of the pancreas. J Ultrasound Med 2001;20:1229-32.  Back to cited text no. 6
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7.Miao F, Zhan Y, Wang XY, Wang DB, Chen KM, Tang AR, et al. CT manifestations and features of solid cystic tumors of the pancreas. Hepatobiliary Pancreat Dis Int 2002;1:465-8.  Back to cited text no. 7
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8.Dong DJ, Zhang SZ. Solid-pseudopapillary tumor of the pancreas: CT and MRI features of 3 cases. Hepatobiliary Pancreat Dis Int 2006;5:300-4.  Back to cited text no. 8
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9.Zhang H, Liang TB, Wang WL, Shen Y, Ren GP, Zheng SS. Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas. Hepatobiliary Pancreat Dis Int 2006;5:454-8.  Back to cited text no. 9
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10.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. 10
    

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Correspondence Address:
Nisar Ahmad Wani
Department of Radiology, Sher-I-Kashmir Institute of Medical sciences, Srinagar, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.77382

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    Figures

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

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