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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 271-273
Solid pseudopapillary tumor of the pancreas


1 Department of Pathology, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai - 400 008, Maharashatra, India
2 Department of Surgery, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai - 400 008, Maharashatra, India

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   Abstract 

Solid pseudopapillary tumor of the pancreas is considered to be a rare pancreatic tumor. These tumors are typically present in women in their third decade of life. The tumors have a low malignant potential. We report a case of 22-year-old female who presented with intermittent abdominal pain of 3 years duration. Distal pancreatectomy with splenectomy was done as a definitive treatment. The importance of accurate diagnosis and treatment is emphasized.

Keywords: Solid pseudopapillary tumor of pancreas, young females

How to cite this article:
Kamat RN, Naik LD, Joshi RM, Amrapurkar AD, Shetty T S. Solid pseudopapillary tumor of the pancreas. Indian J Pathol Microbiol 2008;51:271-3

How to cite this URL:
Kamat RN, Naik LD, Joshi RM, Amrapurkar AD, Shetty T S. Solid pseudopapillary tumor of the pancreas. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Oct 19];51:271-3. Available from: http://www.ijpmonline.org/text.asp?2008/51/2/271/41692



   Introduction Top


Since being reclassified by WHO in 1996, solid pseudopapillary tumors (SPTs) of pancreas are an internationally accepted entity. SPT are rare tumors and account for 1-2% of all primary tumors of pancreas. They are also called papillary cystic neoplasm, solid-cystic papillary tumor or Gruber-Frantz tumor. Most of these tumors are found in young women in the second or third decade. [1] Rarely, these tumors may be seen in children. [2] The most common clinical presentation is a palpable abdominal mass and an uncharacteristic abdominal pain. These tumors have a low malignant potential and their prognosis is extremely good unlike other tumors of the pancreas. [3],[4]


   Case History Top


A 22-year-old female patient presented in the Surgical Outpatient Department with history of abdominal pain of 3-year duration. The pain was intermittent and not associated with anorexia, nausea, vomiting, weight loss, or any other systemic complaints. The CT scan done 3 years ago had revealed a predominantly solid mass in the body of pancreas. CT-guided FNAC of the pancreatic mass was performed.

The smear was highly cellular and showed papillae and pseudorosettes composed of small uniform tumor cells adherent to delicate fibrovascular core. The blood vessels appeared hyalinized on MGG-stained smear background. Individual cells were uniform, round to plasmacytoid or cuboidal. Nuclei were round with grooves. Nuclear chromatin was finely granular. There was no evidence of nuclear irregularity, pleomorphism or increased mitotic figures. There was no evidence of necrosis or intracellular mucin. The impression was SPT of pancreas.

The patient refused surgery then. At the current presentation, the CT scan revealed a well-defined 5 × 4 × 4 cm, oval, solid mass in the body and tail of pancreas. There was no significant difference in size of tumor as compared to that seen on CT scan done 3 years ago. There was no evidence of locoregional or distant spread on CT scan. Exploratory laparotomy with a distal pancreatectomy and splenectomy was done.

On gross examination of the specimen a 5 × 4 × 4 cm globular, well-defined, predominantly solid mass was seen. Cut surface was greyish white, firm and solid without any areas of haemorrhage and necrosis. Surrounding pancreas, at both resection margins, appeared normal [Figure 1].

Histopathology

On histopathological examination, the tumor was very cellular and well-circumscribed without involvement of adjacent pancreas. Tumor cells were arranged in solid sheets and pseudopapillae. Individual tumor cells were uniform, small to medium sized, polygonal with acidophilic cytoplasm and bland vesicular, ovoid nuclei. Few nuclei showed indentation and indistinct nucleoli. Mitotic figures were few [Figure 2] and [Figure 3]. No vascular invasion was seen. The surrounding stroma was fibrocollagenous with deposition of few cholesterol crystals. With these classical histological features, final diagnosis of SPT of pancreas was made.


   Discussion Top


Solid pseudopapillary tumors of the pancreas are predominantly seen in young women. These are slow-growing tumors with indolent course as was seen in our case. These tumors are commonly located in the body and tail of pancreas.

Most of the SPTs are large with solid and cystic areas. These tumors begin as solid masses in which there are many poorly supported tiny vessels and then the cells farthest from the small vessels undergo swelling and degenerative change, whereas the cells next to the vessels remain intact. This results in a pseudopapillary pattern and cystic spaces. [5] The present case showed only solid tumor without any cystic areas.

The differential diagnosis of SPT is an endocrine neoplasm, cystic tumor of the pancreas and pancreatic pseudocyst and pancreatoblastoma in children. [6] At the time of aspirate, USG and CT scan did not reveal any cystic areas. The aspirate obtained was blood mixed, cellular, without evidence of fluid. Hence, the possibility of other cystic neoplasms of the pancreas was not considered. In this case, the presence of papillae with uniform cell population, the lack of speckled pattern of chromatin and the absence of red cytoplasmic granularity helped us to rule out the possibility of endocrine neoplasm such as islet cell tumor. Lack of nuclear membrane irregularity, hyperchromatic nuclei with coarse chromatin and absence of necrosis ruled out the possibility of low-grade papillary adenocarcinoma of pancreas. In addition, the young age of the patient does not favour the diagnosis of adenocarcinoma. The possibility of pancreatoblastoma was not considered because these tumors are mainly seen in childhood. Histologically, these tumors show uniform epithelial cells arranged in solid sheets, nests and well-formed acinar structures with presence of squamoid corpuscles, which was not seen in our case. The presence of cystic pancreatic mass in the absence of pancreatitis must be regarded with great suspicion and investigated thoroughly. [3] The origin of this tumor remains enigmatic. Some authors have reported that it is probably a neoplasm of uncommitted cells with most cells similar to intercalated duct cells or centroacinar cells. [7] Its infiltrative tendencies can trap acinar or islet cells within the tumor, so such cells should not be regarded as evidence of specific differentiation by the neoplasm.

Nishihara et al [8] , have reported that venous invasion, diffuse growth pattern, extensive tumor necrosis, significant nuclear atypia and high mitotic rate are indicative of aggressive behaviour.

Immunohistochemically, the SPTs show positive reactivity for keratin, desmoplakin, trypsin, chymotrypsin, amylase and vimentin. In addition, focal positivity has been found for neuron-specific enolase and various islet cell hormones such as insulin and glucagons. This might suggest that SPT of the pancreas arises from primitive pancreatic epithelial cells with predominance of exocrine features but having capacity for dual (endocrine and exocrine) differentiation. The presence of progesterone receptors and its well-known predilection for females suggest that it is a hormone-dependent tumor. [9]

Solid pseudopapillary tumors show 15% metastatic rate involving liver and peritoneum. Even in the presence of disseminated disease, the clinical course is usually protracted and overall 5-year survival rate is 97%. [10] The treatment of SPT is surgical. Overall, prognosis is good after limited resections because of its indolent growth.

In conclusion, SPT of the pancreas is a rare pancreatic tumor seen commonly in young women and it is very important to accurately diagnose this tumor, as surgical resection is curative.

 
   References Top

1.Ulusan S, Bal N, Kizilkilic O, Bolat F, Yildirim S, Yildirim T, et al. Solid pseudopapillary tumor of the pancreas associated with dorsal agenesis. Br J Radiol 2005;78:441-3.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Rebhandl W, Felberbauer FX, Puig S, Paya K, Hochschorner S, Barlan M, et al. Solid pseudopapillary tumor of the pancreas (Frantz tumor) in children: Report of four cases and review of literature. J Surg Oncol 2001;76:289-96.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Vincent PJ, Nagpal BM, Singh P, Harjan MM, Nagi GS, Satyanarayan S. Gruber - Frantz tumor. (Medical J Armed Forces India) MJAFI 2003;59:355-7.  Back to cited text no. 3    
4.Gupta ML, Ohri A, Singh J, Singla S, Singh D. Solid pseudopapillary tumor of the pancreas: A case report. Indian J Med Pediatr Oncol 2004;25:42-3.  Back to cited text no. 4    
5.Friendman AC, Lichtenstein JE, Fishman EK, Oertel JE, Dachman AH, Siegelman SS. Solid and papillary epithelial neoplasm of the pancreas: Solid and papillary epithelial neoplasm of the pancreas. Radiology 1985;154:333-7.  Back to cited text no. 5    
6.Kissane JM. Pancreatoblastoma and solid and cystic papillary tumor: Two tumors related to pancreatic ontogeny. Semin Diagn Pathol 1994;11:152-64.  Back to cited text no. 6  [PUBMED]  
7.Pettinato G, Manivel JC, Ravetto C, Terracciano LM, Gould EW, di Tuoro A, et al. Papillary cystic tumor of the pancreas: A clinicopathologic study of 20 cases with cytologic, immunohistochemical, ultrastructural and flow cytometric observations and a review of the literature. Am J Clin Pathol 1992;98:478-88.  Back to cited text no. 7  [PUBMED]  
8.Nishihara K, Nagoshi M, Tsuneyoshi M. Papillary cystic tumor of pancreas: Assessment of their malignant potential. Cancer 1993;71:82-92.  Back to cited text no. 8    
9.Juan R. Ackerman's surgical pathology. 8 th ed. USA: Mosby; 1996. p. 988-9.  Back to cited text no. 9    
10.Tang LH, Aydin H, Brennan MF, Klinstra DS. Clinically aggressive solid pseudopapillary tumor of pancreas: A report of cases with components of undifferentiated carcinoma and a comparative clinicopathologic anlaysis of 34 conventional cases. Am J Surg Pathol 2005;29:51-2.  Back to cited text no. 10    

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Correspondence Address:
Rima N Kamat
F/123, Tarapore Gardens, Off. Andheri Link Road, Andheri (West), Mumbai - 400 053, Maharashatra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.41692

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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    Abstract
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