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CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 3  |  Page : 215-217

Solid pseudopapillary carcinoma of pancreas: A rare case report


Department of Surgery, Kurnool Medical College, Government General Hospital, Kurnool, Andhra Pradesh, India

Date of Web Publication10-Oct-2016

Correspondence Address:
Vudutha Srihari
H. No. 46-1M-1-4, Aditya Nagar, A-Camp, Kurnool, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.191845

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  Abstract 

A 20-year-old female presented with abdominal pain and loss of weight of 3 months duration, initially diagnosed as acid peptic disorder ultra sound examination revealed pancreatic mass and contrast-enhanced computed tomography abdomen confirmed distal pancreatic mass, which was excised and sent for histopathological examination, which revealed solid pseudopapillay tumor of pancreas. Solid pseudopapillary tumors most commonly occur in young women. Till recently the tumors are misclassified as nonfunctioning islet cell tumors or solid cystic tumor of pancreas or papillary cystadeno carcionoma. The tumors are well-encapsulated and cut surfaces show solid and hemorrhagic necrotic patterns. The neoplasms usually behave like a very low grade malignancy; hence complete surgical removal is treatment of choice.

Keywords: Good prognosis, rare exocrine pancreatic tumor, young females


How to cite this article:
Srihari V, Jayaram J, Baleswari G, Kumar M N. Solid pseudopapillary carcinoma of pancreas: A rare case report. J NTR Univ Health Sci 2016;5:215-7

How to cite this URL:
Srihari V, Jayaram J, Baleswari G, Kumar M N. Solid pseudopapillary carcinoma of pancreas: A rare case report. J NTR Univ Health Sci [serial online] 2016 [cited 2019 Dec 16];5:215-7. Available from: http://www.jdrntruhs.org/text.asp?2016/5/3/215/191845


  Introduction Top


Solid pseudo papillary tumor of pancreas accounts for 1-2% of exocrine pancreatic tumors.[1] This uncommon tumor of pancreas is found mainly in Caucasian and Afro-Asian women between second and third decades of life and rare cases have been reported in men and children.[2] Only 15% of these tumors are malignant with vascular and perineural invasion.


  Case Report Top


A house wife from Kallur of Kurnool district, presented with complaints of upper abdominal pain, loss of appetite and loss of weight for 3 months. On examination, there was tenderness in epigastric and left hypochondriac region with no palpable mass. She was taking treatment for acid peptic disorder for the past 3 months and was investigated in our hospital. Ultrasound examination of abdomen revealed intra-abdominal solid soft tissue mass in left hypochondrium? Splenic/pancreatic mass. Contrast-enhanced computed tomography (CECT) abdomen was done to find out the site of origin and it denoted well-defined heterogeneously enhancing mass with areas of necrosis seen in the tail of pancreas pushing splenic hilum laterally and splenic flexure of colon medially probably a pancreatic mass from tail of pancreas [Figure 1] and [Figure 2].
Figure 1: Contrast-enhanced computed tomography abdomen showing tumor arising from distal pancreas

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Figure 2: Well-defined heterogeneously enhancing mass with areas of necrosis seen in the tail of pancreas pushing splenic hilum laterally and splenic flexure of colon medially

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The renal and liver function tests were within normal limits, except for mild anemia and other hematological tests were normal. After preoperative assessment, we have clinically and radiologically confirmed that there is a pancreatic mass arising from the tail of pancreas, with engulfment of the splenic hilum. With the above findings exploratory laparotomy was planned and prepared the patient accordingly.

Exploratory laparotomy was done and lesser sac opened by dividing the gastro colic omentum, we found that the mass is arising from the distal pancreas engulfing the splenic hilum [Figure 3]. Splenic attachments were released and distal pancreatic transaction was done 5 cm away from the tumor margin and splenic hilum identified vascular pedicles ligated separately and splenectomy done, hemostasis secured. Intra- and post-operative period was uneventful. Pneumococcal vaccination and two units of fresh blood was given in the early postoperative period. Patient recovered well and was discharged after 2 weeks of surgery.
Figure 3: Intra-operative photo showing the tumor arising from distal pancreas

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Gross view-growth of size 9 cm × 7 cm × 3 cm, incompletely capsulated with lobulated surface, shows grey white friable tumor tissue with areas of necrosis and cystic change [Figure 4].
Figure 4: Gross view — Growth of size 9 cm × 7 cm × 3 cm capsulated mass. C/s shows grey white friable tumor tissue with areas of necrosis and cystic change

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The histopathological examination of tumor mass showed small uniform tumor cells with round to oval nuclei and indistinct nucleoli. Cells arranged around hyalinized blood vessels in one to several layers, appearing as pseudo papillae with myxoid change in some blood vessels [Figure 5] and [Figure 6]. Features suggestive of solid pseudo papillary tumor, cystic-solid papillary carcinoma of distal pancreas.
Figure 5: Pseudopapillae

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Figure 6: Extensive myxoid change

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  Discussion Top


Solid pseusudopapillary tumors have many synonyms in literature namely Frantz tumor, solid cystic tumor of pancreas and papillary cystic neoplasm of pancreas.

Patients with solid pseudo papillary tumors are asymptomatic and they may present with a gradually enlarging abdominal mass or vague abdominal pain or discomfort. The abdomen is nontender on palpation but obstructive symptoms may occur if tumor grows large enough to compress adjacent viscera.

Usually, there are no abnormalities in biochemical and serological tests the diagnosis is usually made by ultrasound examination and CECT of abdomen done for other reasons.[3]

On gross examination, the mass is usually large (>8 cm), well-encapsulated and contains varying amounts of necrosis, hemorrhage, and cystic degeneration. At microscopic analysis, there are two distinct cellular elements of solid and papillary pattern. The hall mark histological pattern occurs when the tumor cells form papillary configurations composed of a fibrovascular stalk surrounded by several layers of epithelial cells. Solid areas containing foamy macrophages, cholesterol granulomas and calcification also be seen.

Solid pseudo papillary tumors are typically positive for vimentin, neuron specific enolase, alfa-1 antitrypsin and alfa-1 antichymotrypsin and negative for chromogranin epithelial membrane antigen and cytokeratin.[4] On the basis of some similarities between solid pseudo papillary tumors and ovarian surface cells and the proximity between genital ridges and pancreas anlage during early embryogenesis, pseudo papillary tumors might originate from the genital ridge related cells that where incorporated into the pancreas during organogenesis.[5]

This explains the occurrence of those tumors predominantly in young females.[6]


  Conclusion Top


A high index of suspicion is necessary to suspect and diagnose solid pseudo papillary tumors. This diagnosis should be borne in mind when young female patients present with upper abdominal mass. CT scan and endoscopic ultra sound examination are valuable pointers to the preoperative diagnosis. Surgical excision offers best chance for cure and should always be attempted irrespective of the magnitude of resection involved. Patients with solid pseudo papillary tumors have an excellent prognosis after surgical excision.

 
  References Top

1.
Franz VK. Tumors of the pancreas. Atlas of Tumor Pathology. Fasc 27-28, Ser 7. Washington DC: Armed Forces Institute of Pathology; 1959. p. 32-3.  Back to cited text no. 1
    
2.
Mao C, Guvendi M, Domenico DR, Kim K, Thomford NR, Howard JM. 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.  Back to cited text no. 2
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3.
Coleman KM, Doherty MC, Bigler SA. Solid-pseudopapillary tumor of the pancreas. Radiographics 2003;23:1644-8.  Back to cited text no. 3
[PUBMED]    
4.
Kloppel G, Solcia E, Longneccker DS, Capella C, Sobin LH. World Health Organization Institutional Histological Classification of Tumors — Histological Typing of Tumours of the Exocrine Pancreas. 2nd ed. Berlin, Germany: Springer-Verlag; 1996.  Back to cited text no. 4
    
5.
Kosmahl M, Seada LS, Jänig U, Harms D, Klöppel G. Solid-pseudopapillary tumor of the pancreas: Its origin revisited. Virchows Arch 2000;436:473-80.  Back to cited text no. 5
    
6.
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. 6
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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Abstract
Introduction
Case Report
Discussion
Conclusion
References
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