Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 4  |  Page : 478-481  

Solid pseudo papillary neoplasm of the pancreas: A diagnostic dilemma


Department of Pathology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, India

Date of Web Publication17-Sep-2013

Correspondence Address:
Arpana Dharwadkar
Department of Pathology, Pad Dr. D Y Patil Medical College, Pimpri, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.118299

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  Abstract 

Solid cystic tumor of the pancreas (SCPT), also known as Frantz tumor is a rare pancreatic tumor with an incidence less than 2% of all non-endocrine pancreatic tumors. Young women are more often affected than men. We report the case of a 28-year-old female who presented with vague abdominal pain of two-month duration. Ultrasonography (USG) and computed tomography (CT) scan revealed a large cystic mass which was seen to be arising from the tail of the pancreas and attached to the mesentery. Distal pancreatectomy was done, which on histopathological examination was diagnosed as SCPT. Due to its rareness and behavior, this tumor is often associated with diagnostic and management problems.

Keywords: Low-grade malignant neoplasm, pseudopapillary neoplasm, solid cystic tumor of the pancreas


How to cite this article:
Dharwadkar A, Panicker NK, Chaudhari U, Kumar H. Solid pseudo papillary neoplasm of the pancreas: A diagnostic dilemma. Med J DY Patil Univ 2013;6:478-81

How to cite this URL:
Dharwadkar A, Panicker NK, Chaudhari U, Kumar H. Solid pseudo papillary neoplasm of the pancreas: A diagnostic dilemma. Med J DY Patil Univ [serial online] 2013 [cited 2024 Mar 28];6:478-81. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2013/6/4/478/118299


  Introduction Top


Solid pseudopapillary neoplasm is a rare pancreatic neoplasm commonly presenting in young women. [1],[2] The patients may be asymptomatic or may present with vague abdominal discomfort. The tumor can be misdiagnosed due to its cystic nature. Due to the frequent lack of specific clinical and laboratory signs and the overlap of imaging findings between different cystic tumors and between non-neoplastic and neoplastic cystic lesions of the pancreas, the management of these patients is complex. [2]

The objective of this case report is to highlight the diagnostic dilemma we faced, as this tumor is amenable to complete cure after resection.


  Case Report Top


A 28-year-old female presented at our institute with complaints of vague abdominal pain which had persisted for two months. There was no history of vomiting, loss of weight, jaundice, altered bowel/bladder habits, or any other major medical or surgical illness in the past. She had delivered her second child four months back. It was a full-term normal delivery.

Per abdominal examination revealed a mobile and tender mass in the left lumbar region. General examination was unremarkable. The results of hematological and biochemical studies were unremarkable apart from a slightly elevated white cell count. The USG showed a mass measuring 9.0×7.1×7.0 cm in the left lumbar region, probably located in the transverse mesocolon close to the splenic flexure with a thick uniform wall with a smooth surface.

Computed tomography (CT) scan of the abdomen revealed a well-defined rounded mass with heterogeneous density seen in the pancreatic tail region involving the adjacent mesentery [Figure 1]. The lesion measured 8.5×9×9 cm. The findings were thought to represent a rather benign-looking lesion. Hence, a provisional diagnosis of mesenteric desmoid tumor or papillary cyst adenoma of the pancreas was considered.
Figure 1: Computed tomography scan showing a well-defined, rounded mass with heterogeneous density in the pancreatic tail region involving the adjacent mesentery which appeared well capsulated; central hyperdense and nonenhancing hypodense components were seen within it

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Exploratory laparotomy was done. Peri-operatively, a cystic mass was found to be arising from the tail of the pancreas. A spleen-preserving distal pancreatectomy was done.

On gross examination, the mass was well circumscribed, measuring 8×5×5 cm. It weighed 200 g, and was apparently well encapsulated with grayish color. The cut surface showed a large cystic area with hemorrhage and a peripheral rim of a grayish white solid tumor [Figure 2].
Figure 2: Cut surface showing cystic area with hemorrhage and peripheral rim of a solid tumor

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Histological sections showed a large cystic area with extensive hemorrhage surrounded by a fibrocollagenous capsule [Figure 3]. Solid areas showed pseudopapillae covered by a layer of epithelial cells arranged in an organoid pattern [Figure 4]. The tumor cells had round-to-oval grooved nuclei with finely dispersed nuclear chromatin and moderate eosinophilic granular cytoplasm. A few mucinous areas around the vascular septae were noted [Figure 5]. The lymph nodes were free of tumor deposits. Immunohistochemistry (IHC) was positive for progesterone receptor (PR) [Figure 6] with focal positivity for vimentin and cytokeratin. It was negative for chromogranin and synaptophysin.
Figure 3: Cystically dilated areas of hemorrhage with pseudopapillae (hematoxylin and eosin stain, 10×)

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Figure 4: Organoid arrangement of the cells with grooved nuclei (hematoxylin and eosin stain, 40×)

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Figure 5: Myxoid areas

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Figure 6: Immunohistochemistry showing progesterone receptor positivity

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Hence, the diagnosis of solid cystic tumor of the pancreas (SCPT) was made.


  Discussion Top


SCPT, also known as Frantz tumor has been designated with various names over time. [3] But the term accepted by the World Health Organization is solid pseudopapillary neoplasm. [1]

SCPT is a rare tumor with an incidence less than 2% of all non-endocrine neoplasms of the pancreas. It is commonly seen in young women; [1] however, sporadic cases in men are also noted. The tumor is mainly localized in the tail of the pancreas, but cases where it is present in the head and body are also known. [4],[5],[6] It can also be present in an ectopic pancreas. [7] The patients may be asymptomatic or may present with vague abdominal discomfort. Rarely, patients may present with acute pancreatitis. [5] As these tumors are very vascular, they may lead to intraoperative complications such as hemo peritoneum and intra-operative hemorrhage. [8]

Grossly, the tumor is well circumscribed, roughly spherical, and of an average size of 8-10 cm. [2],[9],[10] The cut surface shows large spongy areas of hemorrhage, alternating with both solid and cystic areas. Fine needle aspiration cytology (FNAC) findings are characterized by highly cellular smears showing numerous papillary fragments with slender branching fibrovascular stalks. The tumor cells form two or several layers with a fibrovascular core. Pseudorosette formation maybe present. [8]

Histological appearance is distinctive and diagnostic. The solid areas show a cellular epithelial tumor, hypervascular, but not forming true glandular structures. Areas of degeneration develop into pseudopapillary structures. The cells have round-to-oval grooved nuclei with finely dispersed nuclear chromatin and eosinophilic granular cytoplasm. Mucinous change is also seen in some of the cases. Fibrovascular septae with myxoid or mucinous areas around blood vessels are of diagnostic value and are invariably seen in some areas, if searched for. Mitosis is infrequent. Periodic acid-Schiff (PAS)-positive diastase-resistant hyaline globules may be seen. A collection of foamy macrophages may be seen in degenerate areas. The lymph nodes are generally free of tumor. [1],[8],[10] All these features were observed in our case. Capsular invasion is known to be present in some cases but was not seen in our case.

Origin of the tumor is still controversial. The tumor cells are strongly positive for progesterone receptors, vimentin, alpha 1 antitrypsin, and neuron-specific enolase, whereas they are negative for chromogranin, synaptophysin, and alpha 1 chymotrypsin. [1],[4],[10] It can be assumed that hormones that play an important role in growth and pregnancy exert a powerful augmentative stimulus through the secretion of progesterone as in our case. [11]

The tumor should be differentiated from mucinous cystic tumor, microcystic adenoma, cystadenocarcinoma, and hemangioma. It can also be confused with carcinoma of the pancreas especially with the papillary cystic variant of acinar cell carcinoma. In children, differential diagnosis of pancreatoblastoma should be considered. [4],[9],[12]

The tumor has a low malignant potential. Fifteen percent cases show recurrence. Cases where the tumor has recurred in the regional lymph nodes, liver, and peritoneum are documented. The tumor may metastasize to the liver, omentum, lymph nodes, and spleen. Prognosis is excellent. [2],[4],[10]


  Conclusion Top


Though SCPT is a rare neoplasm, its possibility should be considered in young females with a well-defined cystic abdominal mass. Histopathological examination is characteristic and diagnostic. Surgery is curative with excellent prognosis. Close follow-up is advisable, especially when histopathology is suggestive of an aggressive tumor.

 
  References Top

1.Sternberg S. Diagnostic Surgical Pathology. In: Millis S, Carter D, Grenson J, Reuter V, Stoler M, editors. Pancreas. 5 th ed, Philadelphia: Lippincott Williams and Wilkins; 2010. p. 1469-71.  Back to cited text no. 1
    
2.Fulvio F, Procaccini E, Roberto R, Antropoli M, Manganiello A, Nunziata L, et al. Solid-cystic pseudopapillary tumor of pancreas: Description of two cases and literature review. Tumori 2007;93:522-5.  Back to cited text no. 2
    
3.Frantz VK. Tumour of the pancreas. In Atlas of tumour pathology, Section VII, Fascicles 27 and 28. Washington, DC: Armed Forces Inst Pathol; 1959. p. 32-3.  Back to cited text no. 3
    
4.Huang HL, Shih SC, Chang WH, Wang TE, Chen MJ, Chan YJ. Solid-pseudopapillary tumor of the pancreas: Clinical experience and literature review. World J Gastroenterol 2005;11:1403-9.  Back to cited text no. 4
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5.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. 5
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6.Morana G, Guarise A. Cystic tumors of the pancreas. Cancer Imaging 2006;6:60-71.  Back to cited text no. 6
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7.Petrakis I, Vrachassotakis N, Kogerakis N, Hatzidakis A, Zoras O, Chalkiakis G. Solid pseudopapillary neoplasm of the pancreas: Report of a case after a 10-year follow-up and review of the literature. Pancreatology 2001;1:123-8.  Back to cited text no. 7
    
8.Mehta N, Modi L, Patel T, Shah M. Study of cytomorphology of solid pseudopapillary tumor of pancreas and its differential diagnosis. J Cytol 2010;27:118-22.  Back to cited text no. 8
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9.Lee PS, Ching AS, Chan YL. Solid-cystic Pancreatic Tumour (Frantz's Tumour)-An Unusual Spleno-renal Angle Tumour in a Teenager. J HK Coll Radiol 2002;5:50-3.  Back to cited text no. 9
    
10.Roasi JC. Rosai and Ackerman's pathology. 10 th ed. Vol. 1. St Louis; Missouri. Mosby; 2008. p. 1024-6.  Back to cited text no. 10
    
11.Ganepola GA, Gritsman AY, Asimakopulos N, Yiengpruksawan A. Are pancreatic tumors hormone dependent? A case report of unusual rapidly growing pancreatic tumor during pregnancy, its possible relationship to female sex hormones, and review of the literature. Am Surg 1999;65:105-11.  Back to cited text no. 11
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12.Kasem A, Ali Z, Ellul J. Papillary cystic and solid tumour of the pancreas: Report of a case and literature review. World J Surg Oncol 2005;3:62.  Back to cited text no. 12
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    Figures

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



 

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