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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 365-367
Solid-pseudopapillary neoplasm of the pancreas: A classical presentation with unique paranuclear dot like immunostaining with CD 99


1 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Surgical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
3 Department of Surgical Gastroenterology, KIMS Hospital, Thiruvananthapuram, Kerala, India

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Date of Web Publication14-Aug-2015
 

   Abstract 

A 32-year-old lady presented with a history of abdominal pain and upper abdominal discomfort of 3 months duration. Her imaging studies done at a local hospital showed a solid-cystic mass involving head of the pancreas. The patient was referred to our surgical oncology department. On examination, there was a nontender mass in the epigastrium. An ultrasound scan guided fine-needle aspiration (FNA) was done which was showing classical features of solid-pseudo papillary neoplasm of the pancreas. With this preoperative diagnosis patient was taken up for surgery. Per operatively, there was a solid-cystic mass in the head of the pancreas. Pancreaticoduodenectomy was done. Histopathology and immunohistochemistry (IHC) confirmed the diagnosis of solid-pseudo papillary neoplasm of the pancreas. Apart from the routine IHC panel, CD 99 immunostain was also done which demonstrated the characteristic paranuclear dot-like staining observed in previous studies in the literature.

Keywords: CD 99 immunostain, pancreas, solid-pseudopapillary neoplasm

How to cite this article:
Nair Anila KA, Nayak N, Muralee M, Venugopal BP, Mony RP. Solid-pseudopapillary neoplasm of the pancreas: A classical presentation with unique paranuclear dot like immunostaining with CD 99. Indian J Pathol Microbiol 2015;58:365-7

How to cite this URL:
Nair Anila KA, Nayak N, Muralee M, Venugopal BP, Mony RP. Solid-pseudopapillary neoplasm of the pancreas: A classical presentation with unique paranuclear dot like immunostaining with CD 99. Indian J Pathol Microbiol [serial online] 2015 [cited 2022 Jan 22];58:365-7. Available from: https://www.ijpmonline.org/text.asp?2015/58/3/365/162902



   Introduction Top


Solid-pseudopapillary neoplasm of the pancreas was first reported by Frantz in 1959. [1] It is a rare neoplasm of unknown histogenesis and low malignant potential which occurs predominantly in young women. However, occasional cases have been reported in males. [2] The neoplasm has characteristic cytological features which makes preoperative diagnosis possible in most cases. However, monomorphic population of discohesive cells and eccentric nuclei sometimes makes it difficult to differentiate from other pancreatic tumors especially the neuroendocrine tumors. Though an immunohistochemistry (IHC) panel comprising of vimentin, chromogranin A, CD10, E-cadherin, and β-catenin for diagnosis of solid-pseudopapillary neoplasm of the pancreas is often helpful, these markers are not specific. It is important to diagnose this entity because it is prognostically better than other pancreatic neoplasms with an aggressive course. Management also differs with surgical resection being curative in most cases of solid-pseudopapillary neoplasms, whereas adjuvant therapy may be required in other pancreatic neoplasms. The characteristic CD 99 (MIC 2) paranuclear dot-like positivity seen in solid-pseudo papillary neoplasm of the pancreas is extremely useful in confirming the diagnosis. [3],[4]


   Case Report Top


A 32year-old lady who was on medication for hypertension presented to the local hospital with complaints of abdominal pain and upper abdominal discomfort of 3 months duration. Radiological evaluation detected an 8.5 cm × 8 cm × 7 cm solid cystic mass involving head of the pancreas. The patient was referred to our surgical oncology division. On examination, there was a nontender mass in the epigastrium. Her serum CA 19.9 value was 7.2 u/ml. An ultrasound scan guided fine-needle aspiration was done, the wet fixed papanicolaou stained smears of the material showed characteristic cytological features of solid-pseudopapillary neoplasm. The smears were cellular and showed monomorphic cells lying singly, as loosely cohesive clusters as well as forming delicate pseudo papillae [Figure 1]. Presence of nuclear grooves and background of foamy macrophages and giant cells were also noted. A cytological diagnosis of solid-pseudopapillary neoplasm was made. The patient was taken up for surgery.
Figure 1: Smear showing monomorphic cells forming delicate pseudo papillae (Pap, ´400)


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Per operatively, there was a solid-cystic mass involving head of the pancreas. Body and tail of pancreas were atrophic with fatty infiltration. Whipple's resection was done. The specimen we received showed a large, 8 cm × 7 cm mass involving head of the pancreas. The cut surface was solid with light brown to yellow appearance. There were areas of hemorrhage. Microscopy showed pseudo papillae with poorly cohesive monomorphic cells with round to oval nuclei and finely dispersed chromatin arranged around delicate vascular channels [Figure 2]. Furthermore, seen were solid areas of neoplastic cells with adjacent areas showing cholesterol clefts with foreign-body giant cell reaction. IHC showed strong positivity for vimentin [Figure 3] and progesterone receptors, patchy positivity with CD56 and negative staining with cytokeratin (AE1/AE3). We also did CD 99 immunostain which showed characteristic paranuclear dot-like positivity [Figure 4]. Thus, histopathology and IHC confirmed the preoperative cytological diagnosis of solid-pseudopapillary neoplasm of the pancreas.
Figure 2: Microscopy showing pseudo papillae with poorly cohesive monomorphic cells with round to oval nuclei and finely dispersed chromatin arranged around delicate vascular channels (H and E, ´200)


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Figure 3: Neoplastic cells showing strong positivity for vimentin (IHC, ´400)


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Figure 4: Neoplastic cells showing paranuclear dot-like positivity for CD 99 (IHC, ´1000)


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   Discussion Top


Solid-pseudopapillary neoplasm of the pancreas is a low-grade malignant neoplasm composed of poorly cohesive monomorphic epithelial cells forming solid and pseudopapillary structures. They occur predominantly in young women. Microscopically the main differential diagnoses include well-differentiated neuroendocrine neoplasm and acinar cell carcinomas. Immunohistochemical expression of vimentin, progesterone receptor, CD10; abnormal nuclear labeling with antibodies to beta-catenin and negativity for chromogranin A of the neoplastic cells of solid-pseudopapillary neoplasm will help in reaching a diagnosis.

Burford et al. proposed a panel comprising CD10, E-cadherin and β-catenin for differentiating solid-pseudopapillary neoplasm of the pancreas from pancreatic neuroendocrine neoplasms. [5] However, these markers were found to be positive in intraductal papillary mucinous neoplasm, pancreatoblastoma, and acinic cell carcinoma which sometimes come as a differential diagnosis of solid-pseudopapillary neoplasm. [6],[7],[8] CD 99 immunoreactivity in solid-pseudopapillary neoplasm has been reported, wherein it was demonstrated to have a characteristic paranuclear dot-like positivity. It has been reported that this positivity pattern is quite classical of solid-pseudopapillary neoplasm among the different pancreatic tumors as it was negative in pancreatoblastoma, adenocarcinoma, and acinic cell carcinoma. [3] In pancreatic endocrine tumors, it was either negative or had a membranous pattern of positivity. Thus, CD 99 positivity is extremely helpful in confirming a diagnosis of solid-pseudopapillary neoplasm.

The prognosis of solid-pseudopapillary neoplasm of the pancreas is better when compared to other pancreatic malignancies. Most of the patients are cured after complete surgical resection. [9] The postoperative period of our patient was uneventful and she is on follow-up and is doing well.

To conclude, we agree with previous studies in the literature that CD 99 immunostain with the unique paranuclear dot-like positivity would be of use in diagnosing solid-pseudo papillary neoplasm, especially when other pancreatic neoplasms with similar morphology comes in the differential.

 
   References Top

1.
Frantz VK. Tumors of the pancreas. In: Atlas of Tumor Pathology. 1 st Series. Washington, DC, USA: US Armed Forces Institute of Pathology; 1959. p. 32-3.  Back to cited text no. 1
    
2.
Mohan H, Bal A, Punia RP, Attri AK. Solid and cystic papillary epithelial neoplasm of the pancreas. J Postgrad Med 2006;52:141-2.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.
Guo Y, Yuan F, Deng H, Wang HF, Jin XL, Xiao JC. Paranuclear dot-like immunostaining for CD99: A unique staining pattern for diagnosing solid-pseudopapillary neoplasm of the pancreas. Am J Surg Pathol 2011;35:799-806.  Back to cited text no. 3
    
4.
Ghosh R, Mallik SR, Mathur SR, Iyer VK. CD 99 immunocytochemistry in solid pseudopapillary tumor of pancreas: A study on fine-needle aspiration cytology smears. J Cytol 2013;30:151-5.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Burford H, Baloch Z, Liu X, Jhala D, Siegal GP, Jhala N. E-cadherin/beta-catenin and CD10: A limited immunohistochemical panel to distinguish pancreatic endocrine neoplasm from solid pseudopapillary neoplasm of the pancreas on endoscopic ultrasound-guided fine-needle aspirates of the pancreas. Am J Clin Pathol 2009;132:831-9.  Back to cited text no. 5
    
6.
Abraham SC, Wu TT, Hruban RH, Lee JH, Yeo CJ, Conlon K, et al. Genetic and immunohistochemical analysis of pancreatic acinar cell carcinoma: Frequent allelic loss on chromosome 11p and alterations in the APC/beta-catenin pathway. Am J Pathol 2002;160:953-62.  Back to cited text no. 6
    
7.
Chetty R, Serra S, Salahshor S, Alsaad K, Shih W, Blaszyk H, et al. Expression of Wnt-signaling pathway proteins in intraductal papillary mucinous neoplasms of the pancreas: A tissue microarray analysis. Hum Pathol 2006;37:212-7.  Back to cited text no. 7
    
8.
Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, et al. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas: Frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol 2001;159:1619-27.  Back to cited text no. 8
    
9.
Naresh KN, Borges AM, Chinoy RF, Soman CS, Krishnamurthy SC. Solid and papillary epithelial neoplasm of the pancreas. Diagnosis by fine needle aspiration cytology in four cases. Acta Cytol 1995;39:489-93.  Back to cited text no. 9
    

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Correspondence Address:
Dr. Kunjulekshmi Amma Raveendran Nair Anila
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.162902

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    Figures

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

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