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Year : 2019  |  Volume : 62  |  Issue : 4  |  Page : 624-626
Solid pseudopapillary neoplasm of pancreas metastasizing to spleen in a post menopausal female


Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

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Date of Web Publication14-Oct-2019
 

How to cite this article:
Singla T, Ahluwalia C, Singla G, Kolte S, Singla S, Arora R. Solid pseudopapillary neoplasm of pancreas metastasizing to spleen in a post menopausal female. Indian J Pathol Microbiol 2019;62:624-6

How to cite this URL:
Singla T, Ahluwalia C, Singla G, Kolte S, Singla S, Arora R. Solid pseudopapillary neoplasm of pancreas metastasizing to spleen in a post menopausal female. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Nov 22];62:624-6. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/624/269070




Solid pseudopapillary neoplasm (SPN) also known as gruber frantz tumor or hamoudi tumor is an uncommon entity and reportedly accounts for between 2% and 3% of pancreatic neoplasms and 0.9% to 2.7% of exocrine pancreatic neoplasms.[1] It primarily affects young females with a mean age of 22 years.[2] It is most commonly located in the pancreatic body and tail. Malignant potential is seen only in 10–15% of all cases. The most common sites of metastasis are liver, regional lymph nodes, mesentery, omentum, and peritoneum. Direct spread to spleen has been reported in very few cases. Here we are reporting one such case of metastatic solid pseudopapillary carcinoma of pancreas in a 52-year-old patient.

A 52-year-old post menopausal hypertensive female presented in surgery outpatient department with pain in left upper abdomen since 4 months. It was associated with intermittent fever which lasted for 10-15 days. CT scan suggested a large heterogeneously enhancing mass lesion involving mid and lower pole of spleen [Figure 1], causing indentation over the greater curvature of stomach and with loss of fat planes with adjacent tail of pancreas. Imaging features suggested a possibility of neoplastic mass lesion.
Figure 1: CT scan suggested a large heterogeneously enhancing mass lesion involving mid and lower pole of spleen with loss of fat planes with adjacent tail of pancreas

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Grossly a specimen consisting of spleen along with pancreatic tail was received measuring 19 × 15 × 8 cm. Spleen measured 16 × 10 × 8 cm and pancreatic tail measured 10 × 5 × 2 cm. On cut, a lobulated tumor was identified involving both spleen and the pancreatic tail. The tumor was breaching the splenic capsule. The cut surface was solid with variegated appearance with areas of hemorrhage and myxoid change [Figure 2]. The attached fat did not show any lymph nodes or solid areas.
Figure 2: Grossly specimen shows a lobulated tumor which is solid with a variegated appearance along with areas of hemorrhage

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Microscopically the tumor cells were arranged in pseudopapillary pattern and were also present in sheets, cords, and nests [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. The tumor cells were polygonal with abundant eosinophilic to vacuolated cytoplasm [Figure 3]e. Few of the cells showed nuclear grooving. Dilated thin walled blood vessels, cholesterol clefts and focal areas of hyalinization were also present. Focally mild atypia with hyperchromatic nuclei was noted. No necrosis, vascular, or perineural invasion was noted. The tumor was seen infiltrating the surrounding fat [Figure 3]f. The tumor cells were positive for vimentin, neuron-specific enolase, CD 10, β -catenin, alpha -1 antitrypsin and focally for cytokeratin [Figure 4]. It was however negative for chromogranin and synaptophysin. Ki-67 was <1%. Thus, a diagnosis of solid pseudopapillary neoplasm pancreas with metastasis to spleen was made.
Figure 3: H and E section shows presence of tumor cells arranged in (a and b) Pseudopapillary pattern (10×) (c) cords (20×) (d) sheets (10×). (e) Section shows tumor cells with round to oval nuclei and presence of vacuolated cytoplasm (40×). (f) Section shows tumor cells invading fat (4×)

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Figure 4: Immunohistochemistry section shows (a) cytoplasmic positivity with vimentin (20×) (b) nuclear positivity with NSE (20×) (c) membranous positivity with CD 10 (20×) (d) nuclear positivity with beta- catenin (10×) (e) cytoplasmic positivity with alpha antitrypsin (20×) (f) cytoplasmic positivity with cytokeratin (20×)

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Solid pseudopapillary neoplasm of the pancreas is a rare neoplasm with a low malignant potential. The most common presentation is with non-specific symptoms such as abdominal pain or discomfort along with poor appetite and nausea. These symptoms are related to tumor compression on adjacent organs.[3]

It is often diagnosed during complementary imaging investigations such as ultrasound or CT scan of the abdomen. MRI is better than CT in detecting the cystic or solid components of the tumor. If MRI reveals an encapsulated mass with solid and cystic components as well as hemorrhage without obvious internal septum, SPT of the pancreas should be highly suspected.[4]

The differential diagnosis includes pancreatic neuroendocrine neoplasms, pancreatoblastoma, acinar cell carcinoma. Pancreatic neuroendocrine tumors have a similar morphology as that of solid papillary neoplasm, however presence of speckled (salt-and-pepper) chromatin favors neuroendocrine tumor.[5] The tumor cells are diffusely positive for synaptophysin, chromogranin, and cytokeratin while they are focally positive for synaptophysin, cytokeratin, and negative for chromogranin in SPN.

Pancreatoblastoma which primarily occurs in children is composed of epithelial cells arranged in sheets and nests with acini/ducts. Squamoid corpuscles are common and specific. These tumor cells are positive for AFP, CEA, and mucin and are negative in SPN.[5]

Presence of cohesive clusters of acinic cells with granular cytoplasm, and abundant acinar formation, is the key for the diagnosis of acinar cell carcinoma.[5] The tumor cells are positive for PAS (diastase resistant), BCL 10, CK7, CK 19, and are negative for beta – catenin.

The poor prognostic factors include size >5 cm, male gender, necrosis, cellular atypia, vascular invasion, perineural invasion, and invasion in to adjacent structures.

Only 15% of SPT patients develop metastasis. The prognosis is good even in patients with local recurrence, metastasis or invasion. The overall 5-year survival rate of SPT patients is about 95%. Our case is unique as this tumor is common in younger age group.

In conclusion solid pseudopapillary neoplasm of pancreas is a relatively benign and indolent tumor with a long 5-year survival time. Patients may survive long even with an unresectable tumor. Whenever possible, surgery is justified for local invasion or metastasis of SPT. The role of chemotherapy and radiotherapy remains to be studied.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Santini D, Poli F, Lega S. Solid-papillary tumors of the pancreas: Histopathology. JOP 2006;7:131-6.  Back to cited text no. 1
    
2.
Yan SX, Adair CF, Balani J, Mansour JC, Gokaslan ST. Solid pseudopapillary neoplasm collides with a well-differentiated pancreatic endocrine neoplasm in an adult man. Am J Clin Pathol 2015;143:283-7.  Back to cited text no. 2
    
3.
Yu PF, Hu ZH, Wang XB, Guo JM, Cheng, XD, Zhang YL, et al. Solid pseudopapillary tumor of the pancreas: A review of 553 cases in Chinese literature. World J Gastroenterol 2010;16:1209-14.  Back to cited text no. 3
    
4.
Yu CC, Tseng JH, Yeh CN, Hwang TL, Jan YY. Clinicopathological study of solid and pseudopapillary tumor of pancreas: Emphasis on magnetic resonance imaging findings. World J Gastroenterol 2007;13:1811-5.  Back to cited text no. 4
    
5.
Dinarvand P, Jinping Lai J. Solid pseudopapillary neoplasm of the pancreas: A rare entity with unique features. Arch Pathol Lab Med 2017;141:990-5.  Back to cited text no. 5
    

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Correspondence Address:
Charanjeet Ahluwalia
Department of Pathology, Safdarjung Hospital and Vardhman Mahavir Medical College, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_43_19

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



 

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