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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2019  |  Volume : 24  |  Issue : 2  |  Page : 129-131
 

Solid pseudopapillary neoplasm of the pancreas in a pediatric patient with associated urogenital anomalies


1 Department of Pathology and Laboratory Medicine, Medanta-The Medicity, Gurgaon, Haryana, India
2 Department of Pediatric Surgery, Medanta-The Medicity, Gurgaon, Haryana, India

Date of Web Publication1-Mar-2019

Correspondence Address:
Dr. Meera Luthra
Department of Pediatric Surgery, Medanta-The Medicity, Sector-38, Gurgaon - 122 001, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_175_17

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   Abstract 


We report a case of a 9-year-old girl presented to the outpatient department with abdominal pain and diagnosed as solid pseudopapillary neoplasm (SPN) with urogenital anomalies. SPN can occur in children with extrapancreatic abnormalities, especially urogenital abnormalities, so these anomalies should be looked for in children diagnosed with SPN and vice versa.


Keywords: Children, pancreas, urogenital


How to cite this article:
Sharma R, Sarin H, Luthra M. Solid pseudopapillary neoplasm of the pancreas in a pediatric patient with associated urogenital anomalies. J Indian Assoc Pediatr Surg 2019;24:129-31

How to cite this URL:
Sharma R, Sarin H, Luthra M. Solid pseudopapillary neoplasm of the pancreas in a pediatric patient with associated urogenital anomalies. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2019 Jul 24];24:129-31. Available from: http://www.jiaps.com/text.asp?2019/24/2/129/253335





   Introduction Top


Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm accounting for 0.13%–2.7% of the primary pancreatic tumors and <2% of all exocrine pancreatic neoplasms.[1] These neoplasms were first diagnosed in 1959 by Virginia Frantz, who named them “papillary cystic tumor of the pancreas.”[2] It is most commonly seen in young females with an average age of 28 years and female-to-male ratio of 10:1.[2] The pediatric primary pancreatic neoplasms have an incidence of 1.8 cases per 1,000,000 pediatric population in the United States,[3] of which 20%–25% of the tumors are SPN.[2] This tumor shows low malignant potential, and it metastasizes rarely. Hence, these lesions are managed by surgical excision which warrants an excellent outcome of the patient.


   Case Report Top


An 11-year-old girl presented to the outpatient department with abdominal pain in August 2016. The patient had undergone left partial nephrectomy in 2012 for a left lower nonfunctioning moiety with gross ureterohydronephrosis in a duplex system. The follow-up ultrasound of whole abdomen showed a large left renal cyst. In 2015, she had undergone endoscopy with excision of transverse vaginal septum for hematocolpos.

In August 2016, the patient presented with complaints of abdominal pain. The computed tomography urography revealed an enlarged left kidney with a large 70 mm cystic area with splaying of the upper and middle calyces. Incidentally, heterogeneously enhancing soft tissue density lesion was seen in relation to the tail of the pancreas abutting the spleen and the left kidney with the cyst [Figure 1]a. A tentative diagnosis of a residual urine collection at the left nephrectomy site and an incidental tumor of the tail of the pancreas was discovered. Ultrasound-guided fine-needle aspiration cytology (FNAC) was done from the tail of the pancreas and the renal cyst. The cytological examination of the renal cyst aspirate showed only few inflammatory cells and no malignant cells. The pancreatic FNAC showed smears composed of a monotonous population of neoplastic cells. The cells were arranged in loose groups, singly dispersed, and around fibrovascular hyalinized stalks [Figure 1]c and [Figure 1]d. The cells showed minimal anisonucleosis, with delicate cytoplasm, round to oval, regular nuclei with finely granular chromatin. Pinkish myxoid material was seen between the cell clusters and in the background [Figure 1]e and [Figure 1]f. No mitotic figures or necrosis was identified. The diagnosis was suggestive of SPN of the pancreas. In December 2016, she underwent distal pancreatectomy with splenectomy and recovered uneventfully. Grossly, the specimen showed a solid–cystic lesion measuring 50 mm × 40 mm × 35 mm [Figure 1]b. Histopathology showed neoplastic cells with stippled chromatin, and inconspicuous nucleoli arranged is pseudopapillary pattern with fibrovascular cores [Figure 1]g. Immunohistochemistry showed positivity for beta-catenin, CD10, CD56, nonspecific esterase, and progesterone receptor (PR) and negativity for CD117 [Figure 1]g. The diagnosis of SPN of the pancreas was confirmed. The patient is doing well postsurgery and is under follow-up for the last 9 months.
Figure 1: (a-c) Contrast tomography reveals a heterogeneously enhancing soft tissue density lesion in relation to the tail of the pancreas abutting the spleen and the left cystic kidney (line arrow showing pancreatic tumor and renal cyst in a). (d) Grossly, the specimen showed a solid–cystic lesion measuring 50 mm × 40 mm × 35 mm in the pancreas. (e) Pap stain (×10) shows smears composed of a monotonous population of neoplastic cells. The cells are arranged in loose groups, singly dispersed, and around fibrovascular hyalinized stalks. (f) Giemsa stain (×40) shows minimal anisonucleosis, with delicate cytoplasm, round to oval, regular nuclei with finely granular chromatin. Pinkish myxoid material was seen between cell clusters. (g) Histopathology shows neoplastic cells with stippled chromatin, and inconspicuous nucleoli arranged is pseudopapillary pattern with fibrovascular cores. Immunohistochemistry shows positivity for beta-catenin, CD10, CD56, nonspecific esterase, and PR and negativity for CD117

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   Discussion Top


SPN is rare in children, and as compared to adults, female preponderance is less common in the pediatric population. In children, the most common presentation is palpable mass, whereas, in adults, SPN is detected as an incidental mass. Furthermore, the most common location in adults is body or tail, whereas, in children, it is the head of the pancreas; however, in our child, it was in the tail.[4]

SPN is said to arise from the primitive pancreatic cells or from the cell lines of female genital bud. The molecular alterations typical of SPN are beta-catenin mutation, disruption of Wnt pathway, and disorganization of E-cadherin. In most of the cases, cyclin D1, a transcriptional target of beta-catenin is overexpressed.[3] SPN harbors beta-catenin gene (CTNNB1) mutations in exon 3, which results in the activation of Wnt signaling pathway with activation of c-myc and cyclin D1 and results in tumor genesis. The Wnt signaling pathway and beta-catenin mutations are also associated with urogenital malformations. In our case, the patient was also diagnosed with urogenital anomalies such as left duplex kidney and vaginal septum. It can be assumed that these urogenital abnormalities and SPN were due to similar pathogenic mechanisms, and future molecular studies are needed to support this possibility. The association between these anomalies and SPN needs to be further confirmed, because only one case of such kind has been reported in a 25-year-old female who presented with SPN and solitary kidney and uterus didelphys.[4] Till date, more than fifty cases of SPN have been reported in the pediatric population, but none of them had associated urogenital anomalies.

The radiological examination showed a well-circumscribed, heterogeneous mass with variable solid and cystic areas. The diagnosis can be confirmed by endoscopic ultrasound-guided FNAC (EUS-FNAC), which is an effective and desirable tool for preoperative diagnosis and helps in planning surgical excision of these tumors as in our case.[2] In the pediatric population, the surgical resection proves curative with an overall 5-year survival of 95%.[5]

SPN is a rare tumor in children. EUS-FNAC of pediatric pancreatic neoplasms is a useful and effective tool in preoperative diagnosis of these neoplasms. The surgical resection is the treatment of choice. SPN can occur in children with extrapancreatic abnormalities, especially urogenital abnormalities, so these anomalies should be looked for in children diagnosed with SPN and vice versa. This is the first case of SPN with associated urogenital abnormalities diagnosed in the pediatric age group. The association between urogenital anomalies and SPN and the role of beta-catenin/Wnt pathway needs to be further investigated.

Consent

Written informed consent was obtained from the patient for publication of this case and any accompanying images. No ethical approval was required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for the patient's images and other clinical information to be reported in the journal. The patient's parents understand that the name and initials of the patient will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Uppin SG, Hui M, Thumma V, Challa S, Uppin MS, Bheerappa N, et al. Solid-pseudopapillary neoplasm of the pancreas: A clinicopathological and immunohistochemical study of 33 cases from a single institution in Southern India. Indian J Pathol Microbiol 2015;58:163-9.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Vassos N, Agaimy A, Klein P, Hohenberger W, Croner RS. Solid-pseudopapillary neoplasm (SPN) of the pancreas: Case series and literature review on an enigmatic entity. Int J Clin Exp Pathol 2013;6:1051-9.  Back to cited text no. 2
    
3.
Park JY, Kim SG, Park J. Solid pseudopapillary tumor of the pancreas in children: 15-year experience at a single institution with assays using an immunohistochemical panel. Ann Surg Treat Res 2014;86:130-5.  Back to cited text no. 3
    
4.
Guan ZW, Sun L, Wang YQ, Xu BX. Solid pseudopapillary tumor of the pancreas and concomitant urogenital malformations in a young woman. Diagn Pathol 2013;8:35.  Back to cited text no. 4
    
5.
Chhadi TS, Deshpande AH, Chhadi SA, Kumbhalkar DT, Raut WK. A solid pseudopapillary tumour of the head of pancreas: A rare case report diagnosed by fine needle aspiration cytology. J Clin Diagn Res 2016;10:ED06-8.  Back to cited text no. 5
    

Editorial Comment: Solid Pseudo papillary Neoplasm of the pancreas in children associated with haematocolps and duplex kidney is exceedingly rare. The author has nicely dealt with the tumour. The child had had partial nephrectomy in 2012 and excision of transverse vaginal septum in 2015. The development of renal cyst in the same kindney is not clear. The author did not highlight the operative findings of the left over upper moiety of the left kidney and the related cyst which could have been very interesting to the readers.


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