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J Ped Surg Case Reports 3 (2015) 149e153

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports .com

Solid pseudopapillary neoplasm of the pancreas in pediatricpatients: A case report and institutional case series

Justin B. Mahida a,b, Rajan K. Thakkar a, Jon Walker c, Rulong Shen d, Brian D. Kenney a,Vinay Prasad e, Jennifer H. Aldrink a,*

aDepartmentof Surgery, theOhio StateUniversityWexnerMedical Center,Divisionof Pediatric Surgery,NationwideChildren’sHospital, Columbus, OH,USAbCenter for Surgical Outcomes Research, Nationwide Children’s Hospital, Columbus, OH, USAcDepartment of Internal Medicine, Division of Gastroenterology, the Ohio State University Wexner Medical Center, Columbus, OH, USAdDepartment of Pathology, the Ohio State University Wexner Medical Center, Columbus, OH, USAeDepartment of Pathology, Nationwide Children’s Hospital, Columbus, OH, USA

a r t i c l e i n f o

Article history:Received 29 December 2014Received in revised form9 February 2015Accepted 17 February 2015

Key words:ImmunohistochemistryFrantz tumorSolid pseudopapillary neoplasm

* Corresponding author. Division of Pediatric SurHospital, 700 Children’s Drive, ED320, Columbus, OH,fax: þ1 614 722 3903.

E-mail address: jennifer.aldrink@nationwidechildre

2213-5766/� 2015 The Authors. Published by Elsevier Inhttp://dx.doi.org/10.1016/j.epsc.2015.02.006

a b s t r a c t

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor presenting in adolescent andyoung adult females. A previously healthy 13 year-old female presented to our institution withabdominal pain and emesis. Imaging revealed a pancreatic cystic mass. Endoscopic ultrasound (EUS)with fine needle biopsy suggested SPN. Pathologic evaluation following resection revealed immuno-histochemical (IHC) staining positive for b-catenin and a-1-antitrypsin despite extensive necrosis. Wediscuss this patient as well as our institutional series of SPN of the pancreas, describing the evaluation,management, and histopathology of this rare tumor.� 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND

license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Solid pseudopapillary neoplasm (SPN) of the pancreas is anuncommon tumor of the exocrine pancreas, occurring predomi-nantly in young females [1,2]. Unlike other forms of pancreaticneoplasm, SPN of the pancreas demonstrates an excellent long-term prognosis with adequate resection [3,4]. With accurate pre-operative diagnosis, surgical planning and intraoperative decisionmaking are enhanced.

We present a case of SPN of the pancreas in which the diagnosiswas suggested after fine-needle aspiration cytology on endoscopicultrasound. Definitive diagnosis was made from immunohisto-chemical (IHC) staining, which allowed for appropriate surgicalplanning and adequate resection. Without IHC staining, theextensive necrosis on endoscopic biopsy would have resulted in anotherwise indeterminate diagnosis, demonstrating the importanceof this histologic evaluation. We also present a single-institutionalcase series of patients undergoing resection for SPN of the pancreas,

gery, Nationwide Children’sUSA. Tel.: þ1 614 722 0440;

ns.org (J.H. Aldrink).

c. This is an open access article unde

revealing additional pediatric patients for whom immunochem-istry was valuable in establishing their diagnosis.

1. Case report

A 13-year old previously healthy female presented to ouremergency department with a three day history of abdominal pain,emesis, and malaise. Physical exam revealed mild right-sidedabdominal tenderness. A limited right lower quadrant abdominalultrasound was unremarkable, therefore a computed tomography(CT) scan was obtained demonstrating a predominantly cysticstructure arising from the duodenum and abutting the pancreatichead, without biliary or pancreatic ductal dilation (Fig. 1).

Carcinoembryonicantigen(CEA)andcancerantigen (CA)19-9werenot elevated.Magnetic resonance imaging (MRI) further characterizedthe lesion as a peripherally enhancing round lesion insinuated be-tween the pancreatic head and second portion of the duodenum,(Fig.2) suggestiveof eitheraduplicationcystwith internalhemorrhageor a solid pancreatic head mass. She then underwent endoscopic ul-trasonography (EUS) which revealed that this cystic mass was heter-ogenous in nature and without evidence of internal vascular flow(Fig. 3). Fine needle aspiration revealed monomorphic necrotic cells

r the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Fig. 1. Axial (A) and coronal (B) computed tomographic images of the intra-abdominal cystic mass at diagnosis.

J.B. Mahida et al. / J Ped Surg Case Reports 3 (2015) 149e153150

that on immunohistochemistry were positive for vimentin and b-cat-enin and weakly reactive for synaptophysin and chromogranin.

Given these findings, the patient underwent exploratory lapa-rotomy via a bilateral subcostal incision. A 3 cm mass arising fromthe head of the pancreas and adherent to the second portion of theduodenumwas identified (Fig. 4). A pancreaticoduodenectomy wasperformed for complete resection. The pancreatic duct was notdilated, measuring approximately 2e3 mm. Visceral reconstructionwas achieved with hepaticojejunostomy, pancreaticojejunostomy,and gastrojejunostomy.

On pathological examination, the mass was an encapsulated3 � 3 � 2.5 cm lesion arising from the head of the pancreas. The cutsurface showed yellow brown necrotic tissue with gritty yellowcalcified material. Microscopically, the lesion had a large amount ofnecrosis with focal areas of neoplastic cells, characterized by small tomediumsizedcellsmostly in sheets.Within thenecrotic areas, ghostsof papilliform structureswere present (Fig. 5). On IHC, the tumorwaspositive for b-catenin and a-1-antitrypsin. Testing for CD10 wasindeterminate, and the tumor was not tested for e-cadherin.

The patient’s postoperative recovery was complicated by pro-longed gastroparesis. She received total parenteral nutrition for twoweeks and subsequent nasojejunal feedingwhichwas discontinuedonce she tolerated oral intake. This ultimately resolved and herpostoperative course was otherwise uncomplicated. Final histopa-thology demonstrated this lesion to be a solid pseudopapillaryneoplasm of the pancreas with extensive necrosis and negativemargins. Follow-up imaging has demonstrated no evidence ofrecurrence at 1 year from resection. Given complete resection,adjuvant therapy was not indicated.

Following this case, a review ofmedical and pathological recordsat our institution, revealed eight patients between January 1995

Fig. 2. A) Axial T2-weighted image of the cystic mass (M) with delineation of the plane bdemonstrating its heterogeneity and encapsulation.

and December 2014 who were treated for SPN of the pancreas(Table 1). Three of the patients demonstrated intralesional necrosison histopathology. All eight patients were female and nonerequired adjuvant therapy following resection. With a medianfollow up time of 5 years (range 1e5 years), there were no re-currences. There was one death that was unrelated to the diagnosisor treatment of SPN of the pancreas.

2. Discussion

SPNs of the pancreas are neoplasms of low malignant potentialfound predominantly in young females [1,2]. They account for 1e2%of all exocrine pancreatic tumors [5], but 52e71% of pancreatictumors in children and adolescents [6,7]. The etiology is unknownbut suspected to be genetically distinct from pancreatic ductalneoplasms [2]. Despite their preferential association with youngwomen, there are no reports suggesting an association withendocrine disturbances [8]. These tumors do not demonstrate apreferential localization within the pancreas. These neoplasms areoften found incidentally on routine physical examination or in pa-tients who present with abdominal pain [9,10]. As in our patientand our institutional review, they are not often associated withpancreatic ductal dilation [11,12].

The differential diagnosis for SPN includes pancreatic pseudo-cysts, duplication cysts, and other neoplasms of the pancreasincluding lymphoma and cystic neoplasms [13,14]. Pathology oftenreveals lobulated solid areas with zones of hemorrhage and ne-crosis, and cystic spaces filled with necrotic debris [9,15]. IHC isuseful for diagnosis of SPN of the pancreas [9]. Positive markers fora-1-antitrypsin, a-1-antichymotrypsin, phospholipase A2, CD 10,and CD 56 are suggestive of pancreatic lesions [16]. A combination

etween the mass and the duodenum (D). B) Coronal T2-weighted image of the mass,

Fig. 3. Endoscopic ultrasonographic imaging of intra-abdominal mass, marked withdotted lines.

Fig. 4. Surgical resection specimen with the antrum, duodenum, and pancreatic headmass (3 � 3 � 2.5 cm).

Fig. 5. A) Hematoxylin-eosin staining of the lesion at 100� magnification. Sheets ofsmall to medium-sized, relatively uniform lesional cells growing in an infiltrative sheetare present in the upper portion with normal pancreatic islands of acini in the lowerhalf. A group of normal ductules separate the lesion (L) from normal (N). B) Immu-nohistochemical staining for b-catenin at 100� magnification. Strong, diffuse nuclearstaining in lesional cells within a necrotic area is noted.

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of b-catenin and CD 10 and absence of e-cadherin is most specificfor SPN when compared to other pancreatic neoplasms [2,17e19].Unlike pancreatic adenocarcinoma and neuroendocrine carcinoma,patients do not typically demonstrate elevated CEA or CA 19-9 [20].

Due to these staining characteristics, fine-needle aspiration(FNA) cytology is frequently helpful for making a diagnosis preop-eratively [21]. However, FNA is rarely used in pediatric patients[10,22,23]. EUS-FNA demonstrates improved sensitivity for diag-nosing SPN of the pancreas in adults, correctly identifying over 80%of patients with SPN [11]. In the present case, FNA revealed necroticlaminar cells typical in large neoplasms (>5 cm) and nondiagnosticwhen found in isolation, but the diagnosis was still strongly sus-pected by IHC. Our institutional case series includes several patientsfor whom histopathology revealed central hemorrhage and ne-crosis (Table 1). Others have also reported SPN of the pancreas withextensive cellular degeneration [24].

Complete surgical resection is the treatment of choice whenfeasible. In this case, a pancreaticoduodenectomy was performedgiven the location of the lesion at the head of the pancreas. Otheroptions for treatment include enucleation or subtotal pancreatec-tomy, depending on location of the tumor. Making a correct intra-operative diagnosis is important because of the comparativelyimproved prognosis of SPN as compared to other pancreatic

Table 1Institutional review of patients with SPN of the pancreas.

Age Surgery Pathology Immunohistochemistry Patient status

16 Spleen-preserving distalpancreatectomy

4 � 6 cm solid mass of the pancreas,hemorrhagic center, negative margins.No pancreatic ductal dilation.

No immunohistochemistry performed. NED

11 Pancreatoduodenectomy 14 � 11 � 9 cm mass at head of pancreas,hemorrhagic center, negative margins.

No immunohistochemistry performed. NED

14 Pancreatoduodenectomy 4.2 cm mass in the head of the pancreas,necrotic and hemorrhagic, negative margins.No pancreatic ductal dilation.

Positive: vimentin & NSE.Negative: synaptophysin, keratins andchromogranin.

NED

15 Spleen-preserving distalpancreatectomy

7.8 � 4.6 � 3.2 cm encapsulated mass withnegative clear margins.

Positive: NSE.Negative: synaptophysin, keratins andchromogranin.

NED

14 Pancreatoduodenectomy 2.5 � 1.5 � 1.2 cm mass with pseudopapillaryarchitecture and areas of hemorrhage and necrosis.

No immunohistochemistry performed. NED

13 Pancreatoduodenectomy 3 cm encapsulated mass with pseudopapillaryarchitecture, extensive necrosis, and negativemargins. No pancreatic ductal dilation.

Positive: vimentin, b-catenin, anda-1-antitrypsin.Negative: synaptophysin and chromogranin.

NED

15 Distal spleen-preservingpancreatectomy

7 � 6 � 6 cm encapsulated mass, with centralnecrosis and positive margins. No pancreaticductal dilation.

Positive: NSE and a-1-antitrypsin. Death 3 monthsafter surgery fromrespiratory complicationsof primary illness.

15 Pancreatoduodenectomy,pylorus preserving

5 � 4 � 2.8 cm cystic structure, encapsulated,negative margins. No pancreatic ductal dilation.

Positive: NSE, b-catenin, and a-1-antitrypsin.Negative: chromogranin.

NED

NSE: Neuron-specific enolase. NED: No evidence of disease.Median follow-up was 5 years (range 1 yeare5 years). All patients were female.

J.B. Mahida et al. / J Ped Surg Case Reports 3 (2015) 149e153152

neoplasms [20,25]. In our series, several patients were able to un-dergo pylorus-preserving pancreatoduodenectomy or spleen-pre-serving distal pancreatectomy. None of the patients in our caseseries required adjuvant treatment.

Prognosis of SPN of the pancreas following complete resectionwith negative margins is excellent. Reports suggest that theseneoplasms grow slowly, and recurrence is uncommon followingcomplete resection. Although methods of treatment involvingchemoradiation, radiofrequency ablation and HIPEC have beendescribed, repeat surgical resection is warranted should they recur[3,26e28]. These various adjuvant techniques have all been appliedto adult patients who presented with overwhelming metastasis.Pediatric patients reported in the current literature have typicallypresentedwith resectable disease, with an overall five-year survivalof 95% [3,4].

3. Conclusion

Although SPN of the pancreas is a rare form of pancreaticneoplasm, pediatric patients with pancreatic neoplasms are morelikely to have SPN than other diagnoses. The diagnosis may bedifficult to make preoperatively, but EUS-FNA and IHC arefrequently helpful. With complete surgical resection, patients canexpect an excellent prognosis.

Conflicts of interestThe authors have no financial conflicts of interest to disclose.

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