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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 13  |  Issue : 2  |  Page : 52-55

Surgical management of solid pseudopapillary tumors of the pancreas in female Nigerians: A report of two cases


General Surgery Unit, Department of Surgery, Lagos University Teaching Hospital, Idi-Araba, Lagos, Nigeria

Date of Submission03-Sep-2021
Date of Acceptance31-Dec-2021
Date of Web Publication24-Feb-2022

Correspondence Address:
Dr. Olanrewaju Samuel Balogun
General Surgery Unit, Department of Surgery, Lagos University Teaching Hospital, Idi-Araba, Lagos.
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njgh.njgh_8_21

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  Abstract 

Solid pseudopapillary tumor of the pancreas (SPT) is a rare subtype of pancreatic tumor with a prevalence of 0.13%–2.7%. Most SPTs occur in young females and are slow-growing. The tail of the pancreas is mostly affected. Clinical features are nonspecific. Abdominal computed tomography (CT) is invaluable in the preoperative diagnosis of SPT. Prognosis is good with complete surgical resection.

Keywords: Nigeria, pancreas, pseudopapillary tumors, surgery


How to cite this article:
Balogun OS, Atoyebi OA. Surgical management of solid pseudopapillary tumors of the pancreas in female Nigerians: A report of two cases. Niger J Gastroenterol Hepatol 2021;13:52-5

How to cite this URL:
Balogun OS, Atoyebi OA. Surgical management of solid pseudopapillary tumors of the pancreas in female Nigerians: A report of two cases. Niger J Gastroenterol Hepatol [serial online] 2021 [cited 2022 Jun 30];13:52-5. Available from: https://www.njghonweb.org/text.asp?2021/13/2/52/338255




  Introduction Top


Tumors of the pancreas are broadly classified into endocrine and exocrine varieties.[1] Pseudopapillary tumors are exocrine in origin and exist in solid, cystic, or mixed forms. Solid pseudopapillary tumor (SPT) of the pancreas is one of the subtypes of epithelial exocrine pancreatic tumors with an uncertain direction of differentiation.[1] Pseudopapillary tumors are so-called because on histological examination they show characteristic frond-like features projecting into cystic spaces from hemorrhagic necrosis.[2] SPTs have low-malignant potential and are rare in clinical practice constituting 0.13%–2.7% of all pancreatic tumors.[2] Clinical presentation of SPT may occur at any age. A typical patient with SPT is that of a young female between the second and fourth decade of life with an encapsulated tumor mass in the tail of the pancreas diagnosed at radiological investigations.[3]

Our literature searches at the time of writing this paper revealed no reported case of pancreatic pseudopapillary tumor in Nigeria. Hence, we report the case management of two adult females managed for pseudopapillary pancreatic tumors in our institution.


  Case reports Top


Case 1

A 34-year-old woman presented to the surgical outpatient department of our institution in November 2016 on account of a long-standing (>10 years) epigastric/recurrent left upper abdominal quadrant pain with duration over a decade. Abdominal pain was dull in nature, nondistressing, and nonmigratory. No known aggravating or relieving factors. No other gastrointestinal or genitourinary symptoms and no associated weight loss were observed. She had no medical comorbidity and no previous abdominal surgery. She was previously managed by the gastroenterology unit as a case of chronic pancreatitis. A referral was made to the surgical team on account of her abdominal computed tomographic (CT) scan and abdominal magnetic resonance imaging (MRI) findings, which revealed a lobulated calcified mass of the tail of the pancreas [Figure 1]. The estimated tumor size on the MRI was 20 mm x 24 mm. Her physical examination was unremarkable. The findings of the laboratory blood investigations were within the normal range.
Figure 1: Abdominal CT scan showing a calcified pancreatic tail mass

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She had an elective spleen-preserving distal pancreatectomy about 4 months after presentation. Intraop findings included pancreatic tail mass with fat straddling just right to the splenic hilum. Two pints of blood were transfused postoperation on account of reactionary hemorrhage and peripancreatic hematoma. She was discharged home 6 days after surgery. Left hypochondrial pain from peripancreatic hematoma was managed conservatively.

Histology of resected pancreatic tail mass was initially reported as pancreatic adenocarcinoma. Further review with immunohistochemistry showed positivity to chromogranin, neuron specific enolase (NSE), BerEP4, MIB-1, and Beta-catenin. A confirmatory diagnosis of an SPT of the pancreas was made. The patient had been followed up till date with no clinical or radiological features of recurrence.

Case 2

A 22-year-old woman presented with a 5-year history recurrent epigastric pain radiating to the left side of the back and aggravated by strenuous activities. She also complained of recurrent constipation alternating with diarrhea. No vomiting, hematochezia, melena, anal protrusion, anorexia, or weight loss. She had no medical comorbidity and no previous abdominal surgery.

Her physical examination revealed a large nontender left hypochondrial mass with no definable upper border. She was initially managed by the medical gastroenterologists for peptic ulcer-related dyspepsia. Her gastroscopy and biopsy revealed gastritis with a histopathology report of nonactive, nonatrophic, and chronic HLO-associated gastritis. She was subsequently referred to us based on her abdominal CT scan showing 13 cm by 10 cm distal pancreatic tumor [Figure 2]. Her blood investigations were within the normal limit. She had an open partial (distal) pancreatectomy and en-bloc splenectomy for the large pancreatic tail mass [Figure 3]. Histology of excised mass revealed pseudopapillary tumor of the pancreas. This diagnosis was further confirmed by the tumor positivity to Vimentin and Progesterone receptor (PgR) on immunohistochemistry. Patient had been followed up till date without recurrence.
Figure 2: Abdominal MRI showing a large solid pseudopapillary tumor of the pancreas

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Figure 3: A well-encapsulated solid pseudopapillary tumor of the pancreas arising from the tail of the pancreas

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  Discussion Top


In 1959 Frantz described a rare solid/cystic papillary tumor of the pancreas with low-grade malignant potential occurring typically in young females and curable by surgical resection.[2] SPT typically involves the tail of the pancreas but may also be found in other regions of the pancreas. SPT was originally coined Frantz’s tumor but was renamed by the World Health Organization (WHO) as SPT of the pancreas in 1996.[4] To the best of our knowledge, the two SPT cases being reported are probably the first in Nigeria.

The rarity of SPT limits information on the epidemiology of this lesion. Most available series on SPT of the pancreas were in case reports and case series involving an average of nine patients most of whom were females.[5] In two systematic reviews, SPT was reported to be nine times as common in females compares to males.[2],[5] Similar to the findings in these reports, the two cases being reported were young females in the twenties (case 2) and thirties (case 1), respectively.

Clinical features of SPT are mostly described as nonspecific.[2] Many cases of SPT had been diagnosed in otherwise asymptomatic patients during routine examination with abdominal CT scan.[6] The long duration of symptoms of the two cases presented tends to suggest an indolent nature of SPT. Recurrent upper gastrointestinal symptoms like epigastric and left hypochondrial pains were principal symptoms in both patients. The second patient was initially managed for suspected peptic ulcer disease until endoscopy and CT of the abdomen revealed the diagnosis.

Tumor size and characteristics of SPT vary. The two patients presented could be described as representing extremes of tumor dimensions in SPTs.[7] There was no proportionality between the duration of symptoms and tumor size as the first patient with a longer duration of symptoms had a smaller tumor. Tumor calcification seen on abdominal CT scan in the first case is characteristic of an atypical type of SPT and is found in small-sized (≤3 cm) sharply marginated tumors[6] [Figure 1]. Preoperative tissue diagnosis of SPT using Endoscopic Ultrasound-Fine Aspiration (EUS-FNA) with immunohistochemistry has been reported.[5],[6] This modality of investigation is not available in our setting.

Surgery is the mainstay of treatment for SPTs. Curative surgical resection is feasible in most patients with localized SPTs.[2] The type and extent of surgery for SPTs are dictated by the location of the tumor, local infiltration of surrounding structures, and intraoperative frozen section findings.[7],[8] In most cases, SPTs have good prognosis.[8],[9] Procedures ranging from tumor enucleation (pancreatic head SPT in children) to Whipples’ pancreaticoduodenectomy and de-bulking for unresectable/metastatic disease have been described.[2] Spleen-sparing distal pancreatectomy was feasible in the first case because of the small size (≤3 cm) of the tumor. Our surgical experience and follow-up of these two cases had resulted in a favorable outcome. We were also able to show the feasibility of spleen preservation at surgery for small-sized lesions.[2] However, in patients with advanced/metastatic disease, there were reports of partial response and improved quality of life with chemotherapy and radiotherapy prior to surgical resection in selected patients.[2],[10]


  Conclusion Top


SPTs in the two patients presented were indolent localized pancreatic tumors with typical and atypical features on radiological imaging. Clinical features in both cases were non-specific. Abdominal CT scan was invaluable in making the pre-operative localization and diagnosis of SPT in both cases. SPTs in the two patients were amenable to complete surgical resection. Definitive diagnosis was made by immunohistochemistry.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Klimstra DS, Pitman MB, Hruban RH An algorithmic approach to the diagnosis of pancreatic neoplasms. Arch Pathol Lab Med 2009;133:454-64.  Back to cited text no. 1
    
2.
Papavramidis T, Papavramidis S Solid pseudopapillary tumors of the pancreas: Review of 718 patients reported in English literature. J Am Coll Surg 2005;200:965-72.  Back to cited text no. 2
    
3.
Choi JY, Kim MJ, Kim JH, Kim SH, Lim JS, Oh YT, et al. Solid pseudopapillary tumor of the pancreas: Typical and atypical manifestations. AJR Am J Roentgenol 2006;187:W178-86.  Back to cited text no. 3
    
4.
Bhutani N, Kajal P, Singla S, Sangwan V Solid pseudopapillary tumor of the pancreas: Experience at a tertiary care centre of Northern India. Int J Surg Case Rep 2017;39:225-30.  Back to cited text no. 4
    
5.
Law JK, Ahmed A, Singh VK, Akshintala VS, Olson MT, Raman SP, et al. A systematic review of solid-pseudopapillary neoplasms: Are these rare lesions? Pancreas 2014;43:331-7.  Back to cited text no. 5
    
6.
Baek JH, Lee JM, Kim SH, Kim SJ, Kim SH, Lee JY, et al. Small (<or=3 cm) solid pseudopapillary tumors of the pancreas at multiphasic multidetector Ct. Radiology 2010;257:97-106.  Back to cited text no. 6
    
7.
Yao J, Song H A review of clinicopathological characteristics and treatment of solid pseudopapillary tumor of the pancreas with 2450 cases in Chinese Population. Biomed Res Int 2020;2020: 2829647.  Back to cited text no. 7
    
8.
Lanke G, Ali FS, Lee JH Clinical update on the management of pseudopapillary tumor of pancreas. World J Gastrointest Endosc 2018;10:145-55.  Back to cited text no. 8
    
9.
Shuja A, Alkimawi KA Solid pseudopapillary tumor: A rare neoplasm of the pancreas. Gastroenterol Rep (Oxf) 2014;2:145-9.  Back to cited text no. 9
    
10.
Czarnecka AM, Moszczuk B, Korniluk J, Nowak-Dement A, Szczylik C Chemotherapy of pancreatic solid pseudopapillary carcinoma: A case report and a literature review. Cancer Treat Commun 2016;7:47-51.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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