Pediatric Pancreatoblastoma: A Case Report

Document Type : case report

Authors

1 Department of Radiology, Emam Reza Hospital, Tabriz University of Medical Sciences, Tabriz, Iran

2 Pediatric Research Center, Children hospital, Medical Faculty, Tabriz University of Medical Sciences, Tabriz, Iran

3 Department of Pathology, Tabriz University of Medical Sciences, Tabriz, Iran

4 Medical Radiation Sciences Research Group, Tabriz University of Medical Sciences, Tabriz, Iran

5 Department of Radiology, Zanjan University of Medical Sciences, Zanjan, Iran

6 Medical student, student research committee, Tabriz University of Medical Sciences, Tabriz, Iran

Abstract

Background: Pancreatoblastoma (PB) is an exceedingly uncommon pancreatic tumor arising from pancreatic exocrine cells. It is the most common malignant pancreatic tumor in childhood.
Case presentation: We report a case of PB in a 5-year-old girl who presented to our institution with severe abdominal pain, anorexia, vomiting, and jaundice. Laboratory tests were compatible with cholestasis. Ultrasound imaging showed mild intra- and extrahepatic bile duct dilation as well as a pancreatic mass. A Computed Tomography (CT) scan confirmed a large mass in the head of the pancreas, which was associated with periportal lymphadenopathy as well as anterior and inward displacement of superior mesenteric vessels. Although the mass was unresectable at the time of admission to our center, an open biopsy of the tumor was performed, which revealed a diagnosis of PB. Following six months of neoadjuvant chemotherapy, the size of the tumor was dramatically decreased, allowing the complete resection of it.
Conclusion: When a child presents with a massive solid cystic tumor in the pancreas, the possibility of pancreatoblastoma must be considered. Surgery is utilized to completely remove the tumor, while pathology and immunohistochemistry are used to confirm the diagnosis. Patients with huge tumors or extensive lymphadenopathy typically need neoadjuvant chemotherapy before surgery to downstage their disease.

Keywords

References

  1. Huang Y, Yang W, Hu J, Zhu Z, Qin H, Han W, Wang H. Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. Pediatr Surg Int. 2019; 35(11):1231-8.
  2. Chisholm KM, Hsu CH, Kim MJ, Rangaswami A, Gray Hazard FK. Congenital pancreatoblastoma: report of an atypical case and review of the literature. J Pediatr Hematol Oncol. 2012; 34(4):310-5.
  3. Dhebri AR, Connor S, Campbell F, Ghaneh P, Sutton R, Neoptolemos JP. Diagnosis, treatment and outcome of pancreatoblastoma. Pancreatology. 2004; 4(5):441-51; discussion 52-3.
  4. Lachiewicz AM, Berwick M, Wiggins CL, Thomas NE. Survival Differences between Patients with Scalp or Neck Melanoma and Those with Melanoma of Other Sites in the Surveillance, Epidemiology, and End Results (SEER) Program. Archives of Dermatology. 2008; 144(4):515-21.
  5. Liu T, Zhao T, Shi C, Chen L. Pancreatoblastoma in children: Clinical management and literature review. Translational Oncology. 2022; 18:101359.
  6. Vasireddi AK, Leo ME, Squires JH. Magnetic resonance imaging of pediatric liver tumors. Pediatr Radiol. 2022; 52(2):177-88.
  7. Zhu AX, Dayyani F, Yen CJ, Ren Z, Bai Y, Meng Z, Pan H, Dillon P, Mhatre SK, Gaillard VE, Hernandez S, Kelley RK, Sangro B. Alpha-Fetoprotein as a Potential Surrogate Biomarker for Atezolizumab + Bevacizumab Treatment of Hepatocellular Carcinoma. Clin Cancer Res. 2022; 28(16):3537-45.
  8. Li J, Peng C, Fan X, Wang L, Wang J. Adult pancreatoblastoma: a case report. J Int Med Res. 2021; 49(8):3000605211039565.
  9. Sheng M, Zhang R, Ma X, Zhou H. CT manifestations of childhood pancreatoblastoma. World Journal of Pediatric Surgery. 2022; 5(3):e000398.
  10. Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: imaging findings in 10 patients and review of the literature. Radiology. 2000; 214(2):476-82.
  11. Shet NS, Cole BL, Iyer RS. Imaging of Pediatric Pancreatic Neoplasms with Radiologic-Histopathologic Correlation. American Journal of Roentgenology. 2014; 202(6):1337-48.
  12. Zhang X, Ni S-j, Wang X-h, Huang D, Tang W. Adult pancreatoblastoma: clinical features and Imaging findings. Scientific Reports. 2020; 10(1):11285.
  13. Chung EM, Travis MD, Conran RM. Pancreatic Tumors in Children: Radiologic-Pathologic Correlation. RadioGraphics. 2006; 26(4):1211-38.
  14. Suemitsu Y, Ono Y, Mizukami Y, Ye J, Yamakawa K, Takamoto T, Nakano-Narusawa Y, Mukai Y, Takamatsu M, Nakazawa A, Mino-Kenudson M, Kumasaka T, Matsuda Y. A Case of Adult Pancreatoblastoma with Novel APC Mutation and Genetic Heterogeneity. Frontiers in Oncology. 2021; 11.
  15. Imamura A, Nakagawa A, Okuno M, Takai S, Komada H, Kwon AH, Uetsuji S, Kamiyama Y, Sakaida N, Okamura A. Pancreatoblastoma in an Adolescent Girl: Case Report and Review of 26 Japanese Cases. The European Journal of Surgery. 1998; 164(4):309-12.
  16. Qiu L, Trout AT, Ayyala RS, Szabo S, Nathan JD, Geller JI, Dillman JR. Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation. RadioGraphics. 2021; 41(6):1766-84.
  17. Rosenbaum DG, Abramson SJ, DeLappe E, Teruya-Feldstein J, La Quaglia MP, Fox JJ, Price AP. Pancreatic Involvement in Neuroblastoma With Radiologic-Pathologic Correlation: A Single-Institution Experience. American Journal of Roentgenology. 2013; 201(1):W141-W6.
  18. Galgano S, Royal S. Primary pancreatic neuroblastoma presenting with opsoclonus-myoclonus syndrome. Radiol Case Rep. 2016; 11(1):36-40.
  19. Yang Z, Gong Y, Ji M, Yang B, Qiao Z. Differential diagnosis of pancreatoblastoma (PB) and solid pseudopapillary neoplasms (SPNs) in children by CT and MR imaging. Eur Radiol. 2021; 31(4):2209-17.
  20. Bien E, Roganovic J, Krawczyk MA, Godzinski J, Orbach D, Cecchetto G, Barthlen W, Defachelles AS, Ferrari A, Weldon CB, Brecht IB, Schneider DT, Bisogno G, Kolenova A, Ben-Ami T, Martinova K, Virgone C, Stachowicz-Stencel T, Kachanov D, Reguerre Y. Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatric Blood & Cancer. 2021; 68(S4):e29112.
  21. Defachelles AS, Rocourt N, Branchereau S, Peuchmaur M. [Pancreatoblastoma in children: diagnosis and therapeutic management]. Bull Cancer. 2012; 99(7-8):793-9.
Journal of Pediatric Perspectives
Volume 11, Issue 3
March 2023
Pages 17527-17536

Files

Share

How to cite

Statistics