Pancreatoblastoma in previously pancreatic pseudocysts in a 14-year-old female: a case report

Authors

  • Monica Bellynda General Surgery Residency Programme, Faculty of Medicine, Universitas Sebelas Maret, Dr. Moewardi Hospital, Surakarta, Indonesia
  • Marsih General Surgery Residency Programme, Faculty of Medicine, Universitas Sebelas Maret, Dr. Moewardi Hospital, Surakarta, Indonesia
  • Yohanes Adinugroho General Surgery Residency Programme, Faculty of Medicine, Universitas Sebelas Maret, Dr. Moewardi Hospital, Surakarta, Indonesia
  • Suwardi Department of Pediatric Surgery, Faculty of Medicine, Universitas Sebelas Maret, Dr. Moewardi Hospital, Surakarta, Indonesia
  • Muhammad Riza Department of Pediatric Hematology-Oncology, Dr. Moewardi Hospital, Faculty of Medicine, Universitas Sebelas Maret, Surakarta, Indonesia
  • Faizal Muhammad Department of Neurology, Faculty of Medicine, Universitas Sebelas Maret, Dr. Moewardi Hospital, Surakarta, Indonesia https://orcid.org/0000-0002-5607-571X

DOI:

https://doi.org/10.13181/mji.cr.236351

Keywords:

pancreas, pancreatic neoplasm, pancreatic pseudocysts, pancreatoblastoma

Abstract

Pancreatoblastoma is a rare tumor characterized by uncontrolled growth of pancreatic epithelial cells with a mix of squamous nests and acinar differentiation. Diagnostic modalities include abnormal liver enzyme, pancreatic enzyme, and imaging findings. Treatment options include surgical resection, sometimes combined with chemotherapy, depending on the tumor’s size and grade. We reported a pancreatoblastoma in a 14-year-old female with prior pancreatic pseudocysts. The transformation from pseudocysts to pancreatoblastoma is believed to be caused by the heterozygosity molecular loss on the 11p chromosome and several genetic mutations. Magnetic resonance cholangiopancreatography showed a well-defined, heterogeneous mass in the pancreatic head, with 70% of the mass composed of cysts. A partial pancreatectomy was performed because a complete pancreatectomy may harm the adjacent structures. However, a complete resection combined with chemoradiation may be the best option for long-term survival and complete remission. In this case, she was disease-free until 30 months after the chemotherapy protocol.

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References

Ellerkamp V, Warmann SW, Vorwerk P, Leuschner I, Fuchs J. Exocrine pancreatic tumors in childhood in Germany. Pediatr Blood Cancer. 2012;58(3):366-71. https://doi.org/10.1002/pbc.23211

Omiyale AO. Adult pancreatoblastoma: current concepts in pathology. World J Gastroenterol. 2021;27(26):4172-81. https://doi.org/10.3748/wjg.v27.i26.4172

Huang Y, Yang W, Hu J, Zhu Z, Qin H, Han W, et al. Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. Pediatr Surg Int. 2019;35(11):1231-8. https://doi.org/10.1007/s00383-019-04524-y

Argon A, Çelik A, Öniz H, Özok G, Barbet FY. Pancreatoblastoma, a rare childhood tumor: a case report. Turk Patoloji Derg. 2017;33(2):164-7. https://doi.org/10.5146/tjpath.2014.01268

Glick RD, Pashankar FD, Pappo A, Laquaglia MP. Management of pancreatoblastoma in children and young adults. J Pediatr Hematol Oncol. 2012;34 Suppl 2:S47-50. https://doi.org/10.1097/MPH.0b013e31824e3839

Reid MD, Bhattarai S, Graham RP, Pehlivanoglu B, Sigel CS, Shi J, et al. Pancreatoblastoma: cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. Cancer Cytopathol. 2019;127(11):708-19. https://doi.org/10.1002/cncy.22187

Agalianos C, Passas I, Sideris I, Davides D, Dervenis C. Review of management options for pancreatic pseudocysts. Transl Gastroenterol Hepatol. 2018;3:18. https://doi.org/10.21037/tgh.2018.03.03

Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, et al. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas: frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol. 2001;159(5):1619-27. https://doi.org/10.1016/S0002-9440(10)63008-8

Salman B, Brat G, Yoon YS, Hruban RH, Singhi AD, Fishman EK, et al. The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. J Gastrointest Surg. 2013;17(12):2153-61. https://doi.org/10.1007/s11605-013-2294-2

Bläker H, Hofmann WJ, Rieker RJ, Penzel R, Graf M, Otto HF. Beta-catenin accumulation and mutation of the CTNNB1 gene in hepatoblastoma. Genes Chromosomes Cancer. 1999;25(4):399-402. https://doi.org/10.1002/(SICI)1098-2264(199908)25:4<399::AID-GCC14>3.0.CO;2-X

Raoul JL, Oziel-Taieb S, Lecomte T, Adelaide J, Guille A, Chaffanet M, et al. Case report: two cases of metastatic pancreatoblastoma in adults: efficacy of folfirinox and implication of the wnt/v-catenin pathway in genomic analysis. Front Oncol. 2021;11:564506. https://doi.org/10.3389/fonc.2021.564506

Berger AK, Mughal SS, Allgäuer M, Springfeld C, Hackert T, Weber TF, et al. Metastatic adult pancreatoblastoma: multimodal treatment and molecular characterization of a very rare disease. Pancreatology. 2020;20(3):425-32. https://doi.org/10.1016/j.pan.2020.02.017

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;31:2209-17. https://doi.org/10.1007/s00330-020-07309-3

de Jong K, Bruno MJ, Fockens P. Epidemiology, diagnosis, and management of cystic lesions of the pancreas. Gastroenterol Res Pract. 2012;2012:147465. https://doi.org/10.1155/2012/147465

Joshi U, Poudel P, Ghimire RK, Basnet B. Pancreatic pseudocyst or mucinous cystadenocarcinoma of pancreas? A diagnostic dilemma. Clin Case Rep. 2017;5(4):501-4. https://doi.org/10.1002/ccr3.887

Changazi SH, Ahmed Q, Bhatti S, Siddique S, Abdul Raffay E, Farooka MW, et al. Whipple procedure: a five-year clinical experience in tertiary care center. Cureus. 2020;12(11):e11466. https://doi.org/10.7759/cureus.11466

Özmen MM, ?ahin TT, Gündo?du E. Laparoscopic distal pancreatectomy: techniques, pearl tips and pitfalls. Eur J Endosc Laparosc Surg. 2014;1(3):132-8. https://doi.org/10.5578/ejels.8734

Mahdi M, Abu Alnasr M, Almehman BA, Nassan S, Bin Yahib S. A rare case of pancreatoblastoma in a pediatric patient. Cureus. 2020;12(1):e6779. https://doi.org/10.7759/cureus.6779

Published

2023-02-27

How to Cite

1.
Bellynda M, Marsih, Adinugroho Y, Suwardi, Riza M, Muhammad F. Pancreatoblastoma in previously pancreatic pseudocysts in a 14-year-old female: a case report . Med J Indones [Internet]. 2023Feb.27 [cited 2024Nov.4];31(4):269-73. Available from: http://mji.ui.ac.id/journal/index.php/mji/article/view/6351

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Case Report/Series
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