Pancreatoblastoma in previously pancreatic pseudocysts in a 14-year-old female: a case report
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.
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/β-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, Şahin TT, Gündoğdu 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
Copyright (c) 2023 Monica Bellynda, Marsih, Yohanes Adinugroho, Suwardi, Muhammad Riza, Faizal Muhammad
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with Medical Journal of Indonesia agree to the following terms:
- Authors retain copyright and grant Medical Journal of Indonesia right of first publication with the work simultaneously licensed under a Creative Commons Attribution-NonCommercial License that allows others to remix, adapt, build upon the work non-commercially with an acknowledgment of the work’s authorship and initial publication in Medical Journal of Indonesia.
- Authors are permitted to copy and redistribute the journal's published version of the work non-commercially (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in Medical Journal of Indonesia.