Clinical Journal of Gastroenterology

, Volume 11, Issue 2, pp 161–166 | Cite as

Pancreatoblastoma: an unusual diagnosis in an adult patient

  • Gonçalo Nunes
  • Hélder Coelho
  • Marta Patita
  • Rita Barosa
  • Pedro Pinto Marques
  • Lídia Roque Ramos
  • Maria José Brito
  • Aurora Tomaz
  • Jorge Fonseca
Case Report
  • 90 Downloads

Abstract

The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis. The patient underwent an endoscopic ultrasound (EUS) with fine needle aspiration (FNA) from the pancreatic mass and liver metastasis with cytological evaluation consistent with a pancreatoblastoma, later confirmed through a percutaneous US-guided liver biopsy. During the inpatient period, liver function deteriorated and acute kidney injury developed. Severe progressive cachexia was observed. The patient was discharged on renal replacement therapy and palliative care. Death occurred 3 months after diagnosis. Pancreatoblastoma is an uncommon pancreatic malignant epithelial cancer of the pancreas, typically occurring in the paediatric population. Adult pancreatoblastoma is extremely rare, with about 40 cases reported in the literature and generally presenting a more aggressive biologic and clinical behaviour. Surgical resection is the treatment of choice, but most cases are detected in advanced stages. This case underlines the ability to establish a pancreatoblastoma cytology-based diagnosis with EUS-FNA, and confirms the associated poor outcome.

Keywords

Adult pancreatoblastoma Fine needle aspiration Cytology 

Notes

Acknowledgements

There are no acknowledgements regarding this manuscript.

Compliance with ethical standards

Conflict of interest

Gonçalo Nunes, Hélder Coelho, Marta Patita, Rita Barosa, Pedro Pinto Marques, Lídia Roque Ramos, Maria José Brito, Aurora Tomaz and Jorge Fonseca declare that they have no Conflict of interest.

Human rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Not applicable.

References

  1. 1.
    Savastano S, d’Amore ES, Zuccarotto D, et al. Pancreatoblastoma in an adult patient. A case report. JOP. 2009;10(2):192–5.PubMedGoogle Scholar
  2. 2.
    Naik VR, Jaafar H, Leow VM, et al. Pancreatoblastoma: a rare tumour accidentally found. Singapore Med J. 2006;47(3):232–4.PubMedGoogle Scholar
  3. 3.
    Zouros E, Manatakis DK, Delis SG, et al. Adult pancreatoblastoma: a case report and review of the literature. Oncol Lett. 2015;9(5):2293–8.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Zhang D, Tang N, Liu Y, et al. Pancreatoblastoma in an adult. Indian J Pathol Microbiol. 2015;58(1):93–5.  https://doi.org/10.4103/0377-4929.151199.CrossRefPubMedGoogle Scholar
  5. 5.
    Du E, Katz M, Weidner N, et al. Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature. Arch Pathol Lab Med. 2003;127(11):1501–5.PubMedGoogle Scholar
  6. 6.
    Charlton-Ouw KM, Kaiser CL, Tong GX, et al. Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. JOP. 2008;9(6):733–8.PubMedGoogle Scholar
  7. 7.
    Hammer ST, Owens SR. Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. Arch Pathol Lab Med. 2013;137(9):1224–6.  https://doi.org/10.5858/arpa.2013-0272-CR.CrossRefPubMedGoogle Scholar
  8. 8.
    Vilaverde F, Reis A, Rodrigues P, et al. Adult pancreatoblastoma—case report and review of literature. J Radiol Case Rep. 2016;10(8):28–38 (eCollection 2016 Aug).PubMedPubMedCentralGoogle Scholar
  9. 9.
    Salman B, Brat G, Yoon YS, 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.CrossRefPubMedGoogle Scholar
  10. 10.
    Cavallini A, Falconi M, Bortesi L, et al. Pancreatoblastoma in adults: a review of the literature. Pancreatology. 2009;9(1–2):73–80.  https://doi.org/10.1159/000178877.CrossRefPubMedGoogle Scholar
  11. 11.
    Benoist S, Penna C, Julié C, et al. Prolonged survival after resection of pancreatoblastoma and synchronous liver metastases in an adult. Hepatogastroenterology. 2001;48(41):1340–2.PubMedGoogle Scholar
  12. 12.
    Rajpal S, Warren RS, Alexander M, et al. Pancreatoblastoma in an adult: case report and review of the literature. J Gastrointest Surg. 2006;10(6):829–36.CrossRefPubMedGoogle Scholar
  13. 13.
    Crawford JM. Gallbladder, extrahepatic biliary tract, and pancreas tissue processing and normal histology. In: Odze RD, Goldblum JR, editors. Odze&Goldblum surgical pathology of the GI tract, liver, biliary tract and pancreas. Saunders 2014, Philadelphia, p 968.Google Scholar

Copyright information

© Japanese Society of Gastroenterology 2017

Authors and Affiliations

  • Gonçalo Nunes
    • 1
  • Hélder Coelho
    • 2
  • Marta Patita
    • 1
  • Rita Barosa
    • 1
  • Pedro Pinto Marques
    • 1
  • Lídia Roque Ramos
    • 1
  • Maria José Brito
    • 2
  • Aurora Tomaz
    • 3
  • Jorge Fonseca
    • 1
    • 4
  1. 1.Gastroenterology DepartmentHospital Garcia de OrtaAlmadaPortugal
  2. 2.Pathology DepartmentHospital Garcia de OrtaAlmadaPortugal
  3. 3.Palliative Care Team, Hospital Garcia de OrtaAlmadaPortugal
  4. 4.CiiEM, Centro de Investigação Interdisciplinar Egas MonizMonte da CaparicaPortugal

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