Pancreatic Adenocarcinoma in a Patient with IgG4-Related Autoimmune Pancreatitis: Diagnostic Challenge

IgG4 Pancreatitis vs Cancer: A Steroid-Refractory Case

Authors

  • Letícia Vieira dos Santos Ribeiro Universidade Federal de Santa Catarina (UFSC), Curso de Graduação em Medicina. Florianópolis (SC), Brasil. https://orcid.org/0009-0001-5934-8488
  • Vítor Paulino dos Santos Universidade Federal de Santa Catarina (UFSC), Curso de Graduação em Medicina. Florianópolis (SC), Brasil. https://orcid.org/0009-0008-6335-796X
  • Gilberto Kremer Universidade Federal de Santa Catarina (UFSC), Hospital Universitário Polydoro Ernani de São Thiago, Divisão do Aparelho Digestivo. Florianópolis (SC), Brasil. https://orcid.org/0009-0001-3330-1403
  • Flávia Cristina de Novaes Gerber Universidade Federal de Santa Catarina (UFSC), Hospital Universitário Polydoro Ernani de São Thiago, Serviço de Anatomia Patológica. Florianópolis (SC), Brasil. https://orcid.org/0009-0002-2184-9831
  • Leandro Marins Universidade Federal de Santa Catarina (UFSC), Hospital Universitário Polydoro Ernani de São Thiago, Serviço de Anatomia Patológica. Florianópolis (SC), Brasil. https://orcid.org/0009-0009-6441-0758
  • Fabiana Barroso Thomaz Universidade Federal de Santa Catarina (UFSC), Hospital Universitário Polydoro Ernani de São Thiago, Serviço de Radiologia. Florianópolis (SC), Brasil. https://orcid.org/0000-0003-4020-086X
  • Leonardo de Lucca Schiavon Universidade Federal de Santa Catarina (UFSC), Departamento de Clínica Médica. Florianópolis (SC), Brasil. https://orcid.org/0000-0003-4340-6820
  • Janaína Luz Narciso-Schiavon Universidade Federal de Santa Catarina (UFSC), Departamento de Clínica Médica. Florianópolis (SC), Brasil https://orcid.org/0000-0002-6228-4120

DOI:

https://doi.org/10.32635/2176-9745.RBC.2025v71n4.5240

Keywords:

Autoimmune Pancreatitis, Adrenal Cortex Hormones/therapeutic use, Pancreatic Neoplasms

Abstract

Introduction: IgG4-related autoimmune pancreatitis (AIP) type 1 is a fibroinflammatory condition. An atypical case of pancreatic adenocarcinoma arising in a patient with IgG4-related AIP, which progressed despite corticosteroid therapy, raising the hypothesis of a missed malignancy at initial presentation or tumor development in the setting of chronic inflammation is reported. Case report: A 46-year-old male presented with abdominal pain, fever, and jaundice. Imaging revealed a pancreatic head mass with calcifications, pancreatic tail atrophy, and peripancreatic lymphadenopathy, raising suspicion of malignancy. During hospitalization for diagnostic workup, the patient developed cholangitis. Magnetic resonance cholangiopancreatography revealed a common bile duct stricture, managed with endoscopic retrograde cholangiopancreatography and biliary stent placement. An endoscopic ultrasound-guided biopsies were non-diagnostic. An exploratory laparotomy with open biopsy confirmed IgG4-related AIP. Despite corticosteroid therapy, the patient showed clinical deterioration with worsening abdominal pain, progressive biliary strictures, and significant weight loss. During follow-up, biliary stent exchanges became increasingly difficult due to stricture progression. Another endoscopic ultrasound-guided biopsy ultimately confirmed pancreatic adenocarcinoma. The patient was referred for palliative chemotherapy. Conclusion: The diagnosis of IgG4-related pancreatitis must be carefully established, and lack of treatment response warrants diagnostic reevaluation, as in this case, where it ultimately represented pancreatic malignancy.

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Published

2025-06-30

How to Cite

1.
Ribeiro LV dos S, Santos VP dos, Kremer G, Gerber FC de N, Marins L, Thomaz FB, Schiavon L de L, Narciso-Schiavon JL. Pancreatic Adenocarcinoma in a Patient with IgG4-Related Autoimmune Pancreatitis: Diagnostic Challenge: IgG4 Pancreatitis vs Cancer: A Steroid-Refractory Case. Rev. Bras. Cancerol. [Internet]. 2025 Jun. 30 [cited 2025 Dec. 5];71(4):e-045240. Available from: https://rbc.inca.gov.br/index.php/revista/article/view/5240

Issue

Vol. 71 No. 4 (2025): oct./nov./dec.

Section

CASE REPORT

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