16 April 2026
: Case report 
Granulomatosis with Polyangiitis (Wegener Granulomatosis) Presenting as a Pancreatic Mass: A Rare Malignancy Mimic
Unusual clinical course, Challenging differential diagnosis, Unusual setting of medical care
Soumyadip Sain
ABEF 1*, Subhashish Das ADF 1, Vinant Bhargava BDF 2, Sunila Jain BCD 3, Samiran Nundy BCDEF 1
DOI: 10.12659/AJCR.951707
Am J Case Rep 2026; 27:e951707
Table 1 Review of previous literature of cases reporting GPA presenting as a pancreatic mass.
| Author, year | Age/sex | Clinical features | Pancreatic presentation | Serology (C-ANCA/ P-ANCA/IgG4) | Treatment/ surgery |
|---|---|---|---|---|---|
| O’Neil et al [], 19927 | 62/M | Painless jaundice, lung mass | Head mass | C-ANCA+ | Steroids + cyclophosphamide |
| Christl et al [], 20048 | 50/F | Weight loss | Tail mass | C-ANCA+ | Distal pancreatectomy + steroids |
| Marroun et al [], 20069 | 68/F | Jaundice, sinusitis, orchitis | Head mass | C-ANCA+ | Whipple + steroids + cyclophosphamide |
| Tinazzi et al [], 200710 | 48/F | Epigastric pain | Pancreatic head mass | C-ANCA+ | Whipple procedure |
| Hamilton et al [], 201111 | 78/F | Pulmonary nodules | Not specified | C-ANCA+ | Steroids + cyclophosphamide |
| Cindoruk et al [], 2012 (Case 1)6 | 37/F | Recurrent pancreatitis | Pseudocystic lesion | Not reported | EUS-guided drainage + steroids |
| Cindoruk et al [], 2012 (Case 2)6 | 54/F | Recurrent pancreatitis | Pseudocyst | Not reported | EUS-guided drainage + steroids |
| Valerieva et al [], 2013 (Case 1)1 | 62/F | Fever, otitis, sinusitis | Tail mass | C-ANCA+ | Distal pancreatectomy + steroids + AZA |
| Valerieva et al [], 2013 (Case 2)1 | 62/F | Epigastric pain, fever | Pancreatic tail mass | C-ANCA+ | Distal pancreatectomy + steroids + AZA |
| Kontis et al [], 2014 (Case 1)5 | 57/M | Abdominal pain, nausea | Head mass | C-ANCA+ | Distal pancreatectomy + bowel resection |
| Kontis et al [], 2014 (Case 2)5 | 68/F | Abdominal pain, jaundice | Diffuse pancreatic involvement | C-ANCA+ | Biliary drainage + total pancreatectomy |
| Reddy et al [], 20174 | 20/F | RUQ pain, jaundice | Cystic-solid head mass | ANCA negative | Whipple procedure |
| Castillo et al [], 20193 | 73/M | Fever, weight loss, sinus pain, rash | Head mass (FDG-avid) | C-ANCA+ /IgG4 normal | Rituximab + steroids (no surgery) |
| Present Case, 2025 | 62/M | Fever, weight loss, hemoptysis | Tail mass with FDG uptake | C-ANCA++/ IgG4−/C3/C4 high | Rituximab + steroids (no surgery) |
| GPA – granulomatosis with polyangiitis; C-ANCA – cytoplasmic anti-neutrophil cytoplasmic antibody; P-ANCA – perinuclear anti-neutrophil cytoplasmic antibody; IgG4 – immunoglobulin G4; EUS – endoscopic ultrasound; AZA – azathioprine; RUQ – right upper quadrant; FDG – fluorodeoxyglucose; M – Male; F – Female. | |||||