BENIGN MASQUERADE OF A MALIGNANT LESION-WHAT THE MIND DOES NOT KNOW , THE EYES DO NOT SEE!!
Deepti Ramachandra*1, Puneet Dhar1, Gourav Kaushal2, Nirjhar R. Rakesh1, Abhishek Agrawal1, Satya Vati Rana1, Anvin Mathew1, Anuj Goyal1, Mithun Nariampalli Karthyarth1
1Surgical Gastroenterology, All India Institute Of Medical Sciences , Rishikesh, Rishikesh, Uttarakhand, India; 2All India Institute of Medical Sciences - Bathinda, Bathinda, India
Background: Immunoglobulin G4 (IgG4) is a minor subtype of immunoglobulin , accounting for about 3-6% of all circulating immunoglobulins IgG. It is associated with a rare multiorgan fibroinflammatory disorder called immunoglobulin G4 (IgG4) – related disease (IgG4-RD), characterized by lymphoplasmacytic infiltrate with abundant IgG4 -positive plasma cells and storiform fibrosis . Differentiating IgG4-RD from malignant process poses a diagnostic challenge as IgG4 immune related reaction involves some malignant lesions of pancreas, biliary tree, gall bladder and ampullary region. Malignant process poses a diagnostic challenge as IgG4 immune-related reaction involves some malignant lesions of the pancreas, biliary tree, gall bladder, and ampullary region. In addition, carcinogenesis upregulates regulatory T cells ( T-reg), producing interleukin 10 (IL-10) eliciting IgG4 reaction, suggesting similar pathogenesis in IgG4 RD and carcinoma.
Case Report: An elderly gentleman presented with painless progressive obstructive jaundice for six months with associated anorexia , weight loss, and, occasionally low-grade fever spikes.Contrast computed tomography of abdomen( CECT triple phase) revealed a heterogenously enhancing hilar block. Initial management of cholangitis constituted antibiotics , endoscopic biliary drainage. Bile cytology did not reveal malignant cells, and biopsy from lesion was negative for malignancy . But however, serum IgG4 level was elevated 750mg/dl ( normal range 80-135mg/dl). The patient was planned for a short trial of steroids in view of elevated IgG4 after the resolution of cholangitis. But however, he was lost to follow up. Four months later , he presented again to outpatient and was re-evaluated with a CECT triple phase which showed a similar hilar lesion with suspected omental nodules. Fine needle aspiration from the omental nodule revealed adenocarcinoma. In view of disseminated malignancy patient was referred for palliative therapy.
Conclusion: Distinguishing cholangiocarcinoma from IgG4-RD is difficult yet is pivotal to avoid grave consequences . Serum IgG4 level confers a poor sensitivity and specificity. Therefore, the peritumoral infiltrate of IgG4 seen in cholangiocarcinoma and pancreatic carcinoma must be interpreted cautiously. Serum IgG4 elevation can be misleading and misinterpreted in inexperienced hands . However, a good clinical correlation, radiological interpretation, histopathological assessment for typical storiform fibrosis along with IgG4 positive cells, and response to a trial of steroids can point towards benign diagnosis rather than a malignant one.
Back to 2022 Posters