CASE REPORT
A 74 years old male had previously presented to a different hospital with a painless jaundice and weight loss of 10kg without additional symptoms. The patient had a medical history of heavy smoking, hypertension, hyperlipidaemia, insulin dependent diabetes mellitus and a simultaneous carcinoma of the bladder and prostate followed by prostatectomy and cystectomy with urinary bladder reconstruction 15 years ago. His family history was notable for a brother who died of an acute leukaemia at the age of 9 years, a brother with lung cancer at the age of 60 and a father with an adenocarcinoma of the prostate at the age of 75. On physical examination, the patient was thin, with apparent jaundice but without lymphadenopathy or organomegaly. In the laboratory blood tests, an increased tumour marker CA 19-9 (757 U/ml, normal reference 0-37 U/ml) was present. A whole body computer tomography (CT) scan revealed a space occupying lesion (SOL) in the head of the pancreas with a regional lymphadenopathy.
The patient underwent a Whipple procedure. Histo-pathology (HP) and immune-histochemistry (IHC) examinations from the SOL biopsy revealed a chronic lympho-plasmocytic infiltrate with a storiform pattern and a fibrotic inflammatory infiltrate prominent with eosinophils and plasma cells with an immune-stain positive for IgG4. No evidence of malignancy was found. A repeated revision of the biopsy that was performed at a different medical center has confirmed these findings. After the operation, serum IgG4 and CA-19-9 levels were within the normal limits. The diagnosis of autoimmune pancreatitis (AIP) type 1was established.
Eight months after the procedure, the patient was referred to our hospital for a routine follow-up. Physical examination was unremarkable. Blood tests showed normal levels of IgG4 and CA-19-9, and liver enzymes levels were slightly elevated but similar to the levels that were detected before the development of jaundice (Table 1). He underwent a positron emission tomography (PET-FDG) scan that found no signs of disease. One year after the operation, a routine abdominal magnetic resonance imaging (MRI) has displayed a SOL with cystic component of 20 mm in the tail of the pancreas with a contrast enhancement (Figure 1A). The patient underwent an endoscopic ultrasound (EUS) followed by a biopsy utilizing a fine needle aspiration (FNA) from the SOL which was not conclusive. A surgical removal of the tail of the pancreas was recommended, but the patient refused further investigation. Three months later, the patient reported a gradual weight loss of 5kg without additional complaints. A follow-up PET-FDG scan showed a hyper-metabolic consolidation in the tail of the pancreas, and an increase of the SOL to 24 mm was seen on additional MRI (Table 1). Since the patient did not consent to additional procedures and the differential diagnosis included recurrence of IgG4-RD or malignancy, empiric treatment with prednisone at a dosage of 0.5 mg/kg/day was started. At this time point, CA-19-9 and IgG4 in serum were still within the normal limits. A month later, he had a MRI which detected an increase in the size of the known SOL to 29 mm and several new consolidations in the liver suspected to be liver metastases, that were confirmed by PET-FDG (Figure 1B, Figure 2). Steroid treatment was discontinued. HP of the biopsy from the liver lesions revealed a chronic inflammation without evidence of malignancy. IgG4 immuno-stain was negative. For the first time, increased CA 19-9 levels were documented (Table 1). An additional EUS of the tail of the pancreas with FNA has revealed poorly differentiated adenocarcinoma. The patient refused further treatment with chemotherapy and immunotherapy and followed a conservative management. Afterwards, he was lost to follow-up.