Disguised as a tumor

An 81-year-old female patient was admitted due to persistent deterioration in her general condition, weight loss (15 kg in the last approximately four months), dysphagia and increasing dyspnea.

This was preceded by three long outpatient hospitalizations, first for complicated choledocholithiasis, then for purulent cholangitis and, most probably, for benign distal stenosis of the hepatocholedochal duct (HCD). Finally, septic shock occurred in which Klebsiella pneumoniae could be detected in blood culture and multiple small liver abscesses with hypodense liver lesions were suspected to have occurred during the course.

There was a history of severe bronchial asthma and long-standing hypothyroidism. In 2014, cholecystectomy and three endoscopic stone extractions for cholecystitis and choledocholithiasis were performed.

Normal vital signs

Current findings: Physically, there were no significant findings other than a significantly reduced general condition. Vital signs were normal and there was no fever. In the laboratory, only moderately increased inflammatory parameters were observed (leukocytes 10.2, CRP 56.6 mg/l, ferritin 864 ng/ml), as well as a slight increase in alkaline phosphatase (AP 190 U/l) and gamma GT (GGT 70 U/l). The chest radiograph was unremarkable.

The main sonographic finding was a central, hypoechoic, solid-appearing hepatic lesion measuring 10 × 4 cm with multiple hypoechoic satellites (see Figure 1). There was no cholestasis. Immediately following contrast-enhanced sonography showed (iso-)contrast enhancement of the arterial lesion (see image 2a) with a rapid onset of hypocontrast in the portal venous and late phases (see image 2b).

Thus, at the first clinical and imaging synopsis, advanced tumor disease was suspected, most likely an extensive cholangiocellular carcinoma (CCC). Although there were doubts about the feasibility of chemotherapy due to the greatly reduced general condition, a diagnostic liver puncture (test excision, EP) was arranged with the patient.

Liver biopsy: Liver biopsy surprisingly revealed a granulomatous inflammatory reaction of epithelioid cells with extensive necrosis, highly suspicious for tuberculosis (TB). Infiltrates of a malignant tumor were not detectable (see Figure 3).

A diagnosis of TB was made, but there was no evidence of tuberculosis infection in either the imaging studies, the Quantiferon test, or the gastric juice. A repeat liver EP for molecular pathological examination for mycobacteriosis was also negative.

Tumor invasion into the duodenum

Because of dysphagia, gastroscopy was performed before obtaining liver histology. If the esophagus was normal, tumor invasion into the duodenum was suspected, as was dislocation and partial occlusion of the bile duct stent. Endoscopic retrograde cholangiopancreatography (ERCP) was then performed, which resulted in hemobilia with hemorrhagic shock (see Figure 4).

Tako-Tsubo cardiomyopathy also developed and the patient had to be treated in the intensive care unit for several days. Histologically, the supposed tumor invasion later appeared to be duodenitis with eosinophilia.

Tumor turns into rheumatism: After repeated liver scans showed no evidence of mycobacteriosis, granulomatous inflammation continued to be the main finding. Small lung densities had previously been seen on computed tomography (CT) but were now enlarging and were classified as granulomas (see Figure 5).

When sarcoidosis was suspected, the patient was transferred to rheumatology at the Ludwigshafen Clinic. Here, an infectious cause of the granulomas (bacteria, parasites, protozoa) was ruled out serologically, additional environmental diagnostics (CT of the paranasal sinuses, ophthalmological examination), bronchoalveolar lavage (BAL) with a lymphocytic pattern and finally the detection of eosinophilia (normal in our admission laboratory).

Analyzing the findings together, the diagnosis of “eosinophilic granulomatosis with polyangiitis” was made. This disease, previously known as Churg-Strauss syndrome, is a small vessel vasculitis associated with antineutrophil cytoplasmic antibody (ANCA) with extravascular infiltrates, usually predominantly in the lungs.

According to the criteria of the American College of Rheumatology and the European Alliance of Associations for Rheumatology, at least six points are required for the diagnosis of “eosinophilic granulomatosis with polyangiitis”, provided that vasculitis is present.

In addition to the liver, the sites of manifestation in our patient’s case were the lungs (long-standing asthma, multiple pulmonary nodules), peripheral eosinophilia, and the duodenum. Cardiac magnetic resonance imaging (MRI) did not reveal any evidence of cardiac involvement, which often determines the prognosis.

Therapy with systemic steroids and methotrexate was initiated. With this medication, the patient improved rapidly, although complications with Pneumocystis jirovecii pneumonia subsequently developed.

Conclusion for practice:

Liver involvement with tumor-mimicking infiltrates is an uncommon and very rare manifestation of eosinophilic granulomatosis with polyangiitis. Eosinophilia only appeared later in the course of the disease, an eosinophilic infiltrate had only been observed in the small intestine and was not considered relevant;

For a long time, the gastroenterological focus was very restricted to the bile ducts, but the findings (purulent cholangitis, gram-negative bacteria in the blood culture, supposed hepatic microabscesses, then a large tumor lesion) were both impressive and plausible.

In retrospect, it can be discussed whether the long-standing history of bronchial asthma and the lung lesions already described on previous CT scans should not have suggested a systemic disease earlier, but the procedure probably would not have made a decisive difference.

Fortunately, liver puncture (less invasive) was performed despite the apparently unfavorable situation.