Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory and idiopathic autoimmune disorder. IgG4-RD can be characterized by the presence of pseudotumors. Inflammatory pseudotumors may involve any part of a human organ. There are few reports of sinus lesions in IgG4-RD. An 82-year-old man has a history of chronic sinusitis for the last several years and no remarkable family history. Two years before disease presentation, the patient experienced intermittent nasal bleeding, stuffy nose, dizziness, and fatigue. Blood test revealed positive (160X) antinuclear antibody with a mixed speckled and nucleolar pattern, IgG level of 1370 mg/dL, and IgG4 level of 99.7 mg/dL. Computed tomography (CT) of the sinus revealed several calcifications in the sphenoid sinus. Surgical findings revealed tumor-like materials. Pathological examination of the soft tissues revealed acute and chronic granulomatous inflammation. Immunohistochemical analysis demonstrated high levels of positive-affinity markers of IgG, IgG4, and CD138 and a IgG4/IgG ratio > 40%. IgG4-RD with pseudotumor was diagnosed. The initial treatment was intravenous methylprednisolone 120 mg daily for three days and oral prednisolone 10 mg twice a day and azathioprine 50 mg daily. The efficacy of the treatment was insufficient, and nasal bleeding did not decrease. Subsequently administered intravenous rituximab 1000 mg monthly for 2 months. Following this treatment, nasal bleeding stopped. CT revealed reduction in nasal mucosal swelling compared with that in a previous scan. This report highlights that in cases with an inflammatory mass mimicking malignancy, IgG4RD should always be considered, and rituximab treatment is recommended upon failure of steroid and azathioprine therapy.
Primary squamous cell carcinoma (SCC) of the liver is a rare disease that is difficult to diagnose until the pathology is confirmed. The age of the patients generally ranges from 18 to 83 years. The pathogenesis of primary SCC of the liver remains unclear and therapeutic guidelines have not yet been established. The overall survival rate may be less than 1 year. The prognosis for patients without surgery is worse than that for patients who undergo surgery. Herein, we report a case of primary SCC of the liver that responded well to intravenous carboplatin and 5-flurouracil (5-FU) with the aim of providing an alternative therapeutic option. A 61-year-old woman with no history of alcohol use disorder, cirrhosis, exposure to hepatotoxic chemicals, or a remarkable family history presented to our hospital with a complaint of epigastric pain, poor appetite, and fatigue, which had occurred 3 days before presentation. Blood tests revealed levels of alpha-fetoprotein of <2.0 ng/mL, carcinoembryonic antigen of 4.39 ng/mL, carbohydrate antigen (CA) 19-9 of 1306.15 U/mL, CA 125 of 66.3 U/mL, CA 153 of 19.7 U/mL, and SCC antigen of 8.5 ng/mL. Computed tomography scans of the abdomen showed a 5.8-cm lobulated soft-tissue mass with central necrosis in segment 6 of the liver, which caused compression of the common hepatic duct. Pathological examination of the masses revealed squamous cell carcinoma with focal glandular differentiation. The patient underwent palliative chemotherapy with intravenous carboplatin 150 mg (day 1) and 5-FU 1000 mg (days 1–4) instead of surgery. After two cycles of chemotherapy, jaundice and liver function improved. The patient was discharged in stable condition and was followed up in our outpatient department. Although the patient refused to undergo surgery, no tumor recurrence or distant metastasis was found during the 8-month follow-up period. This report highlights that neoadjuvant chemotherapy with carboplatin and 5-FU can be considered for primary SCC of the liver before a liver resection.
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