模仿腎盂尿路上皮癌的IgG4相關性疾病之病例報告

吳宗憲、康智雄

高雄長庚紀念醫院泌尿科

IgG4 related disease from renal pelvis mimicking urothelial carcinoma: a case report

Tsung-Hsien Wu, Chih Hsiung Kang

Department of Urology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan

Introduction

IgG4-related disease (IgG4-RD) is an inflammatory fibrosis disease which is able to affect multiple organs and the fibrosis usually has a characteristic of storiform pattern. It is also a tumor-like lesion, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and lymphadenopathy. IgG4-RD has now been reported in nearly every organ. We present a rare case of a patient with image and the literature review.

Case presentation

This 65-year-old female had underlying disease of asthma, with sabutamol inhaler control. She was a case of recurrent bladder cancer state post several times of transurethral resection of bladder tumor and regularly followed up at urological clinic. She denied symptoms including frequency, urgency, dysuria or hematuria. There was no fever and body weight change noticed. Physical Examination revealed no flank pain and no abdominal tenderness. Lab data showed WBC: 4800/uL, Hemoglobin: 12.9g/dL, Creatinine: 0.76mg/dL, urine WBC: 0/uL, urine RBC: 0/uL. However, recent CT revealed newfound enhancing soft tissue masses in left renal pelvis, suspected urothelial tumor or renal sinus tumor encasing collecting system. Under the impression of left urothelial carcinoma, hand assisted retroperitoneoscopic nephroureterectomy was done. The pathology showed no tumor identified grossly in the renal pelvis, but the renal parenchyma measures 1.7 cm in the greatest thickness. The renal pelvis and surrounding soft tissue reveal fibrosis, dense lymphoplasmacytic infiltration and lymphoid follicle proliferation. The histomorphology and the result of the immunostains are consistent with IgG4-related disease (IgG4/IgG>40%). We also check lab data showed IgG: 1490mg/dL (700-1600) and IgG4:516mg/dL (3~201 mg/dL). The patient had no postoperative complications. She is regularly followed up bladder cancer and treats IgG4 related disease with low dose steroid.