• Dapeng Li, Wei Zhang, Jun Wang, An Wang, Mingming Wu, and Yangguang Wei.
• Department of Urology, Rongcheng People's Hospital, The Affiliated Hospital of Jining Medical University, Weihai, Shandong, China.
• Medicine (Baltimore). 2023 Mar 24; 102 (12): e33410e33410.

RationaleMucinous adenocarcinoma of the renal pelvis is extremely rare in malignancy of the renal pelvis, and <100 cases have been reported so far. However, horseshoe kidneys appear to be more prone to this mucinous gland metaplasia. Due to the lack of a specific diagnostic basis, it is difficult to confirm the diagnosis before surgery, which is easy to cause misdiagnosis.Patient ConcernsWe report a 64-year-old male patient who found a cystic mass in the left kidney during physical examination. CT examination showed a horseshoe kidney with a cystic mass 9.5 × 8.0 cm in front of the left kidney, lacking obvious diagnostic features.DiagnosisIt was misdiagnosed as cystic teratoma before the operation, and was diagnosed as mucinous adenocarcinoma of the renal pelvis through pathological examination after the operation.InterventionsResection of the tumor by nephron-sparing surgery and postoperative chemotherapy.OutcomesNo tumor recurrence was found at 6 years of follow-up. After 7 years, the patient had multiple metastases in the abdominal wall and peritoneum, and no tumor recurrence was found in the urinary system. The patient received chemotherapy again and survived well.LessonsThe prognosis of nephron-sparing tumor resection for MRAP is not significantly different from that of radical nephroureterectomy + bladder cuff excision. Because it can reduce the risk that patients can not tolerate follow-up chemotherapy due to abnormal renal function after surgery, which may be more beneficial in patients with kidney abnormalities or chronic disease.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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