Work

Primary enteric-type adenocarcinoma of the renal parenchyma in a patient with bilateral renal malrotation: a case report and review of the literature

Year: 2026

Type: article

Abstract: Background Primary enteric-type adenocarcinoma of the renal parenchyma is an exceptionally rare malignancy, accounting for less than 1% of renal epithelial tumors. Due to its morphological and immunohistochemical overlap with metastatic gastrointestinal malignancy, establishing a primary renal origin presents a considerable diagnostic challenge. Radical nephroureterectomy has traditionally been the standard surgical treatment. Case presentation We report a 47-year-old asymptomatic female with an incidental 68 mm cystic-solid left renal mass detected during a trauma evaluation. Imaging suggested Bosniak category IV on computed tomography and cystic teratoma on magnetic resonance imaging. Notably, the patient had bilateral renal malrotation. Serum tumor markers (CEA and CA19-9) were within normal limits. The patient underwent successful robotic-assisted partial nephrectomy with a warm ischemia time of 15 minutes. Histopathology revealed adenocarcinoma with an enteric immunophenotype: positive for CK20, CDX-2, SATB2, and Villin, with complete loss of PAX-8 and GATA-3. Postoperative gastrointestinal endoscopy and 18 F-FDG PET-CT excluded primary gastrointestinal or distant occult malignancies, confirming a primary renal parenchymal origin. At one-month follow-up, the patient showed no evidence of recurrence without adjuvant therapy. Conclusion This case represents the first reported association of primary enteric-type renal adenocarcinoma with bilateral renal malrotation and demonstrates that robotic-assisted partial nephrectomy can be a feasible nephron-sparing approach for selected patients with this aggressive tumor. A systematic diagnostic approach including surgical resection, immunohistochemical profiling, and exclusion of gastrointestinal metastasis is essential for accurate diagnosis and optimal management.

Source: Frontiers in Oncology

DOI: 10.3389/fonc.2026.1977843