Primary enteric-type adenocarcinoma of the renal parenchyma in a patient with bilateral renal malrotation: a case report and review of the literature
- 1Department of Urology, The First Affiliated Hospital of Zhengzhou University
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.
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BibTeX
@article{wu2026primary,
title = {{Primary enteric-type adenocarcinoma of the renal parenchyma in a patient with bilateral renal malrotation: a case report and review of the literature}},
author = {Yuanqi Wu and Haoke Zheng and Gaurab Pokhrel and Rongyu Zhao and Shouchang Wu and Shuanbao Yu and Xuepei Zhang},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1977843},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1977843/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Primary enteric-type adenocarcinoma of the renal parenchyma in a patient with bilateral renal malrotation: a case report and review of the literature
AU - Yuanqi Wu
AU - Haoke Zheng
AU - Gaurab Pokhrel
AU - Rongyu Zhao
AU - Shouchang Wu
AU - Shuanbao Yu
AU - Xuepei Zhang
PY - 2026
DA - 2026/10/14
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1977843
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1977843/full
AB - 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.
SN - 2234-943X
ER -