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Peer-reviewedCC BYCase reports in urology

Extrarenal Papillary Renal Cell Carcinoma: A Unique Case With Bilateral Involvement of the Adrenal Glands.

  • Margarida André1
  • Brigitta Cismasiu2
  • Luísa Moreira1
  • Marta Vasconcelos1
  • Maria Isabel Santos1
  • João Rodrigues-Alves3
  • Eduardo Fernandes4
  • João Paulo Rosa1
  • Miguel Carvalho1
  • Helder Mansinho5
  • Carlos Luz-Correia2
5 affiliations
  1. 1Urology Department Almada-Seixal Local Health Unit Garcia de Orta Hospital Almada Portugal hgo.pt.
  2. 2Surgery Department Almada-Seixal Local Health Unit Garcia de Orta Hospital Almada Portugal hgo.pt.
  3. 3Pathology Department Almada-Seixal Local Health Unit Garcia de Orta Hospital Almada Portugal hgo.pt.
  4. 4Radiology Department Almada-Seixal Local Health Unit Garcia de Orta Hospital Almada Portugal hgo.pt.
  5. 5Medical Oncology Department Almada-Seixal Local Health Unit Garcia de Orta Hospital Almada Portugal hgo.pt.
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Abstract

Primary extrarenal renal cell carcinoma (RCC) is an exceptionally rare malignancy. We report a unique case of bilateral adrenal papillary extrarenal RCC in a 62-year-old man, managed through sequential surgical interventions over a prolonged follow-up. Initial right adrenalectomy revealed a papillary RCC with high-grade oncocytic features; immunohistochemical analysis confirmed PAX8 positivity, supporting a renal-type origin in the absence of a renal primary. Subsequent contralateral adrenal involvement and local recurrence required laparoscopic adrenalectomy and multiorgan resection. Despite complete macroscopic resections, the disease progressed to hepatic, pulmonary, peritoneal, and cerebral metastases, resulting in the patient's death due to postoperative intracranial hemorrhage. No renal involvement was identified throughout the disease course. This case highlights the aggressive behavior of extrarenal RCC and underscores the importance of comprehensive immunohistochemical workup, prolonged surveillance, and multidisciplinary management in the absence of standardized guidelines.

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Cite

BibTeXRIS
BibTeX
@article{andre2026extrarenal,
  title = {{Extrarenal Papillary Renal Cell Carcinoma: A Unique Case With Bilateral Involvement of the Adrenal Glands.}},
  author = {Margarida André and Brigitta Cismasiu and Luísa Moreira and Marta Vasconcelos and Maria Isabel Santos and João Rodrigues-Alves and Eduardo Fernandes and João Paulo Rosa and Miguel Carvalho and Helder Mansinho and Carlos Luz-Correia},
  journal = {Case reports in urology},
  year = {2026},
  month = nov,
  doi = {10.1155/criu/9413935},
  url = {https://europepmc.org/article/MED/42997264},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Extrarenal Papillary Renal Cell Carcinoma: A Unique Case With Bilateral Involvement of the Adrenal Glands.
AU  - Margarida André
AU  - Brigitta Cismasiu
AU  - Luísa Moreira
AU  - Marta Vasconcelos
AU  - Maria Isabel Santos
AU  - João Rodrigues-Alves
AU  - Eduardo Fernandes
AU  - João Paulo Rosa
AU  - Miguel Carvalho
AU  - Helder Mansinho
AU  - Carlos Luz-Correia
PY  - 2026
DA  - 2026/11/06
JO  - Case reports in urology
DO  - 10.1155/criu/9413935
UR  - https://europepmc.org/article/MED/42997264
AB  - Primary extrarenal renal cell carcinoma (RCC) is an exceptionally rare malignancy. We report a unique case of bilateral adrenal papillary extrarenal RCC in a 62-year-old man, managed through sequential surgical interventions over a prolonged follow-up. Initial right adrenalectomy revealed a papillary RCC with high-grade oncocytic features; immunohistochemical analysis confirmed PAX8 positivity, supporting a renal-type origin in the absence of a renal primary. Subsequent contralateral adrenal involvement and local recurrence required laparoscopic adrenalectomy and multiorgan resection. Despite complete macroscopic resections, the disease progressed to hepatic, pulmonary, peritoneal, and cerebral metastases, resulting in the patient's death due to postoperative intracranial hemorrhage. No renal involvement was identified throughout the disease course. This case highlights the aggressive behavior of extrarenal RCC and underscores the importance of comprehensive immunohistochemical workup, prolonged surveillance, and multidisciplinary management in the absence of standardized guidelines.
SN  - 2090-696X
SN  - 2090-6978
ER  -