Case report shows kidney preservation after renal hilar pheochromocytoma surgery

A 37-year-old man with a catecholamine-producing adrenal tumor and a hypertensive crisis underwent radical removal of the mass while retaining his left kidney through an unusual combination of ex vivo surgery and autotransplantation. The tumor was too tightly integrated with the renal vessels to be separated in place, so surgeons removed the kidney, performed vascular and ureteral preparation on a cold back table, excised the tumor in the abdomen and then returned the preserved organ to the left iliac fossa. Two years later, the patient had normal renal function, required no antihypertensive medication and showed no evidence of tumor recurrence or metastasis on whole-abdomen computed tomography. The case report provides evidence of feasibility and satisfactory mid-term follow-up in one patient, not proof that the technique is broadly effective.

The emergency began with 16 hours of sudden-onset severe pain in the left upper quadrant and a blood pressure of 260/110 mmHg at the referring hospital. Contrast-enhanced CT showed a retroperitoneal mass next to the left renal artery, with arterial narrowing. Blood pressure control remained poor despite sodium nitroprusside, and intravenous urapidil at the receiving hospital produced only partial improvement. A repeat scan identified a 4.0 by 3.2 cm mixed-density mass in the left adrenal region, including cystic degeneration and strongly enhancing solid components. The lesion was difficult to distinguish from the adrenal gland, while the adjacent renal artery was compressed and attenuated and the fat plane between the structures was obscured.

Laboratory findings supported the diagnosis of a pheochromocytoma crisis while also suggesting secondary activation of the renin–angiotensin–aldosterone system caused by renal artery involvement. Supine renin was 414.5 pg/mL, compared with a reference range of 4–24 pg/mL, and supine aldosterone was 418.3 pg/mL, compared with a reference range of 10–160 pg/mL. Angiotensin II remained within normal limits, and serum creatinine was 67 μmol/L. Preoperative preparation combined oral terazosin at 2 mg twice daily for 14 days with metoprolol succinate at 47.5 mg once daily. Daily intravenous administration of 1,500 mL of normal saline, together with a regular diet, expanded the circulating volume. During this period, blood pressure was maintained at 110–120/60–70 mmHg, while hemoglobin fell from 162 to 140 g/L and hematocrit from 47.3% to 39.9%, changes the clinical team interpreted as evidence of effective volume restoration.

Operation on February 1, 2023, initially used a transperitoneal laparoscopic approach. Exploration revealed dense adhesion between the tumor and the left renal vein, with the renal artery running through the tumor parenchyma. Because the kidney could not be separated while preserving the mass, the surgeons converted to an open transperitoneal operation through an approximately 20 cm left pararectus incision. The adrenal central vein was divided first, a step intended to limit catecholamine release triggered by manipulation of the tumor. The renal artery was then ligated and divided before it entered the lesion. The kidney and ureter were removed after five minutes of warm ischemia and immediately perfused with 500 mL of cold University of Wisconsin solution at 4°C by gravity from a height of one metre over five minutes.

The operation relied on two teams working simultaneously. After kidney retrieval, the primary team continued the in situ tumor resection, while a second team trimmed the renal vessels and ureteral stump on a back table, keeping the organ in ice slush at 0°C throughout bench preparation. Once the tumor had been removed and frozen-section examination confirmed pheochromocytoma, hemostasis was secured and the operative field was re-draped for implantation. The renal artery was anastomosed end-to-end to the internal iliac artery, and the renal vein end-to-side to the iliac vein in the left iliac fossa. The ureter was connected to the bladder by ureteroneocystostomy. After unclamping, the graft reperfused and produced visible urine. Total cold ischemia time was 90 minutes, and the operation lasted seven hours, including the initial attempt to preserve the kidney in situ before conversion to open surgery.

Postoperative imaging showed that the transplant was functioning promptly. Serum creatinine was 91 μmol/L on the first day. Contrast-enhanced ultrasonography on the second day demonstrated renal artery opacification at seven seconds, good cortical perfusion and no segmental anastomotic stenosis; the resistance index was 0.81. Definitive histopathology described a 6.0 by 5.5 by 4.5 cm tumor made up of nests with comedo-like necrosis and cellular atypia, together with focal extension into periadrenal soft tissue. Resection margins were negative, as were capsular invasion, perineural invasion and vascular invasion. Immunostaining was positive for chromogranin A, synaptophysin and CD56, supporting neuroendocrine chromaffin-cell origin. Negative steroidogenic factor-1 and pan-cytokeratin staining argued against an adrenocortical tumor, while a Ki-67 index of 1% and negative phosphohistone H3 indicated low proliferative activity.

The tumor received a Pheochromocytoma of the Adrenal Gland Scaled Score, or PASS, of 6 and a Grading System for Adrenal Pheochromocytoma and Paraganglioma, or GAPP, score of 5, indicating moderate differentiation. The authors classified it as a pheochromocytoma with malignant potential. That designation is distinct from an established diagnosis of metastatic disease: under the 2022 World Health Organization classification, pheochromocytomas and paragangliomas are not divided into benign and malignant categories, and malignancy is confirmed through the occurrence of metastases. Histological features associated with recurrence or metastasis can nevertheless indicate greater risk. The patient recovered without major complications, his blood pressure normalized and he no longer needed medication. At one year, creatinine was 70 μmol/L and CT urography showed good excretion from the graft without evidence of recurrence.

At the two-year follow-up, whole-abdomen CT showed an unremarkable graft and no recurrence or metastasis, while renal function remained normal. These observations support the report’s narrower conclusion that the combined procedure can enable radical tumor resection and kidney preservation in a carefully selected patient. The approach is not a routine alternative to nephrectomy based on this single case. Its rationale is that tumors closely surrounding the renal artery and vein may be difficult or unsafe to dissect in situ because manipulation can tear major vessels or provoke a surge of catecholamines. Traditionally, ipsilateral nephrectomy has therefore been favored for tumors invading or encasing those vessels.

The available literature likewise provides very limited evidence for autotransplantation in this setting. A review of 269 pheochromocytoma cases identified only 10 with coexisting renal artery lesions and only one renal autotransplantation, performed for renal artery stenosis rather than tumor involvement. By contrast, a report of six complex renal tumors treated with nephrectomy, bench surgery and autotransplantation reported no postoperative dialysis and no significant rise in creatinine at 30 days, but those cases did not establish outcomes for pheochromocytoma specifically. The present strategy requires vascular-surgery and transplantation expertise, two-team coordination and prolonged cold preservation. The authors note that patients who are young, have a solitary kidney or have impaired contralateral renal function stand to benefit most, although that proposed advantage has not been evaluated in a comparative study.

Important limitations temper the two-year result. Catecholamine biochemical testing was not completed before surgery, so the preoperative diagnosis rested on the clinical crisis, imaging and later histopathology rather than complete biochemical confirmation. Only one patient underwent the procedure, and there was no comparison group. The absence of recurrence after two years does not eliminate the possibility of very delayed recurrence or metastasis, particularly in a tumor with periadrenal soft-tissue invasion and a PASS score of 6. Continued long-term surveillance therefore remains necessary. Even with those constraints, the case demonstrates a technically coherent response to a rare surgical problem: by moving delicate vascular reconstruction away from the tumor-bearing operative field and placing the kidney under cold protection, surgeons removed the pheochromocytoma, re-established renal blood flow and ureteral drainage, and achieved normal renal function and blood-pressure control at mid-term follow-up.

Subject of Research: Case report of kidney preservation using ex vivo surgery and autotransplantation for pheochromocytoma involving renal hilar vessels

Article Title: Renal autotransplantation for pheochromocytoma involving the renal hilar vessels with kidney preservation: a case report and literature review

Article References: Chen, X., Fan, X., Weng, G., & Lou, J. (2026). Renal autotransplantation for pheochromocytoma involving the renal hilar vessels with kidney preservation: a case report and literature review. Frontiers in Oncology, 16, Article 1986562. https://doi.org/10.3389/fonc.2026.1986562

Image Credits: AI Generated

DOI: 10.3389/fonc.2026.1986562

Keywords: pheochromocytoma, renal autotransplantation, kidney preservation, bench surgery, renal hilar vessels, hypertensive crisis, adrenal tumor, vascular surgery, case report, Renal, autotransplantation, involving