Venous Extension and Complex Local Surgery in ACC

Localized Disease Management

Venous extension and complex local surgery in adrenocortical carcinoma (ACC) refers to anatomically advanced but potentially localized disease in which the primary adrenal tumor extends into major veins or directly invades adjacent organs and vascular structures. In ACC care, this sits within localized disease management and surgical oncology, because the central question is whether complete macroscopic resection remains feasible despite caval, renal venous, hepatic, or occasional atrial involvement.¹² These operations may require coordination across endocrine, vascular, hepatobiliary, transplant, or cardiothoracic surgical teams when venous control or reconstruction is complex.³⁴

The available literature suggests that venous tumor thrombus does not by itself make ACC unresectable, and published experience repeatedly describes successful thrombectomy or multivisceral resection in selected nonmetastatic patients.¹⁵ However, the evidence base is limited and highly selected. Most data come from retrospective institutional series, technical reports, and case reports, so the literature is more reliable for defining operative feasibility and anatomic principles than for estimating comparative survival benefit.⁶³

This distinction is clinically important because surgery remains the only potentially curative modality for localized ACC, while systemic therapy alone is not an established curative alternative in this setting.²¹ At the same time, recurrence risk remains high even after apparently complete resection, and the morbidity of major vascular or multivisceral operations may be substantial.⁶⁷ As a result, management decisions depend less on the mere presence of venous extension than on disease distribution, expected margin status, patient fitness, and the capabilities of the treating center.⁴⁸

Diagnostic and anatomic context

ACC may spread intraluminally from the adrenal vein into the renal vein and inferior vena cava (IVC), with rare progression into the right atrium, or it may directly invade surrounding organs and the caval wall.³⁹ Right-sided tumors more often extend directly into the IVC, whereas left-sided tumors may progress through the renal vein before entering the cava, a pattern that has implications for operative exposure and reconstruction planning.³¹⁰

Clinical presentation is variable and may reflect local mass effect, hormonal excess, incidental detection during staging, or complications of venous obstruction and embolic risk.¹¹¹² Hormone secretion and symptoms may help establish the broader clinical picture, but they do not reliably define technical resectability. The more dependable determinants are thrombus extent, vessel wall involvement, adjacent organ invasion, and the presence or absence of distant metastatic disease.³¹³

These anatomic features make preoperative imaging central to decision-making. Cross-sectional imaging is used to define the cranial extent of thrombus, hepatic vein or intrapericardial involvement, possible atrial extension, and whether the vessel is merely occupied by thrombus or appears directly invaded.¹³¹⁴ Imaging can usually support operative planning, but it may not fully predict wall invasion or the ease of thrombectomy, so final strategy often depends on intraoperative findings.³⁸

Major patterns of complex local disease

Venous tumor thrombus

The most characteristic pattern is venous extension into the adrenal vein, renal vein, IVC, or, rarely, the right atrium.¹² Across the literature, thrombus level is treated primarily as a marker of operative complexity rather than an automatic contraindication to surgery. More cranial extension generally implies more difficult exposure, greater embolic risk, and a higher likelihood that bypass or combined thoracoabdominal access will be needed.¹³³

What is relatively consistent is that selected patients with cavo-atrial extension can undergo complete gross resection at experienced centers.¹¹⁸ What is less certain is whether thrombus level independently predicts long-term oncologic outcome apart from its association with operative risk and advanced local disease. In practice, thrombus extent is most useful for surgical planning and perioperative risk assessment rather than for declaring futility on its own.¹²⁹

Direct invasion and multivisceral resection

A second pattern involves bulky localized ACC with direct invasion of adjacent organs or major vascular structures, sometimes requiring liver resection, caval wall excision, venous grafting, or other en bloc procedures to achieve negative margins.¹⁵⁵ The underlying oncologic principle remains the same as in less complex ACC surgery: removal of all gross disease with acceptable operative risk.

The literature supports technical feasibility in carefully selected patients, particularly in high-volume centers with multidisciplinary support.¹⁶⁴ Broad survival conclusions are less reliable because reports are heterogeneous in stage, margin status, adjuvant treatment, and follow-up. The practical implication is that extensive local involvement raises the threshold for surgery but does not, by itself, exclude curative-intent resection.⁶⁴

Operative planning and surgical strategies

Because anatomy drives feasibility, management usually begins with multidisciplinary resectability assessment. Depending on thrombus level and adjacent organ involvement, planning may require input from vascular, hepatobiliary, transplant, or cardiothoracic surgeons in addition to adrenal or surgical oncology teams.⁴⁷ This need for coordinated planning is one of the most reproducible themes in the literature; the exact thresholds for specialty involvement or choice of approach are less standardized and often center-specific.³¹⁴

Open adrenalectomy with thrombectomy is the dominant reported approach for major venous involvement.¹⁶¹³ For limited thrombus, direct vascular control and thrombectomy may be feasible without extracorporeal support, while selected reports describe intrapericardial IVC control or vessel reconstruction techniques intended to avoid sternotomy or preserve renal drainage in favorable anatomy.¹⁶¹⁰⁷ A practical implication is that kidney-sparing or vessel-sparing strategies may be possible in selected left-sided or renal-vein-involved cases, but these remain individualized technical decisions rather than standard expectations.¹⁰⁷

As disease extends into the suprahepatic IVC or right atrium, reported operations more often use cardiopulmonary bypass, and sometimes deep hypothermic circulatory arrest, to facilitate safe thrombus extraction and reduce the risk of embolization or hemodynamic compromise.¹¹¹³⁸ These approaches appear feasible, but comparative evidence is insufficient to show that one strategy is superior across centers or thrombus levels. Clinically, the operative plan is best tailored to anatomy and institutional expertise rather than inferred from isolated technical successes.⁶⁹

Outcomes, risks, and limitations

The most reliable conclusion from the published literature is that aggressive local surgery for ACC with venous extension is technically possible, including in some cases with retrohepatic caval or atrial involvement.⁵⁴ The least reliable conclusion is the size of any oncologic advantage compared with nonoperative or less aggressive strategies, because no robust prospective comparative studies exist and reported cohorts are subject to strong referral and publication bias.⁶⁷

Perioperative morbidity may be considerable and includes major hemorrhage, transfusion requirement, embolic events, cardiopulmonary complications, and morbidity related to cavotomy, vascular reconstruction, bypass, or multivisceral resection.¹¹¹² Thrombus extension into the retrohepatic cava or atrium appears to correlate more consistently with operative hazard than with categorical unresectability.³⁸ Anatomic variation in adrenal venous drainage may further complicate dissection and vascular control, underscoring the value of meticulous imaging review and open operative expertise.¹⁷

Some historical and nonhuman reports support broad surgical principles such as the importance of exposure, cranial thrombus level as a risk marker, and the occasional feasibility of vessel-sparing thrombectomy, but they have limited direct applicability to human ACC.^1819202122 Their main relevance is contextual rather than practice-defining.

Role in management and research

Within contemporary ACC management, venous extension and complex local invasion are best understood as indications for specialized resectability assessment rather than automatic exclusion from curative-intent surgery.¹³ Compared with routine adrenalectomy, decision-making depends more heavily on whether complete gross resection appears achievable, whether distant disease is absent or limited, and whether the center can safely provide advanced vascular or cardiac support.²⁴

Important evidence gaps remain. The literature would be strengthened by multicenter reporting that standardizes thrombus level, distinguishes intraluminal thrombus from wall invasion, and separately analyzes perioperative outcomes, margin status, recurrence patterns, and long-term survival.³⁷ Current evidence supports referral of selected patients for complex surgical evaluation, but claims about prognosis or superiority of particular operative techniques should remain cautious because they continue to rest mainly on retrospective and case-based data.⁶⁸

Included Articles

• PMID 7771009: This review and case report highlights that adrenocortical carcinoma can extend as tumor thrombus into the renal vein, inferior vena cava, and even the right atrium, and argues that such venous invasion should not automatically preclude curative-intent radical one-stage resection with thrombectomy when disseminated disease is absent.¹

• PMID 9366311: This review of 29 ACC cases with intracaval tumor thrombus emphasizes that complete surgical extirpation offers the best chance of survival and that cardiopulmonary bypass can facilitate safer resection of caval or atrial extension. It also highlights the need to assess the vena cava carefully in patients with large, especially right-sided, adrenal tumors.²

• PMID 16643609: This case series includes one adrenocortical carcinoma with tumor thrombus extending through the inferior vena cava into the right atrium, managed by transthoracoabdominal resection with cardiopulmonary bypass. The report suggests this approach can permit complete thrombus extraction with acceptable perioperative technical safety, though long-term survival benefit remains uncertain.⁶

• PMID 16860633: This case report describes virilizing ACC with inferior vena cava and right atrial extension causing risk of embolization and heart failure. It highlights operative management with preoperative caval assessment and resection using cardiopulmonary bypass and hypothermic circulatory arrest for extensive venous tumor thrombus.¹¹

• PMID 17174512: A technical note describes transabdominal transdiaphragmatic intrapericardial isolation of the inferior vena cava to enable en bloc resection of tumors with vena caval thrombus reaching the hepatocaval junction without sternotomy, thoracotomy, or cardiopulmonary bypass. In one included ACC case, this approach was feasible with IVC resection and PTFE graft reconstruction.¹⁶

• PMID 18758230: This case report describes aggressive surgical management of locally advanced left-sided ACC with tumor thrombus extending through the inferior vena cava into the right atrium. It emphasizes that detailed preoperative evaluation and complete locoregional resection, including vascular and contralateral renal vein graft reconstruction when necessary, may be feasible in carefully selected symptomatic patients.¹⁵

• PMID 18816449: In a laparoscopic adrenalectomy series, early identification and ligation of the adrenal vein was feasible in all cases, with venous variants found infrequently. One ACC case had a duplicated right adrenal venous drainage pattern into the inferior vena cava and right renal vein, highlighting operative anatomic variation relevant to adrenal surgery.¹⁷

• PMID 22096307: This case describes surgical management of pediatric adrenocortical carcinoma with tumor thrombus extending from the inferior vena cava to the right atrial junction, using endoluminal balloon occlusion to reduce embolization risk during thrombectomy. It highlights major operative hazards including massive bleeding, transfusion needs, and potential pulmonary embolism.¹²

• PMID 22278755: This case report describes curative-intent resection of a very large right ACC with liver invasion and tumor thrombus extending through the inferior vena cava into the right atrium, using cardiopulmonary bypass, partial IVC wall resection, and limited hepatic resection to achieve negative margins.⁵

• PMID 27127363: This case report describes curative-intent resection of left-sided ACC with tumor thrombus extending from the adrenal vein through the renal vein into the inferior vena cava, using adrenalectomy, caval thrombectomy, and IVC wall resection while preserving the ipsilateral kidney via lumbar and gonadal venous drainage.¹⁰

• PMID 28523067: This case report describes ACC with contiguous tumor thrombus extending from the right adrenal gland through the inferior vena cava into the right atrium and across the tricuspid valve. It emphasizes that precise imaging defines operative extent and that complete resection for cavo-atrial extension may require combined cardiac-abdominal surgery with cardiopulmonary bypass.¹³

• PMID 36780100: This correspondence discusses surgical management of ACC with venous tumor invasion and emphasizes that operative planning depends on precise classification of tumor thrombus extent. It highlights differing right- versus left-sided venous extension patterns and outlines thrombus levels from adrenal or renal vein involvement up to retrohepatic IVC and right atrial extension.³

• PMID 36795567: This case illustrates curative-intent management of localized nonsecreting ACC with tumor thrombus extending through the inferior vena cava into the right atrium using multidisciplinary planning, preoperative arterial embolization, and one-stage radical adrenalectomy with simultaneous caval and atrial thrombectomy under cardiopulmonary bypass.⁴

• PMID 37774418: A tertiary-center case within a mixed-malignancy IVC thrombectomy series describes ACC with tumor thrombus extending from the adrenal vein/IVC into the right atrium managed by radical resection using sternotomy-laparotomy exposure, cardiopulmonary bypass, deep hypothermic circulatory arrest, and complete thrombus extraction.⁸

• PMID 41103879: A tertiary-center case describes localized ACC with renal vein and inferior vena cava thrombus managed by open adrenalectomy plus thrombectomy, preserving the ipsilateral kidney. The report emphasizes multidisciplinary operative planning for complex vascular involvement and documents short-term disease control after resection.⁷

• PMID 24127376: A 2013 BMJ Case Report describes ACC with tumor thrombus extending into the right atrium. It supports the note’s existing framing that atrial extension is rare but surgically relevant and mainly documented through case-based experience.⁹

• PMID 32558442: A 2020 review on surgical management of abdominal tumors involving the inferior vena cava is not specific to ACC, but it adds broader surgical context for preoperative assessment, vascular control, and reconstruction planning in cases with caval involvement.¹⁴

• PMID 39219601: A 2024 veterinary case report describes feline adrenocortical carcinoma with ipsilateral renal vein invasion treated by adrenalectomy and renal vein venotomy without nephrectomy, with preserved renal function and prolonged follow-up. Its relevance to human ACC is indirect, but it illustrates the organ-preserving rationale behind selected kidney-sparing approaches when renal vein thrombus can be removed safely.²²

• PMID 23725435: A retrospective canine adrenalectomy series including adrenocortical carcinoma suggests that vena caval invasion, particularly more extensive cranial thrombus, increases short-term perioperative mortality, while not necessarily precluding longer-term survival among postoperative survivors. Its relevance to ACC is indirect but it adds useful nuance to risk stratification by thrombus extent.²⁰

• PMID 24392699: A veterinary series of right adrenalectomy described an intercostal approach that improved exposure of the right adrenal bed and was used in dogs with adrenal tumors, including some adrenocortical carcinomas with phrenicoabdominal vein or caval invasion. Although indirect and nonhuman, it adds a technical nuance relevant to exposure and vascular control in complex right-sided adrenal surgery.¹⁸

• PMID 28990687: A small canine retrospective series described phrenicoabdominal venotomy as a possible alternative to cavotomy for selected adrenal tumors with limited vena caval thrombus arising through the phrenicoabdominal vein. Its relevance to ACC is indirect and mainly technical, suggesting a vessel-sparing thrombectomy concept for carefully chosen anatomy rather than changing current human surgical practice.¹⁹

• PMID 31034643: A 2019 multicenter veterinary study of adrenalectomy with cavotomy found substantial perioperative mortality, with postdiaphragmatic thrombus extension associated with worse perioperative and overall outcomes, while longer survival remained possible in animals surviving surgery. Its relevance to ACC is indirect because the cohort was nonhuman and mostly consisted of pheochromocytoma rather than adrenocortical carcinoma.²¹

References

Footnotes

1. Adrenal cortical carcinoma with tumor thrombus invasion of inferior vena cava.. Urology. 1995. PMID: 7771009. Local full text: 7771009.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶

2. Adrenocortical carcinoma with intracaval extension.. J Urol. 1997. PMID: 9366311. Local full text: 9366311.md ↩ ↩² ↩³ ↩⁴ ↩⁵

3. Re: Olivero A, et al. Adrenocortical carcinoma with venous tumor invasion: is there a role for mini-invasive surgery?. Langenbecks Arch Surg. 2023. PMID: 36780100. Local full text: 36780100.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶ ↩⁷ ↩⁸ ↩⁹ ↩¹⁰ ↩¹¹ ↩¹²

4. Adrenocortical carcinoma with tumor thrombus extension into the right atrium.. Pol Arch Intern Med. 2023. PMID: 36795567. Local full text: 36795567.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶ ↩⁷ ↩⁸

5. Adrenocortical carcinoma with intracaval extension to the right atrium: resection on cardiopulmonary bypass.. Ann Surg Oncol. 2012. PMID: 22278755. Local full text: 22278755.md ↩ ↩² ↩³ ↩⁴

6. Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atrium.. Int J Urol. 2006. PMID: 16643609. Local full text: 16643609.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶ ↩⁷

7. Demographics, Clinical Profiles, and Outcomes of Patients With Adrenal Disorders in a Tertiary Care Center: A Retrospective Study.. Cureus. 2025. PMID: 41103879. Local full text: 41103879.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶ ↩⁷

8. Inferior vena cava tumor thrombus: clinical outcomes at a canadian tertiary center.. Perfusion. 2024. PMID: 37774418. Local full text: 37774418.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶ ↩⁷

9. Adrenocortical carcinoma with tumour thrombus extension to right atrium: a rare finding in uncommon tumour.. BMJ Case Rep. 2013. PMID: 24127376. Local full text: 24127376.md ↩ ↩² ↩³ ↩⁴

10. Adrenocortical carcinoma with inferior vena cava thrombus: Renal preserving surgery.. Indian J Urol. 2016. PMID: 27127363. Local full text: 27127363.md ↩ ↩² ↩³ ↩⁴

11. Virilizing adrenocortical carcinoma with cavoatrial extension.. Am J Surg. 2006. PMID: 16860633. Local full text: 16860633.md ↩ ↩² ↩³ ↩⁴ ↩⁵

12. Anesthetic management for removal of adrenocortical carcinoma with thrombus in the inferior vena cava extending to the right atrium.. J Anaesthesiol Clin Pharmacol. 2011. PMID: 22096307. Local full text: 22096307.md ↩ ↩² ↩³ ↩⁴

13. Extension of Adrenocortical Carcinoma into the Right Atrium.. Pak J Med Sci. 2017. PMID: 28523067. Local full text: 28523067.md ↩ ↩² ↩³ ↩⁴ ↩⁵ ↩⁶

14. Surgical Approach to Abdominal Tumors Involving the Inferior Vena Cava.. Isr Med Assoc J. 2020. PMID: 32558442. Local full text: 32558442.md ↩ ↩² ↩³

15. Cardiac and caval invasion of left adrenocortical carcinoma.. Urol Int. 2008. PMID: 18758230. Local full text: 18758230.md ↩ ↩²

16. Intrapericardial isolation of the inferior vena cava through a transdiaphragmatic pericardial window for tumor resection without sternotomy or thoracotomy.. Eur J Surg Oncol. 2007. PMID: 17174512. Local full text: 17174512.md ↩ ↩² ↩³ ↩⁴

17. Experience in identifying the venous drainage of the adrenal gland during laparoscopic adrenalectomy.. Clin Anat. 2008. PMID: 18816449. Local full text: 18816449.md ↩ ↩²

18. Intercostal approach for right adrenalectomy in dogs.. Vet Surg. 2014. PMID: 24392699. Local full text: 24392699.md ↩ ↩²

19. Phrenicoabdominal venotomy for tumor thrombectomy in dogs with adrenal neoplasia and suspected vena caval invasion.. Vet Surg. 2018. PMID: 28990687. Local full text: 28990687.md ↩ ↩²

20. Evaluation of risk factors for outcome associated with adrenal gland tumors with or without invasion of the caudal vena cava and treated via adrenalectomy in dogs: 86 cases (1993-2009).. J Am Vet Med Assoc. 2013. PMID: 23725435. Local full text: 23725435.md ↩ ↩²

21. Perioperative morbidity and mortality in dogs with invasive adrenal neoplasms treated by adrenalectomy and cavotomy.. Vet Surg. 2019. PMID: 31034643. Local full text: 31034643.md ↩ ↩²

22. Kidney sparing during surgical treatment of an adrenocortical carcinoma with renal vein invasion in a cat.. Can Vet J. 2024. PMID: 39219601. Local full text: 39219601.md ↩ ↩²