Large sarcomatoid chromophobe renal cell carcinoma with tumor thrombus extending into the inferior vena cava: a case report on the challenges and lessons from a fatal outcome

Introduction

Renal cell carcinoma (RCC) affects around 400,000 people annually, with incidence rates expected to rise within the next decade (1). Its indolent nature has resulted in advanced-stage presentation in 25–30% of newly diagnosed cases (2,3). Among the aggressive subtypes, sarcomatoid renal cell carcinoma (sRCC) represents a rare undifferentiated variant with over 75% of cases presenting with metastasis (4,5). One rare progression of RCC is tumor thrombus (TT), a neoplastic extension of the tumor into the inferior vena cava (IVC) or the right atrium that is associated with poorer overall survival (6,7). Despite the promising potential of immune checkpoint inhibitors (ICIs), surgical intervention continues to be the preferred initial treatment modality, with cytoreductive nephrectomy (CN) emerging as a novel approach that significantly reduces tumor burden while enhancing ICI efficacy (8,9). Successful tumor resection with CN has 5-year survival rates exceeding 30%, however, early postoperative mortality ranges from 15–78%, with a 30-day mortality of approximately 2–10% (10-12). Common complications include hemorrhage, pulmonary embolism, sepsis, and acute renal failure, with increased risks associated with higher TT levels (13). Ultimately, these risks must be carefully considered when evaluating all management options.

Here, we report the case of a left-sided RCC with IVC level II TT with metastatic disease involving the liver, duodenum, colon, and retroperitoneum. The patient underwent CN with multiorgan resection and IVC thrombectomy, of which the postoperative course was complicated by septic bowel perforation associated with multiorgan failure. The purpose of this case presentation is to describe the importance of early recognition and comprehensive surgical planning particularly when pursuing CN in the context of high-risk RCC. We present this case in accordance with the CARE reporting checklist (available at https://atm.amegroups.com/article/view/10.21037/atm-2025-1-162/rc).

Case presentation

A 63-year-old male had been evaluated over several months for anemia, with repeated laboratory assessment without definitive identification of the underlying cause. He also reported weight loss for the last 6 months, associated with generalized weakness and abdominal and back pain. Imaging was not pursued until later in the course of the workup, at which point a large left kidney mass with a level II TT extending into IVC above the renal orifice. Figure 1A was identified. The radiological imaging showed that the left kidney mass was attached to the pancreas, spleen, second and third portions of the duodenum, descending colon, and small bowel with liver metastasis. Imaging also demonstrated a pulmonary embolus, likely a manifestation of a TT that embolized to the pulmonary arteries as seen in other reported cases (14).

Figure 1 Imaging and surgical findings of a left renal mass with tumor thrombus extension. (A) Computed tomography showing a left renal mass of 37 cm and with tumor thrombus going into the inferior vena cava (white arrow). (B,C) Different computed tomography views (B: coronal view; C: axial view) of the left renal mass. (D) Surgical specimen of large left renal involving the left colon.

All procedures performed in this case report were in accordance with the ethical standards of the University of Miami Institutional Review Board and with the Declaration of Helsinki and its subsequent amendments. The patient was informed of the risks of surgery, including infection, bleeding, blood transfusions, pulmonary emboli, and the impossibility of complete surgical excision. The patient was presented to a multidisciplinary team, and the decision was management with a CN surgical approach. Written informed consent was obtained for the publication of this case and accompanying images prior to surgery and discussion with family members was also done. A copy of the written consent is available for review by the editorial office of this journal.

Procedure in detail

A bilateral Chevron incision was performed approximately two fingerbreadths below the costal margins. A Thompson retractor was used to elevate the costal margin. The left kidney with the large tumor (Figure 1B,1C) was dissected laterally and posteriorly (with the help of two other surgeons, due to the size and weight of the mass) and then mobilized medially. The renal artery was posteriorly identified, ligated, and divided (15). The extensive collateral circulation collapsed, but due to the size of the mass, blood loss was not minimized. Subsequently, pancreas and spleen mobilization were performed using transplant techniques (16). Further dissection of the mass required separation of the tumor from the spine and posterior wall. The renal vein and IVC were then dissected, and the TT extending into IVC was removed. The mass also infiltrated the descending colon, where the 3^rd and 4^th portions of the duodenum had to be transected and repaired. The mass was finally removed from the bowel, spine, and aorta. Following this resection, the paraaortic lymph nodes were removed. Following node removal, a segment of the jejunum was removed and sown proximally and distally with an anastomosis between the small bowels. There was an estimated 15 liters of intraoperative blood loss, prompting transfusion of 20 units of packed red blood cells, 16 units of fresh frozen plasma, 2 units of platelets, 9 liters of crystalloid, and 2.5 liters of albumin. Pathology examination revealed a left kidney mass and IVC as a 5,550-gram total nephrectomy specimen, measuring 46 cm × 30 cm × 28 cm (Figure 1D). Ancillary analysis of the tumor revealed chromophobe and sarcomatoid components in morphology with lymphovascular invasion and metastatic sarcomatoid chromophobe RCC in five of six lymph nodes, the largest of which was 3 cm, with classification of the tumor as pT4M1N1 according to American Joint Committee on Cancer (AJCC) (8^th edition) (17).

In the early postoperative period, the patient developed acute right heart failure secondary to a pulmonary embolism managed by anticoagulation and an IVC filter. On postoperative day 5, the patient developed acute mental status changes with new-onset upper extremity weakness. Imaging was remarkable for evolving subacute infarcts in the bilateral frontal and parietal lobes, an acute subdural hematoma, and a right frontal hygroma, which remained stable on repeat imaging. The postoperative period was further complicated by the development of sepsis with cultures obtained from the Jackson-Pratt (JP) drain output identifying gram-positive and gram-negative bacteria. By postoperative day 13, the patient had multiorgan failure due to sepsis. At this point, a decision was made to pursue comfort care in alignment with the patient’s wishes and discussions with the patient’s multidisciplinary care team. The patient expired on postoperative day 15.

Discussion

Advanced RCC with TT presents a rare and complex clinical challenge that demands highly individualized care. Despite significant progress in immunotherapy, CN with thrombectomy remains the gold standard in appropriately selected patients (18). Metastatic disease and non-clear cell RCC (nccRCC) subtypes are often managed with surgical resection due to the variable clinical benefit of ICIs. For instance, several studies have reported that chromophobe variants of nccRCC, identified in the histopathology of this patient, have objective response rates ranging from 0% to 11% when treated with ICI-based regimens (19,20). With the demonstrated decreased efficacy of ICIs, surgical intervention continues to play a critical role in RCC disease control and overall survival. Several studies have also demonstrated significantly improved long-term outcomes with CN in the setting of metastatic disease, with 5-year survival rates reaching approximately 58% compared to 34% in patients with ICIs alone (21). Although these results are noteworthy, it is essential to acknowledge that this data is primarily derived from a high-volume center with increased expertise with complex cases, like our own, thereby emphasizing the importance of individualized decision-making in each respective case.

In this case, the patient presented with unrecognized metastatic disease that contributed to his advanced presentation and extensive tumor burden. Preoperative imaging played a crucial role in surgical planning by delineating hepatic metastatic lesions and invasion into surrounding viscera. The patient was preoperatively staged as pT4M1N1 and classified as intermediate risk per Memorial Sloan Kettering Cancer Center (MSKCC) criteria. The detection and characterization of the TT was a critical aspect of the case, with the thrombus having been classified as level II according to the Mayo Clinic Classification (22). While the prognostic value of TT level remains controversial, several studies have demonstrated notable increases in operative complications with increased extension of the thrombi (23). Consequently, the surgical procedure was technically demanding as it required meticulous removal of the TT from critical vascular structures along with widespread resection of tumor-involved segments of the bowel, spleen, pancreas, spine, and posterior abdominal wall. With an estimated blood loss of approximately 15 liters, this represented the largest complication of this case that was effectively managed with initiation of massive transfusion protocol (MTP). In addition to significant bleeding requiring blood transfusion, pulmonary embolism, infectious, and cardiopulmonary events are the most frequently reported complications of complex and challenging CNs (24,25). Factors such as degree of tumor invasion, TT level, and patient’s comorbidities such as elevated body mass index (BMI) and cardiovascular disease, have been found to substantially increase CN associated morbidity and mortality (26). Yet despite the considerable perioperative risks that this operation carries, successful surgical resection continues to offer significantly favorable long-term oncologic outcomes.

As described above, pathology revealed mixed chromophobe and sarcomatoid components with lymphovascular invasion, both unfavorable prognostic features. In particular, sarcomatoid variants are associated with a poorer prognosis, reduced overall survival rates and a higher propensity for metastasis (27,28). Though a biopsy was not obtained during the preoperative period, this may have better guided conversations with the family on the patient’s likely prognosis.

Given the complexity of surgical intervention, several ethical considerations emerge in guiding clinical decision-making, especially when weighing conservative treatment against surgical options (29). While the advancement of surgical technique has allowed for the resection of previously inoperable malignancies, the risks and complications of such surgeries remain significant as previously discussed (30). In this case, at the time of surgical decision-making, the patient was referred with a diagnosis of renal tumor with venous TT and with metastatic disease. Following multidisciplinary evaluation, the patient and family were counseled extensively regarding the advance nature of the disease, the high preoperative risk, and the limited oncologic benefit of surgery. All therapeutic options, including conservative and palliative management, were discussed in detail.

Despite recommendations favoring non-operative management given the anticipated surgical risk and uncertain survival benefit, the patient and family expressed a strong preference for surgical intervention. After thorough informed consent and shared decision-making, surgery was undertaken with palliative intent, aiming to relieve local disease burden and potential thrombus-related complications. While the outcome was unfavorable, the decision to proceed with surgery reflected patient autonomy and beneficence, informed consent, surgical experience of similar complex tumor by the surgical team and the clinical information available at the time rather than retrospective knowledge of disease extent.

Accordingly, as multidisciplinary teams engage in discussions with the patient, it is crucial to acknowledge and transparently communicate the potential limitations of surgical treatment, particularly when the expected benefit is uncertain and significant postoperative risks are significant (31,32). Supporting ethical and autonomous decision-making requires that patients receive comprehensive information, allowing them to consider the full spectrum of possible outcomes, including prolonged hospitalization and loss of functional independence. This empowers patients to make decisions that are truly consistent with their values and informed consent. Ultimately, the decision to operate was guided by ethical principles; the patient was fully informed of the risks, alternatives, and likely outcomes, and decisional capacity was confirmed. The surgical approach was not pursued as curative therapy but as patient-driven, palliative intervention.

While our team has shown prior success in the resection of complex renal tumors, each case demands individualized evaluation to determine the intervention that offers the most meaningful chance of recovery for the patient (33). Ultimately, the assessment of surgical success has shifted beyond technical achievement to encompass patient-centered outcomes, including functional recovery, quality of life, and alignment with patient goals (34).

Lastly, this case highlights the importance of comprehensive evaluation in patients presenting with unexplained anemia, especially in older adults, where malignancy should remain a differential consideration as a possible presentation as a paraneoplastic syndrome (35). While access to imaging and specialist consultation may vary across healthcare systems, a timely and thorough investigation is critical for identifying underlying neoplastic processes before they become unresectable or life-threatening. This case also serves as a reminder of the systemic vulnerabilities that can contribute to delayed diagnoses, including fragmented care, limited access to diagnostics, and cognitive biases such as anchoring during clinical evaluation.

Conclusions

In conclusion, we present a case of advanced RCC with a level II TT managed with CN and IVC thrombectomy in the setting of metastatic disease. This case demonstrates the complexity in decision-making in advanced disease and highlights the critical role of integrating ethical considerations in guiding management between surgical intervention and conservative or palliative care.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://atm.amegroups.com/article/view/10.21037/atm-2025-1-162/rc

Peer Review File: Available at https://atm.amegroups.com/article/view/10.21037/atm-2025-1-162/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://atm.amegroups.com/article/view/10.21037/atm-2025-1-162/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this case report were in accordance with the ethical standards of the University of Miami Institutional Review Board and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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