https://orcid.org/0000-0003-0784-9157
Abstract
Chromophobe renal cell carcinoma (chRCC) with sarcomatoid and rhabdoid differentiation represents a rare variant of renal cancer. Duplicated inferior vena cava (DIVC) constitutes an uncommon vascular anomaly. To date, no previous reports have documented the coexistence of DIVC and chRCC with sarcomatoid and rhabdoid differentiation. A 64-year-old Caucasian male presented with a left renal mass. Computed tomography revealed a DIVC, an enlarged retroperitoneal lymph node, and a solid mass in the left kidney. The patient underwent left radical nephrectomy with retroperitoneal lymph node dissection (RPLND). The postoperative course was complicated by deep vein thrombosis, managed with anticoagulation for 3 months. Histopathological analysis confirmed chRCC with sarcomatoid and rhabdoid differentiation, with metastasis identified in one of the 22 excised lymph nodes. The patient survived for 16 years following surgery. Preoperative identification of venous anatomical variations is essential for surgical planning and facilitates safe radical nephrectomy with RPLND in comparable cases.
Introduction
Duplication of the inferior vena cava (DIVC) is a rare congenital vascular anomaly that occurs in ~0.7% of the population [1]. It alters retroperitoneal anatomy and may complicate uro-oncologic surgical procedures, especially if lymphadenectomy is required.
Chromophobe renal cell carcinoma (chRCC) accounts for ~5% of renal malignancies and has a more favourable prognosis than clear cell renal cell carcinoma (ccRCC). Sarcomatoid differentiation is observed in 8%–9% of chRCC cases and is associated with a more aggressive clinical course, including higher rates of lymph node and distant metastases [2, 3]. Complete surgical excision is the primary treatment; retroperitoneal lymph node dissection (RPLND) is reserved for patients at risk of lymph node involvement.
This report documents previously unreported coexistence of DIVC and chRCC with sarcomatoid and rhabdoid differentiation.
Case report
A 64-year-old Caucasian male presented with a left renal mass and mild left flank discomfort, without haematuria, fever, or weight loss. Physical examination revealed varicose veins. His body mass index was 27 kg/m2. Baseline laboratory results were within reference ranges. He had a 5-year history of well-controlled hypertension. Both parents had hypertension, while his brother died from lung cancer.
Contrast-enhanced computed tomography (CT) scan revealed a 7 cm solid left renal mass (Fig. 1a), DIVC (Fig. 1b), and an enlarged lymph node. After detailed consideration, an open adrenal-preserving left radical nephrectomy with RPLND was recommended. Thromboembolic prophylaxis was initiated with nadroparin 0.3 ml subcutaneously 12 hours before surgery and compressive stockings on the day of surgery. Ceftriaxone 2 g was administered 1 hour prior to incision.
(a) Coronal contrast-enhanced CT scan demonstrates a left renal mass; (b) coronal contrast-enhanced CT scan demonstrates DIVC; the black asterisk indicates the right IVC trunk, and the white asterisk indicates the left IVC trunk.
Surgery was performed under general anaesthesia. The patient was positioned supine with a slight dorsal tilt. A midline xiphoid-pubic laparotomy was performed. A paraduodenal peritoneal incision at the ligament of Treitz facilitated early ligation and division of the left renal artery and vein. After medial mobilization of the colon, the left radical nephrectomy was completed, preserving the adrenal gland. An intraoperative finding of a detectable lymph node in the left interaortocaval compartment prompted RPLND. Dissection began in the left para-aortic compartment from the renal hilum to the diaphragmatic crus, followed by the left paracaval, precaval, retrocaval, and interaortocaval compartments, as well as the preaortic, retroaortic, and right interaortocaval, precaval, and retrocaval compartments (Fig. 2).
Intraoperative field following RPLND demonstrates DIVC; the black asterisk indicates the right IVC trunk, and the white asterisk indicates the left IVC trunk.
The patient received intravenous antibiotics, nadroparin for thromboprophylaxis, and standard supportive therapy. The nasogastric tube, urinary catheter, and drains were removed on postoperative Days 2, 4, and 5, respectively. He was discharged on postoperative Day 5 with instructions to continue thromboprophylaxis as an outpatient.
Seven days after discharge, the patient was readmitted with clinically confirmed left-sided deep vein thrombosis (DVT), classified as a Clavien-Dindo grade II complication, and treated with anticoagulation for 3 months.
Histopathological analysis confirmed chRCC with rhabdoid and sarcomatoid differentiation (Fig. 3a). Metastasis was found in one of 22 excised lymph nodes (Fig. 3b). Surgical margins were clear. Final staging was pT3a, pN1, M0.
(a) Histopathological slide demonstrates chRCC with rhabdoid and sarcomatoid differentiation (H&E, ×40); (b) histopathological slide demonstrates lymph node metastasis of chRCC with rhabdoid and sarcomatoid differentiation (H&E, ×40).
The patient was followed regularly for 10 years with no evidence of disease recurrence or progression. Sixteen years after surgery, he died from cerebrovascular complications related to chronic hypertension and prolonged anticoagulant therapy.
Discussion
Congenital anomalies of the inferior vena cava (IVC) occur in ~0.2% to 3% of the population [4]. Among them, DIVC is the most common. It arises during the 6th to 10th week of embryogenesis due to the persistence of the supracardinal veins, resulting in bilateral IVC trunks flanking the aorta. It is classified into three types: major, minor, and asymmetric [2].
Most individuals with DIVC are asymptomatic, although some may develop DVT or pulmonary embolism [5]. Several authors have suggested that DIVC may be associated with an increased risk of thrombosis [6]. In this case, despite pharmacological and mechanical thromboprophylaxis, the patient developed DVT, likely due to extensive retroperitoneal dissection, malignancy, and venous stasis consistent with Virchow’s triad [7]. Therefore, limiting lymphadenectomy to retroperitoneal compartments with suspicious lymph nodes may reduce the risk of thrombosis in patients with DIVC.
Contrast-enhanced CT offers high spatial resolution, facilitating the detection of both the tumour and DIVC. Accurate preoperative identification of anatomical anomalies through radiological imaging is essential for surgical planning and minimizing intraoperative complications.
The standard seven-compartment template for RPLND is complicated by the presence of an additional left-sided IVC trunk, which creates extra interaortocaval, precaval, retrocaval, and paracaval compartments on the left side of the aorta. Three intraoperative steps are critical: early identification and careful dissection of both caval trunks to prevent vascular injury; ligation of the lumbar veins before lymphadenectomy; and meticulous compartment-based lymph node dissection, with continuous attention to vascular structures to minimize intraoperative bleeding. In this case, omission of RPLND in the right paracaval region was based on institutional experience, indicating that lymph node metastases are rare lateral to the opposite major vessel and are unlikely to increase the risk of residual disease. The observed long-term survival suggests that selected patients with limited nodal metastasis may benefit from complete tumour removal. Further prospective studies are necessary to validate this observation.
A comprehensive search of the English-language literature in Medline and Scopus identified only seven reports describing the coexistence of DIVC and confirmed RCC [8–14]. Details are summarized in Table 1. RPLND was performed in two cases, although procedural details were not provided [8, 9].
Summary of published cases of DIVC and RCC
| Authors | Year of publication | Age (year) | Gender | Tumour size (cm) | Tumour side | Surgical approach | Type of surgery | RPLND | Histopathology |
|---|---|---|---|---|---|---|---|---|---|
| Pescatori et al. [8] | 1991 | 42 | F | 15 (right) 5 (left) | Bilateral | NS | Right RNx thrombectomy; left NSS | NS (nodes negative) | ccRCC |
| Habuchi et al. [9] | 1993 | 77 | M | NS | Left | Open transabdominal | RNx and en bloc cava resection | Limited para-aortic | RCC with no lymph node metastasis |
| Shingleton et al. [10] | 1994 | 63 | M | Large | Right | Open thoracoabdominal | RNx and liver and colon resection | NS | Hypernephroma |
| Ito et al. [11] | 2007 | 59 | M | 4 | Left | Laparoscopic | RNx | NP | ccRCC |
| Chaud et al. [12] | 2022 | 79 | M | 4 | Left | Open thoracoabdominal | RNx and thrombectomy; anastomosis of left to right IVC | NS | ccRCC |
| Dall et al. [13] | 2022 | 39 | M | 3.8 & 3.5 | Bilateral | Open chevron | Bilateral RNx | NS | ccRCC |
| 2022 | 57 | M | 4.5 | Left | Robot-assisted | NSS | NP | Smooth muscle-rich ccRCC | |
| Yusof et al. [14] | 2022 | 55 | M | 10 | Left | Laparoscopic | RNx | NP | ccRCC |
M, male; F, female; NS, not specified; NP, not performed; RNx, radical nephrectomy; NSS, nephron-sparing surgery; IVC, inferior vena cava; RCC, renal cell carcinoma; ccRCC, clear cell renal cell carcinoma.
This case report has several limitations. The retrospective design and single-patient presentation limit the generalizability of the findings. The absence of comprehensive molecular and genetic analyses restricts insight into the tumour’s aggressiveness. Potential author bias may overemphasize the significance of RPLND.
Conclusion
This case documents a previously unreported coexistence of chRCC with sarcomatoid and rhabdoid differentiation and DIVC. Timely recognition of venous anomalies is essential for effective surgical planning. Accurate preoperative imaging and meticulous surgical technique facilitate successful resection in complex anatomical scenarios. The favourable long-term outcome underscores the importance of complete tumour excision.
Conflicts of interest
The authors declare that they have no competing interests.
Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or non-profit sectors.
Ethics approval
Ethical approval was not required for this case report under institutional policy. The Institutional Review Board granted an exemption as the patient did not receive experimental treatment and anonymity was fully maintained.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
