Novel surgical approach - cadaveric inferior vena cava graft reconstruction following leiomyosarcoma resection: A case report

Abstract

BACKGROUND

Inferior vena cava (IVC) leiomyosarcomas are rare and aggressive tumors. Complete cure depends on achieving R0 resection, which often requires circumferential resection and reconstruction. Synthetic grafts have traditionally been used when venous continuity must be restored. However, the use of cadaveric IVC grafts for reconstruction has not been widely reported.

CASE SUMMARY

Herein, we present the case of a 64-year-old woman diagnosed with an intrahepatic IVC leiomyosarcoma with local invasion. The patient responded favorably to chemotherapy and subsequently underwent an en bloc right hepatectomy, retrohepatic IVC resection, and reconstruction with an interpositional cadaveric IVC graft. Serial imaging follow-ups until 2 years after the operation showed persistent patency of the graft and no graft-related complications.

CONCLUSION

Cadaveric IVC grafts are an alternative to synthetic grafts for reconstruction, with acceptable outcomes. Larger, long-term studies are necessary to validate these findings.

Key Words: Inferior vena cava leiomyosarcoma; Vascular sarcoma; Vascular reconstruction; Venous cadaveric graft; Case report

Core Tip: We share our experience with an unprecedented approach using a cadaveric venous graft to reconstruct the inferior vena cava (IVC) after IVC leiomyosarcoma resection. The unavailability of a synthetic graft of an appropriate size led us to use the cadaveric graft, which showed durability and patency over a 27-month period, encouraging us to share our results.

INTRODUCTION

Inferior vena cava (IVC) leiomyosarcomas are rare yet aggressive tumors originating from the endothelium of a vein[1]. Although systemic treatment plays a crucial role in managing these patients, complete cure depends on achieving R0 resection, which often requires circumferential resection and reconstruction[1,2]. Synthetic grafts have traditionally been used when venous continuity must be restored. However, the use of cadaveric IVC grafts for reconstruction has not been widely reported[3].

Here, we present the case of a 64-year-old woman diagnosed with an intrahepatic IVC leiomyosarcoma with local invasion. The patient responded favorably to chemotherapy and subsequently underwent an en bloc right hepatectomy, retrohepatic IVC resection, and reconstruction with an interpositional cadaveric IVC graft.

CASE PRESENTATION

Chief complaints

The patient had pain in the right hypochondriac.

History of present illness

The patient was a 64-year-old woman who was referred to our hepatobiliary surgery service in 2019. She had a 5-month history of worsening right hypochondriac pain, with no accompanying nausea, vomiting, altered bowel habits, jaundice, fever, or weight loss.

History of past illness

The patient had type 2 diabetes mellitus.

Personal and family history

The personal and family history was unremarkable.

Physical examination

Physical examination showed no remarkable findings.

Laboratory examinations

All initial laboratory results were within normal ranges.

Imaging examinations

Computed tomography (CT) of the chest, abdomen, and pelvis revealed a heterogeneously enhancing, lobulated soft tissue mass measuring 4.6 cm × 3 cm × 6 cm. The mass involved the intrahepatic IVC, surrounding liver tissue, and part of the right adrenal gland. The lungs were free of metastatic deposits (Figure 1).

Figure 1 Computed tomography abdomen and pelvis showing heterogeneously enhancing lobulated soft tissue lesion enhancing. A: The portal phase; B: Delayed phase.

Magnetic resonance imaging confirmed that the lesion originated from the intrahepatic segment of the IVC and invaded segments 6 and 7 of the liver, as well as the right crus of the diaphragm.

MULTIDISCIPLINARY EXPERT CONSULTATION

The case was reviewed by a multidisciplinary tumor board comprising hepatobiliary surgeons, radiologists, pathologists, and medical oncologists. Owing to the tumor’s abutment to the IVC and the involvement of the left, middle, and right hepatic veins, the tumor was deemed unresectable. Systemic chemotherapy with doxorubicin, ifosfamide, and Mesna was initiated.

Following four cycles of chemotherapy, CT revealed mild progression of the tumor and necrosis in the liver lesion. The patient was switched to a second-line chemotherapy regimen of gemcitabine and docetaxel for six additional cycles. Subsequent imaging revealed tumor stabilization. One year after the initial diagnosis, follow-up imaging demonstrated a significant reduction in the tumor size (from 6 cm × 4.6 cm to 2.8 cm × 1.8 cm), with no evidence of systemic metastatic spread. Given the significant response to systemic therapy, the tumor board reconsidered the patient and recommended curative surgical resection.

FINAL DIAGNOSIS

An intrahepatic IVC leiomyosarcoma with invasion of the diaphragm, liver, and right adrenal gland, along with hepatic metastatic deposits.

TREATMENT

The patient underwent an L-shaped laparotomy, en bloc right hepatectomy, caudate lobectomy, right adrenalectomy, and retrohepatic IVC resection. Reconstruction was performed using a cadaveric IVC graft obtained from a deceased donor liver. The decision to use a cadaveric graft with an acceptable diameter was made because a synthetic conduit of the proper size was unavailable.

Our transplant anesthesia team was skilled in managing intraoperative IVC clamping and performed the procedure without the need for cardiopulmonary bypass. The patient was stabilized using intravenous fluids and inotropes before clamping, and invasive monitoring was performed throughout the operation. The anastomosis extended from the infrahepatic IVC, including the right renal vein, to the suprahepatic segment.

Postoperative histopathological examination confirmed leiomyosarcoma of the IVC with negative surgical margins. Severe hepatic steatosis was observed in the excised liver tissue (Figure 2).

Figure 2 Histopathology images of the final specimen. A: Tumor cells seen within the inferior vena cava. Astrex denoting wall muscle wall; B: Bizarre shaped cells with multiple mitotic figures; C: Tumor cells invading the liver with a background of hepatic steatosis.

OUTCOME AND FOLLOW-UP

The patient was monitored regularly during clinical and radiological follow-up to assess the graft patency and detect recurrence. One month post-surgery, follow-up CT revealed suspicious pulmonary nodules in the right lower lobe but no evidence of recurrence at the surgical site. The IVC and hepatic veins remained patent (Figure 3). The patient was administered six additional cycles of gemcitabine and dacarbazine to reduce the risk of recurrence.

Figure 3 Follow up computed tomography scan, obtained 1 month post op. A: No evidence of oral recurrence and patent inferior vena cava graft; B: Newly found lung nodule.

Unfortunately, the pulmonary nodules progressed despite chemotherapy. The tumor board recommended a right thoracotomy and resection of the metastatic lesion, followed by stereotactic body radiation therapy for a left-sided lung lesion.

Despite all locoregional and systemic treatments, the patient later developed bilateral pulmonary and duodenal metastases, and after receiving three cycles of eribulin, her condition worsened due to pneumonia, ascites, and anemia. She died 27 months after the abdominal surgery, following a family meeting that resulted in a do-not-resuscitate order.

DISCUSSION

Vascular leiomyosarcomas are slow-growing rare tumors that account for 0.5%-1% of all soft tissue sarcomas in adults. IVC leiomyosarcomas are the most common primary tumors of the IVC. Despite their rarity, the prognosis is generally poor, with a 5-year survival rate of only 31% after complete resection. Negative surgical margins are the most important prognostic factors for survival[4,5].

Leiomyosarcomas typically affect women, with a 3:1 female-to-male ratio. The median age at diagnosis is in the fifth decade of life[1,2]. Symptoms are usually nonspecific and depend on the location of IVC involvement. Patients with hepatic vein involvement or extension into the right atrium often have a worse prognosis[4,6].

Complete surgical resection with free margins remains the only curative option, while chemotherapy and radiation play uncertain roles. Studies have shown that incomplete resection or nonsurgical treatment alone results in 0% survival at 5 years.

The segment-based classification of IVC involvement helps guide surgical approaches[2-5]. Our case involved a segment III tumor necessitating right hepatectomy and adrenalectomy, along with IVC resection and reconstruction[6,7].

Various synthetic and autologous grafts have been used for IVC reconstruction, including Dacron, polytetrafluoroethylene (PTFE), reversed superficial femoral vein grafts, and common autologous venous tube grafts[6-10]. However, PTFE has been the graft of choice, owing to its superior patency rate due to its compression-resistant rings[3,5,9]. Several studies have reported complications related to the use of synthetic grafts, including leakage, graft-related infections, pseudointima formation, thrombosis, stenosis, venous hypertension, pulmonary embolism, and anticoagulation-related complications[1,8,9,10]. Moreover, graft size selection plays an important role in the development of thrombotic complications[11]. While there is no universal formula dictating an ideal graft size, a large graft diameter has been shown to result in thrombotic events due to low blood velocity via the conduit; in contrast, although a small graft diameter may enhance the blood flow and mitigate blood stagnation, it may eventually develop a thick pseudointima and subsequent thrombosis or occlusion[11,12]. The synthetic graft available at the time of the operation was too small for the already widened IVC and precluded safe anastomosis formation, which caused us to deviate from the usual practice of using a synthetic graft in light of the availability of a potential alternative with an acceptable diameter.

Cadaveric venous grafts, which are not commonly reported, have been effective in selected patients with higher infection resistance and patency rates[13-18]. In our patient, the cadaveric IVC graft demonstrated long-term patency with no complications related to graft thrombosis or infection during the 27 months of follow-up.

CONCLUSION

Cadaveric IVC grafts, although not readily available, are viable alternatives for reconstruction following IVC resection. Our patient demonstrated successful graft patency for two years. However, larger controlled studies are necessary to validate these findings and assess long-term outcomes.