Renal cell carcinoma with inferior vena cava thrombus extending to the right atrium diagnosed during pregnancy (2024)

Abstract

Keywords: inferior vena cava, pregnancy, renal cell carcinoma, right atrial mass, tumor thrombus

Introduction

Renal tumors in pregnancy are rare. Renal cell carcinomas (RCC) account for 50% of primary renal neoplasms in pregnancy, thus making it the most common urologic malignancy of pregnancy.^1,2 As of February 2015, there were 106 reported cases of RCC during pregnancy.³ Pregnant patients with RCC commonly present with a palpable mass (88%), pain (50%), hematuria (47%), fever (21%), or hypertension (18%). The classic triad of a palpable mass, flank pain, and hematuria is only seen in approximately 26% of cases.² As the use of cross-sectional imaging has increased, there has been a rise in the incidental detection of RCC during pregnancy, including on fetal ultrasound.⁴

An estimated 4–10% of all patients with RCC have intraluminal extension of the tumor into the inferior vena cava (IVC).⁵ RCC IVC thrombi are classified as shown in Table 1.

At the present date, there exists only one other report of RCC with IVC tumor thrombus diagnosed during pregnancy.⁶ In this report, the tumor thrombus extended only to the level of the infrahepatic IVC (level II tumor thrombus). We present the case of an RCC with IVC tumor thrombus extending into the right atrium (level IV) diagnosed at 24 weeks of pregnancy.

Case presentation

A 37-year-old woman at 24 weeks of pregnancy with a history of iron-deficiency anemia and two spontaneous abortions was incidentally found to have a solid mass in the lower pole of the right kidney on fetal ultrasound subsequently confirmed by magnetic resonance imaging (MRI) of the abdomen and pelvis (without intravenous gadolinium) to be 7.1 × 11 cm. At 29 weeks of pregnancy, the patient presented to an outside hospital for dyspnea. Doppler ultrasound of the bilateral lower extremities demonstrated thrombi in the right common femoral vein and right femoral vein; however, a ventilation-perfusion (VQ) scan was indeterminate for pulmonary embolus (PE) and a transthoracic echocardiogram (TTE) showed normal biventricular function. Blood tests indicated that the patient was lupus anticoagulant positive; therefore, the patient was discharged on therapeutic enoxaparin on hospital day 5. At 9 days later, at 30 weeks of pregnancy, the patient presented again to an outside hospital with worsening dyspnea, tachycardia, and anemia to a hemoglobin of 7.0 g/dl, requiring transfusion of 2 units of packed red blood cells. TTE at this time revealed a previously undiagnosed right atrial mass. The patient was transitioned from enoxaparin to a heparin drip, received a dose of betamethasone for fetal pulmonary maturation, and was transferred to the Cardiac Care Unit of Mount Sinai Medical Center, NY, USA. A computed tomography (CT) angiogram of the chest demonstrated extensive bilateral pulmonary emboli, a left lower lobe pulmonary infarct, intraventricular septal bowing, and a 1.7 × 2.8 cm right atrial mass. MRI of the abdomen and pelvis confirmed that the right renal mass extended into the right renal vein, the IVC, an accessory hepatic vein, and the right atrium (level IV IVC thrombus). Additionally noted on MRI of the abdomen and pelvis were infrarenal IVC thromboses with extensive retroperitoneal, periportal, and paraspinal venous collaterals (Figure 1).

A multidisciplinary team consisting of specialists in urology, cardiothoracic surgery, maternal fetal medicine, obstetric anesthesia, cardiothoracic anesthesia, and pulmonary critical care was assembled to determine the optimal course of care of the patient and her fetus. The multidisciplinary team decided to perform a staged operation in order to optimize survival for both the patient and her fetus: first, delivery of the fetus by cesarean section after 30 weeks of gestation, followed by right radical nephrectomy with IVC and right atrial thrombectomy under cardiopulmonary bypass (CPB) several days later, after the patient had recovered from the delivery and was medically stable.

The patient underwent an uncomplicated cesarean delivery of a healthy preterm neonate under general anesthesia at 30 weeks and 2 days of gestation with an estimated blood loss of 800 ml. The intraoperative course was uncomplicated, with hemodynamic stability maintained throughout. After 4 days, the patient underwent open right radical nephrectomy, IVC thrombectomy, right atrium and ventricle exploration, and pulmonary artery embolectomy under partial CPB, where the circulation is partially bypassed but some native ejection is still allowed to occur. Operative and CPB times were 519 min and 106 min respectively (20 min on full CPB). During thrombectomy, it was noted that the tumor thrombus extended into the contralateral renal vein but appeared not to invade the wall of either the renal veins or the IVC. The procedure was complicated by the dislodgement of the right atrial mass with embolization of a large fragment into the left proximal pulmonary artery as confirmed by transesophageal echocardiography. This fragment was retrieved under direct visualization. Intraoperatively the patient developed severe coagulopathy (secondary to use of cardiotomy suckers while on partial CPB) requiring transfusion of 14 units of packed red blood cells, 2 units of platelets, 8 units of fresh frozen plasma, 10 units of cryoprecipitate, and infusion of 6 liters of crystalloid fluids. The remainder of the intraoperative course was uncomplicated and the patient was transferred intubated to the cardiac care unit. She was extubated on postoperative day 1.

Pathologic evaluation of the specimen revealed a 13 cm World Health Organization/International Society of Urological Pathology nuclear grade 2 clear cell RCC with tumor thrombus of the bilateral renal veins, IVC, and right atrium (pT3c) (Figure 2). The tumor thrombus was found to invade the wall of the right renal vein.

On postoperative day 4, a CT scan of the abdomen and pelvis revealed complete occlusion and distension of the infrahepatic IVC and thrombi of the right common iliac, right external iliac, and right common femoral veins. A small nonocclusive thrombus was present in the left common femoral vein, but the left common and external iliac veins were patent.

On postoperative day 8, the patient was discharged to home in a stable condition on furosemide and therapeutic enoxaparin with scheduled urologic, cardiothoracic, and oncologic follow up. At 4 postoperative weeks, a CT scan of the chest, abdomen, and pelvis was performed revealing a normal left kidney with no renal mass, a patent intrahepatic IVC and persistent occlusion of the infrahepatic IVC, bilateral iliac and bilateral common femoral veins. Given that this patient had extension of the tumor thrombus into her pulmonary artery and given the presence of several pulmonary nodules, at 9 postoperative weeks, she initiated anti-vascular endothelial growth factor therapy with sunitinib.

Discussion

Surgical management is the mainstay of treatment for RCC diagnosed during pregnancy. Timing and surgical approach are of paramount importance in maximizing both maternal and fetal survival. Antepartum, at parturition, and postpartum timing for surgical resection have been described. Various surgical approaches have also been reported for the management of RCC during pregnancy: open versus laparoscopic versus robotic approaches; transperitoneal versus retroperitoneal approaches; and radical versus partial nephrectomy.

The timing for nephrectomy during pregnancy must balance fetal and maternal morbidity and mortality risks. Nephrectomy may be safely performed in the 1st or 3rd trimester. During the 1st trimester, the small fetal size minimizes injury to the fetus. During the 3rd trimester, nephrectomy may occur at the time of parturition or postpartum if the risk of hemorrhage precludes a concurrent procedure.⁷

At 28 weeks of gestation, a fetus has a >90% survival rate.⁸ Treatment, such as antenatal steroids to enhance fetal lung maturity and decrease neonatal morbidity and mortality as well as magnesium prophylaxis for fetal neuroprotection for fetuses <32 weeks gestation, can be performed to improve neonatal outcome. Surgery during pregnancy with a viable fetus may induce uterine contractions, potentially resulting in premature delivery. Hemodynamic changes during surgery may impact the fetal heart rate, raising the issue of the need for fetal monitoring depending on gestational age and patient preference.

The presence of a right atrial mass poses unique management challenges. Although it is sometimes possible to remove such masses via the IVC, in this case, the size and extent of the mass made this impossible, and the use of CPB and an open approach was deemed necessary. Cardiopulmonary bypass can cause severe utero-placental dysfunction, and the fetal mortality from CPB is high, 18.6% in a recent review.⁹ Thus the strong consensus among the care team was for a staged procedure that pre-empted exposing the fetus to the risks of CPB.

The large intra-atrial mass, and the presence of acute and chronic PE, presented substantial cardiopulmonary risk to the mother as well. Acute and chronic PE can cause severe right heart strain leading to right ventricular (RV) failure and death. The intra-atrial mass could embolize to the main or branch PA (as it did), also leading to acute RV failure. Finally, there can be RV inflow occlusion from a ‘ball-valve’ effect of the mass entering and obstructing the tricuspid valve, leading to acute loss of preload and cardiovascular collapse. All of these factors contributed to the urgency of the case and need for a multidisciplinary approach involving a cardiac anesthesiologist and surgeon for both procedures.

Maternal health may be threatened by the risk of tumor invasion and metastasis, thromboembolism, and operative complications such as infection, hemorrhage, organ injury, multisystem organ failure, and death. The mean uniaxial dimension doubling time for a RCC tumor is 300 days; therefore, surgery is not always emergent.¹⁰ The risk of thromboembolic events in this patient was increased by the hypercoagulable state associated with pregnancy, the presence of lupus anticoagulant antibodies, and the IVC tumor thrombus extending into the right atrium. As in this case, the risk of intraoperative bleeding is increased by the presence of venous collaterals that form in the pregnancy-related hyperdynamic state and as a consequence of chronic IVC occlusion. The technique of partial CPB described above also contributed to the severe coagulopathy that was observed.

No population data are currently available about the prognosis of pregnant patients with RCC IVC tumor thrombi since only one other case has been reported in the literature. For the nonpregnant population with RCC IVC thrombi, a poor prognosis is associated with a higher-grade tumor and nodal involvement. For this population, local tumor invasion appears to have a greater prognostic role than the level of IVC tumor extension. There is a survival benefit associated with nephrectomy plus immunotherapy only for patients with IVC thrombi with no nodal involvement or metastases.^11,12

Conclusion

The management of RCC with IVC tumor thrombus during pregnancy requires a multidisciplinary coordinated approach to determine the optimal timing and approach to surgery in order to minimize fetal and maternal morbidity and mortality. Some benefit has been seen with nephrectomy plus immunotherapy in patients with IVC thrombi but no nodal involvement or metastases. Collective data need to be studied to guide treatment for optimal outcomes in this patient population.

Footnotes

Contributor Information

Efe C. Ghanney, Department of Urology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Jaime A. Cavallo, Department of Urology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Matthew A. Levin, Departments of Anesthesiology and Genomics and Genetic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Ramachandra Reddy, Department of Cardiothoracic Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Jeffrey Bander, Division of Cardiology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Maria Mella, Department of Obstetrics, Gynecology, and Reproductive Science, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Joanne Stone, Department of Obstetrics, Gynecology, and Reproductive Science, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Myron Schwartz, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Kenneth Haines, Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Umesh Gidwani, Division of Cardiology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA. Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Reza Mehrazin, Assistant Professor, Department of Urology, Icahn School of Medicine at Mount Sinai, One Gustave L. Levy Place, Box 1272, New York, NY 10029, USA.

References