Keywords
renal cell carcinoma - embolization - Budd–Chiari syndrome - Stauffer's syndrome
Introduction
Renal cell carcinoma (RCC) is the most common renal malignancy comprising 85% of all
malignant renal tumors. Venous invasion occurs in 4 to 10% of patients with renal
neoplasms, and within this group, 2 to 16% have tumors extending into the right atrium.[1] The presence of venous extension connotes stage III disease in the Robson classification
and T3 tumor in the TNM staging system. T3 tumors are further subdivided into the
degree of central extension of the tumor thrombus with important implications for
surgical management. Tumor with venous invasion extending to the supradiaphragmatic
inferior vena cava (IVC) is T3c.
When possible, surgery with complete resection of tumor and the associated thrombi
may be attempted with curative intent. Unfortunately, many patients are not surgical
candidates at the time of diagnosis. Renal artery embolization has been proposed as
a palliative treatment for unresectable RCC in patients unfit or unwilling to undergo
surgery.[2] Renal artery embolization for RCC was first described in 1969; since then, various
techniques and embolic materials have been described.
We present a case in which we performed an embolization for a RCC with T3c tumor thrombus.
The patient had severe coagulopathy (international normalized ratio [INR] 2.8) secondary
to rapidly progressive liver dysfunction and was declined for surgical intervention.
The goal was to devascularize and shrink tumor thrombus, thereby improving hepatic
venous outflow. This technical review was performed according to the World Medical
Association Declaration of Helsinki.
Technique
A 70-year-old female patient presented with acute-onset weakness, dyspnea, and decrease
in appetite with an associated 10 lb weight loss. She also reported hematochezia.
A colonoscopy was unrevealing. Initial laboratory evaluation revealed her liver function
tests were aspartate aminotransferase (AST) 1,161 and alanine aminotransferase (ALT)
773. Abdominal ultrasound demonstrated a large right renal mass with extension into
the IVC. Subsequent computed tomography (CT) scan demonstrated a 6 cm right lower
pole renal mass with tumor extension into the IVC and right atrium ([Fig. 1a, b]). An echocardiogram confirmed a 2 to 3 cm right atrial mass consistent with tumor
thrombus.
Fig. 1 (a, b) CT images demonstrating tumor thrombus within the intrahepatic IVC and right renal
lower pole mass. CT, computed tomography; IVC, inferior vena cava.
The patient was evaluated by the tumor board, including the urologist, oncologist,
and nephrologist, and given the high risk of mortality in such a complex surgery,
the patient was referred to interventional radiology for tumor embolization, in the
hope that would result in tumor shrinkage and recanalization of the thrombus.
The patient consented for the procedure after being explained the risks and benefits.
After gaining access via the right common femoral artery, the right renal artery was
selected and subsequently, the lower pole segmental renal artery. Obvious tumor vascularity
in the lower pole was noted, extending into the right renal vein and IVC ([Fig. 2a]). Embolization was performed using 150 to 250 polyvinyl alcohol particles to stasis
([Fig. 2b]).
Fig. 2 (a, b) Digital subtracted angiography image demonstrating tumor vascularity within the
right renal lower pole and tumor thrombus extending into the IVC (a) and postembolization
DSA image demonstrating no residual tumor (b). IVC, inferior vena cava; DSA, digital
subtracted angiography.
After embolization, there was a notable improvement in her laboratories with an AST
41 and ALT 63 after 14 days ([Fig. 3]). There was also gradual improvement of her INR during her hospitalization, which
decreased to 1.5 (from 2.8) in the same time frame. Repeat CT scan only 5 days after
embolization demonstrated axial intrahepatic IVC cross-sectional diameter decreased
from 44 to 39 mm.
Fig. 3 Graph of liver enzyme AST/GOT before and after embolization. AST/GOT, aspartate aminotransferase.
Although the acute phase of Budd–Chiari syndrome (BCS) resolved, the patient was deemed
to be extremely deconditioned due to her complex medical problems, renal tumor, and
thrombus burden. The patient was ultimately discharged to a skilled nursing facility
with plans for palliative care.
Discussion
BCS is defined as obstruction of the hepatic veins resulting in congestive hepatopathy.
The clinical presentation can vary from asymptomatic radiographic findings to severe
fulminant hepatic failure. The classic triad of presentation is abdominal pain, ascites,
and hepatomegaly.[3] The etiology can be divided into primary and secondary causes. Primary BCS results
from the direct occlusion of hepatic veins from an intraluminal source of the thrombus
(most common). Secondary BCS is caused by an extrinsic compression of either the IVC
or the hepatic veins.[3]
RCC, along with hepatocellular carcinoma and adrenal tumors, are the major malignant
conditions capable of direct IVC invasion and venous obstruction. Once the IVC is
obliterated, collateral veins may develop to reestablish venous return to the right
atrium. Collaterals drain via the azygous vein using its extensive communications
with the lumbar and renal veins, or extensive perirenal collaterals.[4]
Usually, the ideal treatment for advanced RCC causing BCS is surgical excision with
removal of the tumor thrombus from the IVC and/or hepatic veins in a select group
of patients. In other patients, treatment for BCS may include anticoagulation and
percutaneous angioplasty with stenting. This treatment may be harmful, with the potential
of dislodging a fragment of the tumor resulting in pulmonary embolism.[3] Surgery before developing decompensated liver disease is paramount. Once the patient
develops hepatic failure following hepatic vein involvement, the outcome is poor.
Kume et al[5] reported four cases presenting at their center over 7 years along with eight cases
reported in international literature. Seven patients had liver failure at the time
of diagnosis, and surgical resection was possible in only one patient.
BCS caused by RCC can be classified into two groups: with or without acute liver failure.
There are no reports in the literature that describe survival in those patients presenting
with acute liver failure secondary to BCS from RCC. Severe sudden onset of BCS is
not a common presentation of RCC and has a very poor prognosis.[6]
However, our patient's liver function improved dramatically after embolization. Repeat
CT scan demonstrated a slight decrease in the size of the intracaval tumor thrombus.
We hypothesize that this slight size change allowed enough room for the hepatic veins
to decompress and resolve the BCS. However, an alternative explanation for the patient's
presentation was considered. In 1961, Dr. Maurice Stauffer, an American gastroenterologist,
recorded liver dysfunction in a patient with RCC without hepatic metastases.[7] Stauffer's syndrome, seen in 3 to 20% of RCC patients, is characterized by elevations
in liver enzymes as well as abnormal levels of hepatic synthetic products. Elevations
of liver enzymes and prothrombin time exist in 66% of cases but usually resolve with
nephrectomy.[7]
RCC embolization was an effective means to bridge the gap between liver decomposition
and the time of surgery. To our knowledge, this is the first reported case of RCC
transcatheter embolization for liver failure secondary to BCS.
