Stage
ENSAT
Definition
I
T1 (≤5 cm), N0, M0
T1: Tumor ≤5 cm, localized
II
T2 (>5 cm), N0, M0
T2: Tumor >5 cm, localized
III
T1 or T2, N1, M0
T3 or T4, N0/1, M0
T3: Tumor infiltration into the surrounding adipose tissue
T4: Tumor invasion into adjacent organs or a tumor thrombus in the vena cava/renal vein
IV
M1
Presence of distant metastasis
8.3 Preoperative Workup
Adrenocortical carcinomas with IVC invasion are mainly diagnosed on clinical symptoms associated with hormonal oversecretion or secondary to mass effect (abdominal pain, palpable mass, or signs of compression). Clinical history and examination should assess (1) symptoms related to excess hormone production, including cortisol excess, Cushing’s syndrome, androgen/estrogen excess (virilization in females, feminization in males), signs/symptoms suggestive of multiple hormonal oversecretion, and high blood pressure [14–16] and (2) local compressive symptoms of a large mass, usually nonsecreting, including abdominal or flank pain, abdominal distension, early satiety, nausea/vomiting, weight loss, leg edema, and development of parietal collateral veins [14–17].
Biochemical and hormonal assessment (Table 8.2) should be complete to detail any abnormal oversecretion including steroid precursors, androgens, and cortisol and exclude a malignant pheochromocytoma (24-h urinary meta- and normetanephrine dosage).
Table 8.2
Minimal biochemical assessment for suspected adrenocortical carcinoma
Glucocorticoid | Midnight serum cortisol Basal serum ACTH 24-h urinary-free cortisol 1-mg overnight dexamethasone suppression test |
Androgens or precursor oversecretion | DHEA-S (serum) 17-OH-progesterone, compound S (serum) Androstenedione (serum) Testosterone (serum) 17β-estradiol (serum), in men and menopausal women Deoxycorticosterone (DOC; serum) |
Mineralocorticoid oversecretion | Potassium Aldosterone and renin serum levels |
Catecholamine oversecretion | 24-h urinary metanephrines and normetanephrines |
The goals of imaging workup are to stage the tumor (locoregional extension, venous invasion, distant metastasis) and assess the surgical resectability. This should include thoracoabdominal CT scan, abdominal MRI (Fig. 8.1), and [18] F-fluorodeoxyglucose positron emission tomography (PET scan). The second step of imaging workup is to specify the upper level of the thrombus and direct invasion of the caval wall. This is best achieved by the combination of angio-CT scan and angio-MRI, which are currently considered the most accurate modalities for assessment of ACC with IVC extension [18–21]. Both these noninvasive techniques provide direct evidence of tumor thrombus by demonstrating enlargement of the IVC, and multiplanar reconstruction (MPR) might help to visualize intravenous extension on axial, sagittal, and coronal sections [22]. In most cases, the diagnosis of tumor invasion of caval wall is not reliably detected by preoperative imaging studies and usually made during surgical exploration. In return, the distinction between tumoral and upstream fibrinocruoric thrombus of the IVC can be assessed by the different sequences and multiplanar reconstructions of MRI. In case of thrombus extension, through the cavo-atrial junction or into the right atrium, transesophageal ultrasonography and cardiac MRI can both assess the anatomical upper limits of intracardiac extension and provide useful myocardial and hemodynamic parameters in patients presenting right atrial involvement. These complementary studies should be in our experience performed when the thrombus extends beyond the hepatic veins and when either intrapericardial hepatic vascular exclusion or cardiopulmonary bypass might be required. Conventional cavography is no more used in the preoperative workup of ACC with IVC involvement. Lower extremity deep venous thrombosis should furthermore be excluded by venous Doppler ultrasonography prior to surgical resection of ACC with IVC invasion.
Fig. 8.1
Large right adrenocortical carcinoma invading the inferior vena cava
8.4 Neoadjuvant Treatment
In patients with borderline resectable ACC, preoperative chemotherapy might represent an option, in order to select nonprogressive patients and obtain a significant shrinkage of the tumor thrombus [23]. Nevertheless, in view of the few data available, the limited response rate of current chemotherapy schedules, and risks of tumor thrombus embolism, upfront surgery remains the best therapeutic option in patients with borderline resectable ACC with IVC invasion.
8.5 Surgical Management
Surgical treatment should be considered a priority in patients with resectable ACC with IVC invasion. In metastatic ACC or borderline resectable ACC with IVC invasion, the decision for surgery should be made within the context of a multidisciplinary tumor board. This should take into account several factors including age and presence of comorbidities, risk of tumoral pulmonary embolism, severity of hormonal oversecretion, clinical symptoms of an important adrenal mass, and expertise of the surgical team. The diagnostic and therapeutic pitfalls specific to ACC extending into the IVC were first described in 1972 by Castleman et al. [24]. The use of cardiopulmonary bypass (CPB) techniques to facilitate tumor resection in patients with caval involvement was reported 4 years later by Scully et al. [25]. In 1989, Shahian et al. [26] reported the first procedure using cardiopulmonary bypass (CPB) with hypothermic circulatory arrest (HCA).
Resection of a large ACC raises additional specific problems. The difficulty of achieving “en bloc” resection of a friable tumor without rupturing the peripheral capsule is enhanced by the presence of tumor neovascularization and development of venous collateral circulation due to both tumor size and caval obstruction. The fragility of tissue secondary to prolonged steroid oversecretion may furthermore increase the risks of hemorrhage or tumor disruption. Most ACC with IVC are represented by large stage III (regional involvement of adjacent organs) or stage IV (pulmonary and/or liver metastases) tumors [9]. In stage III disease, ipsilateral nephrectomy and locoregional lymphadenectomy are in our experience constantly required to ensure adequate “en bloc” and safe resection of the primary, which may be associated with right hepatectomy for right ACC or distal pancreatectomy with splenectomy for left ACC in case of locoregional invasion to adjacent organs. This furthermore allows primary vascular control of arterial inflow to the tumor and reduction of blood loss. In stage IV metastatic disease, after careful discussion of surgical indication, ipsilateral nephrectomy and locoregional lymphadenectomy are usually performed together with resection of liver metastases in the absence of concomitant lung metastases, whereas lung metastases are not considered for simultaneous resection.
The optimal approach for surgical resection of ACC with IVC extension depends on the size of the primary tumor, upper limit of intravenous extension, and need for associated procedures (nephrectomy, lymphadenectomy, hepatectomy, distal pancreatectomy, and splenectomy). Bilateral subcostal approach combined with a midline vertical incision appears in most cases the best option for a large exposure, mobilization of the liver, and exposure of the subdiaphragmatic IVC. This approach can be extended to vertical sternotomy when cardiopulmonary bypass with hypothermic cardiac arrest is requested. In favorable patients, midline incision combined with right transverse extension may be sufficient, whereas others have advocated the use of a thoracoabdominal approach extending from the xiphoid appendix to the costochondral junction and extension into the seventh intercostal space.
The modality used for venous control depends on the location and extent of tumor involvement and the amount of collateral venous circulation.
8.5.1 Tumor Thrombus Located Below the Suprahepatic Veins
8.5.1.1 Thrombus Extension Limited to Infrahepatic IVC
This represents the most frequent clinical situation (Fig. 8.2). Cross-clamping of the IVC can be sufficient if the upper limit of tumor thrombus is located below the suprahepatic veins. Thrombectomy is effective for resection of tumor thrombus not involving the venous wall. IVC should be freed up to 2 cm above the upper limits of the thrombus. Vertical cavotomy can be confined to the anterior wall of the IVC, or phlebotomy can be initiated in the renal or adrenal vein and extended to the IVC. Tumor thrombus associated with ACC has a jellylike, friable consistency similar to that of the primary tumor [27]. Resection usually begins with dissection of minor adherences to the caval wall [28] occurring at the level of the ostia of the right adrenal vein or left renal vein. Next, retrograde thrombectomy is performed by gently pulling the endoluminal mass down through the cavotomy. Closure of cavotomy can usually be done by direct suture. The use of prosthetic (Fig. 8.3) or peritoneal patch should be considered if narrowing exceeds 50 % or if there is a significant risk of postoperative thrombosis.
Fig. 8.2
Intraoperative view of a right adrenocortical carcinoma invading the inferior vena cava
Fig. 8.3
Intraoperative view after tumor resection and inferior vena cava prosthetic replacement
8.5.1.2 Thrombus Extension to Retrohepatic IVC
In cases of retrohepatic IVC involvement by ACC, IVC reconstruction is typically recommended because collateral circulation is often reduced during dissection and nonadherent thrombus is not always associated with extensive collaterals [29–31]. Most of the time, the homolateral kidney to the lesion is removed en bloc. This situation always requires full right liver mobilization, 360° dissection of the retrohepatic IVC, up to the IVC below the hepatic vein confluence. IVC reconstruction can require reimplantation of contralateral renal veins whenever possible, avoiding acute renal failure. This is particularly true for the right kidney, which has a short vein without collaterals precluding renal function preservation in cases of simple ligation. Nevertheless a slanting IV section can often preserve the contralateral renal ostium avoiding renal vein clamping and kidney warm ischemia. Reconstruction of the IVC is not required, when collateral circulation is well developed because gradual occlusion of the IVC allows the development of venous collaterals [32]. End-to-end renal vein anastomosis after a retrohepatic IVC resection including the renal vein confluence should be considered as an alternative option for preserving the right kidney when IVC reconstruction is not possible or should be avoided [33]. If IVC replacement is required because of tumor extension to the venous wall, a ringed polytetrafluoroethylene (PTFE) graft should be used. Its size should be a little bit smaller than the native IVC to promote faster velocities through the graft segment and avoid thrombus formation.
8.5.2 Tumor Thrombus Extending Above the Suprahepatic Veins and Below the Cavo-Atrial Junction
It is important to note that most of the time the tumor thrombus is mobile at least on its upper portion. After total mobilization of the right liver, full 360° liberation of the IVC, hepatic vascular exclusion (HVE) is the technique of choice for patients with extension into the retrohepatic or interhepato-diaphragmatic IVC. As previously reported [34], control of the suprahepatic veins or IVC often requires a 5 to 7 cm-diaphragm incision 2–3 cm above the vena caval foramen. It is often necessary to ligate the termination of the left and right inferior phrenic veins at both ends. After, the dissection plan follows the space between the inferior part of the pericardium and the diaphragm. When the supradiaphragmatic IVC is identified, it is dissected on both side; a large blunt dissector is used to tape the intrapericardial portion of the vena cava. Lowering the liver during the procedure puts the IVC in tension and opens the space between the pericardium and the diaphragm. It is important during the dissection to pay a special attention to the right pleura that can be accidentally open. Once the HVE is performed, the thrombus, when free, is first extracted, allowing the replacement of the clamp below the hepatic vein, limiting HVE duration and warm hepatic ischemia. Despite the theoretical risk of engorgement of the liver, we recommend that the upper clamp be placed on the suprahepatic IVC immediately after clamping of the portal triad in order to lower the risk of massive tumor embolization. Tolerance of HVE is generally good in patients with well-developed collateral circulation, but fluid expansion can be performed if necessary [35]. Clamping of the supraceliac aorta can limit blood loss during resection of highly vascularized tumors but is rarely needed. In patients with suprahepatic IVC involvement but below the cavo-atrial junction, cross-clamping of the intrapericardial IVC or partial clamping of the right atrium during HVE can be performed. Usually, thrombectomy, at least of the proximal part of the thrombus, is possible, making unnecessary the replacement of all the IVC with hepatic vein reimplantation.
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