“The Way to a Man’s Heart” - Adrenocortical Carcinoma With Vascular Invasion Into the Right Atrium and Ventricle

Background: Adrenocortical carcinoma (ACC) is a rare aggressive malignant neoplasm which may present with intravascular extension into the inferior vena cava (IVC) and rarely into the right atrium (RA). Clinical Case: 62-year-old male with no prior known significant medical history presented to ED with 2-day history of mild hematuria with 3-week history of headache. Vital signs were normal other than blood pressure of 198/88 while physical exam was unremarkable. Headache subsided and blood pressure improved to 130/60 range after IV labetalol administration. CT abdomen and pelvis with contrast revealed a large right suprarenal mass extending into the right hepatic vein, IVC, and RA. The right adrenal gland was not visualized while the left adrenal gland and bilateral kidneys were normal. MRI chest, heart and abdomen with contrast showed heterogeneously enhancing lobulated right adrenal mass measuring 11.4 x 11 x 14 cm (AP, transverse, CC, respectively) with extensive tumor thrombus invading the right hepatic vein, IVC, RA and notably protruding into the right ventricle (RV) through the tricuspid valve during diastole. Technitium-99m MDP whole body scan did not show any uptake suspicious for metastases. Pre-op lab assessment showed mildly abnormal 1 mg dexamethasone suppression test but no evidence of ACTH suppression, elevated catecholamines or excess adrenal steroidogenesis. He underwent combined cardiothoracic and abdominal surgery on cardiopulmonary bypass with resection of adrenal mass, removal of thrombus from IVC, RA, RV and patch angioplasty of IVC with bovine pericardium. Pathology report was consistent with ACC (AJCC stage III). On 1 month postoperative follow-up, patient is clinically doing well with plans to start mitotane with addition of etoposide/doxorubicin/cisplatin (EDP) chemotherapy. Conclusion: ACC is a rare, highly aggressive malignancy which may produce extensive intravascular invasion. It may rarely extend to the RA and even rarer into the RV; with 42 and 1 reported cases, respectively. No study has conclusively found that vascular extension of ACC is a poor prognostic factor, hence surgical management is the primary strategy including cases with RA/RV involvement. There is lack of data and consensus regarding adjuvant or palliative medical therapy. However, in phase II trials combination of EDP chemotherapy and mitotane have shown response rates ranging from 11% to 54%. Reference: Alghulayqah, Abdulaziz, et al. “Long-term recurrence-free survival of adrenocortical cancer extending into the inferior vena cava and right atrium: Case report and literature review.” Medicine 96.18 (2017).

Preserving inferior right hepatic vein enabled bisegmentectomy 7 and 8 without venous congestion: a case report

Background In hepatectomy, the preservation of portal perfusion and venous drainage in the remnant liver is important for securing postoperative hepatic function. Right hepatectomy is generally indicated when a hepatic tumor involves the right hepatic vein (RHV). However, if a sizable inferior RHV (IRHV) exists, hepatectomy with preservation of the IRHV territory may be another option. In this case, we verified the clinical feasibility of anatomical bisegmentectomy 7 and 8 with RHV ligation, averting the right hepatic parenchyma from venous congestion, utilizing the presence of the IRHV. Case presentation A 70-year-old man was presented with a large hepatic tumor infiltrating the RHV on computed tomography during a medical checkup. The patient was diagnosed with hepatocellular carcinoma (HCC), T2N0M0, stage III. Right hepatectomy was first considered, but multi-detector computed tomography (MDCT) also revealed a large IRHV draining almost all of segments 5 and 6, suggesting that IRHV-preserving liver resection may be another option. The calculated future remnant liver volumes were 382 mL (26.1% of the total volume) after right hepatectomy and 755 mL (51.7% of the total volume) after anatomical bisegmentectomy 7 and 8; therefore, we scheduled IRHV-preserving anatomical bisegmentectomy 7 and 8 considering the prevention of postoperative liver failure and increased chance of performing repeat resections in cases of recurrence. Preoperative three-dimensional simulation using MDCT clearly revealed the portal perfusion area and venous drainage territories by the RHV and IRHV. There was an issue with invisibility of the anatomical resection line of segments 7 and 8, which was completely dissolved by intraoperative ultrasonography using Sonazoid and the portal dye injection technique with counter staining. The postoperative course in the patient was uneventful, without recurrence of HCC, for 30 months after hepatectomy. Conclusions IRHV-preserving anatomical bisegmentectomy 7 and 8 is a safe and feasible procedure utilizing the three-dimensional simulation of the portal perfusion area and venous drainage territories and the portal dye injection technique.

Feasibility of Right Upper Transversal Hepatectomy in the Absence of an Inferior Right Hepatic Vein: New Insights regarding This Complex Procedure

Background. Right upper transversal hepatectomy (RUTH) is defined as the removal of liver segments 7, 8, and 4A with ligature of the right and middle hepatic veins and is considered one of the most complex techniques of parenchymal-sparing hepatectomies. This procedure can be performed, without venous reconstruction, if collateral veins are present communicating within remnant liver segments to a large inferior right hepatic vein and/or to the left hepatic vein. This venous network could maintain outflow from the inferior right segments (S5, S6) to the left liver when a RUTH is performed, even in the absence of an inferior right hepatic vein. The aim of this study is to present our experience with RUTH without venous reconstruction in patients with and without the presence of an inferior right hepatic vein (IRHV). Methods. Patients submitted to RUTH for treatment of liver metastases were selected from our database. The presence of an IRHV, clinical and surgical characteristics of the patients, immediate outcomes, viability of liver segments 5 and 6, and long-term survival were analyzed. Results. RUTH was successfully performed in four patients. In two patients, IRHV was not present, but intrahepatic communicating veins between proximal right and middle hepatic veins and left hepatic vein were present. No venous reconstructions were performed. Mild congestion of the inferior right segments occurred in the patients where there was no IRHV but no immediate, early, or late complications were observed. Conclusions. RUTH is feasible and can be performed even in the absence of an IRHV, without venous reconstruction. Some degree of congestion of the right inferior liver segments might occur when an IRHV is absent, yet this is not clinically significant when communicating veins are present. Maximum parenchyma preservation might prevent postoperative liver failure and allow repeated resections in case of hepatic recurrence.