HCC with TT in the RA (RATT) should be treated as an oncologic emergency because of the risk of sudden cardiac death and pulmonary embolism. However, there is no consensus on the therapeutic options for HCC with IVC or RA thrombi [6-8]. The practice guidelines of the American Association for the Study of Liver Diseases  and the Barcelona Clinic Liver Cancer guidelines  recommend sorafenib for advanced HCC, including HCC exhibiting major vascular invasion . However, the prognosis remains unsatisfactory because the survival time is only 8.1 months if major vascular invasion is present . Therefore, the only potentially curative option for HCC with TT in the IVC or RA is liver resection with thrombectomy.
Li et al.  classified TT based on the anatomic location relative to the heart. Type I inferior hepatic TT involves the IVC below the diaphragm, type II involves the IVC above the diaphragm but outside the RA, and type III is TT above the diaphragm that reaches the RA. TT in the IVC or RA poses an increased risk of sudden cardiac arrest, liver failure, and pulmonary embolism. This TT classification is useful for selecting the operative procedure, especially for patients requiring CPB . Considering the well-known potential intraoperative adverse effects of CPB, such as coagulation abnormalities with heparin, and postoperative complications, such as immunosuppression and cerebral infarction, its use should be considered carefully . Several reports have described metastasis from a primary tumor after heart surgery using CPB. Hasegawa et al.  describe two mechanisms through which CPB might contribute to dissemination of tumor cells. First, tumor cells that contaminate the reservoir blood may spread through the arterial cannula. Second, disruption of homeostasis by CPB might liberate tumor cells that were spread preoperatively but whose migration or growth were suppressed by the immune system. As CPB is a complex technique with major systemic effects, surgical treatment with simple THVE should be performed if possible . We previously reported that THVE was useful for hepatectomy in patients with tumor invasion into the hepatic vein and IVC or TT in the IVC . Veno-venous bypass maintains cardiac venous return in cases where the tumor is localized to the IVC and suprahepatic IVC clamping is sufficient for safe removal of the tumor. In addition, Sakamoto et al.  mentioned that the RATT may be pulled downward into the IVC by mobilizing the liver caudally if the thrombus has only just entered the RA. By contrast, Ariizumi et al.  strongly advocated that cavo-atrial thrombectomy prior to hepatectomy for HCC with RATT should be performed under CPB, because they experienced a pulmonary embolism due to RATT in a man with HCC who underwent hepatectomy prior to thrombectomy without CPB. CPB was crucial in providing the ability to incise the atrial and IVC walls to easily visualize and precisely extirpate the intravascular and intra-atrial parts of the tumor. However, in this case, the tumor was adherent to the wall of the IVC and atrium. Therefore, “pulling” the intra-atrial tumor en-bloc with the resected liver lobe without a sufficient viewpoint might have resulted in inappropriate resection and possible tearing and embolism of the tumor. In addition, to prevent potential intraoperative dissemination by intraoperative handling, Shirabe et al.  cited the benefit of thrombectomy before hepatic transection. Therefore, we decided to perform CPB for this patient to remove the RATT prior to the liver resection to prevent both pulmonary embolism and dissemination of malignant cells. THVE without CPB might scatter tumor cells during extirpation of the tumor or lead to incomplete removal of the TT if invasion to the wall is present . Further, Wakayama et al.  experienced two cases of intra-IVC recurrence due to incomplete resection of TT on the IVC wall, so the IVC wall should be resected if invasion to the wall is suspected. Moreover, cardiocirculatory arrest seems to be superior to THVE in obtaining a better and exhaustive cleaning from the TT . There are several surgical reports of HCC with RATT simultaneously resected under CPB. Our search of the English and Japanese literature revealed 28 cases of resection of HCC with RATT, in addition to the present case (Table 1) [3, 6, 17, 19-37]. We have found that the mean age is 59.62 ± 10.49 years, which is younger than that of ordinary HCC patients, and only five patients are in seventies. There have been only two previous reports of cases in which thrombectomy under CPB was performed prior to hepatectomy. Liver recurrence and lung metastasis occurred in the 10 patients and in the 9 patients, respectively. Eleven patients including the present case are already deceased due to recurrence of HCC. Prior to our case, the longest one was 56-month survival reported by Yogita et al. 
Hepatectomy with extracorporeal circulation is normally employed only under specific conditions [26–28] because of the technical aspects associated with the procedure. Further, after hepatic resection performed during CPB, uncontrollable bleeding may occur as a result of coagulation abnormalities caused by cirrhosis and the use of heparin. In addition, a variety of complications, including disseminated intravascular coagulation and adult respiratory distress syndrome, may occur after CPB in patients with cirrhosis . Therefore, CPB has not been widely performed for hepatectomy. Even if aggressive surgery for HCC with RATT is performed, the prognosis is not necessarily good. The present case describes the longest survival of a patient with HCC with RATT who underwent simultaneous resection of the liver and TT under CPB. The long survival in this case suggests that the heart-first approach could be better than the liver-first approach from an oncological point of view. In addition, the heart-first approach prevents not only sudden cardiac arrest and pulmonary embolism but also uncontrollable bleeding due to inflow obstruction by the ball valve effect. However, Ariizumi et al.  reported that a large amount of blood loss could occur during liver surgery due to heparinization. We also experienced a large volume of blood loss, probably due to heparin administration.
Almost all HCC patients with TT formation in the vasculature experience recurrence after resection with curative intent . The first recurrence most frequently occurs in the remnant liver, followed by multiple metastases in other organs, including the lungs . As long as the recurrence is confined to the liver, several effective options are available, including repeated hepatectomy, RFA, and TACE . Here, we adopted TACE because of the patient’s preference.
Although thrombectomy alone has been performed in similar cases to avoid sudden death, the outcomes of those cases were poor . Surgical resection is generally contraindicated for patients with unresectable metastates because incomplete resection is a decisive factor for poor prognosis. R1/2 resection has a negative impact on the prognosis, and R0 resection should be attempted . Kasai et al.  altered their criteria for resection and performed preoperative hepatic arterial injection chemotherapy (HAIC) instead of up-front resection for patients with advanced HCC. Notably, preoperative HAIC seems to be associated with a reduced risk of recurrence . Therefore, it could be a promising option for patients with HCC with RATT.
Intense follow-up should be performed during the patient’s life, and multidisciplinary therapies are essential to achieve long-term survival of HCC patients. Our vigorous repeated treatments, including TACE, systemic chemotherapy, and radiotherapy, might have contributed to our patient’s long survival. HCC with RATT is a very rare but critical entity, and aggressive surgical resection using CPB is the only method for obtaining curative treatment and long survival.
In conclusion, we report a case of HCC with RATT treated by aggressive surgery following multidisciplinary therapies after recurrence. Cavo-atrial thrombectomy prior to hepatectomy under CPB provides a safe, controlled approach for resection. Furthermore, CPB is conclusive for providing the ability to visualize the RATT, thereby ensuring satisfactory oncologic margins. This is the only reported case of HCC with RATT with a long survival time of approximately 15 years after surgery.