A Case of Metastatic Fumarate Hydratase-Deficient–like Renal Cell Carcinoma Successfully Managed by Ipilimumab plus Nivolumab

We report a 62-year-old male with metastatic fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) without fumarate hydratase (FH) mutation (FH-deficient–like RCC). The International Metastatic RCC Database Consortium risk score was intermediate, and immunotherapy with nivolumab and ipilimumab (Ipi/ Nivo) was initiated. Four cycles of Ipi/Nivo and 5 cycles of nivolumab resulted in a complete response of the metastases. Hypophysitis occurred as an immune-related adverse event after four cycles of Ipi/Nivo. The prognosis of patients with FH-deficient RCC is generally poor. Few reports of FH-deficient RCC successfully treated with Ipi/Nivo have been published. Ipi/Nivo can be effective for treating FH-deficient RCC

Sphingosine-1-phosphate receptor 1 expression in angiosarcoma : Possible role in metastasis and a potential therapeutic target

Sphingosine-1-phosphate (S1P) is a potent lipid mediator that has been implicated in the migration of lymphocytes and endothelial cells through S1P receptors. S1PR1 is strongly expressed in angiosarcoma, a malignant tumor of endothelial cell origin that has a high propensity for metastasis and poor prognosis; however, the pathological significance of S1PR1 expression is not clear. In this study, we investigated the effect of S1PR1 modulation on cell migration, and examined its potential role as a therapeutic target against metastatic dissemination of angiosarcoma. S1PR1 expression in the human angiosarcoma cell line MO-LAS was assessed by immunocytochemical examination and Western blotting. Effects of S1PR1- specific small interfering RNA (siRNA) and that of FTY720-P (a functional S1PR1-antagonist) on MO-LAS cell chemotactic migration towards sphingosine-1-phosphate (S1P) or 10% fetal bovine serum (FBS) were assessed by Transwell migration assay; wound healing assays for random cell migration were performed using a live cell analyzer. Immunostaining revealed high expression of S1PR1 on the MO-LAS cell membrane. Transwell and wound-healing assays showed that S1P enhanced chemotactic and random migration of MO-LAS cells, respectively. Inhibition of S1PR1 expression with siRNA significantly attenuated chemotaxis of cells towards S1P and 10% FBS. Further, FTY720-P strongly induced the internalization and degradation of S1PR1 even in the presence of serum containing S1P. It attenuated chemotactic cell migration of MO-LAS towards both S1P and serum, as well as the random motility of cells at nanomolar concentrations. These results suggest that the S1P/S1PR1 axis may be a potential therapeutic target for inhibition of angiosarcoma metastasis by controlling its cell motility

Robotic Renal Autotransplantation: First Case Outside of North America

A 38-year-old woman with a 2.7-cm left ureteral stenosis requiring chronic ureteral stent exchange elected to undergo robotic renal autotransplantation. Left ureteropelvic junction obstruction (UPJO) was also suspected. Robotic donor nephrectomy contributed to the fine dissection for desmoplastic changes. The kidney was removed through a Gelport and examined on ice. UPJO was not seen. An end-to-side robotic anastomosis was created between the renal and external iliac vessels. The console time was 507 min, and the warm ischemia time was 4 min 5 sec. She became stent-free. Robotic renal autotransplantation is a new, minimally invasive approach to renal preservation

Feasible kidney donation with living marginal donors, including diabetes mellitus

Objectives: To compare the donor outcomes of living donor kidney transplantation between standard donors (SDs) and marginal donors (MDs) including diabetic patients (MD + DM). Methods: MDs were defined according to Japanese guideline criteria: (a) age >70-years, (b) blood pressure 25 to = 70 to 6.2 or Results: No kidney function parameters were different between SDs and MDs. When comparing SD and MD + DM, MD + DM had a lower postoperative eGFR (48 vs. 41 (1 (month), p = .02), 49 vs. 40 (12, p = 2 risk factors. Conclusions: Although long-term observation of donor kidney function is necessary, careful MD + DM selection had the potential to expand the donor pool

ABO Blood Incompatibility Positively Affects Early Graft Function: Single-Center Retrospective Cohort Study

Background We investigated the association between ABO-incompatible (ABO-I) kidney transplantation and early graft function. Methods We retrospectively analyzed 95 patients who underwent living donor kidney transplantation between May 2009 and July 2019. It included 61 ABO-compatible (ABO-C) and 34 ABO-I transplantations. We extracted data on immunologic profile, sex, age, cold ischemic time, type of immunosuppression, and graft function. Two definitions were used for slow graft function (SGF) as follows: postoperative day (POD) 3 serum creatinine level >3 mg/dL and estimated glomerular filtration rate (eGFR) Results The characteristics between the ABO-C and ABO-I were not different. ABO-I received rituximab and plasma exchange. Patients also received tacrolimus and mycophenolate mofetil for 2 weeks and prednisolone for 1 week before transplantation as preconditioning. Of the 95 study patients, 19 (20%) and 21 (22%) were identified with SGF according to POD 3 serum creatinine level or eGFR, respectively. Multivariable analysis revealed that ABO-I significantly reduced the incidence of SGF (odds ratio, 0.15; 95% confidence interval, 0.03-0.7; P = .02), and cold ischemic time >150 min increased the incidence of SGF (odds ratio, 6.5; 95% confidence interval, 1.7-25; P = .006). Similar results were identified in POD 3 eGFR. Inferior graft function in patients with SGF was identified up to 6 months after transplantation. Conclusion ABO-I reduces the incidence of SGF, which is associated with an inferior graft function up to 6 months

Robotic Renal Autotransplantation: A Feasibility Study in a Porcine Model

We investigated the feasibility of robotic renal autotransplantation (RAT) in a porcine model to reduce invasiveness of RAT. Five pigs underwent robotic RAT using the da Vinci® robotic system. A robotic left nephrectomy was performed in all cases. Robotic RAT was performed on the left side in all but one case. Four ports were used. In 3 cases, the kidney was taken out through the GelPort® and irrigated on ice with Ringer’s solution. In 2 cases, a complete intracorporeal robotic RAT was performed. An end-to-side anastomosis was performed between the renal vein and the external iliac vein and between the renal artery and the external iliac artery. Ureteroneocystostomy was also performed in 2 cases. All cases were performed robotically without open conversion. The median (IQR) console time was 3.1 (0.7) h, and the operative time was 3.8 (1.1) h. The estimated blood loss was 30 (0) ml. The warm ischemia time was 4.0 (0.2) min, and the cold ischemia time was 97 (17) min. Intracorporeal transarterial hypothermic renal perfusion was feasible in the 2 complete intracorporeal robotic RAT cases by using a perfusion catheter through a laparoscopic port. Robotic RAT has the potential to be a new minimally invasive substitute for conventional open surgery

Combined Laparoscopic and CT Monitoring of the Ice-Ball Margin during Cryoablation for Renal Cell Carcinoma Associated with von Hippel-Lindau Disease: First Case

We report a 47-year-old Japanese female with 10 previous treatments for multiple bilateral renal cell carcinoma (RCC) associated with von Hippel-Lindau disease. The 14-mm right lower pole renal tumor was in contact with the right ureter. Laparoscopic cryoablation was performed to protect the ureter wrapped with gauze. Computed tomography (CT) monitoring was used to confirm the precise ≥ 6 mm ice-ball margin. There was no local progression at 6-months post-surgery. The serum creatinine has been stable. This is apparently the first report of combined laparoscopic and CT monitoring of an ice-ball formation and its margin during cryoablation for RCC

成人発症の難治性胞巣型横紋筋肉腫に対する Vincristine Irinotecan Temozolomide 療法を用いた治療経験

　成人発症の胞巣型横紋筋肉腫は，まれであり，再発しやすく予後が悪いと言われている．さまざまな化学療法が試されているが効果的な報告は少ない．我々は，難治性胞巣型横紋筋肉腫に対してVincristine Irinotecan Temozolomide（VITA）療法を施行し，長期完全奏効（Complete Response：CR）を維持している症例を経験したため報告する．症例は20歳代の男性で，トルコ鞍斜台に腫瘍を認め，摘出生検を施行し胞巣型横紋筋肉腫と診断された．残存腫瘤に対して放射線治療を施行し，CT 検査で病変の消失を確認した．術後３か月後に右頸部リンパ節腫大が出現し，リンパ節郭清術を施行した．その２か月後に左頸部リンパ節腫大が出現したため，Childrens Oncology Group ARST0431レジメン（VI 療法とVDC/IE 交代療法およびVAC 療法によるブロックから構成される計54週間の治療）を施行し，PET/CT でFDG 集積の消失を確認し，CT でもリンパ節腫大病変は消失した．しかし，１年後に左頸部リンパ節腫大が再度出現．再発と判断し，VITA 療法を開始した．６コース施行しCT にてCR を確認した．以後，外来で経過観察中でありCR を維持している．難治性胞巣型横紋筋肉腫に対してVITA 療法は有用であると考えられ，治療法の選択枝の一つとして考慮すべきである．　Adult onset alveolar rhabdomyosarcoma is rare, but recurrence is common and prognosis is poor. Various types of chemotherapy regimens have been attempted, but few studies have reported information on efficacy. Here, we report our experience with a patient who received Vincristine Irinotecan Temozolomide (VITA) therapy and in whom long-term (complete response: CR) was maintained. The patient was a 20-year-old man who developed alveolar rhabdomyosarcoma in the clivus of the sella turcica. Following enucleation and postoperative radiotherapy, lesion disappeared by CT was achieved. However, right cervical lymphadenopathy was soon apparent, and a lymphadenectomy was performed. Because left cervical lymphadenopathy was apparent after 2 months, the Children\u27s Oncology Group ARST0431 regimen (54 weeks of therapy: blocks of therapy with VI, interval compression with VDC/IE and VAC) was initiated, negative PET imaging and lesion disappeared by CT was achieved. However, reappearance of left cervical lymphadenopathy was noted after 1 year. Recurrence was diagnosed in the patient, and VITA therapy was initiated again. CR was achieved after 6 courses, and subsequent outpatient follow-up has revealed that CR has been maintained. Hence, VITA therapy may be useful for treatment-resistant alveolar rhabdomyosarcoma and should be considered as a treatment option