Introduction: Resecability of liver tumors is exclusively depending on the future liver remnant (FLR). The remnant can be hypertrophised using portal vein occlusion techniques. The latest hypertrophising method is Associating Liver Partition and Portal vein ligation for Staged hepatectomy (ALPPS), which provides the most significant induced hypertrophy in the shortest time. Morbidity and mortality of this procedure were initially unacceptably high. Aim: Reducing complications by better patient selection and modified surgical technique. Method: The First Department of Surgery, Semmelweis University, Budapest, prefers the ‘no touch’ technique, instead of ‘complete mobilization’. For optimizing patient selection, an international registry (including our patients’ data) was established. In addition to the surgical, we collected demographic, disease, liver function, histology, morbidity (Clavien–Dindo) and mortality parameters. Volume and function measurements were performed by using CT-volumetry and 99mtechnecium-mebrofenin SPECT/CT. Data were analyzed by multivariate analysis (significance: p<0.05). Results: We performed 20 ALPPS procedures from 2012 to 2018. The relative volume increment and resectability in our department and among the 320 registry patients were 96% vs. 86% and 95% vs. 98%. Using ‘no touch’ technique, the Clavien–Dindo III–IV morbidity and mortality rates were significantly lower (22%–0%) than with ‘complete mobilization’ (63%–36%) (p<0.05). Based on the multivariate analysis of the registry patients, age over 60 years, liver macrosteatosis, non-colorectal liver tumor, >300 minutes operation time, >2 units of red blood cell transfusion, or insufficient FLR function before stage 2 were identified as independent factors influencing mortality (p<0.05). Conclusion: Mortality and morbidity of ALPPS can be reduced by proper patient selection and ‘no touch’ surgical technique. Orv Hetil. 2019; 160(32): 1260–1269.

Introduction Rhabdomyolysis is a syndrome characterized by a rapid necrosis of muscle fibers and the release of muscle-derived metabolic products into the circulatory system. A rare cause of rhabdomyolysis is paraneoplastic polymyositis. Case presentation A 67-year-old man was diagnosed with paraneoplastic polymyositis and rhabdomyolysis caused by hepatocellular carcinoma (HCC). Intravenous steroid was used as a symptomatic therapy for rhabdomyolysis, and the tumour was removed by left hemihepatectomy to treat the underlying cause. After muscle strength gradually improved, steroid therapy was discontinued. The patient was reoperated multiple times due to bleeding and bile leakage. Following the operations, his overall state and muscle strength further improved. Despite that, the patient's condition worsened again, and eventually, he died of candida albicans pneumonia and sepsis. Discussion HCC is an extremely rare cause of paraneoplastic polymyositis and rhabdomyolysis. Treatment is challenging, as none of the few available case reports record long term survival and less than half of the reports record muscle strength improvement. In our case, the patient was treated with systemic steroid therapy and resection of the tumour. The patient's muscle strength temporarily improved, but subsequently, the patient died. Conclusion Our case confirms the importance of a definitive treatment of HCC, as we achieved a significant improvement in muscle strength by removing the tumour. On the other hand, our paper highlights the dangers of double-sided steroid therapy, which, combined with the essential, effective treatment of rhabdomyolysis, may have contributed to the development of postoperative complications and candida sepsis leading to death.

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Összefoglaló. A vena cava inferior leiomyosarcomája a vena tunica mediájából kiinduló igen ritka rosszindulatú daganat. Tünetei és radiomorfológiai jelei nem mindig juttatnak pontos kórisméhez, szövettani mintavétele pedig elhelyezkedése miatt veszélyes lehet. Diagnózisa ezért sokszor jelent kihívást a klinikusok számára. Kezelése elsősorban sebészi, amelyet együtt vagy monoterápiában alkalmazott radioterápia és kemoterápia egészít ki. Esetünkben egy 74 éves nőbeteg tünetmentes, a v. cava inferior jobb v. renalis fölötti részének jobb mellékvesével összefüggő tumorát találtuk. Endokrinológiai kivizsgálása során a szérum kortizol, adrenalin, noradrenalin, adenocorticotrop hormon (ACTH), teljes és szabad tesztoszteron, dehidroepiandroszteron-szulfát (DHEA-S), nemi hormon kötőfehérje (sex hormone binding globulin, SHBG) normál tartományban volt, a tumor hormonszekréciót nem mutatott. A tumor sebészi eltávolítása mellett döntöttünk. Preoperatív biopsziát annak kockázatai miatt nem végeztünk. A műtét során a v. cava inferior daganatát találtuk, a tumor eltávolítását végeztük a vena falának resectiójával, a jobb mellékvese eltávolításával és a jobb v. renalis neoimplantatiójával. A jobb feltárás érdekében a műtét elején cholecystectómiát is végeztünk. A műtét után szövődmény nem jelentkezett. A beteg adjuváns radioterápiában részesült. A tumor korai diagnózisának és en bloc resectiójának köszönhetően jelentősen növeltük a beteg gyógyulási esélyeit, az elvégzett radioterápiával pedig csökkentettük a recidíva valószínűségét. Summary. Leiomyosacroma of the inferior vena cava is an extremely rare malignancy originating from the tunica media of the venous wall. Its symptoms and radiomorphological signs do not always lead to an accurate diagnosis. Histological sampling can be dangerous due to its location. Therefore the diagnosis is often a challenge for clinicians. Its treatment is primarily surgical, supplemented by radiotherapy and chemotherapy applied together or in monotherapy. In our case, an asymptomatic 74-year-old female patient was diagnosed with a tumor of the inferior caval vein located just above the right renal vein and involving the right adrenal gland. As serum cortisol, epinephrine, norepinephrine, adrenocorticotropic hormone (ACTH), total and free testosterone, dehydroepiandrosterone sulfate (DHEA-S), sex hormone binding globulin (SHBG) was at normal level, the tumor showed no hormone secretion. Primary surgical resection was planned. Preoperative biopsy was not performed due to its high risk of complications. During operation, tumor resection was performed by resection of the venous wall, removal of the right adrenal gland and neoimplantation of the right renal vein. For better exposure, cholecystectomy was also performed at the beginning of surgery. There were no postoperative complications. The patient received adjuvant radiation therapy. Thanks to the early diagnosis of the tumor and the complete resection, we significantly increased the patient’s chances of total recovery. With the radiotherapy performed, we reduced the likelihood of tumor recurrence.

Összefoglaló. A focalis nodularis hyperplasia (FNH) a máj második leggyakrabban előforduló benignus góca. Nem malignizálódik, így a jelenlegi ajánlások szerint eltávolítása relatív indikációt képez. Kezelését illetően több különböző módszer is ismert. A közlemény célja, hogy összefoglalót adjon az FNH ellátásáról és két esetben bemutassa az alternatív kezelési lehetőségeket. Egy 40 éves nőbetegnél hasi panaszok miatt indult kivizsgálás során a májban CT-morfológiailag FNH igazolódott. A laesio lokalizációját figyelembe véve, a sebészeti reszekció magas rizikója miatt, transarterialis embolisatio (TAE) mellett döntöttünk. Többszörös kezelést követően a kontroll képalkotó vizsgálatok alapján jelentős regressziót értünk el, mindemellett a beteg panaszmentessé vált. Egy 25 éves nőbetegnél hasi panaszok miatt történt MR-vizsgálat igazolt FNH-t. Az elhelyezkedésre tekintettel TAE-t végeztünk, a beteg azonban ezúttal nem vált teljesen panaszmentessé. Definitív megoldásként májreszekció történt, melyet követően panaszai megszűntek. Tünetmentes FNH felfedezésekor elegendő lehet a betegek hosszú távú követése. Tünetek, illetve nagy műtéti kockázat esetében a malignus májtumoroknál rutinszerűen alkalmazott TAE az FNH-ban akár önmagában vagy sebészi kezeléssel kombinálva is eredményesen alkalmazható. Kisebb rizikó esetén a legmegfelelőbb választás a primer laparoszkópos reszekció. Orv Hetil. 2022; 163(15): 606–612. Summary. Focal nodular hyperplasia (FNH) is the second most common benign mass of the liver. According to the current recommendations, removal makes relative indication. Several different treatment methods are known. The purpose of the paper is to provide a summary of FNH care and to present alternative treatment options in two cases. A 40-year-old woman was investigated for abdominal complaints; CT scan confirmed FNH of the liver. Given the localization of the lesion, we chose transarterial embolization (TAE) due to the high risk of surgical resection. After multiple treatments, regression was achieved, and the patient became asymptomatic. Painful FNH in a 25-year-old female was confirmed by MRI. The lesion was dominantly seen in segment 1, causing vena cava compression and collaptiform episodes. As a definitive solution, liver resection was performed, after which her complaints ceased. Long-term follow-up of patients may be sufficient when asymptomatic FNH is detected. In the case of symptoms or high risk of surgery, TAE can be used effectively by FNH either alone or in combination with surgical treatment. For lower-risk patients, primary laparoscopic resection is the most appropriate choice. Orv Hetil. 2022; 163(15): 606–612.