Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review.

A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms "perivascular epithelioid cell tumor", "PEComa", "liver", and "hepatic". All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality.

Short- and long-term outcomes of surgery for colorectal and non-colorectal liver metastasis: a report from a single center in the Baltic country.

BACKGROUND: The liver is a major target organ for metastases of various types of cancers. Surgery is a well-established option for colorectal liver metastases (CRLM). Regarding the improved surgical and anesthetic techniques, the safety of liver resection has increased. Consequently, the interest in the surgical management of non-colorectal liver metastases (non-CRLM) has gained significant attention. Therefore, this study was designed to investigate the surgical treatment outcomes for non-CRLM and to compare it with an outcome of CRLM in a tertiary care center in the Baltic country-Lithuania. METHODS: We retrospectively analyzed data from all patients who underwent liver resection for CRLM or non-CRLM between 2010 and 2017 in a tertiary care center-Vilnius University hospital Santaros Clinics. Demographic and metastasis characteristics, as well as disease-free and overall survival, were compared between the study groups. RESULTS: In total, 149 patients were included in the study. Patients in the CRLM group were older (63.2 ± 1.01 vs 54.1 ± 1.8 years, p < 0.001) and mainly predominant by males. Overall postoperative morbidity rate (16.3% vs 9.8%, p = 0.402) and major complications rate (10% vs 7.8%, p = 0.704) after liver resection for CRLM and non-CRLM was similar. Kaplan-Meier analysis showed higher disease-free survival in the CRLM group with 89.4% vs 76.5% and 64.9% vs 31.4% survival rates at 1 and 3 years, respectively (p = 0.042), although overall survival was not different between the CRLM and non-CRLM groups with 89.4% vs 78.4% and 72.0% vs 46.1% survival rates at 1 and 3 years, respectively (p = 0.300). CONCLUSIONS: In this study, we confirmed comparable short- and long-term outcomes after liver resection for CRLM and non-CRLM. Surgical resection should be encouraged as an option in well-selected patients with non-CRLM.

The Impact of Chemotherapy and Transforming Growth Factor-ß1 in Liver Regeneration after Hepatectomy among Colorectal Cancer Patients.

An ongoing debate surrounds the impact of chemotherapy on post-hepatectomy liver regeneration in patients with colorectal cancer liver metastases (CRLM), with unclear regulatory mechanisms. This study sought to delve into liver regeneration post-resection in CRLM patients, specifically examining the roles of hepatocyte growth factor (HGF) and transforming growth factor ß1 (TGF-ß1). In this longitudinal observational study, 17 patients undergoing major liver resection for CRLM and 17 with benign indications as controls were enrolled. Liver regeneration within 30 postoperative days was assessed via CT, considering clinicopathological characteristics, liver enzymes, liver stiffness by elastography, and the impact of HGF and TGF-ß1 on liver regeneration. The results revealed that the control group exhibited significantly higher mean liver regeneration volume (200 ± 180 mL) within 30 days postoperatively compared to the CRLM group (72 ± 154 mL); p = 0.03. Baseline alkaline phosphatase (AP) and TGF-ß1 blood levels were notably higher in the CRLM group. Immunohistochemical analysis indicated a higher proportion of CRLM patients with high TGF-ß1 expression in liver tissues compared to the control group (p = 0.034). Correlation analysis showed that resected liver volume, baseline plasma HGF, AP, and albumin levels significantly correlated with liver regeneration volume. However, in multivariable analysis, only resected liver volume (ß: 0.31; 95% CI: 0.14-0.47, p = 0.01) remained significant. In conclusion, this study highlights compromised liver regeneration in CRLM patients post-chemotherapy. Additionally, these patients exhibited lower serum TGF-ß1 levels and reduced TGF-ß1 expression in liver tissue, suggesting TGF-ß1 involvement in mechanisms hindering liver regeneration capacity following major resection after chemotherapy.

Malignant mixed epithelial and stromal tumour of the kidney: a case report and a literature review.

INTRODUCTION: Mixed epithelial and stromal tumour of the kidney (MEST) is a rare and distinctive neoplasm accounting for 0.2% of all renal cancers. Most of these tumours behave in a benign fashion but 13 cases with malignant transformation have already been reported. We present the first case of an extremely aggressive MEST with rapid recurrence after radical treatment, demonstrating objective response to chemotherapy. CASE PRESENTATION: A 31-year-old female presented to the hospital complaining of gross hematuria. Computed tomography (CT) revealed an intraparenchymal mass in the left kidney forming a tumour thrombus in the inferior vena cava (IVC). Metastatic disease was ruled out and, under the clinical diagnosis of renal cell carcinoma, left radical nephrectomy with IVC thrombectomy was performed. The histopathological examination confirmed malignant MEST of the kidney. At the follow-up 12 months after surgery, a recurrent tumour in the left paravertebral area and a tumour thrombus in the IVC were detected. A second surgery was recommended and the mass from the paravertebral area was removed, so resection of the IVC with prosthetic replacement was performed. The histopathologic examination confirmed a recurrent malignant MEST. At the follow-up three months after the second surgery disease progression was diagnosed, so chemotherapy with ifosfamide and doxorubicin was initiated. The CT scan performed 14 months after the chemotherapy confirmed a stable process of the disease with no signs of progression. CONCLUSIONS: A literature review and our case report confirm the existence of extremely aggressive malignant MEST that shows response to chemotherapy. However, more reports are needed to improve our understanding about the biology of the MEST to develop any recommendations on personalized therapy.

Fatal Liver and Lung Alveolar Echinococcosis with Newly Developed Neurologic Symptoms due to the Brain Involvement.

The fox tapeworm Echinococcus multilocularis causes human alveolar echinococcosis, commonly affecting the liver. However, in ∼1% of cases, systematic spread of the disease involves the brain as well. A patient had a 6-year history of liver and lung alveolar echinococcosis that was considered not suitable for surgery, and treatment with albendazole was introduced. After the appearance of neurologic disturbances, an intracranial mass lesion was demonstrated by radiologic imaging. The lesion was surgically removed, and histologic analysis revealed metacestode tissue of E. multilocularis . Despite the surgical resection of the lesion, the patient died of progression of systemic alveolar echinococcosis. The authors highly recommend implementing neurologic monitoring to the follow-up algorithm for patients with systemically disseminated alveolar echinococcosis. When neurologic symptoms occur, radiologic imaging of the brain should be obtained immediately. Surgery should be considered for all intracranial echinococcal lesions, unless the lesion is located in the eloquent brain area.

Echinococcus infections in the Baltic region.

In the Baltic countries, the two zoonotic diseases, alveolar echinococcosis (AE) caused by Echinococcus multilocularis, and cystic echinococcosis (CE) caused by Echinococcus granulosus, are of increasing public health concern. Observations from Estonia, Latvia and Lithuania indicate that the distribution of both parasites is wider in the Baltics than previously expected. In this paper, we review and discuss the available data, regarding both parasitoses in animals and humans, from the Baltic countries and selected adjacent regions. The data are not easily comparable but reveal a worrisome situation as the number of human AE and CE cases is increasing. Despite improvements in diagnostics and treatment, AE has a high morbidity and mortality in the Baltic region. For the control of both zoonoses, monitoring transmission patterns and timely diagnosis in humans as well as the development of local control programs present major challenges.

Orthotopic liver transplantation: the first experience and results of the Vilnius University Hospital Santariskiu Klinikos.

BACKGROUND: Liver transplantation has become the treatment of choice for chronic and acute end-stage liver failure as well as for selected cases of malignancies and metabolic disorders. We report our first experience of the orthotopic liver transplantation. MATERIAL/METHODS: Between 2005 and 2008 16 cadaveric orthotopic liver transplantations in 16 adults (12 males, 4 females, mean age 44 years) were performed. Main indications for orthotopic liver transplantation were cholestatic liver disease (31%), viral-induced cirrhosis (25%), alcoholic liver disease (19%), hepatocellular carcinoma associated with hepatitis virus infection (13%), autoimmune cirrhosis (6%), cryptogenic acute liver failure (6%). Mean follow-up was 15 month (range: 4 days - 43 month). RESULTS: Intraabdominal haemorrhage was observed in 6 patients (37.5%). Vascular complications were observed in 3 patients (18.75%). Biliary complication were observed in 3 patients (18.75%). Overall 1 year patient survival was 87,5%. Four (25%) patients died during follow-up. All patients died because of sepsis and multiorgan system failure. CONCLUSIONS: Our first results showed that secret of successful liver transplantation is perfect interdisciplinary team approach, including selection of the recipient and timing of transplantation, the operative procedure itself, prevention and treatment of complications, the perioperative anaesthesiological and intensive-care management, and careful follow up after transplantation.