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Undifferentiated embryonal sarcoma of the liver (UESL) is very rare and has a very poor prognosis. UESL metastases have been reported in 5%-13% of the children with UESL and most metastases reported in the literature are present at diagnosis. Metastases reported in the literature belong to the lungs, pleura, and peritoneum. Radiological diagnosis of the UESL remains a poorly understood problem due to its rarity. Most of the reports published in the literature are also based on a relatively small number of patients. Approximately 200 cases have been reported regarding imaging features of this tumor. We reported a girl with UESL, who applied to the emergency department with abdominal pain. The lesion was solid and had cystic areas on ultrasound and there were peripherally enhanced serpenginous vessels in the lesion on Computed Tomography and MRI. Immunohistochemical diagnosis of the lesion was UESL. 26 months after surgery and adjuvant chemotherapy extradural and subcutaneus metastases were detected. These metastasis sites were first described for UESL.

Purpose Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant lesion in adults. Here, we present an extremely rare case of UESL in a pregnant woman. However, this was the first case, wherein one surgery covered all the procedures needed to be performed, including delivery of the neonate, hysterectomy, and resection of the left liver lobe to ensure the safety of both the mother and the newborn. Case presentation A 32-year-old woman at 37-weeks age of gestation was admitted at Bach Mai Hospital (Hanoi, Vietnam) due to epigastric pain, chest pain, and breathing difficulty. Blood tests showed an increased total alpha-fetoprotein level of 1085 ng/mL. Fetal ultrasound showed a fetal heart rate of 127 beats/minute, biparietal diameter of 92 mm, femur length of 70 mm, and fetal weight of 3287 g. Magnetic resonance imaging indicated a large tumor (12 × 14 × 18 mm) in the left liver, with organ signals increased on T2-weighted and decreased on T1-weighted. The mass was lobulated, developed along a downward pattern, and did not invade the portal vein. The surgeries performed were cesarean section, hysterectomy due to the inelasticity of the uterus, and left hepatectomy to ensure the safety of both the mother and newborn. The operation was performed safely and without any complications, and the patient was discharged from the hospital after 10 days. Conclusion This case report with distinct characteristics has been the first case ever reported in Vietnam, as well as in the literature. We believe that this case report will be useful for the clinical evidence pool and for educational purposes.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumour that occurs mainly in children. Herein, we aimed to identify any differences in clinical characteristics and survival between adult and paediatric patients with UESL.From 1975 to 2015 in the Surveillance, Epidemiology and End Results (SEER) database, patients diagnosed with UESL were identified and divided into paediatric (18 years) and adult (≥18 years) groups. We then compared the clinical characteristics, management, and overall survival (OS) of adults and children diagnosed with UESL.We analysed 113 patients with UESL (81 children and 32 adults). UESL was significantly more common in adult male than paediatric male patients (71.9% vs. 48.2%; P = 0.022). When compared to adult patients, paediatric patients were more likely to receive chemotherapy (93.8% vs. 65.6%; P 0.001). Adults had a significantly worse OS than paediatric patients (5-year OS, 30.0% vs. 81.2%; P 0.001). Univariate analysis found that adult age, surgical therapy and chemotherapy were associated with OS. Multivariate analysis revealed that adult age, SEER summary stage and surgical therapy were independent prognostic factors for OS.UESL had a male predominance among adult patients. Moreover, the prognosis of adult patients with UESL was significantly worse than that of paediatric patients. Surgery and chemotherapy should be considered in the treatment of patients with UESL.

We present a case of undifferentiated embryonal sarcoma of the liver (UES), an uncommon malignant mesenchymal tumor that occurs predominately in children and is very rarely seen in adults. Our patient is a 32-year-old pregnant female without significant past medical history, who presented to the emergency department with worsening abdominal pain and a large palpable abdominal mass. Imaging identified a large, hemorrhagic right hepatic lobe mass that in retrospect had imaging characteristics most suggestive of UES. Though extremely rare in adults, it is still important to recognize the imaging features of UES as to avoid mistaking it for other more common benign and malignant hepatic masses.

Liver tumors in children can be benign or malignant. Although several clinical factors are important in the evaluation of these lesions, MRI is particularly important for lesion characterization and tumor staging. In children, use of a hepatobiliary contrast agent is recommended to evaluate a known or suspected liver lesion. In this review, we discuss the most common benign and malignant pediatric liver tumors, including vascular tumors, mesenchymal hamartoma, focal nodular hyperplasia, hepatocellular adenoma, hepatoblastoma, hepatocellular carcinoma, fibrolamellar hepatocellular carcinoma, undifferentiated embryonal sarcoma and metastases, with emphasis on relevant clinical information and imaging appearance at MRI using hepatobiliary agents.

BACKGROUND Undifferentiated embryonal sarcoma of the liver (UESL) is a neoplasm that rarely develops in adults. The main treatments for UESL are upfront gross total surgical resection and adjuvant multiagent chemotherapy. Here, we report a case of recurrent UESL in an adult treated with pembrolizumab and discuss a method to identify proper candidates for antibody of programmed cell death protein 1 (anti-PD-1) treatment. CASE SUMMARY A 69-year-old woman was admitted for abdominal pain that developed for 1 wk. Computed tomography showed a 16 cm mass in the right lobe of the liver. Right hemihepatectomy and lymphadenectomy were performed, and histological diagnosis was UESL. Six months later, the patient suffered from painless obstructive jaundice, and positron emission tomography-computed tomography revealed multiple metastases. Then, percutaneous transhepatic cholangial drainage was applied to reduce jaundice, and radiofrequency ablation was used to control the lesion near the hepatic hilum. However, the patient suffered from a serious fever caused by the tumor. The patient received treatment with pembrolizumab, and the prescribed dosage was 2 mg/kg every 3 wk. After the seventh dose, positron emission tomography-computed tomography revealed that the multiple metastases had nearly disappeared. Radiologic exam was used to evaluate the disease state, and no new lesions were found. Next-generation sequencing and immunohistology were applied to determine the reason why the patient had such a favorable response to pembrolizumab. Tumor mutation burden, microsatellite instability, and programmed death ligand 1 expression can be combined to predict the effect of PD-1 antibodies. When every one of these biomarkers are detected in a tumor patient, the patient may be a proper candidate for PD-1 antibodies. CONCLUSION Anti-PD-1 treatment for tumors needs further research to identify indications and proper biomarkers.

The undifferentiated embryonal sarcoma of the liver is a rare tumor with a poor prognosis, with improved outcomes being reported with more recently multimodality treatments. We report a case of a 6-year-old girl with an incidentally diagnosed and histologically proven localized undifferentiated embryonal sarcoma of the liver. The divergence between solid appearance at US and cystic-like appearance on CT/MRI, which has been attributed to the presence of myxoid component frequently described with this tumor, was crucial for the diagnosis and subsequent treatment.

Resumen Objetivo Los tumores hepaticos son infrecuentes en la poblacion pediatrica. Entre los mas frecuentes se encuentran el hamartoma mesenquimatoso y el sarcoma embrionario indiferenciado, de diferente estirpe, pero con similitudes de imagen. El proposito de este articulo es repasar los hallazgos caracteristicos en las imagenes y sus diagnosticos diferenciales. La ecografia es el metodo inicial para su estudio. La resonancia magnetica y la tomografia computarizada son utiles para una mejor caracterizacion tumoral y planificacion quirurgica. Conclusion El radiologo debe estar familiarizado con las caracteristicas de imagen de las diferentes patologias y evaluarlas junto a la edad, antecedentes personales y analisis sericos del paciente.

Background Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver malignancy originating from primary mesenchymal tissue. The clinical manifestations, laboratory tests, and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high. The overall prognosis is poor and survival rate is low. Aim To investigate the diagnosis, treatment, and prognosis of UESL. Methods We performed a retrospective, single-center cohort study in Shengjing Hospital of China Medical University, which is a central hospital in northeast China. From 2005 to 2017, we recruited 14 patients with pathologically confirmed UESL. We analyzed the clinical manifestations, laboratory tests, imaging examinations, pathological examinations, therapy, and prognosis of these patients. Results There were nine males and five females aged 2-60 years old included in the study. The major initial symptoms were abdominal pain (71.43%) and fever (57.14%). Preoperative laboratory tests revealed that seven patients had increased leukocyte levels, four showed a decrease in hemoglobin levels, seven patients had increased glutamyl transpeptidase levels, nine had increased lactate dehydrogenase levels, and three showed an increase in carbohydrate antigen 199. There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children (6/6 vs 5/8, P = 0.091). The survival rate after complete resection was 6/10, while that after incomplete resection was 0/4 (P = 0.040), suggesting that complete resection is important to improve survival rate. In total, five out of the eight children achieved survival. During the follow-up, the maximum survival time was shown to be 11 years and minimum survival time was 6 mo. Six adult patients relapsed late after surgery and all of them died. Conclusion Preoperative imaging examination for UESL has a high misdiagnosis rate. Multidisciplinary collaboration can improve the diagnostic accuracy of UESL. Complete surgical resection is the first choice for treatment of UESL.

Abdominal ultrasound in a 50-year-old Japanese man revealed a cystic lesion on the caudate lobe of the liver. Four-month follow-up imaging showed a rapid increase in the size of the cystic lesion. The patient underwent laparoscopic partial hepatectomy because of a suspicion and perceived risk that the lesion might be malignant. The initial histological diagnosis was a hepatic cyst. Eleven months later, computed tomography showed a giant cystic lesion in the abdominal cavity and multiple liver metastases. The patient underwent excision of the giant cystic lesion and a partial hepatectomy. Immunohistochemistry for the recurring lesion revealed undifferentiated embryonal sarcoma of the liver.

Undifferentiated embryonal sarcoma of the liver is a rare entity. It is a malignant primitive mesenchymal tumor seen in the pediatric age group often between 6 to 10 years of age. It involves the right lobe of the liver commonly and is usually asymptomatic. Acute presentation in these cases is secondary to its rupture/ wall dehiscence. Alfa fetoprotein, a tumor marker elevated in most of the hepatic malignant tumors is however normal in undifferentiated embryonal sarcoma. Imaging wise it is a large encapsulated multiseptated lesion. It shows a “paradoxical appearance” with a predominantly solid appearance on ultrasonography and cystic appearance on CT/MRI. This is a peculiar feature that can help in the early diagnosis of this entity. Besides, normal serum alfa fetoprotein levels favor its diagnosis. Hereby we present a case of a 5-year-old female child, presented with complaints of acute onset abdominal pain and distension which on imaging investigation showed a liver mass with typical paradoxical appearance on ultrasonography, CT and MRI as described.

Background Treatment of undifferentiated embryonal sarcoma of the liver (UESL) is a great clinical challenge due to its rarity. This study aims to examine the long-term survival of UESL patients after treatment using different therapeutic modalities. Methods A pooled analysis of individual data was performed on all UESL patients obtained from literature search (n = 307) and our institution (n = 1). Results The 5-year overall survival rate of the 308 patients was 65.8%, 70% for partial hepatectomy group (n = 271), 78.9% for liver transplantation group (n = 14) and 6.6% for nonsurgical treatment group (n = 23). For patients receiving partial hepatectomy, paediatric patients, radical resection and combined chemotherapy were independent predictors for improved survival. Conclusion Radical hepatectomy combined chemotherapy should be considered as the preferred treatment option for USEL. Liver transplantation appears to be a reasonable alternative for unresectable disease.

Highlights • First Case Report about ALPPS for the treatment of an undifferentiated embryonal sarcoma of the Liver. • The feasibility of the ALPPS procedure in a newly faced tumor-entity. • Importance of an intraoperative histological evaluation of the parenchyma to estimate the individual risk for liver failure., Introduction Embryonal sarcomas of the liver (ESL) are extremely rare solid tumors appearing mainly in children. The therapeutic standard for an ESL is a margin free resection combined with chemotherapy. The Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) procedure as a surgical therapy offers a curative approach for liver tumors of various origins where the future liver remnant (FLR) would be insufficient after a one-staged (extended) hemihepatectomy. Presentation of Case A 19-year-old patient was diagnosed with an undifferentiated embryonal sarcoma of the liver (UESL) in the right liver lobe with oligometastatic spread to the lungs. After neoadjuvant chemotherapy remission was enough to plan a resection of the liver tumor. During the operation we changed our strategy from one-stage hepatectomy to ALPPS because of borderline FLR and macroscopic and histologic liver damage to avoid posthepatectomy liver failure. The interstage and postoperative course of the patient was uneventful beside postoperative bile leakage, which was treated by interventional drainage and stenting. Discussion The ALPPS-procedure as a comparatively new surgery was considered over a portal vein ligation or embolization. ALPPS shows a faster hypertrophy compared to standard one-staged hemihepatectomy with decreased or similar proliferation, apoptosis or angiogenesis (at least for CRLM) Conclusion In experienced centers the ALPPS-procedure is evolving as the safer approach in hemihepatectomys where the FLR is critical. Additionally, ALPPS can serve as an intraoperative option when liver volume and quality seem not to be sufficient and is to be considered when facing new tumor-entities.

Undifferentiated embryonal sarcoma (UES) is an uncommon paediatric hepatic tumour that clinically simulates a liver abscess when present with fever. This report describes a case of UES in a 12-year-old boy, who presented with abdominal pain, swelling and fever, all simulating a liver abscess. The possibility of UES was considered at imaging, based on the solid appearance on ultrasound and cystic appearance with serpiginous peripheral vessels on computed tomography/magnetic resonance imaging. The diagnosis was confirmed at histopathology.

Background Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. Methods In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected. Results A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH. Conclusions In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive neoplasm that occurs predominantly in children. Like mesenchymal hamartoma of the liver (MHL), UESL harbors recurrent rearrangements involving 19q13.3 and 19q13.4, a region of the genome that contains a primate-specific cluster of micro-RNAs. Here, we present a case of a high-grade neoplasm that arose in the left hepatic lobe of a 5-year-old male and gave rise to widespread lymph node, visceral, and soft tissue metastases. The tumor was composed of sheets, tubules, and papillae of epithelioid cells with rhabdoid morphology. INI1 and BRG1 expression were retained. Tumor cells diffusely expressed epithelial markers, including multiple keratins. While the morphologic and immunophenotypic features were suggestive of poorly differentiated carcinoma with rhabdoid features, the tumor was found to harbor the t(11;19)(q13;q13.3) translocation characteristic of UESL, as well as a TP53 mutation. Given the clinical presentation, imaging, clinical course, the tumor was classified as UESL with unusual, carcinoma-like histopathologic features. In the context of an unclassified high-grade hepatic tumor in a young child, molecular or cytogenetic testing for chromosome 19q13 alterations should be considered.

Malignant hepatic tumors in children are rare, comprising 1.3% of all pediatric malignancies. Following hepatoblastoma, hepatocellular carcinoma is the second most common. Other malignant hepatic tumors seen in childhood include those of mesenchymal origin including undifferentiated embryonal sarcoma, angiosarcoma, rhabdomyosarcoma and epithelioid hemangioendothelioma, as well as biliary tumors such as cholangiocarcinoma. Diagnosis can be challenging because of their rarity, and the recognition of distinctive imaging features for certain tumors such as epithelioid hemangioendothelioma and biliary rhabdomyosarcoma can focus the differential diagnosis and expedite the diagnostic process. A complete MRI examination with hepatocyte-specific contrast media and diffusion-weighted imaging helps to focus the differential diagnosis, and, although findings are often nonspecific, in some cases typical features on MRI can be helpful in diagnosis. Histopathological analysis is usually required for definitive diagnosis. Hepatic tumors tend to be aggressive, and full staging is imperative to establish disease extent. Significant proportions are not amenable to upfront surgical resection and often require a multimodality approach including neoadjuvant chemotherapy within a multidisciplinary setting. Facilitating complete surgical resection is usually required for better survival. In this review, we emphasize pathology and imaging features for rare liver tumors that are useful in reaching a prompt diagnosis. We also discuss general clinical findings, prognosis and management of these tumors.

Undifferentiated embryonal sarcoma of the liver (UESL) is a primitive hepatic neoplasm that presents in a variety of forms on ultrasonography, computed tomography, and magnetic resonance imaging. In this case report, we present an UESL with fluid-fluid cysts mimicking a radiographic presentation commonly seen in venolymphatic malformation on magnetic resonance imaging. This is the first described case of UESL, with this radiographic presentation and outlines, the importance of considering this malignant lesion when evaluating liver tumors in children. Keywords: Undifferentiated embryonal sarcoma of the liver, Primary liver tumors, Magnetic resonance imaging, Venolymphatic malformation

This study evaluates the clinicopathological characteristics and outcomes of children vs. adults with undifferentiated embryonal sarcoma of the liver (UESL). A retrospective analysis of 82 children (&lt, 18 years) and 41 adults (≥18 years) with UESL registered in the National Cancer Database between 2004–2015 was conducted. No between-group differences were observed regarding tumor size, metastasis, surgical treatment, margin status, and radiation. Children received chemotherapy more often than adults (92.7% vs. 65.9%, p &lt, 0.001). Children demonstrated superior overall survival vs. adults (log-rank, p &lt, 0.001) with 5-year rates of 84.4% vs. 48.2%, respectively. In multivariable Cox regression for all patients, adults demonstrated an increased risk of mortality compared to children (p &lt, 0.001), while metastasis was associated with an increased (p = 0.02) and surgical treatment with a decreased (p = 0.001) risk of mortality. In multivariable Cox regression for surgically-treated patients, adulthood (p = 0.004) and margin-positive resection (p = 0.03) were independently associated with an increased risk of mortality. Multimodal treatment including complete surgical resection and chemotherapy results in long-term survival in most children with UESL. However, adults with UESL have poorer long-term survival that may reflect differences in disease biology and an opportunity to further refine currently available treatment schemas.

Liver tumors are a heterogeneous and complex mix of benign and malignant neoplasms that may arise in the setting of chronic liver injury or due to no prior insult. In children, hepatoblastoma is the most common malignant primary liver tumor and hepatocellular carcinoma is rare. In adults, however, hepatocellular carcinoma is most common and undifferentiated embryonal sarcoma is vanishingly rare. Liver Cancer explores these, and the myriad of other entities in between, with a depth and precision that is highly informative and practical to the modern physician scientist. Descriptions of grossing techniques, histopathologic features, ancillary testing modalities, molecular/genetic abnormalities, imaging characteristics, treatment options, clinical signs/symptoms and surgical approaches are contemporary contributions to this exciting field. Liver Cancer represents the latest knowledge of primary liver tumors. Refreshingly, it focuses on tumors and underlying processes that affect both children and adults. Written by and for practicing Pathologists, Oncologists, Surgeons, Gastroenterologists and Radiologists, its comprehensive content makes it a valuable reference for primary liver tumors.

A minimally invasive monitoring technique, intraoperative transesophageal echocardiography (TEE), has been utilized to provide real-time data on volume status and ventricular function in patients undergoing liver transplantation. In this case, TEE was utilized in an 8-year-old female undergoing undifferentiated embryonal sarcoma of the liver resection to monitor for pulmonary emboli, particularly a saddle embolus. In addition to visualization of cardiac structures, TEE can also be utilized to monitor the liver, lungs, spleen, and kidneys. Monitoring for echocardiographic findings of pulmonary embolism in this high-risk patient was an integral part of effective intraoperative management.

Hepatic embryonal sarcoma is a malignancy classified among soft tissue tumors of uncertain differentiation. It is also known as liver embryonal sarcoma, embryonal sarcoma of the liver, undifferentiated embryonal sarcoma, malignant mesenchymoma of the liver, and mesenchymal sarcoma of the liver.

Adult undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignant tumor. As UESL is rare, the literature predominantly includes case reports, with a limited number of small case series. The aim of the present study was to investigate the presentation, treatment modalities and outcomes of this rare tumor. The present study includes a case series of adult UESL and a systematic review. A single-institution case series of adult UESL were retrospectively analyzed, and a systematic review of adult UESL was performed by searching MEDLINE, Web of Science, EMBASE, the Google Scholar database and the Cochrane Library. For all identified adult UESL cases, the demographic variables, treatments and survival were analyzed. Three female adult patients with UESL (median age, 21 years) were successfully treated by complete tumor resection, with or without adjuvant chemotherapy, at Beijing Tsinghua Changgung Hospital between 2015 and 2018. Of these patients, two are currently alive (follow-up, 9 and 41 months), and one died after pulmonary recurrence 17 months post-diagnosis. The present systematic review identified 108 cases of adult UESL. Among all 111 analyzed cases, the median overall and disease-free survival rates were as follows: 1-year, 72 and 67%; 3-year, 56 and 40%; and 5-year, 47 and 35%, respectively. Treatment strategies combining complete tumor resection and chemotherapy promoted improved overall and disease free survival time compared with radical tumor resection alone. The present analysis included one of the largest case series of UESL in adults, and is the first such study to present survival rates. The results of the present study confirmed that survival was improved by treatment strategies combining complete tumor resection and chemotherapy.

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

Aim To analyse incidence, treatment and outcomes of paediatric liver malignancies in Finland during 1987-2017. Methods Medical records and national cancer registry data of 47 children with liver malignancies were reviewed. Survival was calculated with the Kaplan-Meier method. Results During follow-up, liver malignancy incidence remained stable at 1.1:10(6). Altogether, 42 patients with hepatoblastoma (n = 24), hepatocellular carcinoma (n = 11) and undifferentiated embryonal sarcoma (n = 7) underwent surgery at median age 4.6 (interquartile range, 2.0-9.6) years and were followed up for 13 (7.0-19) years. Cumulative 5-year survival was 86% for hepatoblastoma, 41% for hepatocellular carcinoma and 67% for undifferentiated embryonal sarcoma. Five-year survival was decreased among hepatoblastoma patients aged >= 2.4 years (73% versus 100%, P = .040), with PRETreatment EXTent of disease IV (PRETEXT, 60% vs 100%, P = .004), and with recurrent disease (67% vs 88%, P = .029). Recurrent/residual disease associated with decreased 5-year survival in hepatocellular carcinoma (0% vs 83%, P = .028). Survival was similar among 19 transplanted and 23 resected patients. In total, 14 deaths occurred either for the underlying malignancy (n = 8), adverse effects of chemotherapy (n = 5) or unrelated reasons (n = 1). Conclusion Outcomes for PRETEXT I-III hepatoblastoma and un-metastasized hepatocellular carcinoma were encouraging. Adverse effects of chemotherapy significantly contributed to mortality.

Medical laboratory tests are becoming more reliable with increased specificity and sensitivity, leading to their use as definitive diagnostic tests for many medical conditions. Enzyme-linked immunosorbent assay (ELISA) tests are convenient, sensitive, and standardly used for rapid detection and quantification of antigens or patient antibodies against specific antigens. However, based upon the specificity and sensitivity of an ELISA test, the results may not be definitive for a specific disease but merely suggestive, due to potential cross-reactivity of antigens and antibodies. Here, we present a case of a 15-year-old male who presented with fever, nausea, and right upper quadrant pain. Computed tomography scan showed an 18-cm liver mass with cystic features. Biopsy results confirmed a diagnosis of undifferentiated embryonal sarcoma of the liver; however, the clinical picture was complicated by positive ELISA results for Echinococcus, Entamoeba histolytica, and histoplasmosis. Due to the absence of travel and positive ELISA result for three different infectious agents, we hypothesize that tumor molecular mimicry might have led to false-positive ELISA results in the absence of infection in this case, demonstrating a limitation of ELISA serology. Critical appraisal of all possible evidence to ensure alignment when assigning the final diagnosis is essential for optimal patient outcomes.

Primary sarcomas of the liver are unusual neoplasms developing in adults. They constitute a heterogeneous group of neoplasms including undifferentiated embryonal sarcoma. Patients usually present with an abdominal mass and abdominal pain. Case 1: A 53-year-old woman presented with abdominal pain. Computed tomography showed an occupying mass in the right lobule and an intra-auricular multi-lobulated mass suggestive of a secondary deposit. Biopsy of the hepatic lesion revealed undifferentiated embryonal sarcoma. Despite radiotherapy and supportive measures, her overall status progressively worsened until cardiac arrest. Case 2: A 41-year-old woman presented with hepatomegaly. Abdominal imaging showed cystic lesions in the right hepatic lobule with multiple septae. The patient underwent extended right hepatectomy and a histopathological study reported high-grade undifferentiated embryonal sarcoma. Two years after surgery, a new cystic lesion in the surgical site was recorded and chemotherapy was scheduled. The lesion remained stable for three years when disease progression was observed and second-line chemotherapy was initiated. Although undifferentiated embryonal sarcoma of the liver has poor prognosis, early diagnosis is essential to increase the chances of survival. Currently, surgical resection and chemotherapy are the primary treatment modalities.

Many parts of India are endemic for hydatid cyst, and hence, the most common diagnosis of a hepatic cyst in such regions is hydatid cyst. Undifferentiated embryonal sarcoma of the liver (UESL) is a rare differential diagnosis for hepatic hydatid cyst as the clinical and radiological features of hydatid cysts and UESL overlap. Here, we report a 4-year-old boy with hepatic cyst, who was initially diagnosed as hepatic hydatid cyst, which was later confirmed as UESL. The child was successfully treated with chemotherapy. This case report highlights the need to spread awareness about UESL, as prompt diagnosis and treatment improves prognosis of the same.

Undifferentiated embryonal sarcoma (UES) is an uncommon primary hepatic tumour of childhood. The mass usually shows paradoxical features of being cystic on CT and solid on ultrasound. These lesions are usually hypovascular. Very rarely they may present as hypervascular liver masses with macroaneurysms and arteriovenous (AV) shunt, with only less than six cases reported in literature. We report a case of an 11-year-old child who presented with progressive abdominal distention, and CT revealed a large exophytic hypervascular mass of liver with multiple macroaneurysms, pooling of contrast and a high-flow AV shunt. Histopathology, along with immunohistochemistry, revealed the mass to be UES. The child underwent neoadjuvant chemotherapy followed by successful surgery. The prognosis of this tumour depends on prompt diagnosis and early intervention. We present this case to highlight the atypical presentation of UES, which will encourage radiologists to keep this differential in relevant clinical settings.

Introduction: Undifferentiated embryonal sarcoma of liver (USEL) is a rare disease that 90% occurs in children between age group 6-10 years with no gender discrepancy. Diagnosis of USEL relies on postoperative pathology and immunohistochemistry. Objective : This is a rare case report of an 8 year female patient who underwent excision of liver mass with left side lobectomy for a liver mass which turned out to be Undifferentiated Embryonal Sarcoma of Liver(USEL) on Histopathological analysis and Immunohistochemical examination. Results : Patient was diagnosed to be having Gastrointestinal Stromal Tumor of stomach on radiological investigations but intraoperatively turned out to be a liver mass for which patient underwent excision of liver mass with left side lobectomy for a liver mass which turned out to be Undifferentiated Embryonal Sarcoma of Liver(USEL) on Histopathological analysis and Immunohistochemical examination. Patient underwent chemotherapy including ifosfamide, mesna and doxorubicin. Currently, the patient’s physical status is normal with no distant metastases shown in PET-CT. Conclusion : Undifferentiated Embryonal Sarcoma of Liver is a rare entity but with adequate surgical procedure and followup chemotherapy or radiotherapy has excellent prognosis

Intrahepatic chlangiocarcinomas (CCs), the second most common primary malignant liver tumours, usually occur in non-cirrhotic liver, and can be classified into three types based on gross morphology: mass-forming; periductal infiltrating; and intraductal growing. Among them, mass-forming intrahepatic CCs are the most common type and characterized by homogeneous mass with an irregular but well-defined margin with peripheral enhancement on late arterial phase and delayed enhancement in central portion of tumours corresponding to the fibrous stroma. Several imaging features such as enhancement pattern and degree of diffusion restriction have been suggested as prognostic markers for mass-forming CCs. Hepatocellular carcinomas (HCCs) are the most common primary malignant liver tumors, and usually arise from the cirrhotic liver. However, approximately 20% of HCCs involve the non-cirrhotic liver (hereafter, non-cirrhotic HCC), and non-cirrhotic HCCs are often detected at an advanced stage due to the lack of surveillance for patients with non-cirrhotic liver. Other primary malignant liver tumours other than CCs and HCCs including angiosarcoma, undifferentiated embryonal sarcoma are quite rare, and imaging diagnosis is often difficult. This review offers a brief overview of epidemiology, risk factors and imaging features of primary malignant tumours in non-cirrhotic liver. Understanding of radiologic appearance and predisposing clinical features as well as differentials of primary malignant tumour in non-cirrhotic liver can be helpful for radiologists to adequately assess these tumours, and subsequently to make optimal management plan.

Background . Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mes-enchymal tumor that occurs predominantly in children. Diagnosis delay often happens due to the deficiency of clinical symptoms, radiological findings, and serological markers. Objektive was to describe the imaging features of the undifferentiated embryonal sarcoma of the liver in children. Materials . Six children with UESL (5 girls and 1 boy) were observed and treated at the Research Center of Pediatric Oncology between 2008 and 2016. The median age was 6.5 years (from 4 months to 11 years). The diagnosis was confirmed by histological and immunohistochemical analysis. Abdominal ultrasonography (US), abdominal computed tomography (CT), and magnetic resonance imaging (MRI) were performed. Results . The enormous solitary well circumscribed mass with irregular contours without invasion of liver vessels were revealed by US, CT, and MRI. In 5 cases, the tumors didn’t disturbed the fibrocapsule. Five tumors showed solid appearance with some little anechoic areas in abdominal US, while at CT and MRI UESL had predominantly cyst-like structure due to the high water content of the prominent myxoid stroma. After intravenous contrast administration predominantly peripheral enchancement and solid parts of the mass was determined at CT and MRI in all cases. Conclusion . Imaging data reveal discrepancy between US and CT/MRI appearance of UESL that can help to suspect this tumor. It is necessary to perform the morphological investigation when UESL is suspected.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive malignancy. Though the molecular underpinnings of this cancer have been largely unexplored, recurrent chromosomal breakpoints affecting a noncoding region on chr19q13, which includes the chromosome 19 microRNA cluster (C19MC), have been reported in several cases. We performed comprehensive molecular profiling on samples from 14 patients diagnosed with UESL. Congruent with prior reports, we identified structural variants in chr19q13 in 10 of 13 evaluable tumors. From whole transcriptome sequencing, we observed striking expressional activity of the entire C19MC region. Concordantly, in 7 of 7 samples undergoing miRNAseq, we observed hyperexpression of the miRNAs within this cluster to levels >100 fold compared to matched normal tissue or a non-C19MC amplified cancer cell line. Concurrent TP53 mutation or copy number loss was identified in all evaluable tumors with evidence of C19MC overexpression. We find that C19MC miRNAs exhibit significant negative correlation to TP53 regulatory miRNAs and K-Ras regulatory miRNAs. Using RNA-seq we identified that pathways relevant to cellular differentiation as well as mRNA translation machinery are transcriptionally enriched in UESL. In summary, utilizing a combination of next-generation sequencing and high-density arrays we identify the combination of C19MC hyperexpression via chromosomal structural event with TP53 mutation or loss as highly recurrent genomic features of UESL., Author summary We perform the most comprehensive molecular analysis to date of undifferentiated embryonal sarcoma of the liver, utilizing whole exome sequencing, RNA sequencing, miRNA sequencing, and SNP arrays. We find that UESL tumors harbor aberrant transcriptional start sites within C19MC region that is driven by structural rearrangement, leading to extreme overexpression of C19MC miRNAs. We further find that C19MC miRNAs negatively correlate with TP53 and K-Ras regulatory miRNAs. UESL also harbors highly recurrent TP53 mutation or copy number loss. Therefore, we provide for the first time a genomic landscape of UESL and describe cellular consequences of the discovered foundational genomic changes.

Undifferentiated embryonal sarcoma of the liver (UESL) is a highly malignant neoplasm occurring predominantly in children. Complete resection is the only curative treatment. We encountered an 11-year-old boy with UESL arising in the right lobe of the liver extending to the pulmonary valve. He was treated with IREC (irinotecan, etoposide, and carboplatin) neoadjuvant chemotherapy, concurrent extended radical right hepatectomy, and intracardiac tumorectomy under cardiopulmonary bypass, and adjuvant IREC chemotherapy. He remains in remission without complications 48 months after diagnosis. Multidisciplinary treatment, particularly the combination of radical surgery and effective neoadjuvant chemotherapy, may contribute to UESL patients' survival.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare, malignant, mesenchymal tumor that is commonly observed in children. The symptoms and imaging characteristics of UESL are not specific and can be mimicked by other tumors or liver abscesses. The tumor often appears as a large, heterogeneous, echoic solid mass with small cystic areas, presenting a cyst-like appearance on computed tomography (CT) and magnetic resonance imaging (MRI). The histopathological manifestations of UESL consist of stellate-shaped and spindle cells scattered on a myxoid background, with high mitotic counts. Cells with multiple or bizarre nuclei can also be observed. Here, we illustrate a 9-year-old male diagnosed with UESL by describing the imaging and histopathological characteristics that resulted in the diagnosis.

Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6–10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described. To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses. We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997–2015 and analysed the clinical, biological and imaging data. We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children. Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.

Undifferentiated embryonal sarcoma of the liver (UESL) is rare primary hepatic sarcoma and is known to occur in pediatric patients. This case is the UESL occurred in a 51-year old male patient. Multilocular cystic lesion was composed of primitive spindle cells without specific differentiation. This rare case would help to review differential diagnosis of primary sarcoma in liver and cystic neoplasm of the liver.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive pediatric malignancy. The purpose of this study was to review the clinical, radiologic, and pathologic features and outcome of children with UESL at our institution, in the United Network of Organ Sharing database and to review the existing literature to define the state of the art for children with UESL. Six children were diagnosed with UESL at the Texas Children's Cancer Center between 1993 and 2014, 12 children underwent liver transplantation registered in the United Network of Organ Sharing database, and 198 children with UESL were described in 23 case series during 1978 to 2014. Patients were treated with multimodal treatment approaches including primary surgical resection, neoadjuvant and/or adjuvant chemotherapy, and liver transplantation resulting in overall survival reported between 20% and 100% with significant improvement over the recent years. We show that complete tumor removal remains the key element of treatment and our single-institutional experience and data in the published literature suggest that combination chemotherapy with ifosfamide and doxorubicin to facilitate complete surgical resection is an effective approach to cure children with UESL.

The embryonal sarcoma of the liver is an extremely malignant, aggressive and infrequent condition of the liver, predominantly affecting children in the age groups of four to ten. The disease manifests with fever, abdominal pain or mass, nausea and laboratory findings suggestive of liver cell injury. The diagnosis can be made using tumor markers, imaging, immunohistochemistry and histopathology. The multimodal management protocol focuses the use of surgical resection, chemotherapy and radiation to significantly improved survival rates and quality of life in these patients.