Undifferentiated Embryonal Sarcoma Of Liver Research Articles

Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Entity

Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a rare hepatic malignant mesenchymal neoplasm in the paediatric population, typically affecting children between 6-10 years of age without sex predilection. It accounts for 9-15% of paediatric liver malignancies, with an incidence of 0.6-1.2 cases per one million patients. Here, the authors present a case of an 11-year-old male, who presented with right upper quadrant pain, loss of appetite for the past one month, and high-grade fever for the past three days. On examination, a firm mass in the right hypochondrium and epigastrium, about 4 cm below the right costal margin, was noted, moving with respiration. Blood work showed normal values and normal alpha-fetoprotein levels. An ultrasonogram revealed a cystic lesion, while a contrast-enhanced Computed Tomography (CT) scan showed a large hepatic space-occupying lesion with cystic and solid architecture, with the following differential diagnosis as, embryonal sarcoma and hepatoblastoma. The patient underwent surgery for a hepatic hydatid cyst, and the procedure performed was percutaneous aspiration, irrigation and respiration. Gross examination revealed multiple grey-brown and grey-white fragments with a variegated appearance. Microscopic analysis showed a malignant neoplasm composed of spindle cells, stellate cells, multinucleated giant cells in a myxoid stroma, with many atypical mitoses present. Periodic Acid-Schiff (PAS) positive eosinophilic hyaline globules were observed in the tumour cell cytoplasm, along with extensive areas of haemorrhage and necrosis. The final diagnosis of embryonal sarcoma was made after a panel of Immunohistochemistry (IHC) markers. The present case is presented here due to its rarity and diagnostic challenge, arising from the lack of a characteristic clinical presentation, serological markers and inconclusive radiological findings.

Undifferentiated embryonal sarcoma of liver arising within mesenchymal hamartoma in a child "case report"

BackgroundPrimary pediatric liver tumors are scarce. Undifferentiated embryonal sarcoma of the liver (UESL) embraces nine to fifteen percent of the pediatric hepatic neoplasms. Mesenchymal hamartoma of the liver (MHL) is the second most prevalent benign pediatric hepatic neoplasm following infantile hemangioendothelioma.Case presentationA 6-year-old female child presented with vague diffuse abdominal pain and palpable right lumbar mass by clinical examination. The US showed a well-circumscribed large right hepatic complex multilocular cystic lesion with multiple internal septations and some turbid loculi. The constellation of CT and dynamic MRI findings raised the presence of atypical suspicious complex cystic hepatic mass lesion with hemorrhagic, calcific entities and internal soft tissue enhancement. The histopathological correlation proved UESL on the background of MHL.ConclusionsA case of a pediatric hepatic complex cystic lesion, which had typical ultrasound features of MHL, however, CT and MRI had atypical suspicious criteria. Finally, our case is presented as UESL in the background of MHL based upon pathological diagnosis. The radiologists must be familiar with overlapped entities between MHL, UESL, and other differential diagnosis entities regarding pediatric hepatic neoplasms.

Undifferentiated embryonal sarcoma of liver

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 liver in a 9-year-old: case report

<p>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.</p>

CT and MRI features of undifferentiated embryonal sarcoma of liver in adults

Objective To analyze the CT and MRI manifestations of undifferentiated hepatic embryonal sarcoma in adults with an aim to improve the preoperative diagnosis of this disease. Methods A retrospective analysis was carried on the clinical data of 11 patients with undifferentiated hepatic embryonal sarcoma treated in Wenzhou People’s Hospital of Zhejiang and Wenzhou Central Hospital of Zhejiang between January 2008 and December 2018. There were 5 males and 6 females. The age ranged from 46 to 76 years (median 64 years). The site, size, morphology, margin, and pattern and degree of of the tumors were analyzed. Results Among the 11 patients, 10 patients had a single tumor, only 1 patient had multiple tumors. A total of seven patients' tumors were located in the right liver, 2 in the left liver, and 2 involved both the left and right livers. The maximum diameter of the tumors ranged from 5.5 cm to 21.2 cm, the maximum diameter (10.2±4.8 cm). The edge of tumors was clear in 9 patients and unclear in 2 patients. The tumors were elliptical in 4 patients or irregular in 7 patients. Plain CT scan showed 4 patients' tumors had mainly a cystic component, while 5 patients had both cystic and solid components. Plain MRI scan showed that five tumors were cystic and solid, with mixed internal signals. T1 weighted imaging (T1WI) showed slightly lower and lower signals; T2 weighted imaging (T2WI) showed slightly higher and higher signals. There was a low signal stripe separating inside; Diffusion-weighted imaging showed a high signal and apparent diffusion coefficient showed a low signal. The was characterized by in and fast out (marked in the arterial phase and fast clearance in the venous phase), and enhancement (mild to moderate in the arterial phase and even more obvious in the venous or delayed phase). Conclusion Adult undifferentiated embryonic sarcoma has certain CT and MRI features. Its shape is elliptical or irregular, and mostly with a clear boundary. There were intratumoral cystic or cystic solid components, with mixed density/signal on CT and MRI, being characterized by in and fast out and enhancement on intravenous contrast enhanced scans. Key words: Liver neoplasms; Tomography, X-ray computed; Magnetic resonance imaging; Undifferentiated embryonal sarcoma

Undifferentiated embryonal sarcoma of liver (UESL) - a case report

Undifferentiated embryonal sarcoma of liver (UESL) - a case report

Clinicopathologic observation of pediatric undifferentiated embryonal sarcoma of liver

Clinicopathologic observation of pediatric undifferentiated embryonal sarcoma of liver

Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Report

Undifferentiated embryonal sarcoma of liver (USEL) is a rare primary hepatic malignancy principally affecting patients of pediatric age group. We present a case undifferentiated embryonal sarcoma of liver in a 1-year old female. She presented with complaints of abdominal distension and vomiting for 2 weeks. Ultrasonography showed grossly enlarged liver with multiple heterogenous lesions. Computed tomography revealed presence of large exophytic lesion of size 11.3 × 7 cm involving left lobe of liver. Ultrasound guided biopsy was performed, and specimen was sent for histopathological examination. On Microscopic examination undifferentiated round to spindle cells in myxoid background were seen. Immunohistochemistry revealed positivity for vimentin and AE1 thus proving undifferentiated embryonal sarcoma of liver. Diagnosis is often challenging because of overlapping epidemiological, clinical, radiological findings with those of other liver tumors.

Undifferentiated Embryonal Sarcoma Of Liver- A Rare Entity At Tertiary Cancer Centre

Undifferentiated Embryonal Sarcoma Of Liver- A Rare Entity At Tertiary Cancer Centre

Undifferentiated embryonal liver sarcoma in childhood: A case report.

In order to improve the diagnosis and therapy of undifferentiated embryonal liver sarcoma (UELS), the present study presents the case of a 9-year-old female with UELS and discusses UELS in childhood. The patient presented with abdominal pain and fever. The laboratory tests, radiographic examination and pathological features presented by the female were similar to those of typical cases of UELS reported in childhood. The patient initially received surgical treatment and the immunohistochemical findings suggested that the patient had UELS. The patient’s parents refused adjuvant chemotherapy and demonstrated a right prerenal mass 6 months post-surgery. Microscopic examination revealed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. Previous studies have shown that patients who receive combined therapy with complete tumor resection and adjuvant chemotherapy have a longer survival time than those who undergo surgical therapy alone. Complete tumor resection combined with adjuvant chemotherapy may reduce the risk of recurrence and enhance the survival time in patients with UELS.

A Single-institution Retrospective Cases Series of Childhood Undifferentiated Embryonal Liver Sarcoma (UELS)

Undifferentiated embryonal liver sarcoma (UELS) makes up 9% to 15% of all malignant liver tumors in children. UELS is characteristically diagnosed between the ages of 6 and 10 years and presents with abdominal pain, vomiting, and an abdominal mass. There is currently no standardized treatment for UELS except attempt at complete surgical resection. There have been only about 150 cases of UELS reported in the literature all with historically poor overall survival of <37.5% at 5 years. This report is one of the largest single-institution reports of UELS consisting of 5 patients over 2 decades. The purpose of this study is to characterize presentation and to report treatment success in UELS in children, adolescents, and young adults and the use of liver transplantation and, lastly, to suggest a use of positron emission tomography/computed tomography (PET/CT) in monitoring of this disease process. We conducted an Institutional Review Board-approved retrospective chart review. Data were collected from UELS patients younger than 21 years seen at the University of California Los Angeles over the past 20 years (January 2001 to September 2011). Descriptive analysis was conducted including multiple parameters of patient demographics, tumor characteristics, treatment modalities, and morbidity and mortality. Five patients with UELS were identified. Patients initially presented with fever, abdominal pain, or nausea. Ages ranged from 10 to 19 years old (median age 13 y old), and there was a 4:1 male-to-female predominance. Tumor size ranged from 6 to 22 cm in largest diameter. One patient presented with metastatic disease to the lungs and heart and 1 patient recurred 2 years from diagnosis with bilateral paraspinal masses. Treatment included local control surgery with neoadjuvant and adjuvant chemotherapy with an anthracycline/alkylating agent combination. One patient with recurrent and refractory disease achieved local control with an orthotopic liver transplantation (OLT). Metastatic disease was controlled with surgery and radiation therapy. 18-Fluorodeoxyglucose PET/CT was a useful imaging tool for judging response to therapy with complete loss of metabolic activity in tumor after neoadjuvant chemotherapy in 2 representative cases. Although follow-up is short for some patients, overall survival in these 5 patients was 100% with follow-up ranging from 21 to 68 months. Disease-free survival ranged from 8 to 46 months with no patients with residual disease. UELS is an aggressive high-grade primary liver sarcoma with high metastatic potential. This report represents one of the largest single-institution studies of UELS. Using multimodality therapy, patients have achieved 100% overall survival even in the setting of extensive disease, metastases, and recurrence. In cases of unresectable primary tumor or recurrent and refractory disease isolated to the liver, OLT is a potential therapeutic option. We report success with adjuvant chemotherapy and complete surgical resection with OLT as an alternative in unresectable or refractory cases. We also suggest a possible utility of PET/CT in monitoring treatment response in this disease.

A long-term survival case of adult undifferentiated embryonal sarcoma of liver

Undifferentiated embryonal sarcoma of the liver (USEL) is a rare malignant hepatic tumor with a poor prognosis that is usually observed in children (aged 6 to10 years) and rarely seen in adults. We present a case of USEL in a 27-year-old woman with no previous history of the disease. Laboratory tests performed on admission showed that the patient had mildly elevated levels of aspartate aminotransferase, alanine transaminase, alkaline phosphatase, lactate dehydrogenase, and γ-glutamyl transpeptidase. The levels of viral hepatitis and tumor serum markers were all within normal limits. Computed tomography showed a large mass involving the right lobe and the medial segment of the liver. Right trisectionectomy was performed. Microscopically, the tumor was composed of pleomorphic and polynuclear dyskaryotic cells in a myxoid stroma with focal eosinophilic globules and no clear differentiation to muscle. Histological diagnosis showed undifferentiated embryonal sarcoma. Adjuvant therapy with cisplatin, vincristine, doxorubicin, cyclophosphamide, and actinomycin D was initiated. We administered a high dose of etoposide to extract the patient’s peripheral blood stem cells and performed radiation therapy and peripheral blood stem cell transplantation. At 5-year follow-up, the patient was alive without any evidence of recurrence. Here, we describe the clinical and histopathological features of USEL as well as the therapeutic options for USEL in adults with this disease.

Undifferentiated (embryonal) liver sarcoma: synchronous and metachronous occurrence with neoplasms other than mesenchymal liver hamartoma

Undifferentiated (embryonal) liver sarcoma (UELS) is a rare tumor that typically occurs in children. The association of UELS with neoplasm other than mesenchymal liver hamartoma is exceedingly rare. The aim of the study was to report 3 cases of UELS, 2 of them being interesting because of their association with another neoplasm, vaginal embryonal rhabdomyosarcoma in a teenage girl and B-acute lymphoblastic leukemia in a middle-aged woman. Besides, one of our cases of UELS, in a 58-year-old woman, is an extremely rare presentation of such a tumor in a middle-aged adult. The patient's clinical features, therapy, and pathologic results were reviewed; immunohistochemical analysis and, in 2 cases, electron microscopy were performed. In this study, all 3 patients were females aged 13, 13, and 58 years. Histopathologic evaluation of resected liver tumors confirmed the diagnosis of UELS in all of them. In 2 of the cases, metachronous occurrence of UELS with vaginal embryonal rhabdomyosarcoma in a teenage girl and B-acute lymphoblastic leukemia in a middle-aged woman is described. Careful clinical analysis, histologic studies, and immunohistochemistry are mandatory to distinguish UELS from other hepatic malignancies with similar or overlapping features and to exclude the possibility of other tumors that may be considered in the differential diagnosis. The association of UELS with another neoplasm is exceedingly rare.

Undifferentiated liver embryonal sarcoma in adults: A report of four cases and literature review

To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone. UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.

CT and pathological findings in undifferentiated embryonal sarcoma of liver in adults

CT and pathological findings in undifferentiated embryonal sarcoma of liver in adults

Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis

Purpose The aim of the study was to report on 3 cases of childhood undifferentiated embryonal liver sarcoma (UELS) and to highlight the clinical features, laboratory findings, diagnosis, and management of this rare disease. Methods The patients' age, sex, clinical features, laboratory findings, pathologic results, and therapy were reviewed. Immunohistochemistry analysis was performed on the resected mass sections. Results In this study, 2 cases were female (aged 8 and 12 years) and 1 was male (aged 9 years). The causes of hospitalization were mainly abdominal pain, mass, or fever. An elevated erythrocyte sedimentation rate was noted in 2 available cases, and α fetoprotein (AFP) was within the normal range. Imaging findings indicated a well-defined heterogeneous large mass in the right lobe. Histopathologic evaluation of the mass confirmed the diagnosis of UELS. Immunohistochemical staining showed that vimentin and CD68 antigen were positive in all samples, whereas desmin was positive in one sample. Surgery with chemotherapy was performed in 2 cases. Conclusion The diagnosis of UELS depends mainly on the pathologic findings. Undifferentiated embryonal liver sarcoma should be included in the differential diagnosis of mass in the liver, especially with well-defined heterogeneous imaging findings and normal AFP. Diagnosis and management should be made early as UELS is a potentially treatable tumor.

Undifferentiated embryonal sarcoma of liver in an old female: Case report and review of the literature

The clinical characteristics of undifferentiated (embryonal) sarcoma of the liver (UESL) were investigated and the best treatment modalities were recommended. Both histology and immuno-histochemistry demonstrated the cellular features of this peculiar tumor. The tumor size was 12 cm multiply 9 cm multiply 8 cm in the right liver lobe. The patient underwent surgical resection of the tumor. The postoperative recovery was uneventful and she died eight months after diagnosis. The tumor showed mixed spindle and polygonal cells within the myxoid matrix. Some tumor cells contained eosinophilic hyaline globules that were positive for resistant diastase. Immunohistochemistry showed positive vimentin. Stellate and spindle cells were positively stained with alpha-1-antichymotrypsin (AACT) and CD68. This case indicates that UESL is not obviously differentiated in old-aged adults.

Fine needle aspiration biopsy in pediatric space-occupying lesions of liver: a retrospective study evaluating its role and diagnostic efficacy

Background Liver space-occupying lesions (SOLs) in the pediatric population form a unique and distinct spectrum as compared with those in the adult population. There are no large studies available in the English literature regarding fine needle aspiration cytology (FNAC) in pediatric liver SOLs. Aim This study was done to familiarize with the cytomorphological features of these distinctive benign and malignant lesions of liver and to determine the specificity, sensitivity, and diagnostic efficacy of FNAC. Methods A total of 41 FNACs from pediatric liver SOLs were reviewed, and the diagnoses were correlated with clinical and radiological findings, histopathological diagnoses, and/or follow-up information. These were categorized into malignant, benign, and indeterminate groups. Results There were 20 malignant lesions that included hepatoblastoma (7), hepatocellular carcinoma (4), undifferentiated embryonal sarcoma of liver (3), and metastases (6). The 12 benign lesions included abscess (8), 1 case each of mesenchymal hamartoma, focal nodular hyperplasia, hydatid cyst, and tuberculosis. In the overall FNAC performance calculation, the specificity of FNAC was 100%, sensitivity 95%, positive predictive value 100%, negative predictive value 92.3%, and diagnostic accuracy 96.9%. Conclusion The recognition of the distinct cytomorphological features of pediatric focal liver lesions, in conjunction with the clinical data and radiological findings, make FNAC a useful primary diagnostic modality in these unique and distinctive lesions.