Hepatic Undifferentiated Embryonal Sarcoma Research Articles

Treatment of Undifferentiated Embryonal Sarcoma of the Liver in Children.

Undifferentiated embryonal sarcoma of the liver is a rare mesenchymal tumor with a highly malignant potential. It occurs almost exclusively in the pediatric population and typically has a poor outcome. Although previous studies have reported dismal prognoses, recent advances in combined treatment modalities, e.g., surgery and chemotherapy, have given cause for optimism. Even in those diseases not amenable to complete surgical resection or refractory diseases, other treatment modalities, such as liver transplant, have yielded promising results. This paper provides a review of the current treatment modalities for hepatic undifferentiated embryonal sarcoma in children.

Hepatic undifferentiated embryonal sarcoma in a young adult

Hepatic undifferentiated embryonal sarcoma in a young adult

Liver transplantation for hepatobiliary malignancies: a new era of "Transplant Oncology" has begun.

The indications of liver transplantation for hepatobiliary malignancies have been carefully expanded in a stepwise fashion, despite the fundamental limitations in oncological, immunological, and technical aspects. A new era of "Transplant Oncology," the fusion of transplant surgery and surgical oncology, has begun, and we stand at the dawn of a paradigm shift in multidisciplinary cancer treatment. For hepatocellular carcinoma, new strategies have been undertaken to select recipients based on biological and dynamic markers instead of conventional morphological and static parameters, opening the doors for a more deliberate expansion of the Milan criteria and locoregional therapies before liver transplantation. Neoadjuvant chemoradiation therapy followed by liver transplantation for unresectable perihilar cholangiocarcinoma developed by the Mayo Clinic provided excellent outcomes in a US multicenter study; however, the surgical indications are not necessarily universal and await international validation. Similarly, an aggressive multidisciplinary approach has been applied for other tumors, including intrahepatic cholangiocarcinoma, hepatoblastoma, liver metastases from colorectal and neuroendocrine primary and gastrointestinal stromal tumors as well as rare tumors, such as hepatic undifferentiated embryonal sarcoma and infantile choriocarcinoma. In conclusion, liver transplantation is an important option for hepatobiliary malignancies; however, prospective studies are urgently needed to ensure the appropriate patient selection, organ allocation and living donation policies, and administration of antineoplastic immunosuppression.

Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma

The outcomes of hepatic undifferentiated embryonal sarcoma (HUES) have historically been limited by persistent, unresectable disease and the subsequent development of disease resistance and dissemination. We present our institutional experience with HUES and assess current treatment trends and outcomes in the era of liver transplantation. We conducted a retrospective chart review of cases presenting with HUES at our institution over the past 10 years. The collected data included age, sex, presenting symptoms, imaging and the associated Pretreatment Extent of Disease (PRETEXT) score, pathology, chemotherapy, surgical interventions, and outcomes. Approval was obtained from the institutional review board of the Cincinnati Children's Hospital Medical Center. HUES was identified in 6 patients (4 males and 2 females) with a median age at diagnosis of 11 years (range = 7-13 years). Initial imaging was available for all but 1 patient. The PRETEXT stage for these patients ranged from II to III. One patient was diagnosed with lung metastases. Two patients underwent upfront resection, and 1 patient received neoadjuvant therapy and then conventional resection. Three patients were treated with orthotopic liver transplantation (OLT) after neoadjuvant chemotherapy (primary OLT in 2 cases and salvage OLT for local recurrence in 1 case). Two patients received posttransplant adjuvant chemotherapy. All 6 patients remained in clinical remission with a mean follow-up of 35 months (range = 12-84 months). In conclusion, OLT has rarely been reported as a treatment option for HUES. The addition of liver transplantation as a surgical option for treating patients with HUES can result in improved survival for patients whose tumors are initially unresectable or recur.

Spiral CT findings of hepatic undifferentiated embryonal sarcoma

肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma,UES)少见,有关本病的影像学报道十分有限,且CT检查多为普通增强扫描[1～4],难以准确的反映病灶的强化特征.笔者总结6例UES病人的资料,结合文献分析、总结其螺旋CT表现特征,探讨其诊断和鉴别诊断价值。

Hyaline Globules In Ascitic Fluid In Primary Hepatic Undifferentiated Embryonal Sarcoma

Undifferentiated Embryonal Sarcoma (UES) of the liver is a rare predominantly pediatric malignancy. Only one brief report of cytologic findings in ascitic fluid of a recurrent tumor is available. The characteristic eosinophilic hyaline globules (HG) with immunohistochemistry on fluid cytology, has not previously been documented. A 20-year-old male presented with ascites. Cytology revealed a sparse number of pleomorphic malignant cells. Sections of the cell-block preparation revealed a loose myxomatous background and numerous atypical cells with marked pleomorphism, multinucleation and many intraand extracytoplsmic eosinophilic HG that were periodic acid-Schiff (PAS) positive and diastase-resistant. They displayed a positive reaction to alpha-1-antitrypsin on immunoperoxidase staining. A needle biopsy of a liver mass detected by CT scan confirmed the diagnosis of UES. Detection of sarcoma cells on fluid cytology is not often difficult. But an accurate diagnosis can be achieved only by detailed immunohistochemistry. Preparation of cell-blocks is of value in the work-up and management of patients presenting with malignant effusion.

Undifferentiated embryonal sarcoma of the liver treated with chemotherapy: CT imaging in four patients.

The purpose of this study was to describe the computed tomography (CT) findings of undifferentiated embryonal sarcoma after chemotherapy and to correlate the CT imaging findings with pathologic findings. Ten CT images obtained before and after chemotherapy in four patients with hepatic undifferentiated embryonal sarcoma were retrospectively reviewed and correlated with pathologic findings. After chemotherapy, tumor volume decreased by 50-90% and initially nonresectable tumor or gross residual tumor was successfully excised in three patients. In all patients, enhancing peripheral solid portions and septations changed to low-attenuation areas, and in three patients increased or de novo calcifications were found at the periphery of the tumor. Resected pathologic specimen after chemotherapy showed well-encapsulated masses with central necrosis, fibrosis, and dystrophic calcifications. These CT findings will be useful in monitoring the treatment response of hepatic undifferentiated embryonal sarcoma during chemotherapy.

Hepatic undifferentiated embryonal sarcoma in children

Hepatic undifferentiated embryonal sarcoma in children