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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.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare hepatic tumor in children. Its pathogenesis is largely unknown, but lines of evidence suggest common links to that of mesenchymal hamartoma of the liver (MH). Previously, we found a p53 mutation in a case of pediatric USL. Recently, there was another published report, demonstrating further evidence of p53 alterations in the adult cases. In this study, we analyzed in three cases of pediatric USL and two cases of MH by using PCR-SSCP and direct sequencing technique. The study identified missense mutations in all three cases of USL, but none of MH. The mutations were found specifically in tumor tissue and not detected in the surrounding normal hepatic tissue. Mutation points were localized in exon 7 (Gly245Ser), exon 6 (Arg196Pro), and exon 8 (Arg273Pro), respectively. Immunohistochemical study of p53 protein expression revealed strong immunoreactivity in cases of USL and negative staining in MH. In summary, this study provided a novel data suggesting that mutations of p53 in pediatric USL are not random genetic events and highly possible to be involved in its tumorigenesis.

Background. Primary hepatic sarcoma is a rare tumour with a poor prognosis. Methods. From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution. The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed. There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS). Results. The most common presenting symptoms were right upper quadrant pain and fever. Typical imaging findings were a heterogenous mass with poorly defined margins, pseudocapsule and aberrant vasculature. Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration ( n =5) or angiography ( n =2). Five right hepatectomies and three trisegmentectomies were performed. An R o resection was possible in three cases. Two patients developed complications and there was one death. Adjuvant chemoradiotherapy was administered to 5/7 patients. Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection. Conclusions. The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass. Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy. Patients with resectable tumours should be referred for surgery. Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.

Undifferentiated embryonal sarcoma (UES) of liver is a rare form of liver tumor in children. It may have an atypical presentation leading to delay in diagnosis. The authors report on 3 consecutive children with this condition, each of them presenting in older children who presented with this condition and an initial diagnostic dilemma. One of them presented initially with a solitary cyst, the second with severe abdominal pain, and the third with intermittent fever. Preoperative chemotherapy was successful in shrinking the tumors in 2 children to allow anatomic liver resection with tumor-free margin. Both remain free of recurrence 2 years after completing treatment.

Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare pediatric liver malignancy that is extremely uncommon in middle-aged individuals. We studied 2 cases of UESL in middle-aged adults (1 case in a 49-year-old woman and the other in a 62-year-old man) by histology, immunohistochemistry, and electron microscopy to clarify the cellular characteristics of this peculiar tumor. One tumor showed a mixture of spindle cells, polygonal cells, and multinucleated giant cells within a myxoid matrix and also revealed focal areas of a storiform pattern in a metastatic lesion. The other tumor was composed mainly of anaplastic large cells admixed with few fibrous or spindle-shaped components and many multinucleated giant cells. In both cases, some tumor cells contained eosinophilic hyaline globules that were diastase resistant and periodic acid-Schiff positive. Immunohistochemically, the tumor cells showed positive staining for smooth muscle markers, such as desmin, alpha-smooth muscle actin, and muscle-specific actin, and also for histiocytic markers, such as alpha-1-antitrypsin, alpha-1-antichymotrypsin, and CD68. Electron microscope examination revealed thin myofilaments with focal densities and intermediate filaments in the cytoplasm of tumor cells. Our studies suggest that UESL exhibits at least a partial smooth muscle phenotype in middle-aged adults, and this specific differentiation may be more common in this age group than in children. Tumor cells of UESL with smooth muscle differentiation in middle-aged adults show phenotypic diversity comparable to those of malignant fibrous histiocytoma with myofibroblastic differentiation.

Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor of mesenchymal origin recognized as a unique clinicopathologic entity since 1978. UESL has historically been considered an aggressive neoplasm with an unfavorable prognosis. Survival has improved using recent multimodal approaches, designed for patients with soft tissue sarcomas at other sites. Several small series have reported survival of up to 70% of children. We report a case of a 12-year-old boy from the United Arab Emirates who relapsed after complete surgical resection and was then successfully treated with re-resection followed by chemotherapy and radiotherapy. With a follow-up of 5 years, he is well and asymptomatic, and is leading a healthy life. This case emphasizes the fact that these poorly prognostic tumors may benefit from post-surgery chemotherapy. This case illustrates the improved survival of UESL patients following the multimodality therapy with a relatively long follow-up. This is the first case of UESL reported in this region of the world.

Gastroenteropancreatic neuroendocrine tumors account for 36 % of all neuroendocrine tumors in the pediatric age group. The most common site of occurrence is the appendix. Gastrointestinal stromal tumors are rare in children with the vast majority occurring in the stomach and in girls around the median age of 13 years. Pancreatic malignancy in childhood is distinctly rare, and this chapter discussion is restricted to solid pseudopapillary neoplasm, pancreatoblastoma, and pancreatic neuroendocrine tumors. The liver is the third most common site of origin for solid abdominal tumors in children, and this chapter discusses the more common malignant tumors hepatoblastoma, hepatocellular carcinoma, fibrolamellar hepatocellular carcinoma, and undifferentiated embryonal sarcoma.

With the exception of hemangioma, benign or malignant primary mesenchymal tumors of the liver are seldom encountered. The aim of this review was to discuss the clinical, histopathological, and imaging features of liver hemangiomas (cavernous, capillary, and sclerosed types), liver lipoma, angiomyolipoma, mesenchymal hamartoma, neurofibroma, infantile hemangioendothelioma, epithelioid hemangioendothelioma, myofibroblastoma, angiosarcoma, malignant fibrous histiocytoma, undifferentiated embryonal sarcoma, and nested stromal tumor. In most of these rare liver tumors, radiological findings obtained by cross-sectional imaging may reflect the characteristic pathologic features required for differential diagnosis; however, definitive diagnosis should be confirmed using histopathological examination.

Primary undifferentiated embryonal sarcoma (UES) of the liver is a highly malignant mesenchymal origin tumor and has a peak incidence between the ages of 6 and 10 years. We hereby report a case of primary UES of the liver in a 7-year-old male patient who initially was misdiagnosed and treated as hydatid cyst of the liver. The tumor was occupying almost the entire right lobe of the liver and had a mostly cystic appearance with some solid components in it. Because hydatid disease is endemic in this region, it can often lead to misdiagnosis. The correct diagnosis was established after a biopsy and following neo-adjuvant chemotherapy the patient underwent a successful right hepatic lobectomy with complete resection of the tumor. The patient also received adjuvant chemotherapy and is currently disease - free in the present six month period. Primary UES of the liver has a predominantly solid appearance on US in contrast to its mostly cystic appearance on CT and MRI. These paradoxical imaging findings should be kept in mind in order to be able to distinguish this rare tumor from other entities, especially hydatid cyst. Thus, early diagnosis and prompt surgical resection of these tumors together with adjuvant and/or neo-adjuvant chemotherapy can provide complete remission.

Within the medical community, there is a tendency to describe all cystic lesions in the liver and lungs as Hydatid disease (HD) in areas with HD endemics. This approach may sometimes cause a misdiagnosis. We have three cases with children aged between seven, seven and ten year old, all of whom had been diagnosed, via radiologic imaging, with HD cysts and started on treatment without confirmation. The true diagnoses of these cases were undifferentiated embryonal sarcoma (UES) in two and oesophageal duplication in one case, respectively. The indirect haemagglutination assays (IHA) were obtained in two of them. Although their results were negative, albendazole treatment was started in all cases. Confirmatory tests should be run in dubious cases. An IHA test can aid the diagnosis, although its effectiveness is limited. The possibility of false positive and negative results always exists, especially in lung cysts. A percutanous biopsy is strongly advised for differential diagnosis.

O sarcoma embrionário indiferenciado do fígado é uma entidade rara que acomete, principalmente, crianças acima dos cinco anos de idade. O aspecto macroscópico consiste, caracteristicamente, de grande massa hepática com maior componente sólido, porém apresenta algumas áreas císticas. Curiosamente, a tomografia computadorizada superestima o componente cístico da lesão, sendo a ultra-sonografia um método mais fidedigno na demonstração da consistência do tumor. Os estudos por imagem ajudam a afastar enfermidades não-neoplásicas, como abscessos e hematomas hepáticos, e avaliam a extensão das lesões. O diagnóstico de sarcoma embrionário indiferenciado pode ser corretamente presumido quando se consideram os achados de imagem em conjunto com a idade do paciente e o nível de alfa-fetoproteína. Os autores descrevem um caso de sarcoma embrionário indiferenciado no fígado de uma criança do sexo feminino de dez anos de idade, enfatizando seus aspectos imagenológicos e o diagnóstico diferencial.

Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule.

Undifferentiated embryonal sarcoma (UES) is the third most common hepatic malignancy in children. Previous reports have described a broad range of complex cytogenetic abnormalities in individual cases of hepatic UES. Herein we report the cytogenetic findings of six cases of hepatic UES at our institution analyzed by conventional cytogenetic methods and comparative genomic hybridization (CGH). The CGH demonstrated several chromosomal gains and deletions in each case, but there was no specific abnormality seen in every case. Patterns of chromosomal changes included gains of chromosome 1q (four cases), 5p (four cases), 6q (four cases), 8p (three cases), and 12q (three cases), and losses of chromosome 9p (two cases), 11p (two cases), and chromosome 14 (three cases). The three cases in which CGH showed gains in the 12q region were studied specifically for amplifications of MDM2 and CDK4, two genes that have been shown to be amplified in other soft tissue sarcomas. However, Southern analysis showed no amplification of MDM2 or CDK4 in these three cases. Further analysis will be needed to determine the critical events in the pathogenesis of these malignant pediatric liver tumors.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.

Undifferentiated embryonal sarcoma (UES) is a rare primary malignant tumor of the liver that typically presents in late childhood. We report a case of primary UES, which had a typical paradoxical appearance on different imaging modalities.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.

Undifferentiated embryonal sarcoma of the liver is a rare hepatic malignancy of childhood with a historically poor prognosis. Recent improvements in outcomes have been reported in small numbers of cases with the use of combination therapy involving aggressive surgical resection and chemotherapy. Complete surgical resection is frequently difficult to achieve when the location of the tumor is along the margins of the major hepatic vessels (portal vein, hepatic vein, and hepatic artery). Here we report a case of undifferentiated embryonal sarcoma of the liver that recurred along surgical hepatic vein margins in a 9-year-old boy who subsequently underwent orthotopic liver transplantation from a cadaveric donor. The patient has been in continuous clinical remission for the last 5 years.

Undifferentiated embryonal sarcoma is a rare, highly malignant neoplasm of the liver, mostly affecting children in their late childhood. We present a case of a 5-year-old female with abdominal pain due to an undifferentiated embryonal sarcoma of the right liver lobe and correlate the radiological findings from US, CT and MRI with pathology.

Purpose: This study was undertaken to review the authors' clinical experience with undifferentiated embryonal sarcoma of the liver (UES) in children, focusing on the clinical presentation and results of treatment. Methods: A retrospective analysis of all children who have undergone treatment for UES during the 15-year period from 1984 through 1998 was performed. Results: Seven patients (4 boys and 3 girls) ranging in age from 20 months to 12 years at the time of diagnosis were identified. All presented with large abdominal masses and normal liver function test results. All underwent complete tumor resection; trisegmentectomy was required in 4 of these cases. All patients received postoperative chemotherapy. Two patients suffered tumor recurrence at 12 and 29 months; both of these patients died of their disease. Another patient died of complications related to chemotherapy. The other 4 patients are alive with no evidence of disease after 19 to 150 months' follow-up. Conclusions: Undifferentiated embryonal sarcoma of the liver presents as a large hepatic tumor. Operative resection is difficult, but combined with adjuvant chemotherapy offers the best hope for cure.

Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies. The first patient also had a concurrent cerebellar tumor (pilocytic astrocytoma), for which he first underwent craniotomy and resection, delaying the liver tumor resection by 10 weeks. They are alive and tumor free at 48 months (case no. 1) and 18 months (case no. 2) following neoadjuvant chemotherapy and liver resection.

Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare hepatic tumor and mainly occurs in children. It usually has cystic changes on computed tomography scan, which represents areas of necrosis and hemorrhages. UESL is a rapidly growing tumor and tumor rupture does occur. We reported a girl with a large multicystic hepatic tumor who died of tumor rupture shortly after the diagnosis of UESL. It is mandatory to be aware of this potentially fatal complication especially in patients with UESL of large tumor volume.

The present report describes the case of a 9-year-old girl with an undifferentiated (embryonal) sarcoma of the liver diagnosed by fine-needle aspiration cytology (FNAC). The smears revealed pleomorphic cells, some with cytoplasmic vacuoles and eosinophilic inclusions, as well as spindle cells and myxoid tissue, cytologic features which appear to be distinctive from any other malignant tumor of the liver. Preoperative chemotherapy was then given using a protocol for high-grade soft-tissue sarcomas (MMT95 953 branch B, SIOP). This scheme induced very good response allowing complete surgery 8 months after diagnosis. The patient is alive and well 11 months after surgery. The combined FNAC-preoperative chemotherapy approach may prove useful for this highly malignant tumor.

Hepatic undifferentiated (embryonal) sarcoma is an unusual malignant liver tumor with an overwhelming predilection for children. Fine needle aspiration (FNA) cytology of these tumors rarely has been described in the literature. In this report, we described the clinical, radiologic, cytomorphologic, histopathologic, immunohistochemical, and DNA ploidy features of a similar tumor in an 18 year-old patient. The FNA smears showed clusters and isolated tumor cells with marked variation in morphology including spindle cells, large bizarre anaplastic cells, and giant tumor cells. The histopathologic sections recapitulated the varied morphologic patterns within the tumor cells and also showed characteristic eosinophilic intra- and extracytoplasmic globules. DNA ploidy studies, by image analysis, demonstrated this tumor as an aneuploid tumor, indicating poor prognosis. FNA cytology can be utilized as a sensitive screening tool for these rare tumors.

BACKGROUND Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare malignant mesenchymal hepatic tumor with an incidence among liver tumors of 27.7%. It occurs predominantly in children under the age of 15. CASE The cytologic findings in a case of UESL in fine needle aspiration biopsy material in a 12-year-old girl are described. The patient presented clinically with upper abdominal pain and a palpable mass in the right hepatic lobe. Cytologic features included a combination of polygonal and spindle cells. The polygonal cells were large, with round or lobulated nuclei, and occasionally multinucleated, with one or several nucleoli and variable cytoplasm with poorly defined borders. A few intracytoplasmic and extracytoplasmic eosinophil globules were observed. Histologic and immunohistochemical studies were performed, and staining for vimentin and alpha-1 antichemotrypsin was positive. CONCLUSION It is important to recognize the cytologic features of this type of liver tumor since rapid diagnosis and initiation of early treatment may improve the poor prognosis of these neoplasms.

Adult primary undifferentiated embryonal sarcoma of the liver (UESL) is a rare disease. While the etiology of UESL remains largely unknown, association with systemic inflammatory disorders has been observed. Here, we report a case of UESL in a 46-year-old woman with systemic lupus erythematosus (SLE) and without chronic hepatitis or liver cirrhosis. Systematic review of the publicly available English language medical literature identified only 27 cases of UESL in patients aged >45 years and none with SLE. Our patient presented with abdominal pain and had a 2-year history of SLE. Abdominal ultrasonography and enhanced computed tomography revealed a solid mass in the right lobe of the liver. Presumptive diagnosis of atypical hepatocellular carcinoma was made and the patient was treated with segmentectomy of S5 and S4a and cholecystectomy. The final diagnosis of UESL was made according to the pathology results. Since SLE patients may be at increased risk of malignancy, it is possible that the SLE pathogenesis may have contributed to the development of UESL in our patient. According to this case, UESL should be considered when SLE patients present with hepatic space-occupying lesions.

Background Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL. Materials and methods Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4 months to 17 years (median age, 6 years and 9 months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF + ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx). Results Follow-up from diagnosis ranged from 50 to 222 months (mean 138 months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4 months postoperatively. However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%. Conclusion Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.

BACKGROUND: Undifferentiated sarcoma of the liver is a rare tumor of childhood. The histology has been well documented, but the histogenesis is controversial. The cytologic characteristics of this neoplasm in aspiration material have not been previously documented. CASE: The cytologic features in a 9-year-old male were pleomorphic malignant cells, multinucleate giant cells, numerous hyaline globules and myxoid matrix. CONCLUSION: The cytologic features of undifferentiated sarcoma of the liver are distinctive and different from those of other childhood liver tumors. Awareness of the tumor's cytomorphology can result in a confident preoperative diagnosis.

Objective To examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi-institutional database. Summary Background Data UESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series. Methods The National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012. Results A total of 103 patients (

We have studied one undifferentiated (embryonal) sarcoma of the liver (USL) by fine-needle aspiration cytology (FNAC) and have correlated the cytologic findings with those seen in the histologic sections. The main cytologic and immunocytochemical features were: cellular aspirates, mesenchymal clusters, disparity in individual cell size (small round cells, and multinucleated giant cells, sometimes bizarre), absence of biliary pigment, AFP-negative and vimentin-negative tumor cells, AFP-negative and PAS-positive hyaline globules, and apoptotic cells. Only immunostaining for alpha-1-antitrypsin, alpha-1-antichymotrypsin, and carcinoembryonic antigen were positive in tumor cells in histologic sections. The differential diagnosis should be carried out with rhabdomyosarcomas, hepatoblastomas, malignant fibrous histiocytomas, and poorly differentiated hepatocarcinomas. We think that the cytologic features observed in this case, evaluated both in the appropriate clinical context and in the light of the laboratory findings, may permit the correct diagnosis of this infrequent liver tumor. Nevertheless, more cases need to be studied to assess the reliability of our findings.

Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a tumor highly malignant, of mesenchymal origin. It is a rare finding in adults, though less rare in children. The strategy to be followed and the therapeutic targets to be reached for this tumor, in adult cases, remain ambiguous and controversial. Herein we report the case of a 29 year old female patient with a massive UESL and we describe our therapeutic approach. A 29 year-old female patient was referred to our center with severe intermittent epigastric pain and fever due to a voluminous liver tumor: Needle biopsy was of no specific findings and surgical excision was decided. Right portal vein embolization and selective embolization of the segment's IV branch was performed in order to achieve adequate future liver remnant (FLR). Right trisectonectomy was then performed, with uneventful post operative period and the patient was discharged at the 11(th) post operative day. UESL is a rare tumor that needs aggressive surgical approach and multidisciplinary team management is of paramount importance.

We believe this to be the first case of life-threatening paraneoplastic syndrome (PNS) in a child with undifferentiated embryonal sarcoma of the liver (UESL). We report a case of a 9-year-old child with UESL believed to be unresectable at presentation, who experienced life-threatening, refractory long QTc syndrome, Torsades de pointes, fevers of unknown origin, and secretory diarrhea necessitating emergency surgery. After failure of intravenous immunoglobulin and high-dose steroids to control PNS and despite pancytopenia from chemotherapy, very aggressive surgical resection was life saving and led to rapid resolution of PNS.

We present the case of a 54-year-old gentleman who presented with abdominal distension and a CT scan of his abdomen revealed a large (25 cm) left hepatic lobe tumor. He received chemotherapy for over 1.5 years. The CT scans at the completion of this therapy revealed that the tumor had actually slightly grown in size. He underwent orthotopic liver transplantation without any major complications. The explant histopathology revealed an undifferentiated embryonal cell sarcoma (UECS) composed of relatively bland spindled cells arranged in short fascicles. It is now 10 years and 3 months since his last transplant and the patient remains well with no tumor recurrence.

Undifferentiated embryonal sarcoma of the liver (UESL) represents a heterogeneous group of tumors derived from mesenchymal tissues. Earlier cytogenetic studies in limited cases demonstrated that UESL is associated with a recurrent translocation t(11;19)(q11;q13.3-q13.4) or add(19)(q13.4). In this report, we present our array comparative genomic hybridization (aCGH), fluorescence in situ hybridization (FISH) findings, and a missense mutation of TP53 gene by DNA sequencing in a 19-year-old patient with UESL. The data were compared to laboratory findings reported by previous studies.

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.

The Bochdalek diaphragmatic hernia (BDH) is a common birth defect that is associated with other congenital malformations. Hepatic mesenchymal hamartoma (HMH) is the second most common benign hepatic tumor in children. The association between BDH and HMH or ectopic hepatic tissue is uncommon. We report a case of a female newborn with a left BDH that at surgery was incidentally found to have a tumor of 3.5 cm in maximum dimension with a serous muscular membrane. The histopathological study revealed vascular–mesenchymal stroma with bile ducts entrapped and peripheral normal hepatic tissue. These findings correlate with the diagnosis of hepatic mesenchymal hamartoma (HMH) in ectopic hepatic tissue. Our objective is to report a case of a rare association between HMH and BDH as well as discuss its differential diagnosis.

Six cases of undifferentiated embryonal sarcoma (UES) were reviewed to determine their characteristic features on ultrasonography (US) (n = 5) and computed tomography (CT) (n = 6). US demonstrated a single large, echogenic mass with some anechoic spaces. Contrast-enhanced CT scan revealed a well-demarcated low-attenuation mass with hyperdense septations of variable shape and thickness. Discrepancy of internal architecture on US and CT was one of the important characteristics of UES. CT numbers were 25-47 HU in low-attenuation areas. Enhancing peripheral rim was found in four cases and some solid portions at the periphery or adjacent to the septa were found in all cases. Two patients who had follow-up US and CT without treatment showed enhancing solid portions, changing to hypodense as the tumor grew. When compared with the pathologic findings, US showed a more accurate representation of internal architecture than did CT. Familiarity with these US and CT findings of UES of the liver will be helpful in the differential diagnosis of primary hepatic tumors in childhood.

Summary Undifferentiated embryonal sarcoma of the liver (UESL) is a rare primary liver tumor. Less than 100 adult cases were reported. It has female and right lobe preponderance. In pathological features, focal osteoid picture in UESL is never reported. We present a 63-year-old male patient with left lobe UESL with focal osteoid picture. He was admitted for a palpable solid mass, with left upper quadrant abdominal pain for 4 months. Abdominal computed tomography showed a huge well-circumscribed mass at left upper quadrant, 21.3 × 13 × 27.9 cm 3 in size, with multiple septa in delayed phase. En bloc resection including lateral segmentectomy, splenectomy, and cholecystectomy were performed, but tumor rupture was noted. The pathologic diagnosis was ruptured UESL. The postoperative course was uneventful, and adjuvant radiotherapy without chemotherapy was performed. Peritoneal seeding with massive ascites was noted in the 9 th month after operation. Even after receiving salvage chemotherapy, he died 1 year after operation. Early complete surgical resection with adjuvant chemotherapy may improve prognosis of UESL. But the overall survival of UESL did not improve until recently. We present this case along with a literature review of the clinical pictures, diagnosis, pathology presentation, pathologicogenesis of focal osteoid picture, treatment, and prognosis for UESL of another 23 new reported cases since 2007.

Background. Undifferentiated (embryonal) sarcoma of the liver is a rare, highly malignant, mesenchymal tumor presenting predominantly in late childhood. Four girls, ages 6–13 years, who were treated with combined-modality regimens are reported. Methods. In the first and second patients, hemihepa-tectomy resulted in complete removal of the tumor, and multiple-agent chemotherapy was administered postoperatively. In the third patient, only partial resection could be accomplished initially. By synchronous radiation therapy and chemotherapy, the tumor decreased to an extent that it could be resected completely and was totally devitalized on histologic examination. Postoperative chemotherapy was discontinued after 8 weeks. In the fourth patient, the tumor was not resectable at diagnostic biopsy. Polychemotherapy led to a significant reduction of the tumor size, and resection with clear margins could be performed subsequently. Because histologic necrosis amounted to about 95%, postoperative chemotherapy was also discontinued after 6 weeks. Results. All four patients remain well without evidence of tumor recurrence after 79, 41, 36, and 22 months from diagnosis. Conclusions. The authors suggest that a multimodal therapeutic regimen should be used in patients with undifferentiated hepatic sarcoma.

A 13-year-old boy presented with low-grade fever, upper abdominal pain, loss of appetite, and weight loss for 2 months, with a history of swelling in his upper abdomen for 15 days. On examination, a 17 x 20-cm intraabdominal, smooth, firm, non-tender swelling was noted occupying epigastric and left hypochondrium. The mass moved with respiration, and finger insinuation between mass and right coastal margin was not possible. There was no ascites and further examination was unremarkable. Liver function tests and α-fetoprotein were normal.

AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Background Primary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression. Methods From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years. Results One patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively. Conclusion We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

Undifferentiated (embryonal) sarcoma of liver is a rare tumor with a reputed poor prognosis. Four patients with this tumor are reported, of whom three were alive without recurrence 1.5, 2.5, and 12 years after initial complete surgical resection, and two of whom received no adjuvant therapy. The fourth patient, in whom complete surgical resection of tumor was not achieved, died with recurrent tumor at 13 months. The latter tumor differed histologically and consisted mainly of closely packed smaller undifferentiated cells with a higher mitotic and apoptotic rate. Eosinophilic globules, characteristic of embryonal sarcoma, were found in some cases to contain condensed nuclear chromatin, evidence of origin from tumor cells dying by apoptosis. One tumor mainly contained large cysts lined by biliary-type epithelium; this suggested an origin from a multipotent precursor cell able to differentiate along both stromal and epithelial lines.

Undifferentiated embryonal sarcoma is the uncommon malignant mesenchymal tumor of the liver seen in children and young adults. Radiologic findings reflect the solid to cystic appearances observed in the gross specimens. The discrepancy in the appearances of the mass between the ultrasound scan and computed tomography scan is the hall mark of this tumor. Ultrasonography typically show a large mass which is hyperechoic or isoechoic (solid). Computed tomography shows cystic component of the tumor. This unique radiologic feature will help in the diagnosis of the tumour.

Undifferentiated sarcomas of the liver are very rare and have poor response to chemotherapy and radiation, with resultant poor prognosis. A case of undifferentiated sarcoma of the liver is reported. A 11-year-old woman complained of right hypochondial pain. A giant cystic tumor of the right lobe of the liver was detected by abdominal ultrasonography and CT, and this tumor was hypovascular by angiography. The resected specimen was surrounded by dense materials, with inside including necrotic materials, and no evidense of the tumor. But histological examination revealed immature tumor cells, so we diagnosed it as undifferentiated sarcoma of the liver. Postoperatively combination chemotherapy of VCR, ACD and CPA was performed, and the patient is disease free for 1 year and 5 months after surgery.

Undifferentiated (embryonal) sarcoma of the liver is a primitive mesenchymal neoplasm with predilection for individuals in the first 2 decades of life. In this study (10 boys, 6 girls), children in the age range of 6–10 years were most commonly affected (63%). Clinical features most frequently noted