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hepatic undifferentiated sarcoma

Kyu Ho Lee, Mussin Nadiar Maratovich, Kyoung-Bun Lee
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.
June 2016: Clinical and Molecular Hepatology
Daniela Dueñas, Lourdes Huanca, Mónica Cordero, Patricia Webb, Eloy Ruiz
Undifferentiated (embryonal) liver sarcoma is a rare tumor about 2% of all malignant liver tumors with a poor prognosis and usually occurs in children, this review aims to assess cases of primary embryonal sarcoma of the liver presented at our institution the past 8 years and improve recognition of its variants and evaluate immunohistochemical characteristics that help differentiated it from other tumors. Six cases of undifferentiated liver sarcoma were histologically evaluated and investigated by immunohistochemistry with a panel of antibodies using the equipment “Autostainer Link 48”...
January 2016: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Taizo Hibi, Osamu Itano, Masahiro Shinoda, Yuko Kitagawa
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...
April 29, 2016: Surgery Today
Huina Zhang, Li Lei, Craig W Zuppan, Anwar S Raza
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal neoplasm, relatively unique to the liver that occurs primarily in children and teenagers. Delay in diagnosis is not uncommon due to lack of a characteristic clinical presentation, serological markers and radiological changes. We report a case of UESL in a 9-year-old girl who presented with right upper quadrant pain and a palpable mass. Laboratory and imaging workup revealed a complex hepatic cyst, increased IgE, transient peripheral eosinophilia and a normal alpha-fetoprotein (AFP)...
April 2016: Journal of Gastrointestinal Oncology
Yanzhuo Li, Quanyu Cai, Ningyang Jia, Dong Chen, Lun Lu, Hongyan Cheng
BACKGROUND: To investigate the clinical features of undifferentiated embryonal sarcoma of the liver (UESL) to improve its preoperative diagnostic accuracy. METHODS: The clinical, imaging, and histopathologic findings of 16 UESL patients whose disease was pathologically confirmed but preoperatively misdiagnosed were retrospectively analyzed. RESULTS: Among these 16 patients, 9 were clinically misdiagnosed as primary liver cancer, 3 as hepatoblastoma, and 4 as malignant hepatic mass...
December 2015: Annals of Translational Medicine
Avyakta Kallam, Jairam Krishnamurthy, Jessica Kozel, Nicole Shonka
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease...
December 29, 2015: Rare Tumors
Toshiro Masuda, Toru Beppu, Koichi Doi, Tatsunori Miyata, Shigeki Nakagawa, Hirohisa Okabe, Hiromitsu Hayashi, Takatoshi Ishiko, Ken-Ichi Iyama, Hideo Baba
Undifferentiated embryonal sarcoma of the liver (UESL) in adults, especially over 30 years old, is quite rare. We report two adult UESL patients that one of them survived 62 months and one is now surviving more than 65 months treated with repeated hepatic resections and radio-frequency ablations. Although UESL is an entirely unusual and aggressive tumor, multidisciplinary treatments including repeated hepatic resections and radio-frequency ablations may provide a longer survival.
2015: Surgical Case Reports
Pedro Ventura Aguiar, Carla Dias, Pedro Azevedo, Hugo Neves Silva, Manuela Almeida, Sofia Pedroso, La Salete Martins, Leonídio Dias, Anabela Rodrigues, Ramón Viscaíño, António Cabrita, António Castro Henriques
BACKGROUND: Histiocytic sarcoma (HS) is a rare hematologic neoplasm with a few hundred cases having been described to date. CASE PRESENTATION: We report the case of a 56-year-old woman with a history of hepatitis C infection and chronic kidney disease (CKD), submitted to a kidney transplant in 1984, under maintenance immunosuppression with prednisone and azathioprine. Patient presented with a relentlessly growing mass on her right front thorax. It was painless, smooth, and adherent to the deep muscle...
July 2015: Journal of Nephropathology
Masato Nagaoka, Motohiro Kobayashi, Chie Kawai, Sunil K Mallanna, Stephen A Duncan
Maintenance and differentiation of human pluripotent stem cells (hPSCs) usually requires culture on a substrate for cell adhesion. A commonly used substratum is Matrigel purified from Engelbreth-Holm-Swarm sarcoma cells, and consists of a complex mixture of extracellular matrix proteins, proteoglycans, and growth factors. Several studies have successfully induced differentiation of hepatocyte-like cells from hPSCs. However, most of these studies have used Matrigel as a cell adhesion substrate, which is not a defined culture condition...
2015: PloS One
Hirofumi Tazawa, Toshiyuki Itamoto, Akihiko Oshita, Tsuyoshi Kobayashi, Hironobu Amano, Hirotaka Tashiro, Hideki Ohdan, Shoichi Takahashi, Kazuaki Chayama, Koji Arihiro, Osamu Nakashima
We report a rare case of hepatic carcinosarcoma consisting of two carcinomatous components and four sarcomatous components. A 54-year-old Japanese man presented with sudden right upper abdominal pain. Computed tomography showed a hepatic tumor measuring 17 cm in the greatest dimension and intractable ascites, suggesting rupture of hepatocellular carcinoma (HCC). Transcatheter arterial embolization was repeated at our hospital, resulting in stabilization of the patient's general condition. Right anterior and left median sectionectomies were performed...
April 2010: Clinical Journal of Gastroenterology
D Pariente, S Franchi-Abella
Two-thirds of pediatric liver tumors are malignant, but pseudotumors such as abscesses or hematoma can simulate a tumor. The pediatrician is often the first to discover a hepatic mass in a child. The diagnostic gamut varies depending on the child's age. Before the age of three years, the main diagnoses are hepatoblastoma and hemangioma, while after the age of three, hepatocarcinoma, sarcoma, focal nodular hyperplasia, and adenoma are more frequent. The laboratory findings to search for are alpha-fetoprotein whatever the age (increased in hepatoblastoma and hepatocarcinoma), beta-hCG, and urinary catecholamines in infants...
July 2015: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
A Forlani, P Roccabianca, C Palmieri, G Sarli, D Stefanello, S Santagostino, C Randi, G Avallone
Non-angiomatous-non-lymphomatous sarcomas (NANLs) represent 23%-34% of canine primary splenic sarcomas. Splenic liposarcomas account for 2%-6% of NANLs but myxoid variants are rarely reported and information on their behaviour is fragmentary. An 8-year-old male crossbreed (case 1), a 12-year-old female French bulldog (case 2), and an 11-year-old crossbreed (case 3) underwent splenectomy after the detection of a splenic nodule. Histology, histochemistry, immunohistochemistry, and transmission electron microscopy (TEM) were performed...
2015: Veterinary Quarterly
Yu-Hung Lin, Chih-Che Lin, Allan M Concejero, Chee-Chien Yong, Fang-Ying Kuo, Chih-Chi Wang
BACKGROUND: Primary hepatic sarcoma (PHS) is a rare primary liver malignancy. The histological types of PHS are diverse, and the clinical outcomes and management mainly depend on the histopathology. This study aims to evaluate the results of surgical intervention. METHODS: Between January 2003 and June 2009, 13 adult patients with pathologically proven PHS were identified by record review. The patients' demographic profile, tumor characteristics, treatment modalities, and outcomes were reviewed and analyzed...
2015: World Journal of Surgical Oncology
Juan Putra, Kim Ornvold
Undifferentiated embryonal sarcoma of the liver is an aggressive mesenchymal tumor that occurs predominantly in children. Although this entity has been described for decades, its pathogenesis is still obscure. Its association with mesenchymal hamartoma has been well described on the basis of identical chromosomal abnormalities. The clinical and radiological diagnoses are often difficult, and the diagnosis of undifferentiated embryonal sarcoma of the liver is based on its histology and immunophenotype. It is essential to recognize the characteristic histologic findings and the pattern of the immunohistochemistry staining to rule out other hepatic lesions...
February 2015: Archives of Pathology & Laboratory Medicine
A M Halefoglu, A Oz
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...
July 2014: JBR-BTR: Organe de la Société Royale Belge de Radiologie (SRBR)
Monica M Dua, Jordan M Cloyd, Francois Haddad, Ramin E Beygui, Jeffrey A Norton, Brendan C Visser
INTRODUCTION: Primary hepatic sarcomas are uncommon malignant neoplasms; prognostic features, natural history, and optimal management of these tumors are not well characterized. PRESENTATION OF CASE: This report describes the management of a 51-year-old patient that underwent a right trisectionectomy for a large hepatic mass found to be a liver sarcoma on pathology. He subsequently developed tumor emboli to his lungs and was discovered to have cardiac intracavitary metastases from his primary tumor...
2014: International Journal of Surgery Case Reports
Renumathy Dhanasekaran, Alan Hemming, Elaine Salazar, Roniel Cabrera
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...
2012: Case Reports in Hepatology
Margarida Matias, Miguel Carvalho, Luisa Xavier, José Alberto Teixeira
Paratesticular sarcomas are rare and account for less than 1% of all adult sarcomas. Intrascrotal tumours can be testicular or paratesticular, paratesticular tumours being rarer (7-10%). Only 30% of paratesticular tumours are malignant and 90% of these are sarcomas. Histological subtypes include leiomyosarcoma, rhabdomyosarcoma, liposarcoma and undifferentiated high-grade pleomorphic sarcoma. Recurrence is frequent in this type of tumour and can occur years from initial diagnosis. These reports show two cases of paratesticular sarcoma with very distinct evolutions...
2014: BMJ Case Reports
Elaine O'Meara, Deirdre Stack, Susan Phelan, Naomi McDonagh, Lorna Kelly, Raf Sciot, Maria Debiec-Rychter, Thomas Morris, Doug Cochrane, Poul Sorensen, Maureen J O'Sullivan
Undifferentiated spindle cell sarcoma (UDS) is a poorly defined or understood entity, essentially a waste-basket for cases failing to fulfill criteria for better-established diagnoses based on combined histology, immunohistochemistry, and tumor genetic assays. We identified a novel chromosomal translocation t(17;19)(p13;q13) in a pediatric UDS and have characterized this alteration to show rearrangement of the MLL4 and GPS2 genes, resulting in an in-frame fusion gene MLL4-GPS2, the expression of which promotes anchorage-independent growth...
December 2014: Genes, Chromosomes & Cancer
Zi-Ying Xie, Li-Ping Li, Wei-Jing Wu, DA-Yong Sun, Mei-Hua Zhou, Ya-Gang Zhao
Undifferentiated embryonal sarcoma of the liver (UESL) predominantly occurs in children under the age of 10 years, and ~90% of cases occur in children <15 years old. Patients may complain of abdominal pain, fever or other symptoms. No significant decrease has been identified in the hepatic function or elevation of α-fetoprotein, which differentiates UESL from primary carcinomas of the liver. In the present study, a rare and misdiagnosed case of an UESL arising in a male, which was mistaken for a hepatic abscess and retrospectively re-diagnosed, is reported...
September 2014: Oncology Letters
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