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hepatic undifferentiated sarcoma

Michele A Capozza, Antonio Ruggiero, Palma Maurizi, Stefano Mastrangelo, Giorgio Attinà, Silvia Triarico, Maria Vellone, Felice Giuliante
Definitive diagnosis of pediatric liver masses can be challenging, because clinical manifestations are nonspecific, and ultimate diagnosis may be delayed. We describe 2 patients with liver masses that initially were misdiagnosed and treated as infectious hepatic lesions. Only after histologic examination the correct diagnosis of undifferentiated embryonal sarcoma of the liver was defined. Both patients underwent a complete tumor resection followed by chemotherapy with a favorable outcome.
April 26, 2018: Journal of Pediatric Hematology/oncology
Zarak H Khan, Kamran Ilyas, Hamza H Khan, Haider Ghazanfar, Qulsoom Hussain, Faisal Inayat, Muhammad Yasir, Rizwan Asim
Undifferentiated embryonal sarcoma of the liver (UESL) is a malignancy of mesenchymal origin observed predominantly in the pediatric population and very rarely in adults. We describe the case of a 21-year-old male who presented with acute onset of right upper quadrant pain and distention. Physical examination of the patient revealed right upper quadrant tenderness with the lower border of the liver palpable, 4 cm below the right costal margin. Laboratory tests performed on admission showed that the patient's liver function tests, urinalysis, complete blood count, and basic metabolic panel were within reference range...
October 8, 2017: Curēus
Rajesh Thampy, Khaled M Elsayes, Christine O Menias, Perry J Pickhardt, Hyunseon C Kang, Sandeep P Deshmukh, Kareem Ahmed, Brinda Rao Korivi
Tumours arising from mesenchymal tissue components such as vascular, fibrous and adipose tissue can manifest in the liver. Although histopathology is often necessary for definitive diagnosis, many of these lesions exhibit characteristic imaging features. The radiologist plays an important role in suggesting the diagnosis, which can direct appropriate immunohistochemical staining at histology. The aim of this review is to present clinical and imaging findings of a spectrum of mesenchymal liver tumours such as haemangioma, epithelioid haemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumour, solitary fibrous tumour, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases...
November 2017: British Journal of Radiology
Akiko Mori, Koji Fukase, Kunihiro Masuda, Naoaki Sakata, Masamichi Mizuma, Hideo Ohtsuka, Takanori Morikawa, Kei Nakagawa, Hiroki Hayashi, Fuyuhiko Motoi, Takeshi Naitoh, Keigo Murakami, Michiaki Unno
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: Here, we describe the case of a 65-year-old woman who presented with a huge cystic lesion in the liver. Laboratory studies performed on admission showed modest inflammation, poor nutrition, and elevated levels of total bilirubin, alkaline phosphatase, and γ-glutamyl transferase...
December 2017: Surgical Case Reports
Daniela Treitl, Alexandra Roudenko, Siba El Hussein, Magda Rizer, Philip Bao
Undifferentiated embryonal sarcomas of the liver are extremely rare cases in adults. We report the case of a 30-year-old male who presented with early satiety and abdominal pain due to a massive tumor originating from the left liver and occupying the entire epigastrium. The patient underwent bland embolization in an attempt to decrease the size of the tumor. He then underwent a formal left hepatectomy with resection of liver segments 2, 3, and 4. Extrahepatic inflow control of the portal vein and hepatic artery was performed prior to parenchymal transection...
2016: Case Reports in Surgery
Emil Radu Iacob, Călin Marius Popoiu, Alexandra Nyiredi, LaurenŢiu Mogoantă, Oana Badea, Eugen Sorin Boia
Mesenchymal hamartoma of the liver (MHL) is a benign and rare hepatic lesion, with an uncertain etiology and a potential for developing into an undifferentiated distant embryonal sarcoma after an incomplete resection. It mainly presents as progressive abdominal distension with normal blood works. Most cases are diagnosed in the first two years of life, with a higher frequency in boys and on the right liver. We report the case of a mesenchymal hamartoma of the left liver in an 18-month-old girl, with a rough evolution and a literature review...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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 2017: 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...
February 28, 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
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