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1. Iqbal K, Xian ZM, Yuan C: Undifferentiated liver sarcoma -- rare entity: a case report and review of the literature. J Med Case Rep; 2008;2:20

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  • [Title] Undifferentiated liver sarcoma -- rare entity: a case report and review of the literature.
  • INTRODUCTION: Undifferentiated Liver Sarcoma, also known as Undifferentiated Embryonal Sarcoma of the Liver, is a rare, highly malignant neoplasm which affects mostly the pediatric population, although a few cases have been reported in adults.
  • CASE PRESENTATION: We report a case of undifferentiated liver sarcoma in a 14-year-old Chinese boy who presented with non-specific right hypochondriac pain.
  • Exploratory laparotomy with tumor resection was performed, followed by adjuvant chemotherapy.
  • CONCLUSION: Undifferentiated Liver Sarcoma is a rare, highly malignant hepatic neoplasm affecting almost exclusively the pediatric population.
  • The prognosis is poor but recent evidence shows that long-term survival is possible after complete surgical resection and postoperative chemotherapy.

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  • (PMID = 18218141.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2253550
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2. Bisogno G, Pilz T, Perilongo G, Ferrari A, Harms D, Ninfo V, Treuner J, Carli M: Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer; 2002 Jan 1;94(1):252-7
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  • [Title] Undifferentiated sarcoma of the liver in childhood: a curable disease.
  • BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis.
  • METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995.
  • They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease.
  • Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive.
  • After initial chemotherapy tumor reduction was evident in six of nine evaluable cases.
  • Modern multimodal treatment and supportive therapy have improved survival.
  • [MeSH-major] Liver Neoplasms / therapy. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Remission Induction

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  • [Copyright] Copyright 2002 American Cancer Society.
  • (PMID = 11815984.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Kim KT, Han SY, Park EH, Jang JS, Roh MH, Lee SW, Jeong JS: [A case of the treatment in an adult with hepatic undifferentiated (embryonal) sarcoma]. Korean J Hepatol; 2007 Mar;13(1):96-102
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A case of the treatment in an adult with hepatic undifferentiated (embryonal) sarcoma].
  • Undifferentiated embryonal sarcoma is a rare primary malignant neoplasm of the liver.
  • Undifferentiated sarcoma of the liver in adult is an uncommon hepatic tumor of mesenchymal origin, generally considered an aggressive neoplasm with an unfavorable prognosis.
  • We present a case of undifferentiated sarcoma in a 61-year-old woman.
  • US guided liver biopsy was done and pathological findings of the liver specimen revealed that isolated or grouped round pleomorphic cells and spindle to stellate cells were present.
  • She was diagnosed as having undifferentiated sarcoma of the liver.
  • She received seven courses of VAIA chemotherapy by CWS protocols.
  • Chemotherapy was efficacious and the size of the tumor decreased considerably after the treatment.
  • [MeSH-major] Liver Neoplasms / diagnosis. Liver Neoplasms / drug therapy. Sarcoma / diagnosis. Sarcoma / drug therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dactinomycin / therapeutic use. Doxorubicin / therapeutic use. Female. Humans. Ifosfamide / therapeutic use. Middle Aged. Tomography, X-Ray Computed. Vincristine / therapeutic use

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  • (PMID = 17380080.001).
  • [ISSN] 1738-222X
  • [Journal-full-title] The Korean journal of hepatology
  • [ISO-abbreviation] Korean J Hepatol
  • [Language] kor
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide; VAIA protocol
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4. Lee JA, Kim TW, Min JH, Byon SJ, Jang SH, Choi SY, Kim HJ: [A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult]. Korean J Gastroenterol; 2010 Feb;55(2):144-8
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  • [Title] [A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult].
  • Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children.
  • It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma.
  • We experienced a case of undifferentiated sarcoma in 72-year-old male.
  • Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation.
  • We did ultrasonography guided liver biopsy.
  • These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.
  • [MeSH-major] Liver Neoplasms / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Aged. Bile Ducts, Intrahepatic / pathology. Diagnosis, Differential. Dilatation, Pathologic. Humans. Klatskin Tumor / diagnosis. Male. Positron-Emission Tomography. Tomography, X-Ray Computed. Tuberculosis / diagnostic imaging. Tuberculosis / drug therapy. Ultrasonography. Vimentin / metabolism

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  • (PMID = 20168062.001).
  • [ISSN] 2233-6869
  • [Journal-full-title] The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
  • [ISO-abbreviation] Korean J Gastroenterol
  • [Language] kor
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Vimentin
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5. Chuang WY, Lin JN, Hung IJ, Hsueh C: Undifferentiated sarcoma of the liver. Chang Gung Med J; 2002 Jun;25(6):399-404
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  • [Title] Undifferentiated sarcoma of the liver.
  • Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare primary hepatic malignancy principally affecting patients of pediatric age.
  • Recently, long-term disease-free survival has been achieved in cases which underwent aggressive multimodal treatment.
  • Postoperative chemotherapy was recommended, but was refused by the patient's family.
  • A recurrent liver mass was found 171 days after the operation.
  • Chemotherapy was refused once again by the patient's family, and she was lost to follow-up for 5 months.
  • She was admitted and received chemotherapy with vincristine, ifosfamide, and cisplatin.
  • [MeSH-major] Liver Neoplasms / pathology. Sarcoma / pathology

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  • (PMID = 12173670.001).
  • [ISSN] 2072-0939
  • [Journal-full-title] Chang Gung medical journal
  • [ISO-abbreviation] Chang Gung Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / Tumor Suppressor Protein p53
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6. Kinjo S, Sakurai S, Hirato J, Sunose Y: Embryonal sarcoma of the liver with chondroid differentiation. World J Gastrointest Oncol; 2010 May 15;2(5):247-50

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  • [Title] Embryonal sarcoma of the liver with chondroid differentiation.
  • Computed tomography revealed that the tumor, measuring 14.5 cm × 10.4 cm, occupied the anterior and medial segments of the liver and consisted of multicystic and solid lesions.
  • The preoperative diagnosis was a hepatic cystadenocarcinoma.
  • The final diagnosis was embryonal sarcoma of the liver (ESL).
  • At 6 mo follow-up, the patient is alive with the disease and undergoing chemotherapy.

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  • (PMID = 21160625.001).
  • [ISSN] 1948-5204
  • [Journal-full-title] World journal of gastrointestinal oncology
  • [ISO-abbreviation] World J Gastrointest Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC2999185
  • [Keywords] NOTNLM ; Adult / Chondroid differentiation / Immunohistochemistry / Liver tumor / Undifferentiated sarcoma
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7. Gonçalves-Matoso V, Renella R, Gudinchet F, Schnyder P, Beck-Popovic M, Lepori D: [Undifferentiated sarcoma of the liver: a rare tumor of childhood]. Arch Pediatr; 2005 Nov;12(11):1624-6
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  • [Title] [Undifferentiated sarcoma of the liver: a rare tumor of childhood].
  • Undifferentiated sarcoma of the liver is a rare primary tumor of childhood: only about 150 cases have been reported in the literature.
  • Sonography, then CT-scan and MRI showed a large tumor of the liver.
  • COMMENTS: In the differential diagnosis of primary liver tumors in children, one should think about undifferentiated sarcoma of the liver, especially if imaging shows haemorrhagic foci and if sonography and CT/MRI display a discordant appearance.
  • Survival has improved in the last decade due to agressive surgery and intensive chemotherapy.
  • [MeSH-major] Liver Neoplasms / pathology. Sarcoma / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child. Combined Modality Therapy. Diarrhea / etiology. Humans. Magnetic Resonance Imaging. Male. Prognosis. Tomography, X-Ray Computed. Weight Loss

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  • (PMID = 16198096.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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8. Charfi S, Ayadi L, Toumi N, Frikha F, Daoud E, Makni S, Frikha M, Beyrouti MI, Sellami-Boudawara T: Cystic undifferentiated sarcoma of liver in children: a pitfall diagnosis in endemic hydatidosis areas. J Pediatr Surg; 2008 Jun;43(6):E1-4
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  • [Title] Cystic undifferentiated sarcoma of liver in children: a pitfall diagnosis in endemic hydatidosis areas.
  • Undifferentiated (embryonal) sarcoma (UES) of the liver is a very rare pediatric neoplasm with an aggressive behavior.
  • Multimodal treatment including complete surgical resection and chemotherapy has improved survival.
  • The diagnosis of hydatid cyst was initially presumed.
  • A complete tumor resection after 3 cycles of chemotherapy was performed.
  • Adjuvant chemotherapy was instituted.
  • Differential diagnosis with hydatid cyst may be problematic in endemic hydatidosis areas.
  • [MeSH-major] Echinococcosis, Hepatic / pathology. Liver Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Sarcoma / pathology
  • [MeSH-minor] Abdominal Pain / diagnosis. Abdominal Pain / etiology. Adolescent. Biopsy, Needle. Chemotherapy, Adjuvant. Combined Modality Therapy. Diagnosis, Differential. Endemic Diseases. Endosonography / methods. Female. Follow-Up Studies. Hepatectomy / methods. Humans. Immunohistochemistry. Risk Assessment. Tomography, X-Ray Computed. Treatment Outcome. Tunisia

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  • (PMID = 18558156.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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9. Okajima H, Ohya Y, Lee KJ, Yamamoto H, Asonuma K, Nagaoki Y, Ohama K, Korogi M, Anan T, Hashiyama M, Endo F, Iyama K, Inomata Y: Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure. J Pediatr Surg; 2009 Feb;44(2):e33-8
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  • [Title] Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure.
  • We present the cases of 3 children with huge undifferentiated sarcoma of the liver who were treated with surgical excision including liver transplantation as an option and adjuvant chemotherapy.
  • The primary tumor and the ruptured tissue fragments were removed and he was given postoperative chemotherapy.
  • Preoperative chemotherapy was given but later discontinued owing to severe side effects.
  • He underwent living donor liver transplantation followed by postoperative chemotherapy.
  • He is doing well and is disease free 18 months after the second procedure.
  • Complete removal of the tumor including total hepatectomy and transplantation when indicated and suitable pre- and/or postoperative chemotherapy is an effective treatment for children with undifferentiated sarcoma of the liver.
  • [MeSH-major] Liver Neoplasms / surgery. Liver Transplantation. Sarcoma / surgery


10. Uchiyama M, Iwafuchi M, Yagi M, Iinuma Y, Kanada S, Yamazaki S, Ohtaki M, Shirai Y: Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature. J Hepatobiliary Pancreat Surg; 2001;8(1):87-91
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  • [Title] Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature.
  • 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.
  • 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.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver Neoplasms / drug therapy. Liver Neoplasms / surgery. Neoplasms, Germ Cell and Embryonal / drug therapy. Neoplasms, Germ Cell and Embryonal / surgery

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  • (PMID = 11294295.001).
  • [ISSN] 0944-1166
  • [Journal-full-title] Journal of hepato-biliary-pancreatic surgery
  • [ISO-abbreviation] J Hepatobiliary Pancreat Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 26
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11. Kim DY, Kim KH, Jung SE, Lee SC, Park KW, Kim WK: Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg; 2002 Oct;37(10):1419-23
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  • [Title] Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy.
  • BACKGROUND/PURPOSE: Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignancy found in older children, and the most appropriate treatment strategy has been controversial.
  • The authors report the tumor's clinical characteristics and the results of management in 6 children and recommend the best treatment plans based on these findings.
  • RESULTS: The mean age at diagnosis of USL was 10.3 years (range, 7 to 13 years).
  • Three children underwent primary complete resection without preoperative chemotherapy.
  • However, complete resection was possible at second-look operation after chemotherapy.
  • Two children underwent complete resection after chemotherapy.
  • Preoperative chemotherapy allowed successful resection of 3 USL, which were initially considered unresectable.
  • Chemotherapy according to the the Third Intergroup Rhabdomyosarcoma Study (IRS III) was administered to all except one child who refused further postoperative chemotherapy after having had severe complications during the first cycle of chemotherapy.
  • One child with partial resection died of sepsis at 22 months after diagnosis during postoperative chemotherapy after complete surgical removal of the tumor.
  • Five children are alive without recurrence at 40, 45, 48, 60, and 122 months, respectively, after diagnosis.
  • CONCLUSION: The combined therapy of surgery and chemotherapy in USL can improve the prognosis.
  • [MeSH-major] Liver Neoplasms / drug therapy. Liver Neoplasms / surgery. Mesenchymoma / drug therapy. Mesenchymoma / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Female. Hepatectomy. Humans. Male. Prognosis. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12378446.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article
  • [Publication-country] United States
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12. Jiménez Fuertes M, López Andújar R, de Juan Burgueño M, Moya Herráiz A, Sanjuán Rodríguez F, Montalvá Orón E, Pareja Ibars E, Mir Pallardó J: [Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and literature review]. Gastroenterol Hepatol; 2008 Jan;31(1):12-7
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  • [Title] [Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and literature review].
  • [Transliterated title] Sarcoma indiferenciado (embrionario) de hígado del adulto: informe de un caso y revisión de la literatura médica.
  • Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant hepatic tumor in adults.
  • We report the case of a 40-year-old man with USL who was successfully treated with surgical resection and chemotherapy using ifosfamide and adriamycin.
  • Although the prognosis of the disease remains generally poor, long term survival has been achieved in patients with a combination of surgery and chemotherapy.
  • [MeSH-major] Liver Neoplasms / diagnosis. Sarcoma / diagnosis

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  • (PMID = 18218274.001).
  • [ISSN] 0210-5705
  • [Journal-full-title] Gastroenterología y hepatología
  • [ISO-abbreviation] Gastroenterol Hepatol
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 44
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13. Chowdhary SK, Trehan A, Das A, Marwaha RK, Rao KL: Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst. J Pediatr Surg; 2004 Jan;39(1):E9-12
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  • [Title] Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst.
  • 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.
  • 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.
  • [MeSH-major] Cysts / diagnosis. Liver Diseases / diagnosis. Liver Neoplasms / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Abdominal Pain / etiology. Child. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Fever / etiology. Humans. Male

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  • (PMID = 14694398.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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14. McCarthy FP, Harris M, Kornman L: Management of undifferentiated embryonal sarcoma of the liver in pregnancy. Obstet Gynecol; 2007 Feb;109(2 Pt2):558-60
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  • [Title] Management of undifferentiated embryonal sarcoma of the liver in pregnancy.
  • BACKGROUND: Undifferentiated embryonal sarcoma of the liver is a rare mesenchymal neoplasm with a poor prognosis.
  • Our patient was a woman whose pregnancy was complicated by the recurrence of metastatic undifferentiated embryonal sarcoma of the liver.
  • CASE: In the medical literature only two other women have been described with metastatic undifferentiated embryonal sarcoma of the liver in pregnancy.
  • This is the first reported case of undifferentiated embryonal sarcoma of the liver recurring in and requiring treatment during pregnancy.
  • The patient gave birth to a healthy son before requiring further palliative chemotherapy.
  • CONCLUSION: This case highlights the fact that cancer not infrequently complicates pregnancy and can require treatment, in this case with radio frequency ablation.
  • [MeSH-major] Liver Neoplasms / diagnosis. Pregnancy Complications, Neoplastic / diagnosis. Prenatal Diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Catheter Ablation. Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Infant, Newborn. Magnetic Resonance Imaging. Male. Pregnancy


15. Wei ZG, Tang LF, Chen ZM, Tang HF, Li MJ: Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis. J Pediatr Surg; 2008 Oct;43(10):1912-9
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  • [Title] 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.
  • Histopathologic evaluation of the mass confirmed the diagnosis of UELS.
  • 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.
  • [MeSH-major] Biomarkers, Tumor / analysis. Liver Neoplasms / pathology. Neoplasm Proteins / analysis. Neoplasms, Germ Cell and Embryonal / pathology. Sarcoma / pathology
  • [MeSH-minor] Antigens, CD / analysis. Antigens, Differentiation, Myelomonocytic / analysis. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Desmin / analysis. Dexamethasone / administration & dosage. Doxorubicin / administration & dosage. Fatal Outcome. Female. Hepatectomy. Humans. Ifosfamide / administration & dosage. Male. Neoadjuvant Therapy. Remission Induction. Vimentin / analysis. Vinblastine / administration & dosage

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  • (PMID = 18926232.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Differentiation, Myelomonocytic; 0 / Biomarkers, Tumor; 0 / CD68 antigen, human; 0 / Desmin; 0 / Neoplasm Proteins; 0 / Vimentin; 5V9KLZ54CY / Vinblastine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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16. Küpeli S, Yalçin B, Cil BE, Akçören Z, Büyükpamukçu M: Undifferentiated embryonal sarcoma of the liver in a child complicated by haemorrhage. Pediatr Radiol; 2008 Nov;38(11):1259-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Undifferentiated embryonal sarcoma of the liver in a child complicated by haemorrhage.
  • Undifferentiated embryonal sarcoma (UES) of the liver is a rare malignant neoplasm that mostly affects children younger than 15 years of age.
  • The tumour was managed by embolization of the right hepatic artery and treated successfully with chemotherapy and surgical resection.
  • Prophylactic embolization of the feeding artery should be undertaken before a biopsy procedure if there is the possibility of tumour rupture, in the presence of signs of intratumoral or peritumoral bleeding, or in the presence of a vascular liver mass.
  • [MeSH-major] Hemorrhage / etiology. Liver Neoplasms / complications. Neoplasms, Germ Cell and Embryonal / complications
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biopsy, Needle. Combined Modality Therapy. Diagnosis, Differential. Embolization, Therapeutic / methods. Humans. Male. Tomography, X-Ray Computed

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  • [Cites] J Pediatr Surg. 2002 Oct;37(10):1419-23 [12378446.001]
  • [Cites] J Pediatr Hematol Oncol. 2007 Jan;29(1):63-5 [17230070.001]
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  • (PMID = 18688607.001).
  • [ISSN] 0301-0449
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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17. Anavi BL, Moumdjiev IN, Stoyanova AA, Sapunarova KG, Tashev PV, Dureva-Popova MK: A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type. Folia Med (Plovdiv); 2001;43(3):53-8
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  • [Title] A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type.
  • We present the first case in Bulgaria of cytologically verified undifferentiated (embryonal) sarcoma of the liver.
  • A large formation was found in the right liver lobe on ultrasound examination, which was polycystic on CT scan.
  • In the scanty specimen obtained by fine-needle aspiration biopsy under ultrasound control a characteristic prevalence of polygonal cells within mesenchyme tissue was found.
  • The cells are pathognomonic of undifferentiated (embryonal) sarcoma of the liver.
  • After preoperative chemotherapy with ifosfamide, vincristine and actinomycin D (CEVAIE, CWS--96) resection of the tumor including IV, V, VI and VII liver segments was performed.
  • The cytological diagnosis was histologically verified.
  • The patient had a mechanical subileus episode after the 6th course of chemotherapy.
  • The case indicates that the unique cell appearance in embryonal sarcoma of the liver provides a correct diagnosis even in a scanty specimen.
  • [MeSH-major] Liver Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Sarcoma / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Biopsy, Needle. Child. Combined Modality Therapy. Female. Humans

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  • (PMID = 11930835.001).
  • [ISSN] 0204-8043
  • [Journal-full-title] Folia medica
  • [ISO-abbreviation] Folia Med (Plovdiv)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Bulgaria
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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18. Tokunaga Y, Ryo J, Hoppou T, Kitaoka A, Tokuka A, Osumi K, Tanaka T: Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature. Eur J Gastroenterol Hepatol; 2000 Nov;12(11):1247-51
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  • [Title] Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.
  • 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.
  • 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.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols. Liver Neoplasms / drug therapy. Liver Neoplasms / surgery. Neoplasms, Germ Cell and Embryonal / drug therapy. Neoplasms, Germ Cell and Embryonal / surgery
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Cisplatin. Doxorubicin. Hepatectomy. Humans. Ifosfamide. Male

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  • (PMID = 11111784.001).
  • [ISSN] 0954-691X
  • [Journal-full-title] European journal of gastroenterology & hepatology
  • [ISO-abbreviation] Eur J Gastroenterol Hepatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide; IAP protocol
  • [Number-of-references] 37
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19. Nicol K, Savell V, Moore J, Teot L, Spunt SL, Qualman S, Children's Oncology Group, Soft Tissue Sarcoma Committee: Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a Children's Oncology Group study. Pediatr Dev Pathol; 2007 Mar-Apr;10(2):89-97
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  • [Title] Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a Children's Oncology Group study.
  • Morphologically, the distinction between undifferentiated embryonal sarcoma of the liver (UESL) and biliary tract rhabdomyosarcoma (RMS) can be uncertain because of some shared pathologic similarities.
  • Patients with UESL have been consistently but erroneously enrolled in Children's Oncology Group (COG) treatment protocols because UESL was equated with RMS, despite the differing primary treatment modalities of these entities.
  • Establishing the correct diagnosis of these distinct clinical and pathologic entities is important, as surgery alone may be curative in UESL, whereas initial chemotherapy is often recommended for the treatment of biliary tract RMS.
  • [MeSH-major] Bile Duct Neoplasms / diagnosis. Biomarkers, Tumor / analysis. Liver Neoplasms / diagnosis. Rhabdomyosarcoma / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Child. Child, Preschool. Diagnosis, Differential. Disease-Free Survival. Female. Follow-Up Studies. Humans. Immunohistochemistry. Male. MyoD Protein / metabolism. Myogenin / metabolism. Neoplasm Proteins / metabolism. Retrospective Studies. Survival Analysis. Time Factors. Treatment Outcome


20. Corapçioglu F, Sarper N, Demir H, Güvenç BH, Sözübir S, Akansel G, Berk FS: A child with undifferentiated sarcoma of the liver complicated with bronchobiliary fistula and detected by hepatobiliary scintigraphy. Pediatr Hematol Oncol; 2004 Jul-Aug;21(5):427-33
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  • [Title] A child with undifferentiated sarcoma of the liver complicated with bronchobiliary fistula and detected by hepatobiliary scintigraphy.
  • Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant mesenchymal tumor principally affecting patients of pediatric age.
  • Bronchobiliary fistula is a very rare complication in patients with liver tumor.
  • To the authors' knowledge, this is the first report of a bronchobiliary fistula resulting from tumor invasion in a child with liver sarcoma.
  • A 12-year-old boy was diagnosed to have USL of the right liver lobe, invading the diaphragm.
  • An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface followed by combined chemotherapy regimen.
  • Hepatobiliary scintigraphy is the useful diagnostic procedure to define bronchobiliary fistula in children with liver tumor and clinical suspicion of bronchobiliary fistula.
  • [MeSH-major] Biliary Tract Diseases / complications. Biliary Tract Diseases / radionuclide imaging. Bronchial Fistula / complications. Bronchial Fistula / radionuclide imaging. Liver Neoplasms / complications. Sarcoma / complications. Technetium Tc 99m Disofenin

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  • (PMID = 15205086.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] QTJ2VIW97T / Technetium Tc 99m Disofenin
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21. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky B, Uzieli B, Eid A: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol; 2004 May;30(4):421-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.
  • 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).
  • Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2).
  • Two patients developed complications and there was one death.
  • 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.
  • Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.
  • [MeSH-major] Liver Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Hepatectomy / methods. Humans. Length of Stay. Liver Function Tests. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 15063896.001).
  • [ISSN] 0748-7983
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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22. Dower NA, Smith LJ, Lees G, Kneteman N, Idikio H, Emond J, Rosenthal P: Experience with aggressive therapy in three children with unresectable malignant liver tumors. Med Pediatr Oncol; 2000 Feb;34(2):132-5
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  • [Title] Experience with aggressive therapy in three children with unresectable malignant liver tumors.
  • BACKGROUND: Children with malignant liver tumors often present with unresectable disease but need not be considered incurable.
  • The advent of effective chemotherapy makes aggressive management feasible, as our experience with three such patients demonstrates.
  • Procedure and Results One child with an unresectable undifferentiated sarcoma of the liver and two others with unresectable primary hepatoblastoma and lung metastases were treated with initial chemotherapy, followed by aggressive surgical management.
  • Treatment with chemotherapy followed by hepatectomy and liver transplantation (cadaveric or live donor) in two children has resulted in disease-free survivals of 79 and 38 months.
  • The third patient is alive and well 24 months following chemotherapy and aggressive resection of the primary and 12 metastatic lesions.
  • CONCLUSIONS: Initial chemotherapy for unresectable liver tumors with or without metastases is supported by the review of the literature.
  • Consideration of orthotopic liver transplantation (OLT) from cadaveric or living related donor is warranted when the malignancy is demonstrably chemosensitive, independent of initial staging.
  • [MeSH-major] Liver Neoplasms / therapy
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male

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  • [Copyright] Copyright 2000 Wiley-Liss, Inc.
  • (PMID = 10657875.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
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23. Yagyu S, Morimoto A, Kakazu N, Tamura S, Fujiki A, Nakase Y, Iehara T, Hosoi H, Kuroda H: Late appearance of a Philadelphia chromosome in a patient with therapy-related acute myeloid leukemia and high expression of EVI1. Cancer Genet Cytogenet; 2008 Jan 15;180(2):115-20
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  • [Title] Late appearance of a Philadelphia chromosome in a patient with therapy-related acute myeloid leukemia and high expression of EVI1.
  • A 17-year-old boy developed therapy-related acute myeloid leukemia (t-AML) 3 years after the cessation of chemo- and radiotherapy for undifferentiated sarcoma of the liver.
  • ) karyotype. Despite intensive chemotherapy and two hematopoietic stem cell transplants, he died of the leukemia.
  • Expression of EVI1 in bone marrow cells was remarkably high at the onset of t-AML, although it was not detected at the end of therapy for the sarcoma.
  • [MeSH-minor] Adolescent. Fusion Proteins, bcr-abl / genetics. Fusion Proteins, bcr-abl / metabolism. Gene Expression Regulation, Leukemic. Humans. In Situ Hybridization, Fluorescence. Leukemia, Myeloid, Acute / etiology. Leukemia, Myeloid, Acute / genetics. Liver Neoplasms / therapy. Male. RNA, Messenger / metabolism. Sarcoma / therapy. Up-Regulation

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  • (PMID = 18206536.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / MECOM protein, human; 0 / RNA, Messenger; 0 / Transcription Factors; EC 2.7.10.2 / Fusion Proteins, bcr-abl
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24. Rosito P, Mancini AF, Semeraro M, Paone G, Lima M, Federici S, Domini M, Burnelli R, Baroncini S, Gentili A, Paolucci G, Domini R: [Malignant primary tumors of the liver in childhood]. Pediatr Med Chir; 2002 May-Jun;24(3):200-7
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  • [Title] [Malignant primary tumors of the liver in childhood].
  • The diagnosis was hepatoblastoma (HBL) in 16 cases; hepatocellular carcinoma (HCA) in 3 cases; undifferentiated sarcoma in 1, malignant rhabdoid tumour of the liver in 1.
  • Median age at diagnosis was 1.8 year (1 mounth-13 years).
  • At diagnosis 3 pts presented lung metastases.
  • In 10 patients, initially with unresesectable tumor, chemotherapy was started first.
  • Drugs used were mostly Cisplatinum or Carboplatinum with Doxorubicin.
  • We conclude that excluding metastases at diagnosis (3 deaths), the main prognostic factor is resectability and radical surgery: in our experience 4 patients with unresectable tumor died, as 2 patients with microscopical residual after surgery.
  • [MeSH-major] Liver Neoplasms / epidemiology
  • [MeSH-minor] Algorithms. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Retrospective Studies

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  • (PMID = 12236033.001).
  • [ISSN] 0391-5387
  • [Journal-full-title] La Pediatria medica e chirurgica : Medical and surgical pediatrics
  • [ISO-abbreviation] Pediatr Med Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Italy
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25. Malogolowkin MH, Stanley P, Steele DA, Ortega JA: Feasibility and toxicity of chemoembolization for children with liver tumors. J Clin Oncol; 2000 Mar;18(6):1279-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Feasibility and toxicity of chemoembolization for children with liver tumors.
  • PURPOSE: To determine the feasibility, toxicity, and efficacy of hepatic arterial chemoembolization (HACE) in pediatric patients with refractory primary malignancies of the liver.
  • PATIENTS AND METHODS: Six patients with hepatoblastoma (HB), three with hepatocellular carcinoma (HCC), and two with undifferentiated sarcoma of the liver were treated with HACE every 2 to 4 weeks until their tumors became surgically resectable or they showed signs of disease progression.
  • All but one newly diagnosed patient with HCC had previously received systemic chemotherapy.
  • Elevated liver transaminase and bilirubin levels were seen after each one of the 46 courses of HACE.
  • [MeSH-major] Chemoembolization, Therapeutic. Liver Neoplasms / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma, Hepatocellular / therapy. Child. Child, Preschool. Collagen / administration & dosage. Collagen / therapeutic use. Feasibility Studies. Female. Hepatic Artery. Hepatoblastoma / therapy. Humans. Infant. Male. Sarcoma / therapy. Treatment Outcome

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  • (PMID = 10715298.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 121938-23-6 / Angiostat; 9007-34-5 / Collagen
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26. Berk F, Corapcioglu F, Demir H, Akansel G, Guvenc BH: Bronchobiliary fistula detected with hepatobiliary scintigraphy. Clin Nucl Med; 2006 Apr;31(4):237-9

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  • A 12-year-old boy was diagnosed with undifferentiated sarcoma of the right lobe of the liver invading the diaphragm.
  • Surgery was followed with a combined chemotherapy regimen.
  • For this patient; hepatobiliary scintigraphy, which is routinely used to visualize the liver and biliary tree, provided a noninvasive mean for the precise diagnosis of a bronchobiliary fistula.
  • [MeSH-minor] Bile. Child. Hepatectomy. Humans. Liver Neoplasms / surgery. Male. Radiopharmaceuticals. Sarcoma / surgery. Technetium Tc 99m Disofenin

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  • (PMID = 16550028.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; QTJ2VIW97T / Technetium Tc 99m Disofenin
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