The online version of this article (doi:10.1186/1477-7819-10-65) contains supplementary material, which is available to authorized users.
The author(s) declare that they have no competing interest.
H Yokoo and T Kamiyama was involved in the review of loterature, acquisition of data and drafting and completing the manuscript. K Nakanishi, T Kakisaka, Y Tsuruga, H Kamachi and M Matsushita participated in the critical review of the paper. All authors read and approved the final manuscript.
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.
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Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R: The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 1995, 13: 610-630. PubMed
Kamezaki K, Kikushige Y, Numata A, Miyamoto T, Takase K, Henzan H, Aoki K, Kato K, Nonami A, Kamimura T, Arima F, Takenaka K, Harada N, Fukuda T, Hayashi S, Ohno Y, Eto T, Harada M, Nagafuji K: Rituximab does not compromise the mobilization and engraftment of autologous peripheral blood stem cells in diffuse-large B-cell lymphoma. Bone Marrow Transplant. 2007, 39: 523-527. 10.1038/sj.bmt.1705649. CrossRefPubMed
Kanamaru R, Wakui A, Kambe M, Ouchi K, Kobari M, Matsuno S, Kimura N, Takahashi T, Asamura M, Yoshioka T, Shibata H, Konishi Y: Undifferentiated sarcoma of the liver in a 21-year-old woman: case report. Jpn J Clin Oncol. 1991, 21: 227-232. PubMed
Walker NI, Horn MJ, Strong RW, Lynch SV, Cohen J, Ong TH, Harris OD: Undifferentiated (embryonal) sarcoma of the liver. Pathologic findings and long-term survival after complete surgical resection. Cancer. 1992, 69: 52-59. 10.1002/1097-0142(19920101)69:1<52::AID-CNCR2820690111>3.0.CO;2-L. CrossRefPubMed
Zornig C, Kremer B, Henne-Bruns D: Weh HJ, Schroder S. Brolsch CE: Primary sarcoma of the liver in the adult. Report of five surgically treated patients. Hepatogastroenterology. 1992, 39: 319-321. PubMed
Grazi GL, Gallucci A, Masetti M, Jovine E, Fiorentino M, Mazziotti A, Gozzetti G: Surgical therapy for undifferentiated (embryonal) sarcomas of the liver in adults. Am Surg. 1996, 62: 901-906. PubMed
Urban CE, Mache CJ, Schwinger W, Pakisch B, Ranner G, Riccabona M, Schimpl G, Brandesky G, Messner H, Pobegen W, Becker H, Grienberger H: Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients. Cancer. 1993, 72: 2511-2516. 10.1002/1097-0142(19931015)72:8<2511::AID-CNCR2820720833>3.0.CO;2-M. CrossRefPubMed
Almogy G, Pappo O, Gips M, Lieberman S, Edden Y, Eid A: Improved survival with surgery and systemic chemotherapy for undifferentiated embryonal sarcoma of the liver. Isr Med Assoc J. 2005, 7: 672-673. PubMed
- A long-term survival case of adult undifferentiated embryonal sarcoma of liver
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