Abstract
Undifferentiated embryonal sarcoma (UES) of the liver was first identified as an independent clinicopathologic type of sarcoma in 1978. It is an uncommon hepatic tumor, of mesenchymal origin, usually observed in children, and cases in adults are rare: to our best knowledge, reports of only 51 cases have been published in the past 50 years. We present a case of UES of the liver in a previously healthy 22 year-old woman, admitted to our hospital due to a palpable mass in the right upper abdomen. On admission, laboratory studies showed mildly elevated aspartate aminotransferase, alkaline phosphatase, and γ-GPT. Hepatitis and tumor markers were negative. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) showed a large mass involving the right lobe and the medial segment of the liver, with compression of the bile duct. Right trisectionectomy with bile duct resection and reconstruction was performed. Microscopically, the tumor was composed of pleomorphic spindle cells in a myxoid stroma with focal staining of S-100 by immunohistochemistry. The histologic diagnosis was UES. Adjuvant therapy with vincristine, actinomycin-D, and cyclophosphamide was performed, and at 14 months of follow-up, the patient is alive without any evidence of recurrence. The clinical and histopathological features, as well as the therapeutic choices for adult UES, are described for this patient and in the literature of the past 50 years.
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References
Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Reports of 31 cases. Cancer 1978;42:336–348.
Donovan EJ, Santulli TV. Resection of the left lobe of the liver for mesenchymoma: report of a case. Ann Surg 1946;124:90–93.
Stout AP. Mesenchymoma, mixed tumor of mesenchymal derivates. Ann Surg 1948;127:278–290.
Kelling JW. Liver tumors in infancy and childhood. J Pathol 1971;103:69–85.
Foster JH, Bergman MM. Solid liver tumors. Philadelphia: Saunders; 1977. p. 198–202.
Ishak KG. Primary hepatic tumors in chilhood. In: Popper H, Shaffer F, editors. Progress in liver disease, vol 5. New York: Grune & Stratton; 1976. p. 636–667.
Smithson WA, Telander RL, Carney JA. Mesenchymoma on the liver in chilhood: 5-year survival after combined-modality treatment. J Pediatr Surg 1982;17:70–72.
Tanner AR, Bolton PM, Powell LW. Primary sarcoma of the liver: report of a case with excellent response to hepatic artery ligation and infusion chemotherapy. Gastroenterology 1978;74:121–123.
Ozden I, Bilge O, Erkan M, Cevikbas U, Acarli K. Five years and 4 months of recurrence-free survival iin hepatic angiosarcoma. J Hepatobiliary Pancreat Surg 2003;10:250–252.
Itamoto T, Asahara T, Katayama K, Momisako H, Dohi K, Shimamoto F. Double cancer-hepatocellular carcinoma and intrahepatic cholangiocarcinoma with a spindle-cell variant. J Hepatobiliary Pancreat Surg 1999;6:422–426.
Lise M, Da Pian PP, Nitti D, Miotto D, Balduino M, Bacchetti S. Benign liver tumors in adults: diagnosis and management. J Hepatobiliary Pancreat Surg 1996;3:89–97.
Bottella AG, Valladares LD, Antona EM, Pernaute AS, Aguirre EP, Ortega L, et al. Epithelioid hemangioendothelioma of the liver. J Hepatobiliary Pancreat Surg 2006;13:167–171.
Urban CE, Mache CJ, Schwinger W, Pakish B, Ranner G, Riccabona M, et al. Undifferentiated embryonal sarcoma of the liver in chilhood. Cancer 1993;72:2511–2516.
Bisogno G, Pilz T, Perilongo G, Ferrari A, Harms D, Ninfo V, et al. Undifferentiated sarcoma of the liver in childhood. A curable disease. Cancer 2002;94:252–257.
Lorimer WS. Right hepatolobectomy for primary mesenchymoma of the liver. Ann Surg 1955;141:246–250.
Mattila S, Keskitalo E, Makinem J. Primary non-differentiated sarcoma of the liver. Case report and review of the literature. J Acta Chir Scand 1974;140:303–307.
Blattner WA, Henson DE, Young RC, Fraumeni JF. Malignant mesenchymoma and birth defects. Prenatal exposure to phenytoin. JAMA 1977;283:334–335.
Esposito R, Pollavicini G, de Lalla F. A case of primary undifferentiated sarcoma of the liver. Endoscopy 1977;8:108–110.
Chang WWL, Farooq PA, Morgan WS. Primary sarcoma of the liver in the adult. Cancer 1983;51:1510–1517.
Ellis IO, Cotton RE. Primary malignant mesenchymal tumour of the liver in an elderly female. Histopathology 1983;7:113–121.
Forbes A, Portmann B, Johonson P, Williams R. Hepatic sarcoma in adults: a review of 25 cases. Gut 1987;28:668–674.
Horowitz ME, Ectubanas E, Webber BL, Kun LE, Rao BN, Vogel RJ, et al. Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children. Results of therapy. Cancer 1987;59:396–402.
Miettinem M, Kahlos T. Undifferentiated (embryonal) sarcoma of the liver. Epithelial features as shown by immunohistochemical analysis and electron microscopic examination. Cancer 1989;64:2096–2103.
Kanamaru R, Wakui A, Kambe M, Ouchi K, Kobari M, Matsuno S, et al. Undifferentiated sarcoma of the liver in a 21-year-old woman: case report. Jpn J Clin Oncol 1991;21:227–232.
Lack EE, Schloo BL, Azumi N, Travis WD, Grier HE, Kozakewich PW. Undifferentiated (embryonal) sarcoma of the liver. Clinical and pathologic study of 16 cases with emphasis on immunohistochemical features. Am J Surg Pathol 1991;15:1–16.
McFadden DW, Kelley DJ, Sigmund DA, Barret WL, Dickson B, Aron BS. Embryonal sarcoma of the liver in an adult treated with preoperative chemotherapy, radiation therapy and hepatic lobectomy. Cancer 1992;69:39–44.
Walker NI, Horn ML, Strong RW, Lynch SV, Cohen J, Ong TH, et al. Undifferentiated (embryonal) sarcoma of the liver. Pathological findings and long-term survival after complete surgical resection. Cancer 1992;69:52–59.
Zorning C, Kremer B, Henne-Bruns D, Weh HJ, Schroder S, Brolsch E. Primary sarcoma of the liver in the adult. Report of five surgically treated patients. Hepatogastroenterology 1992;39:319–321.
Zaheer W, Allen SL, Ali SZ, Kahn E, Teichberg S. Primary multicystic undifferentiated embryonal sarcoma of the liver in an adult presenting with peripheral eosinophilia. Ann Clin Lab Sci 1994;24:495–500.
Johnson JA 3rd, White JG, Thompson AR. Undifferentiated (embryonal) sarcoma of the liver in adults. Am Surg 1995;61:285–287.
Grazi GL, Gallucci A. Surgical therapy for undifferentiated (embryonal) sarcomas of the liver in adults. Am Surg 1996;62:901–908.
Lauwers GY, Grant LD, Donnelly W, Meloni A, Foss R, Sanberg A, et al. Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Case report. Am J Surg Pathol 1997;21:1248–1254.
Suarez Y, De Lacy AM, Llovet JM. Intrahepatic bleeding due to undifferentiated (embryonal) hepatic sarcoma. J Hepatol 2000;32:361.
Tokunaga Y, Ryo J, Hoppou T, Kitaoka A, Tokuka A, Osumi K, et al. Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature. Eur J Gastroenterol Hepatol 2000;12:1247–1251.
Begueret H, Trouette H, Vielh P, Laurent C, MacGrogan G, Delson M, et al. Hepatic undifferentiated embryonal sarcoma: malignant evolution of mesenchynal hamartoma? Study of one case with immunohistochemical and flow cytometric emphasis. J Hepatol 2001;34:178–179.
Shufaro Y, Uzieli B, Pappo O, Abramov Y. Pregnancy and delivery in a patient with metastatic embryonal sarcoma of the liver. Obstet Gynecol 2002;99:951–953.
Nishio J, Iwasaki H, Sakashita N, Haraoka S, Isayama T, Naito M, et al. Undifferentiated (embryonal) sarcoma of the liver in middle-aged adults: smooth muscle differentiation determined by immunohistochemistry and electron microscopy. Hum Pathol 2003;34:246–252.
Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, et al. Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol 2004;30:421–427.
Chao-Liu D, Feng X, Hong S, Yong-Qing X, Yong H. Undifferentiated (embryonal) sarcoma of liver in adult: a case report. World J Gastroenterol 2005;11:926–929.
Lepreux S, Rebouisson S, Le Bail B, Saric J, Balabaud C, Bloch B, et al. Mutation of TP53 gene is involved in carcinogenesis of hepatic undifferentiated (embryonal) sarcoma of the adult, in contrast with Wnt or telomerase pathways: an immunohistochemical study of three cases with genomic relation in two cases. J Hepatol 2005;42:424–429.
Agaram NP, Baren A, Antonescu CR. Pediatric and adult hepatic embryonal sarcoma: a comparative ultrastructural study with morphologic correlation. Ultrastruct Pathol 2006;30:403–408.
Sakellaridis T, Panagiotou I, Georgantas T, Micros G, Rontogianni D, Antiochos C. Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis. Case report and review of the literature. World J Surg Oncol 2006;4:9–13.
Scudiere JR, Jakate S. A 51-year-old woman with a liver mass. Arch Pathol Lab Med 2006;130:e24–e26.
Ros PR, Olmsted WW, Dachman AH, Goodman ZD, Ishak KG, Hartman DS. Undifferentiated (embryonal) sarcoma of the liver: radiologic-pathologic correlation. Radiology 1986;160:141–145.
Buetow PC, Buck JL, Pantongrag-Brown L, Marshall WH, Ros PT, Levine MS, et al. Undifferentiated embryonal sarcoma of the liver: pathological basis of imaging findings in 28 cases. Radiology 1997;203:779–783.
Aoyama C, Hachitanda Y, Sato JK. Undifferentiated embryonal sarcoma of the liver. A tumor of uncertain histogenesis showing divergent differentiation. Am J Surg Pathol 1991;15:615–624.
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Pachera, S., Nishio, H., Takahashi, Y. et al. Undifferentiated embryonal sarcoma of the liver: case report and literature survey. J Hepatobiliary Pancreat Surg 15, 536–544 (2008). https://doi.org/10.1007/s00534-007-1265-y
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DOI: https://doi.org/10.1007/s00534-007-1265-y