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Pediatric Surgery International
Published in: Pediatric Surgery International 12/2013

01-12-2013 | Original Article

Embryonal sarcoma of the liver

Authors: G. Chocarro, M. V. Amesty, F. Hernández, B. G. Goiboru Chenu, R. Ortíz, S. Hernández, A. Sánchez, M. Gámez, M. López Santamaría, J. A. Tovar

Published in: Pediatric Surgery International | Issue 12/2013

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Abstract

Purpose

Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center.

Methods

We retrospectively reviewed liver tumors treated between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP protocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints.

Results

Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diagnosis was 81 ± 8.5 months. The most frequent finding was abdominal mass. Alfa-fetoprotein was normal. Imaging depicted heterogeneous tumors with septa, necrosis, and hemorrhagic areas. The diagnosis was ascertained by biopsy. Three tumors were located in the right lobe (PRETEXT II): two were bilobar (PRETEXT III) and one was in the left lobe (PRETEXT I). Two children had metastases at diagnosis and high-risk chemotherapy (vincristine, carboplatin, epirubicin) was administered with poor response. They died without operation 4 and 10 months later. Four patients with local disease underwent typical liver resections after chemotherapy (iphosphamide, vincristine, actinomycin D, and doxorrubicin). Overall actuarial survival at 70 months was 66.6 %.

Conclusions

Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure.
Literature
1.
Exelby PR, Filler RM, Grosfeld JL (1975) Liver tumor in children with particular reference to hepatoblastoma and hepatocellular carcinoma: American academy of pediatric surgical section survey-1974. Pediatr Surg 10:329–337CrossRef
2.
3.
Horowitz ME, Etcubanas E, Webber BL, Kun LE, Rao BN, Vogel RJ et al (1987) Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children. Cancer 59:396–402PubMedCrossRef
4.
Iqbal K, Xian ZM, Yuan C (2008) Undifferentiated liver sarcoma-rare entity:a case report and review of the literature. J Med Case Reports 2:20CrossRef
5.
Leuschner I, Schmidt D, Harms D (1990) Undifferentiated sarcoma of the liver in childhood: morphology, flow cytometry, and literature review. Hum Pathol 21(1):68PubMedCrossRef
6.
Lashkari HP, Khan SU, Ali K, Sashikumar P, Mukherjee S (2009) Diagnosis of undifferentiated embryonal sarcoma of the liver-importance of combine studies of ultrasound and CT scan. J Pediatr Hematol Oncol 31(10):797–798
7.
Bluetow PC, Buck JL, Pantongrag-Brown L et al (1997) Undifferentiated (embryonal) sarcoma of the liver: pathologic basis of imaging findings in 28 cases. Radiology 203:779–783
8.
Chung EM, Lattin GE Jr, Cube R, Lewis RB, Marichal-Hernández C, Shawhan R, Conran RM (2011) From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation. Ragiographics 31:483–507CrossRef
9.
Oral A, Yigiter M, Demirci E, Yildirim ZK, Kantarci M, Salman AB (2011) A case of undifferentiated embryonic liver sarcoma mimicking cystic hydatid disease in an endemic region of the world. J Pediatr Surg 46(11):5e–9eCrossRef
10.
Joshi SW, Merchant NH, Jambhekar NA (1997) Primary multilocular cystic undifferentiated (embryonal) sarcoma of the liver in childhood resembling hydatid cyst of the liver. Br J Radiol 70:314–316PubMed
11.
Mascarello JT, Krous HF (1992) Second report of a translocation involving 19q13.4 in a mesenchymalhamartoma of the liver. Cancer Genet Cytogene 58(2):141–142CrossRef
12.
Bove KE, Blough RI, Soukup S (1998) Third report of t (19q)(13.4) in mesenchymalhamartoma of liver with comments on link to embryonal sarcoma. Pediatr Dev Pathol 1:438–442PubMedCrossRef
13.
Shehata BM, Gupta NA, Katzenstein HM, Steelman CK, Wulkan ML, Gow KW, Bridge JA et al (2011) Undifferentiated embryonal sarcoma of the liver is associated with mesenchymalhamartoma and multiple chromosomal abnormalities: a review of eleven cases. Pediatr Dev Pathol 14(2):111–116PubMedCrossRef
14.
Zeng JM, Tao X, Xu AM et al (2007) Primary and recurrent embryonal sarcoma of the liver: clinicopathological and immunohistochemical. Analysis 51:195–203
15.
Lack EE, Schloo BL, Azumi N et al (1991) Undifferentiated (embryonal) sarcoma of the liver. Clinical and pathologic study of 16 cases with emphasis on immunohistochemical features. Am J Surg Pathol 15:1–16PubMedCrossRef
16.
Lack EE, Schloo BL, Azumi N, Travis WD, Grier HE, Kozakewich HPW (1991) Undifferentiated embryonal sarcoma of the liver. Clinical and pathologic study of 16 cases with emphasis on immunohistochemical features. Am J Surg Pathol 15:615–624CrossRef
17.
Perilongo G, Carli M, Sainati L, Cecchetto G, Mancini A, Cordero di Montezemolo I et al (1978) Undifferentiated (embryonal) sarcoma of the liver in childhood: results of a retrospective Italian study. Tumori 73:213–217
18.
Walker NI, Horn MJ, Strong RW, Lynch SV, Cohen J, Ong TH et al (1992) Undifferentiated (embryonal) sarcoma of the liver in childhood.Successful combined-modality therapy in four patients. Cancer 72:2511f–2516f
19.
Bisogno G, Pilz T, Perilongo G et al (2002) Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer 94:252–257PubMedCrossRef
20.
Dower NA, Smith LJ, Lees G, Kneteman N, Idikio H, Emond J et al (2000) Experience with aggressive therapy in three children with unresectable malignant liver tumors. Med Pediatr Oncol 34:132–135PubMedCrossRef
21.
May LT, Wang M, Albano E, Garrington T, Dishop M, Macy ME (2012) Undifferentiated sarcoma of the liver: a single institution experience using a uniform treatment approach. J Pediatr Hematol Oncol 34(3):114e–116eCrossRef
22.
Urban CE, Mache CJ, Schwinger W et al (1993) Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients. Cancer 72(8):2511PubMedCrossRef
23.
Webber EM, Morrison KB, Pritchard SL, Sorensen PH (1999) Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center. J Pediatr Surg 34:1641–1644PubMedCrossRef
24.
Kim DY, Kim KH, Jung SE et al (2002) Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg 37:1419–1423PubMedCrossRef
25.
Upadhyaya M, Mc Kiernan P, Hobin D et al (2010) Primary hepatic sarcomas in children-a-single-center ecperience over 19 years. J Pediatr Surg 45(11):2124h–2128hCrossRef
26.
Okajima H, Ohya Y, Lee KJ et al (2009) Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure. J Pediatr Surg 44:33e–38eCrossRef
27.
Dower NA, Smith LJ (2000) Liver transplantation for malignant liver tumors in children. Med Pediatr Oncol 34:136–140PubMedCrossRef
28.
Kalicinski P, Ismail H, Broniszczak D et al (2008) Non-resectable hepatic tumors in children- role of liver transplantation. Ann Transplant 13:37–41PubMed
Metadata
Title
Embryonal sarcoma of the liver
Authors
G. Chocarro
M. V. Amesty
F. Hernández
B. G. Goiboru Chenu
R. Ortíz
S. Hernández
A. Sánchez
M. Gámez
M. López Santamaría
J. A. Tovar
Publication date
01-12-2013
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 12/2013
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-013-3344-2

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