Korean J Hepatobiliary Pancreat Surg.  2016 Aug;20(3):144-147. 10.14701/kjhbps.2016.20.3.144.

Undifferentiated embryonal sarcoma of the liver in a young female: treatment with portal vein embolization and liver trisectonectomy

• Giakoustidis DE
• Gargavanis AA
• Katsiki ED
• Salveridis NT
• Antoniadis NA
• Papanikolaou V

• KMID: 2351303
• DOI: http://doi.org/10.14701/kjhbps.2016.20.3.144

Abstract

Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a tumor highly malignant, of mesenchymal origin. It is a rare finding in adults, though less rare in children. The strategy to be followed and the therapeutic targets to be reached for this tumor, in adult cases, remain ambiguous and controversial. Herein we report the case of a 29 year old female patient with a massive UESL and we describe our therapeutic approach. A 29 year-old female patient was referred to our center with severe intermittent epigastric pain and fever due to a voluminous liver tumor: Needle biopsy was of no specific findings and surgical excision was decided. Right portal vein embolization and selective embolization of the segment's IV branch was performed in order to achieve adequate future liver remnant (FLR). Right trisectonectomy was then performed, with uneventful post operative period and the patient was discharged at the 11th post operative day. UESL is a rare tumor that needs aggressive surgical approach and multidisciplinary team management is of paramount importance.

Keyword

Undifferentiated Embryonal Sarcoma; Portal embolization

MeSH Terms

Adult
Biopsy, Needle
Child
Female*
Fever
Humans
Liver*
Portal Vein*
Sarcoma*

Figure

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  Fig. 1 Computed tomography imaging before (A) and after (B) portal vein embolization.

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  Fig. 2 Microphotograph of the tumor showing pleiomorphic bizarre and giant tumor cells with numerous mitoses and eosinophilic globules.

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  Fig. 3 Microphotograph of the tumor showing spindle tumor cells arranged compactly in a fibrous stroma (HE ×40).

Reference

1. Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver: report of 31 cases. Cancer. 1978; 42:336–348. PMID: 208754.

2. 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. PMID: 18218141.

3. Cao Q, Ye Z, Chen S, Liu N, Li S, Liu F. Undifferentiated embryonal sarcoma of liver: a multi-institutional experience with 9 cases. Int J Clin Exp Pathol. 2014; 7:8647–8656. PMID: 25674229.

4. Gao J, Fei L, Li S, Cui K, Zhang J, Yu F, et al. Undifferentiated embryonal sarcoma of the liver in a child: a case report and review of the literature. Oncol Lett. 2013; 5:739–742. PMID: 23426588.

5. Xu HF, Mao YL, Du SD, Chi TY, Lu X, Yang ZY, et al. Adult primary undifferentiated embryonal sarcoma of the liver: a case report. Chin Med J (Engl). 2010; 123:250–252. PMID: 20137381.

6. 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. PMID: 11815984.

7. 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; 37:1419–1423. PMID: 12378446.

8. Lenze F, Birkfellner T, Lenz P, Hussein K, Länger F, Kreipe H, et al. Undifferentiated embryonal sarcoma of the liver in adults. Cancer. 2008; 112:2274–2282. PMID: 18361435.

9. 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. PMID: 16259353.

10. García-Bonafé M, Allende H, Fantova MJ, Tarragona J. Fine needle aspiration cytology of undifferentiated (embryonal) sarcoma of the liver. A case report. Acta Cytol. 1997; 41(4 Suppl):1273–1278. PMID: 9990257.

11. Xie ZY, Li LP, Wu WJ, Sun DY, Zhou MH, Zhao YG. Undifferentiated embryonal sarcoma of the liver mistaken for hepatic abscess in an adult. Oncol Lett. 2014; 8:1184–1186. PMID: 25120683.

12. Ismail H, Dembowska-Bagińska B, Broniszczak D, Kaliciński P, Maruszewski P, Kluge P, et al. Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience. J Pediatr Surg. 2013; 48:2202–2206. PMID: 24210186.

13. Dai CL, Xu F, Shu H, Xu YQ, Huang Y. Undifferentiated (embryonal) sarcoma of liver in adult: a case report. World J Gastroenterol. 2005; 11:926–929. PMID: 15682496.

14. Ma L, Liu YP, Geng CZ, Tian ZH, Wu GX, Wang XL. Undifferentiated embryonal sarcoma of liver in an old female: case report and review of the literature. World J Gastroenterol. 2008; 14:7267–7270. PMID: 19084947.

15. Li XW, Gong SJ, Song WH, Zhu JJ, Pan CH, Wu MC, et al. Undifferentiated liver embryonal sarcoma in adults: a report of four cases and literature review. World J Gastroenterol. 2010; 16:4725–4732. PMID: 20872975.