Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 9  |  Issue : 3  |  Page : 139-141
Undifferentiated (embryonal) sarcoma of the liver: Case report and review of the literature


1 Department of General Surgery, Razi Hospital, P.O. Box 41448-95655, Rasht, Iran
2 Department of Pediatric Oncology, Razi Hospital, P.O. Box 41448-95655, Rasht, Iran
3 Department of Pathology, Razi Hospital, P.O. Box 41448-95655, Rasht, Iran
4 Department of Pathobiology Laboratory, Razi Hospital, P.O. Box 41448-95655, Rasht, Iran

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Date of Submission26-Jan-2003
Date of Acceptance13-May-2003
 

How to cite this article:
Aghajanzadeh M, Riazi H, Kohsari MR, Jafroodi M, Hoda S, Ashtiani MN. Undifferentiated (embryonal) sarcoma of the liver: Case report and review of the literature. Saudi J Gastroenterol 2003;9:139-41

How to cite this URL:
Aghajanzadeh M, Riazi H, Kohsari MR, Jafroodi M, Hoda S, Ashtiani MN. Undifferentiated (embryonal) sarcoma of the liver: Case report and review of the literature. Saudi J Gastroenterol [serial online] 2003 [cited 2020 Oct 23];9:139-41. Available from: https://www.saudijgastro.com/text.asp?2003/9/3/139/33356


Primary hepatic tumors are rare, but unfortunately two-thirds of them are malignant, representing the third most common abdominal cancer encountered in pediatric practice, after Wilms' tumor and neuroblastoma [1],[2] . Undifferentiated (embryonal) sarcoma of liver, which has also been termed "mesenchymal sarcoma", "malignant mesenchynoma", "primary sarcoma of the liver" and "fibromyxosarcoma" is uncommon and typically occurs during the first two decades of life [1],[2] . It is probably the third most common primary malignant hepatic tumor of children, after hepatoblastoma and hepatocellular carcinoma [2],[3] . The typical presentation is that of an abdominal mass and pain but fever is a variable feature [4],[5] . The treatment for the embryonal sarcoma of the liver is surgical resection, with adjuvant chemotherapy and possibly radiation therapy. The prognosis is generally poor [6],[7],[8],[9],[10] .


   Case Report Top


A 14-year-old girl was admitted to the hospital because of the increasing fatigue and right upper quadrant pain of three months duration. Her pain gradually increased and radiated to the right shoulder. On physical examination, the abdomen was soft and flat and the liver edge descended 6cm below the right costal margin. She hadn't any other symptom or sign. Liver function tests and CBC were within normal limits. Chest x-ray revealed slight elevation of the right hemidiaphragm, without pleural effusions. Ultrasonographic examination of the upper portion of the abdomen showed a non­homogenous mass that involved most of the right hepatic lobe, multiple cystic components with septa were seen. Later on, complement fixation test for ecchinococcus granulosus was carried out and found to be negative. Computerized tomography scan of abdomen revealed a large well-circumscribed mass with both cystic and solid components that occupied a large portion of the right hepatic lobe. At celiotomy, a large liver mass in the right lobe was found. Aspiration of the mass revealed gross bloody fluid. An incisional biopsy of the mass was performed and the abdomen was closed. Histological examination revealed undifferentiated (embryonal) sarcoma of the liver and the immunohistochemical study confirmed the diagnosis. Later on, a right hepatic lobectomy was performed through a right thoracoabdominal approach. The patient's postoperative course was uneventful and was discharged from the hospital two weeks after the operation. Thereafter she was referred for adjuvant chemotherapy. Twenty-four months after surgery and chemotherapy, she died because of intraperitoneal recurrence and dissemination of the tumor.


   Discussion Top


Undifferentiated sarcoma of the liver is very rare and usually occur in children during the first two decades of life [1],[2],[11],[12],[13],[14],[15],[16] . In the largest published series to date, Stocker and Ishak found that in 16 of their 31 cases the patients were between 6-10 years of age, although the tumor may occur in adults [1] . There does not seem to be a sex predominance in contrast to other hepatic cancers of childhood [4],[5],[15],[16] .

Embryonal sarcoma of the liver is a rapidly growing malignant tumor [17] . It usually presents with abdominal mass and/or abdominal pain. Other complaints include fever, weight loss, anorexia malaise, lethargy, and nausea and vomiting [12],[15],[16],[17] . Hemorrhage into or rupture of the tumor occurs occasionally [12] . Dyspnea from tumor extending into the right atrium via the inferior vena cava has occurred [17],[18] . The tumor is usually a single large, variably hemorrhagic, necrotic or cystic mass in the right lobe of the liver. Imaging studies (sonography and computerized tomography scan) demonstrate somewhat heterogeneous appearances, ranging from cystic tissue with multiple septa to more solid tissue reflecting the extent of necrosis and cyst formation [4],[5] . Serological investigations and markers are usually negative or unhelpful in identifying the specific nature of the tumor [12],[17] . A nonspecific defect may be seen on an isotope liver scan and on angiographic examination the tumor may appear hypervascular, hypovascular or avascular [4],[12]. Periodic-acid schiff (PAS-positive) intracytoplasmic pink globules are present in the majority of the cases [1],[19],[20] . On immunohistochemical examination the tumor cells are positive for vimentin and some of the cells particularly the larger cells are positive for alpha l-antitrypsin and alpha antichymotrypsin [4],[14],[17],[21] . Unfortunately the tumor cells have characteristics of both fibroblasts and histiocystes and thus resemble the cells of pleomorphic malignant fibrous histiocytomas [19] . There are reports of smooth-muscle [22] or lipoblastic differentiation in some of these tumors [23],[24] . The histogenesis of the undifferentiated sarcoma of the liver remains unresolved [25],[26],[27] . The tumor rarely metastasizes but spreads by direct extension into adjacent organs and sometimes extends into the right atrium via the inferior vena cava. Rupture of the tumor can occur and massive intraperitoneal spread has sometimes been found [17],[18] . The metastases that have been reported were principally in the lung, bone, pleura and peritoneum [2],[3],[7],[24],[25],[26].

The treatment for embryonal sarcoma is surgical resection. Without the surgical resection the mean survival period is less than one year and mortality is greater than 80% [6],[7],[8],[9],[17],[18] . The lesions seldom are resectable and treatment with radiotherapy and chemotherapy have produced disappointing results [10],[11],[12] . The prognosis is generally poor although there have been some reports of five-year survival and local growth is a problem, particularly in cases in which the tumor have been incompletely resected [6],[7],[8],[9],[10].

 
   References Top

1.Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver: Report of 31 cases. Cancer 1987; 42: 336-48.  Back to cited text no. 1    
2.Reynolds M. Pediatric liver tumors. Surg Oncol 1999;16:159.  Back to cited text no. 2    
3.Weinberg AG, Finegold MJ. Primary hepatic tumors of childhood. Hum Pathol 1983: 14: 512-37.  Back to cited text no. 3    
4.Ros PR, Olmsted WW, Dachman AH, Goodman ZD, Ishak KG, Hartman DS. Undifferentiated (embryonal) sarcoma of the liver: Radiologic-pathologic correlation. Radiology 1986; 161: 141-5.  Back to cited text no. 4    
5.Verness M, Collier NA, Mutum SS, Crofton ME. Misleading appearance of a rare malignant liver tumor on computed tomography. Br J Radiol 1984; 57: 262-5.  Back to cited text no. 5    
6.Harris MB, Shen S,Weiner MA, et al. Treatment of primary undifferentiated sarcoma of the liver with surgery and chemotherapy. Cancer 1984; 54: 2859-62.  Back to cited text no. 6    
7.Horowitz ME, Etcubanas E, Webber BL, et al. Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children: Results of therapy. Cancer 1987; 73: 396-402.  Back to cited text no. 7    
8.Perilongo G, Garli M, Sainati L, et al. Undifferentiated (embryonal) sarcoma of the liver in the childhood: Results of A retrospective Italian study. Tumori 1987; 73: 213-7.  Back to cited text no. 8    
9.Ware R, Friedman HS, Filston, et al. Childhood hepatic mesenchynoma: Successful treatment with surgery and multiple-agent chemotherapy. Med Pediatr Oncol 1988; 16: 62-5.  Back to cited text no. 9    
10.Curley S, Izzo F, Delrio P, et al. Radiofrequency ablation of unresectable primary and metastatic hepatic malignancies: Results in 123 patients. Ann Surg 1999; 230: 1.  Back to cited text no. 10    
11.Brian I Carr, John C, Flickinger, Michael T, Lotze. Hepatobiliary cancers In: Vincent T. Devita Jr. Samuel Hellman, Steven A. Rosenberg; Cancer principles and practice of oncology, 5 th edition, 1997: 1108.  Back to cited text no. 11    
12.Michael C, Kew DC. Tumors of the liver In: David Zakin and Thomas D Boyer. Hepatology; A textbook in liver disease, 2"d edition, 1990: 1228.  Back to cited text no. 12    
13.Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Ann Surg 1955; 141: 246.  Back to cited text no. 13    
14.Leuschner I, Schmidt D, Harms D. Undifferentiated sarcoma of the liver in childhood: Morphology, flow cytometry and literature review. Hum pathol 1990; 21: 68.  Back to cited text no. 14    
15.Ming-Hsien Wu, Cheng-Chung Wu, Po-Cheung Kewan. Undifferentiated sarcoma of the liver in an adult: A case report. Formosan J Surg 2001; 34: 138-­42.  Back to cited text no. 15    
16.Padilla-Rodriguez A, Hermandez-Gonzalez M, Alcantara-Uazquez A. Undifferentiated sarcoma of the liver: Report of a case in an adult. Rev Med Hosp Gen Mex 2002; 65: 168-72.  Back to cited text no. 16    
17.Wright TL, Venook AP, Millward-Sadler GH. Hepatic tumors. In: Millward-Sadler, Wright and Arthur. Wright's Liver and Biliary disease, 1992; 3: 1104.  Back to cited text no. 17    
18.Lack EE, Schloo BL, Azumi N, et al. Undifferentiated (embryonal) sarcoma of the liver. Clinical and pathologic study of 16 cases with emphasis on immunohistochemical feature. Am J Surgical Histopathology 1991; 15: 1-16.  Back to cited text no. 18    
19.Keating S, Taylor GP. Undifferentiated (embryonal) sarcoma of the liver: Ultrastructural and immunohistochemical similarities with malignant fibrous histiocytoma. Hum Pathol 1985; 16: 693-9.  Back to cited text no. 19    
20.Abramowsky CR. Cebelin M, Choudhury A, Izant RJ Jr. Undifferentiated (embryonal) sarcoma of the liver with alpha, antitrypsin deposits; immunohistochemical and ultrastructural studies. Cancer 1980; 45: 3108-13.  Back to cited text no. 20    
21.Miettinen 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.  Back to cited text no. 21    
22.Gonzalez-Crussi F. Undifferentiated (embryonal) liver sarcoma of childhood: Evidence of leiomyoblastic differentiation. Pediatr Pathol 1983; 1: 281-90.  Back to cited text no. 22    
23.Cozzutto C, De Bernardi B, Comelli A, Soave F. Malignant mesenchynoma of the liver in children: A clinicopathologic and ultrastructural study. Hum Pathol 1981; 12: 481-5.  Back to cited text no. 23    
24.Gallivan MV, Lack EE, Chun B, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Ultrastructure of a case presenting as a primary intracardiac tumor. Pediatr Pathol 1983; 1: 291-300.  Back to cited text no. 24    
25.Stanly RJ, Dehner LP, Hesker AE. Primary malignant mesenchymal tumors (mesenchynoma) of the liver in childhood: An angiographic-pathologic study of three cases. Cancer 1973; 32: 973-84.  Back to cited text no. 25    
26.Donnelly WH, Talbert JL, Miale T. Malignant undifferentiated stromal tumor of liver (mesenchynoma): An ultrastructural study. Lab Invest 1987; 38: 385-6.  Back to cited text no. 26    
27.Pieterse AS, Smith M, Smith LA, Smith P. Embryonal (undifferentiated) sarcoma of the liver: Fine-needle aspiration cytology and ultrastructural findings. Arch Pathol Lab Med 1983; 109: 677-80.  Back to cited text no. 27    

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Correspondence Address:
Manochehr Aghajanzadeh
Department of General Surgery, Razi Hospital, P.O. Box 41448-95655, Rasht,
Iran
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Source of Support: None, Conflict of Interest: None


PMID: 19861818

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