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Primary pleomorphic liver liposarcoma: A case series and literature review
Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):395-400
Published online August 31, 2021;
Copyright © 2021 The Korean Association of Hepato-Biliary-Pancreatic Surgery.

Chuah Jun Sen1,2, Siaw Jia Yng3, Soon Koon Choon3, Fatin Izni Zamri4

1Department of General Surgery, Pusat Perubatan Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia,
2Department of General Surgery, Hospital Sultanah Aminah, Ministry of Health Malaysia, Johor Bahru, Malaysia,
3Department of General Surgery, Sarawak General Hospital, Ministry of Health Malaysia, Kuching, Malaysia,
4Department of Pathology, Sarawak General Hospital, Ministry of Health Malaysia, Kuching, Malaysia
Correspondence to: Chuah Jun Sen
Department of General Surgery, Hospital Sultanah Aminah, Ministry of Health Malaysia, Jalan Persiaran Abu Bakar Sultan, Johor Bahru 80100, Johor, Malaysia
Tel: +60-16-7727965, Fax: +60-7-2231666, E-mail:
Received February 22, 2021; Revised March 8, 2021; Accepted March 10, 2021.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Primary hepatic liposarcoma is an extremely rare mesenchymal tumor that accounts for only 0.1% to 2% of primary malignant liver tumors. Due to its rarity, there is a lack of knowledge about its clinical course, management, and prognosis. Only 15 cases of primary liposarcoma of the liver have been reported since 1973. Among these 15 cases, only two involved primary liver liposarcoma with a pleomorphic subtype. Here we report the third and fourth cases of primary pleomorphic liver liposarcoma. A 57-year-old female presented with abdominal discomfort and progressive abdominal distension for two weeks. Computed tomography (CT) of her abdomen revealed a large well-defined solid nodule mass with an area of necrosis and hemorrhage occupying segment IV-B of the liver. Wide local excision was performed. She had an uneventful recovery and remained well at six months post-treatment. A 65-year-old male presented with an abdominal mass for two-month. CT demonstrated a mass in the left lobe of the liver with mixed soft tissues and fat attenuation. He underwent wide local excision. He was discharged on day three postoperatively. Histological analysis for both cases revealed liposarcoma of the liver with a pleomorphic subtype.
Keywords : Liver neoplasm; Liposarcoma

Primary hepatic liposarcoma is extremely rare, accounting for only 0.1% to 2% of primary malignant liver tumors [1]. Liposarcoma originating from the mesenchymal tissue is typically found in the shoulder, extremities, and the retroperitoneum space [2]. Primary hepatic liposarcoma can be divided into five subtypes (myxoid, well-differentiated, dedifferentiated, pleomorphic, and myxoid pleomorphic), with pleomorphic liposarcoma being the rarest subtype [3]. The clinical presentation of primary hepatic liposarcoma is highly variable. Early diagnosis of liver liposarcoma is challenging because most patients remain asymptomatic until mass effects such as abdominal pain, abdominal distention, or obstructive jaundice symptoms emerge. Some might experience fever, nausea, vomiting, and weight loss. Physical examination might reveal a palpable abdominal mass. A computed tomography (CT) scan may provide certain typical characteristics of liver liposarcoma, making it the best tool to evaluate the resectability of liver sarcoma. Complete resection with clear margin is likely a curative therapy. To the best of our knowledge, only two cases of primary pleomorphic liver liposarcoma have been reported in the literature. Here, we present two more cases of primary pleomorphic liver liposarcoma with successful curative resection at our center. Written informed consent was obtained from both patients for publication of this case report and accompanying images.


Case 1

A 57-year-old female presented with abdominal discomfort and progressive abdominal distension for two weeks. She denied nausea, vomiting, jaundice, reduced effort intolerance, and constitutional symptoms. She had no risk factors such as drug abuse, alcoholism, or viral infection. She had no significant medical or surgical history. Physical examination revealed a palpable large mass that occupied the right upper quadrant of the abdomen. There was no stigmata of a chronic liver disease such as edema, ascites, splenomegaly, or spider naevi. All blood investigation results were normal. Viral markers of hepatitis B and hepatitis C were negative. Both serum tumor markers alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) were normal. A CT scan of the abdomen revealed a large well-defined predominantly solid nodule mass (11 cm × 7 cm × 9.8 cm) with an area of necrosis and hemorrhage occupying segment IV-B of the liver (Fig. 1). The mass displaced the second part of the duodenum, the head of the pancreas, and the pylorus. No other abnormality was detected in the abdominal viscera. Further investigation and workup showed no other primary lesions.

Fig 1. Axial (A) and coronal (B) computed tomographyof the abdomen showing a well-defined predominantly solid mass with area of necrosis and hemorrhage at segment IV of liver.

An elective laparotomy and wide local excision of the tumor was scheduled. Intraoperatively, we found a huge lipomatic liver mass (12 cm × 11 cm) arising from segment IV-B, extending laterally into segments 5 and 6. The liver was smooth and non-cirrhotic. A macroscopic examination revealed a well-circumscribed and an encapsulated fleshy, pale yellowish tumor. A bi-valved section of the specimen appeared brownish and gelatinous with haemorrhage foci (Fig. 2).

Fig 2. (A) Well circumscribed and encapsulated fleshy pale yellowish tumour. (B) Bivalved section of specimen appearing brownish gelatinous with haemorrhagic foci.

Microscopically, the mass showed heterogeneous histology composed of neoplastic spindled to markedly pleomorphic cells with a variable loose myxoid background. Furthermore, the tumor comprised large cells with markedly pleomorphic vesicular nuclei and moderate-to-ample amounts of variably eosinophilic cytoplasm. There were numerous bizarre tumor giant cells. Several areas with spindled cells arranged in short fascicles were found. Numerous interspersed uni-vacuolated and multi-vacuolated variable pleomorphic lipoblasts were identified. Mitosis was easily seen. Intermittent large foci of coagulative necrosis were noted. Lymphovascular permeation was also observed. However, well-differentiated liposarcomatous areas were not seen. Immunohistochemical staining was performed. The tumor exhibited diffuse Vimentin positively. The resection margin was clear. The histological diagnosis was pleomorphic hepatic liposarcoma (Fig. 3). There were no intra- or postoperative complications. She made an uneventful recovery. She was discharged on postoperative day three. A CT scan of the abdomen at six months postoperatively showed no disease recurrence.

Fig 3. (A) Sheets of pleomorphic tumour cells (H&E, ×40). (B) Multiple bizarre tumour giant cells admixed with lipoblasts (blue arrows) are seen (H&E, ×200). (C) Tumour cells are diffusely positive for Vimentin (×200). (D) S100 stain highlighting lipoblasts (×400).

Case 2

A 65-year-old male without significant past medical or surgical history presented with an abdominal mass for two-month with losses of weight and appetite. He had no risk factors such as drug abuse, alcoholism, or viral infection. A physical examination showed a palpable huge mass in the epigastric region. A CT scan demonstrated a mass in the left lobe of the liver with mixed soft tissues and fat attenuation (Fig. 4). He then underwent a laparotomy and tumor excision. Intraoperatively, there was a huge mass (approximately 15 cm × 15 cm) arising from the undersurface of segment III of the liver (Fig. 5). Otherwise, the rest of the liver was grossly normal. No ascites was noted. Microscopically, the tumor showed a picture of pleomorphic liposarcoma with the presence of pleomorphic malignant cells, epitheloid cells, and central necrosis (about 25% of the tumor mass). The resection margin was clear. Postoperative recovery was uneventful. He was discharged on day three postoperatively. No evidence of primary liposarcoma was found in further history or subsequent investigation. He was lost to a scheduled three-month follow-up postoperatively.

Fig 4. Abdomen computed tomography (A, B) showing a large mass at the left lobe liver with mixed soft tissues and fat attenuation.

Fig 5. Gross specimen of the tumour.

Liposarcoma is one of common soft tissue malignancies that occur in the extremities and visceral areas, particularly in the retroperitoneum. Metastatic spread of soft tissue liposarcomas is relatively common, with liver involvement in 10% of cases [2]. However, primary liver liposarcoma is extremely rare, accounting for only 0.1% to 2% of primary malignant liver tumors. Classification of liposarcoma is based on a combination of two basic histological aspects of the tumor: (1) the stage of lipoblast differentiation based on relative amounts of lipids in the cells and myxoid materials in extracellular spaces; and (2) the overall degree of cellularity and cellular pleomorphism. There are five recognized subtypes: myxoid, well differentiated, dedifferentiated, pleomorphic, and myxoid pleomorphic [3]. To date, only 15 cases of primary liver liposarcoma have been reported in the literature (Table 1). Hence, our knowledge towards this disease remains scarce. Apparently, its long-term prognosis and survival are poor. Among reported cases (Table 1), there was no sex (9 females: 7 males) predilection. It appearred to be more common at the left lobe of the liver. The age range was between 2 and 86 years old, with a median age of 54 years old. The myxoid subtype appeared to be the commonest.

Table 1 . Characteristics of previously reported cases of primary liposarcoma of the liver

NoCase sourceAge (yr) /sexLocationWHO classificationPresentationManagementOutcome
1Kuo et al. [4]61/FRight lobeMyxoidFever, nausea, vomiting, jaundice, tea coloured urine and loss of weightRight hemihepatectomy5 mon survival with recurrence
2Naik et al. [5]42/MLeft lobePleomorphicAbdominal pain, loss of weight and palpable massExtended left lateral hepatectomy-
3Wolloch et al. [8]22/FRight lobe--Right lobectomySurvival 46 days
4Kim and Reyes [9]86/MRight lobeMyxoidRight sided abdominal pain, dyspnea, cough and right shoulder pain for 1 yrSupportive treatmentDied rapidly
5Kim et al. [10]30/FLeft lobeDedifferentiatedAbdominal pain and dyspnea for 4 monLeft lateral subsegmentectomy10 mon disease free
6Soares et al. [11]2/MHilum-Fever, jaundice, choluria, abdominal mass and anorexia for 1 monLaparotomy biopsy, and chemotherapy5 mon survival
7Wright et al. [12]3/MHilumMyxoidRight upper abdominal massWide local excisionDisease free 12 yr. Recurrence.
8Aribal and Berberoglu [13]48/FHilumMyxoidRight abdominal painLaparotomy biopsy, and chemotherapy-
9Nelson et al. [14]54/FRight and left lobeMyxoidAbdominal distension, pain, weight loss, nausea and vomiting for 1 yrLaparotomy and biopsyPost surgery bleeding and death
10Kim et al. [15]63/FLeft lobeWell differentiatedAbnormal serum liver enzymeLeft hemihepatectomy-
11Nakhai and Motabar [16]21/FRight lobe-Fever, dyspnea and abdominal pain for 3 monRight hemihepatectomy and chemotherapy9 mon survival
12Lin et al. [17]64/MLeft lobeMyxoidLeg pain for 1 monLeft hemihepatectomy4 yr disease free
13Binesh et al. [18]83/FLeft lobeMyxoidNausea, vomiting, palpable painful epigastric mass, loss of weight for 6 monTumour resection and chemotherapy19 mon disease free. Recurrence
14Chen et al. [19]43/MLeft lobePleomorphicAbdominal distention, pain, nausea, vomiting, weight loss for 1 yrLeft hemihepatectomy9 mon disease free. Recurrence
15Rayya [20]59/MRight lobeMyxoidNonspecific right hypochondrium pain, weight loss for 9 monLateral partial right hepatectomyMore than 3 mon survival
16Our 1st case57/FLeft lobePleomorphicAbdominal discomfort and distention for 2 wkWide local excisionMore than 6 mon survival (remains disease free)
17Our 2nd case65/MLeft lobePleomorphicAbdominal mass, weight loss for 2 monWide local excisionMore than 3 mon survival

WHO, World Health Organization; F, female; M, male; -, not available.

Early diagnosis of primary liver liposarcoma is difficult because a tumor in the liver is usually undetected unless it is large. In addition, its symptoms are non-specific (Table 1). Most symptoms are due to the mass effect of the tumor on nerves, vessels, the biliary tract, and intestines [4]. Common symptoms reported are fever, nausea, vomiting, loss of weight, jaundice, abdominal distention, abdominal pain, and palpable abdominal mass. In our case series, both patients presented with abdominal distention and a palpable abdominal mass. However, there was no obstructive jaundice symptom because the tumor was confined to the liver lobe without having any compressive effect on the biliary duct.

A CT scan of the abdomen is the imaging modality of choice for identifying certain typical characteristics of liposarcoma [4-6] and for evaluating the resectablitiy of liver liposarcoma prior to surgery. It remains a challenge to differentiate liposarcoma from other liver diseases due to their varied histological compositions including areas of necrosis and hemorrhage.

For well-differentiated liposarcoma, it has a predominantly adipose mass containing non-lipomatous components. The fat component is usually > 75%, while non-lipomatous components are most often seen as prominent thick septa (> 2 mm) with or without nodularity. There might be a presence of focal nodular or globular non-adipose area [6]. For dedifferentiating liposarcoma, it has features similar to those of well-differentiated liposarcoma. However, it has a focal, nodular non-lipomatous region over 1 cm in size. Myxoid liposarcoma may appear normal or as a non-specific soft tissue mass. It has less calcification and radiolucent fat. The fat component is usually < 10% of the lesion. It is often seen in the septa (lacy or linear pattern) or as subtle small nodules in the lesion [6].

Pleomorphic liposarcoma is typically presented as a non-specific soft tissue mass. It is usually associated with an area of necrosis and hemorrhage, accounting for the prominent heterogenecity seen in these lesions (similar to the one in our 1st case). Furthermore, it seldom contains adipose tissues, reflecting a higher degree of anaplasia of the lesion [6]. Both our cases involved pleomorphic type of hepatic liposarcoma, although their CT features were entirely different. The 1st case demonstrated a predominantly solid nodule mass with an area of necrosis and hemorrhage, while the 2nd case showed a mixed soft tissue and fat mass. These non-specific features of pleomorphic liposarcoma can make imaging diagnosis difficult. Tumor markers (such as AFP, CEA, or cancer antigen 19-9) were not increased. Liposarcoma should be one of our differential diagnoses when dealing with a liver mass.

Based on the literature review, the mainstay of treatment is curative surgical resection with clear margins [7]. However, the disease is accompanied by a high rate of tumor recurrence with poor prognosis [4,5,8-20]. Palliative treatment should be offered for those with advanced liposarcoma and metastases. The role of chemotherapy and radiotherapy in hepatic liposarcoma remains less defined with a lack of strong evidence. Among the 15 cases reported, only four of them [11,13,16,18] underwent chemotherapy post-surgical resection, with survival duration ranging from 5 to 19 months. Our patient in the 1st case is disease free currently at six months post-surgical resection of the hepatic pleomorphic liposarcoma. She is still on a regular follow up schedule. However, the patient in the 2nd case had lost his follow up at three months post-surgery. Table 1 shows outcomes of all reported primary hepatic liposarcoma cases in the literature.

In conclusion, our two cases highlight our experience on the clinical course of primary hepatic liposarcoma which is extremely rare. Although there is no clear consensus, surgical resection with clear margin remains to be the mainstay of treatment. Radiotherapy and chemotherapy in hepatic liposarcoma remain uncertain. They might play a role in potentially achieving a long-term survival. Additional strong evidence is required. Based on cases reported in the literature, prognosis of this disease is poor, showing a high recurrence rate.


The authors would like to thank Dr. Tan Jih Huei for contributing his ideas to this study.


No potential conflict of interest relevant to this article was reported.


Conceptualization: CJS, SJY, SKC. Data curation: All authors. Methodology: CJS, SJY. Visualization: CJS, SJY, FIZ. Writing - original draft: CJS. Writing - review & editing: All authors.

  1. Liver Cancer Study Group of Japan. Primary liver cancer in Japan. Clinicopathologic features and results of surgical treatment. Ann Surg 1990;211:277-287.
    Pubmed KoreaMed
  2. Teas S, Ronan SG, Ghosh L. Solitary metastatic liposarcoma of the liver. Arch Pathol Lab Med 1978;102:605.
  3. WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. 5th ed. Lyon: International Agency for Research on Cancer, 2020.
  4. Kuo LM, Chou HS, Chan KM, Yu MC, Lee WC. A case of huge primary liposarcoma in the liver. World J Gastroenterol 2006;12:1157-1159.
    Pubmed KoreaMed CrossRef
  5. Naik PR, Kumar P, Kumar PV. Primary pleomorphic liposarcoma of liver: a case report and review of the literature. Case Reports Hepatol 2013;2013:398910.
    Pubmed KoreaMed CrossRef
  6. Murphey MD, Arcara LK, Fanburg-Smith J. From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. Radiographics 2005;25:1371-1395.
    Pubmed CrossRef
  7. Nassif NA, Tseng W, Borges C, Chen P, Eisenberg B. Recent advances in the management of liposarcoma. F1000Res 2016;5:2907.
    Pubmed KoreaMed CrossRef
  8. Wolloch Y, Dintsman M, Garti I. Primary malignant tumors of the liver. Isr J Med Sci 1973;9:6-11.
  9. Kim TW, Reyes CV. Myxoid liposarcoma mimicking fluid density. J Surg Oncol 1985;30:80-82.
    Pubmed CrossRef
  10. Kim YI, Yu ES, Lee KW, Park EU, Song HG. Dedifferentiated liposarcoma of the liver. Cancer 1987;60:2785-2790.
    Pubmed CrossRef
  11. Soares FA, Landell GA, Peres LC, Oliveira MA, Vicente YA, Tone LG. Liposarcoma of hepatic hilum in childhood: report of a case and review of the literature. Med Pediatr Oncol 1989;17:239-243.
    Pubmed CrossRef
  12. Wright NB, Skinner R, Lee RE, Craft AW. Myxoid liposarcoma of the porta hepatis in childhood. Pediatr Radiol 1993;23:620-621.
    Pubmed CrossRef
  13. Aribal E, Berberoglu L. Primary liposarcoma of the liver. AJR Am J Roentgenol 1993;161:1331-1332.
    Pubmed CrossRef
  14. Nelson V, Fernandes NF, Woolf GM, Geller SA, Petrovic LM. Primary liposarcoma of the liver: a case report and review of literature. Arch Pathol Lab Med 2001;125:410-412.
    Pubmed CrossRef
  15. Kim JL, Woo JY, Lee MJ, Kim KR, Jung JP, Lee NJ, et al. Imaging findings of primary well-differentiated liposarcoma of the liver: a case report. Acta Radiol 2007;48:1061-1065.
    Pubmed CrossRef
  16. Nakhai B, Motabar AR. Primary liposarcoma of the liver: a case report and review of the literature. Med J Islam Repub Iran 2007;21:167-172.
  17. Lin YC, Tsai WC, Liu YC, Yu CP. Primary liposarcoma of liver: a case report and literature review. J Med Sci 2011;31:81-84.
  18. Binesh F, Akhavan A, Kargar S, Navabii H. Primary liposarcoma of liver: a rare case and literature review. BMJ Case Rep 2012;2012:bcr0120125711.
    Pubmed KoreaMed CrossRef
  19. Chen L, Luo J, Wen Q, Chu S, Wang W, Fan S. Primary pleomorphic liposarcoma of liver: a case report and review of the literature. Int J Clin Exp Pathol 2016;9:7629-7633.
    Pubmed KoreaMed CrossRef
  20. Rayya F. Resection of a rare hepatic myxoid liposarcoma: case report. Clin Surg 2017;2:1310.


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