Asian Journal of Surgery (2013) 36, 174e178

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CASE REPORT

Undifferentiated embryonal sarcoma of theliver with focal osteoid picturedA casereport

Jian-Han Chen a, Cheng-Hung Lee a, Chang-Kuo Wei a,Shu-Mei Chang b, Wen-Yao Yin a,c,*

aDepartment of Surgery, Buddhist Dalin Tzu Chi General Hospital, Chiayi, Taiwan, ROCbDepartment of Pathology, Buddhist Dalin Tzu Chi General Hospital, Chiayi, Taiwan, ROCcDepartment of Surgery, College of Medicine, Tzu Chi University, Hualien, Taiwan, ROC

Received 27 December 2011; received in revised form 30 April 2012; accepted 31 May 2012Available online 25 August 2012

KEYWORDSfocal osteoid picture;liver cystic tumor;malignantmesenchymoma;

old age adult;undifferentiatedembryonal sarcomaof liver

* Corresponding author. DepartmentCounty 622, Taiwan, ROC.

E-mail address: yaowen@mail.tcu.

1015-9584/$36 Copyright ª 2012, Asiahttp://dx.doi.org/10.1016/j.asjsur.20

Summary Undifferentiated embryonal sarcoma of the liver (UESL) is a rare primary livertumor. Less than 100 adult cases were reported. It has female and right lobe preponderance.In pathological features, focal osteoid picture in UESL is never reported. We present a 63-year-old male patient with left lobe UESL with focal osteoid picture. He was admitted for a palpablesolid mass, with left upper quadrant abdominal pain for 4 months. Abdominal computedtomography showedahugewell-circumscribedmass at left upper quadrant, 21.3� 13� 27.9 cm3

in size, with multiple septa in delayed phase. En bloc resection including lateral segmentect-omy, splenectomy, and cholecystectomy were performed, but tumor rupture was noted. Thepathologic diagnosis was ruptured UESL. The postoperative course was uneventful, and adju-vant radiotherapy without chemotherapy was performed. Peritoneal seeding with massiveascites was noted in the 9th month after operation. Even after receiving salvage chemotherapy,he died 1 year after operation. Early complete surgical resection with adjuvant chemotherapymay improve prognosis of UESL. But the overall survival of UESL did not improve until recently.We present this case along with a literature review of the clinical pictures, diagnosis,pathology presentation, pathologicogenesis of focal osteoid picture, treatment, and prognosisfor UESL of another 23 new reported cases since 2007.Copyright ª 2012, Asian Surgical Association. Published by Elsevier Taiwan LLC. All rightsreserved.

of Surgery, Buddhist Dalin Tzu Chi General Hospital, No. 2, Minsheng Road, Dalin Township, Chiayi

edu.tw (W.-Y. Yin).

n Surgical Association. Published by Elsevier Taiwan LLC. All rights reserved.12.06.012

Undifferentiated embryonal sarcoma of liver 175

1. Introduction resection, including left lateral segmentectomy, splenec-

Undifferentiated embryonal sarcoma of the liver (UESL)was first described in 1946 as a mesenchymoma, and wassubsequently named “malignant mesenchymoma” by Stout.It was recognized as a unique clinicopathologic entity byStocker and Ishak in 1978. It represents only approximately0.2% of all primary liver tumor.1 The peak age of UESL isbetween 5 and 10 years. Less than 100 adult cases werereported. Adult cases over the age of 60 years are quiteexceptional. Otherwise, it has a female preponderance,and the majority of tumor locations reported were in theright lobe. Only 22% UESL arise in the left lobe. To the bestof our knowledge, there is no report about focal osteoidpicture in UESL in pathology features. We present ourexperience of treating a 63-year-old male with left lobeUESL with focal osteoid picture. This report is presentedalong with a literature review of the clinical pictures,diagnosis, pathology presentation, treatment, and prog-nosis for UESL.

2. Case report

A 63-year-old male patient was admitted to our hospitaldue to a palpable solid mass with left upper quadrantabdominal pain for 4 months. He has no past history ofHepatitis B virus (HBV) or Hepatitis C virus (HCV) infection.Otherwise, he lost 10 kg of body weight within 8 months.Laboratory studies showed no elevation of liver function,alkaline phosphatase, and bilirubin. Abdominal ultraso-nography showed a heterogeneous mass in the intra-abdomen, which was about 10 cm in diameter, witha peripheral cystic-like component near there about 13 cmin diameter. Abdominal computed tomography (CT) showeda huge well-circumscribed mass at left upper quadrant, upto 21.3 � 13 � 27.9 cm3 in size, seemed to have arisen fromthe gastric fundus (Fig. 1A and B), Much inner nonenhancinglow-density collection, with enhancing soft parts, punc-tuate calcifications, and multiple septum in delayed phasewere noted (Fig. 1C). It also exerts mass effect over thegastric body, liver, spleen, and pancreas. Surgical resectionwas recommended and accepted. During operation, a huge,pedunculated, and cystic liver tumor originating frominferior surface of left lateral segment was noted. Thetumor was tightly adhesive to spleen, with multiple foci ofrupture and some attached omental soft tissue. En bloc

Figure 1 (A and B) Abdominal computed tomography before suquadrant which is attached to the spleen and pancreas firmly, wit

tomy, and cholecystectomy, was performed (Fig. 2).On microscopic examination, the tumor was found to be

composed of pleomorphic tumor cells that are spindle andhyperchromatic, distributed in a fibrous or myxomatousstroma. Focally, tumor cells were highly bizarre, withatypical mitoses and mitotic and focal osteoid pictures(Fig. 3B and D). Prominent necrosis and hemorrhage werepresent. There were some eosinophilic granules in themesenchymal part (Fig. 3C). Some dilated or small duct-likestructures were found between the spindle cells in theperipheral area. The resection margin of liver was free oftumor. The immunohistochemistry stains showed positivityfor actin-851, AiACT, and vimentin but no expression forcytokeratin. The pathologic diagnosis was ruptured UESL.

The postoperative coursewas uneventful, and the patientwas discharged 9 days after operation. One month afteroperation, he started to receive radiotherapy for 2 monthswithout any adjuvant chemotherapy. Peritoneal seedingwith massive ascites was noted on abdominal CT in the 9th

monthafter operation.He received salvagechemotherapyasCYVADIC (cyclophosphamide þ vincristine þ adriamycin þdacarbazine). But disease still progressed and he died 1 yearafter operation.

3. Discussion

As we mentioned in the Introduction section, UESL ispredominant in children and very rare in adult. Worldwideonly 75 cases have been reported where patients are olderthan 15 years: 52 adult cases were reported from 1955 to2007 and summarized by Pachera et al2 and another 23 from2007 to 2011, including our case (Table 1)3e18. In summary,the incidence of UESL is related to age. The incidencedecreased with increasing age. In these 75 patients, 21 arebetween 15 and 20 years, 26 between 21 and 40 years, and18 between 41 and 60 years of age, and only 10 patients aremore than 60 years old. The relationship between age andsurvival is not clear now. Otherwise, female preponderance(female/male Z 41/31) is noted. The incidence of rightlobe tumor is higher (40/75, 53.3%) than left lobe (13/75,17.3%) and bilateral lobe (10/75, 13.3%) tumors. Most tumorsizes exceed 10 cm (68/75, 90.6%). There is no staticdifference between the sizes of tumors at different sites(ANOVA test, p Z 0.151).

The most reported symptoms are all nonspecific, such asabdominal mass or hepatomegaly, with or without upper

rgery shows a huge well-circumscribed mass in the left upperh (C) multiple septa (arrows) in the delayed phase.

Figure 2 Gross photograph of undifferentiated embryonal sarcoma of the liver.

176 J.-H. Chen et al.

abdominal pain, but none of them are specific for UESL.Laboratory tests including liver test and tumor markers areusually within normal limit. Only 9.8% patients with eleva-tion of AFP and 1.9% with elevation of CA-125 were noted.So, preoperative diagnosis should be aided by images.Under ultrasonography, UESL often presents as a solid mass.The CT image characters demonstrate it typically as a largemass, predominantly solid or cystic, with well-definedboarders. The solid part of the tumor has been seen atdelayed images with internal septa.12,14 This misleadingcyst-like appearance on CT imaging compared with theultrasonic findings, in both adults and children, is useful inpreoperative diagnosis and in avoiding drainage to largecystic hepatic mass that is considered to be a benignlesion,2,19 such as complicated hydatid cyst.18 The cyto-logical features of fine needle aspiration of UESL along withradiological and other relevant clinical findings may help inthe correct diagnosis of this uncommon primary hepatictumor.10,13,18

Figure 3 (A) Resected specimen under microscope, (B) area ohyperchromatic cells distributed in fibrous or myxomatous stromosteoid picture.

In pathology, the microscopic characters of UESL in ourcase, such as fibrous pseudocapsule with compressedhepatic parenchyma, mediumelarge spindle or satellitecells in solid component, trapped hepatocytes andabnormal bile duct cells at peripheral area, and eosino-philic spherical globules, were similar to those described inprevious journals. There is no specific histological stainingpattern for UESL.2,7 Unlike previously described patholog-ical findings, in our case, focal osteoid picture was noted inthe solid part of tumor. It is an unusual pathologicalpresentation of UESL. To our knowledge, no other casepresented until now has focal osteoid picture.

Focal osteoid presentation was ever mentioned in theteratoid hepatoblasma,20 malignant mixed epithelial andstromal tumor,21 and nested stromal epithelial tumor of theliver.22,23 These tumors have arisen from the stem cell ormesenchymal tissue of liver. Although the histogenesis ofUESL is debated, several journals mentioned that mesen-chymal hamartoma of liver (MHL) may be a premalignancy of

f embryonal sarcoma composed of pleomorphic spindle anda, with (C) eosinophilic globules (arrows) and (D) some focal

Table 1 Twenty-three new cases from 2007 to 2011.

Reference Year Age Sex Site Size (cm) Surgery RT CT Recurrence Treatment after recurrence Followed up

Kim et al3 2007 61 F R 17 Only fine needle biopsy No VAIA No d AWD 12 moMcCarthy et al4 2007 21 F R N/A Resection Yes Ifosfamide

þ etoposideYes, 36 mo Radio frequency ablation

(RFA) for liverþ resection oflung lesion þ palliativeCT after pregnancy

AWD 60 mo

Kim et al5 2007 44 F R 15 Surgical excision N/A N/A N/A N/A N/AGourgiotis et al6 2008 20 M B 20 Extensive left lobectomy No Adjuvant CT Yes N/A DOD 9 moMa et al7 2008 61 F R 12 Right lobectomy No N/A N/A N/A DOD 8 moFuertes et al8 2008 40 M R 28 Right trisegmentectomy

(6 þ 7 þ 8 þ 1)No No Yes, 10 mo Ifosfamide þ adriamycin ANED 36 mo

Yang et al9 2009 46 M R 6.2 Right lobectomy N/A N/A N/A N/A N/AYang et al9 2009 54 M R 13 Right lobectomy N/A N/A N/A N/A N/AYang et al9 2009 26 F R 12.2 N/A N/A N/A N/A N/A N/AYang et al9 2009 20 F B 18.2 N/A N/A N/A N/A N/A N/AKaur et al10 2009 23 M R 15 Partial hepatectomy N/A N/A N/A N/A N/ALi et al11 2010 63 M R 20 Right lobectomy No No Yes, 9 mo N/A DOD 18 moLi et al11 2010 39 M L 16 Left lobectomy No TACE Yes, 30 mo N/A DOD 32 moLi et al11 2010 56 M R 12.5 Right lobectomy No TACE Yes, 14 mo N/A DOD 28 moLi et al11 2010 43 F R 14 Right lobectomy No No Yes, 12 mo N/A DOD 24 moXu et al12 2010 36 F L 30 Right lobectomy N/A N/A N/A N/A N/AFaraj et al13 2010 21 M R 22 Extensive right lobectomy No No Yes, 3 wka Ifosfamide þ etoposide

alternating withactinomycin-D,vincristine

ANED 5 mo

Massaniet al14 2010 47 F R N/A Resection N/A N/A Yes, 24 mo Liver resection þ TACE DOD 29 moYoon et al 15 2010 53 F R 13 Resection No No No d ANED 6 moLee et al16 2010 72 M L 3.4 Only fine needle biopsy N/A N/A N/A N/A N/AGasljevic et al17 2011 58 F N/A N/A N/A N/A N/A N/A N/A N/AKalra et al18 2011 24 M R 14 N/A N/A N/A N/A N/A N/AChen et al(present case)

2011 63 M L 27.9 Lateral segmentectomyþ splenectomyþ cholecystectomy

Yes No Yes, 9 mo CYVADIC DOD 12 mo

ANED Z alive with no evidence of disease; AWD Z alive with disease; CT Z computed tomography; CYVADIC Z cyclophosphamide þ vincristine þ adriamycin þ dacarbazine; DOD Z diedof disease; N/A Z not available; TACE Z transarterial chemoembolization; VAIA Z vincristine þ actinomycin þ ifosfamide þ doxorubicin.a Recurrence site: previous drainage insertion.

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178 J.-H. Chen et al.

UESL.24e26 MHL is an uncommon hamartomatous growth ofmesenchymal tissue or other parts of the liver. This can beindirectly proven that the stem cells in mesenchyma of livermay possibly induce focal osteoid picture, just like the ter-atoid hepatoblasma, malignant mixed epithelial and stromaltumor, and nested stromal epithelial tumor of the liver.

Until recently, radial excision of the tumor seemed toprovide the only chance of cure. Most patients died due totumor recurrence or metastases 1 year after operation. Therisk of recurrence after surgery is relatively high for the first2 years. The recurrence risk is associated with positivemargin and spontaneous or iatrogenic rupture of UESL.Combined chemotherapy and/or radiotherapy has beenused for the treatment of UES and has been found to achievesuperior local control and disease-free survival, even long-term survival. Lenze et al1 analyzed 68 adult patients. Theactuarial 1- and 2-year survival rates for all patients were61% and 55%, respectively. Patients with complete tumorresection followed by adjuvant chemotherapy had signifi-cantly better survival than patients with surgery alone(pZ 0.002). But, if complete resection cannot be achieved,just like in case of our patient, adjuvant chemotherapy hadno benefit on overall and disease-free survivals (p Z 0.07).

In conclusion, UESL is extremely rare in adults. Only in11 reported cases, patients are older than 60 years. Rightlobe and female preponderance are noted. Our case isextremely rare in all adult UESL cases because of thepatient’s age (63 years old, i.e., more than 60 years old)and the tumor’s location (left lobe). Our patient is the firstreported case of focal osteoid picture in UESL, which maydevelop from the stem cells in mesenchyma of liver. OnceUESL is diagnosed, early definite surgical resection withadjuvant chemotherapy may improve survival rate,increase tumor-free survival, and even achieve long-termsurvival. In our case, because of ruptured tumor, incom-plete resection was recognized and the prognosis of thepatient was relatively poor.

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