The incidence of UESL is highest in children between 6 and 10 years of age [
1]. To the best of our knowledge, in the past 50 years, less than 40 cases of UESL have been reported in patients older than 20 years [
5]. UESL accounts for less than 1 % of all primary liver neoplasms in adults, and there are no specific clinical features and tumor makers. Furthermore, the results of imaging studies such as CT, ultrasonography, and magnetic resonance imaging (MRI) are often inconclusive. Thus, UESL must be considered during differential diagnosis of large liver tumors in children and adults. The diagnosis of UESL was delayed in some cases because the lesion presented as a large cystic hepatic mass, suggesting a benign lesion [
6‐
12]. The prognosis of UESL is poor, but cases of long-term survival have been reported. Multidisciplinary treatment (chemotherapy and radiotherapy) has been used to achieve superior and local control and disease-free survival in patients with UESL [
13,
14]. For example, 17 children were reported to show long-term survival after UESL [
14]. In this report, four patients who received complete resection followed by adjuvant chemotherapy achieved complete remission over a follow-up period of 5 years to 10.9 years. One patient was treated with vincristine, actinomycin, cyclophosphamide, and doxorubicin (VACA), two were treated with vincristine, actinomycin, ifosfamide, and doxorubicin (VAIA), and one was treated with ifosfamide, vincristin, and actinomycin (VAIA-IVA). In this report, it was noted that the response to chemotherapy was 62 %. In another report, three children who received complete resection followed by adjuvant chemotherapy of vincristine, actinomycin D, and cyclophsphamide (VADRC-VAC) + CDDP for 1 year, had a comparable prognosis with no evidence of the disease over a follow-up period of 40 to 60 months [
15]. In another report, adjuvant chemotherapy after complete surgical resection of UESL showed significant therapeutic effects in adults [
16]. In this report, all 14 patients who underwent complete tumor resection followed by adjuvant chemotherapy were alive after a median period of 28.5 months (range, 6–204 months). On the other hand, the 1-year and 2-year survival rates for all 14 patients who underwent complete tumor resection without receiving adjuvant chemotherapy was 53 %. Patients who underwent a complete tumor resection followed by adjuvant chemotherapy had significantly better survival rate than patients who underwent only surgery. In five patients who received incomplete surgical resection followed by chemotherapy, one patient was alive without any evidence of the disease after a follow-up of 6 months but all other patients died of sarcoma-associated complications after a median period of 12.5 months (range, 9–24 months). Three patients with UESL, who underwent incomplete surgical resection and received no postoperative chemotherapy, died after a median period of 12 months (range, 5–67 months). The difference in survival rates between these treatment groups was found to be statistically insignificant. Complete excision of the tumor seemed vital for cure. In addition, adjuvant chemotherapy after complete resection seemed be also important for cure. To our knowledge, only three adult cases of UESL (aged > 20 years) with patient survival for more than 60 months have been reported in literature [
9,
16,
17] (Table
4). Complete resections were performed in two of these patients. In the third case, the UESL ruptured during the resection. After the operation, the cancer recurred and the patient underwent two more operations. However, he died because of recurrences at 67.6 months after the first liver resection [
9]. In the other two cases of complete resection, postoperative chemotherapy was performed after complete resections. In one case, the patient had two lesions in the right and left liver. At first, she received hepatic right trisegmentectomy and was treated postoperatively with five cycles of intravenous ifosfamide (2,500 mg/m
2, days 1–3), doxorubicin (50 mg/m
2, day 2), and mensa (2,000 mg/m
2, days 1–3). She also underwent resection of the left-side lesion and received two additional cycles of the chemotherapeutic regimen mentioned above. Both the resection margins were free. At 71 months after the second hepatic operation, she was healthy with no evidence of disease [
17]. In the other case, the patient underwent an extended resection of the right liver lobe and partial resection of the diaphragm. The resection margin was free of tumor infiltration. She did not receive postoperative chemotherapy, and the tumor recurred 4 months later in the region of the former right liver lobe and the pelvis. She was treated with eight courses of intravenous carboplatin (at a dose of 150 mg/m
2 on days 1–4) and etoposide (at a dose of 150 mg/m
2 on days 1–4). After a gap of 4 weeks, she received chemotherapy with doxorubicin (at a dose of 30 mg/m
2 on days 1–2) and ifosfamide (at a dose of 3,000 mg/m
2 on days 1–3). The recurring lesions had reduced. Over a period of approximately 6 years, she received no further chemotherapy or surgical treatment and showed no evidence of recurrence [
16]. No evidence of the disease was seen after complete resection and adjuvant chemotherapy in all three cases showing long-term survival; however, all the adjuvant chemotherapy regimens were different. Hence, we concluded that complete resection and adjuvant therapy were necessary for long-term survival, but the regimens of postoperative standard chemotherapy and radiation are a matter of debate. There are some reports that long-term survival cases of UESL with successful surgery, free margin and after conventional chemotherapy, but one case showed local recurrence despite successful surgery and adjuvant chemotherapy [
16]. Although radiotherapy might be overtreated, we performed radiotherapy after obtaining the patient’s consent in this case. The findings of the cases of adult UESL, including this report, were vital for possible treatment options.
Table 4
Adjuvant therapy, recurrence and outcome of long-term survival cases of adult undifferentiated embronal sarcoma
| 1996 | 25 | Positive | None | Liver | Re-resection, two times | 67.6 | DOD |
| 2005 | 21 | Negative | I + ADM, Re-resectionI + ADM | None | | 77 | NED |
| 2008 | 34 | Negative | None | Liver | CBDCA + VP-16 + ADM + I | 72 | NED |
Present | 2011 | 27 | Negative | DDP + VCR + ADM + ACTRT + PBSCT (VP-16, MCNU, CBDNU, CBDCA, CTX) | None | | 60 | NED |