Long‐term survival outcomes of undifferentiated embryonal sarcoma of the liver: a pooled analysis of 308 patients

Wu, Zhiyong; Wei, You-Zhu; Cai, Zhibin; Zhou, Yanming

doi:10.1111/ans.15684

Cited by 35 publications

(87 citation statements)

References 11 publications

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“…Multimodal therapy consisting of local resection or LT, and neoadjuvant multiagent chemotherapy to facilitate resection, results in survival rates of 65-75%. Several regimens have included alkylators, platinums, podophyllotoxins, anthracyclines, and topoisomerase I. inhibitors, among others [33,66]. There is less agreement about the optimal treatment of MRT.…”

Section: Chemotherapymentioning

confidence: 99%

Liver Transplantation for Pediatric Liver Cancer

Sindhi

Rohan

Tadros

et al. 2020

Cancers

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Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability. In the process, tumor histology, imaging protocols, and tumor staging have also matured to better assess response and LT candidacy. Significant trends include a steady increase in the incidence of and use of LT for hepatoblastoma, and a significant improvement in survival after LT for HCC with each decade. Although LT is curative for most unresectable primary liver sarcomas, such as embryonal sarcoma, the malignant rhabdoid tumor appears relapse-prone despite chemotherapy and LT. Pediatric liver tumors remain rare, and diagnostic uncertainty in some settings can potentially delay treatment or lead to the selection of less effective chemotherapy. We review the current knowledge relevant to diagnosis, LT candidacy, and post-transplant outcomes for these tumors, emphasizing recent observations made from large registries or larger series.

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Section: Chemotherapymentioning

confidence: 99%

Liver Transplantation for Pediatric Liver Cancer

Sindhi

Rohan

Tadros

et al. 2020

Cancers

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“…However, it may have better outcomes compared to angiosarcoma when surgically resected. A recent review article including 271 patients who received partial hepatectomy showed a 5-year survival rate of 70%[ 10 ]. The 5-year survival rate for the undifferentiated sarcoma group in our study was 50%.…”

Section: Discussionmentioning

confidence: 99%

“…Walther et al [ 20 ] studied 3 patients who received LT and remained in clinical remission for a mean of 35 mo. Wu et al [ 10 ] showed 14 patients who received LT for hepatic undifferentiated sarcoma with a 5-year survival rate of 78.9%. When weighing the benefits of LT against the risks for the liver donor or the shortage of deceased donor, LT in hepatic undifferentiated sarcoma is still controversial and needs further research.…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment outcomes of primary hepatic sarcomas: A single-center experience

Kim

Rhu

Kim

et al. 2021

WJH

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BACKGROUND Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it’s rarity. However, with development of medical technology, surgical treatment may lead to better survival. AIM To investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute. METHODS From August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach. RESULTS The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain ( P = 0.002). CONCLUSION Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.

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“…2 Advances in management of these tumors including surgical resection, with or without liver transplantation, along with adjuvant high-dose alkylator-and anthracycline-based chemotherapy provides a curative outcome in the majority of cases. 1,[3][4][5][6][7] Although there is no universally accepted treatment, the systemic approach is generally similar to that of other soft tissue sarcomas. [3][4][5]7 Notably, a recent pooled analysis of UESL demonstrated a 5-year overall survival of 70% for those who were able to undergo surgical resection.…”

Section: Introductionmentioning

confidence: 99%