Pitfalls in the Surgical Management of Undifferentiated Sarcoma of the Liver and Benefits of Preoperative Chemotherapy

Merli, Laura; Mussini, Charlotte; Gabor, Flaviu; Branchereau, Sophie; Martelli, Hélène; Pariente, D.; Guérin, Florent

doi:10.1055/s-0034-1387937

Cited by 16 publications

(23 citation statements)

References 12 publications

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“…A systematic review also showed that out of 14 patients (10 children and 4 adults) undergoing LT for UESL, 3 died [6]. Several other case reports and case series have also demonstrated the ability to achieve long-term survival for UESL with chemotherapy and either primary or salvage LT [12,14,26,37,38]. These findings validate the role of LT in the management of unresectable UESL.…”

Section: Discussionmentioning

confidence: 78%

“…The clinical manifestations (abdominal pain, fever, anorexia) and the radiographic characteristics (solid and cystic components) are nonspecific, and thus UESL may pose a significant diagnostic challenge that can lead to a delay in appropriate management [3][4][5][6][7][8][9]. Although historically treatment was limited to surgical resection and overall survival (OS) was poor [1], the combination of surgical treatment and chemotherapy recently has led to improved OS [10][11][12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

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Undifferentiated Embryonal Sarcoma of the Liver in Children Versus Adults: A National Cancer Database Analysis

Ziogas

Zamora

Lovvorn

et al. 2021

Cancers

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This study evaluates the clinicopathological characteristics and outcomes of children vs. adults with undifferentiated embryonal sarcoma of the liver (UESL). A retrospective analysis of 82 children (<18 years) and 41 adults (≥18 years) with UESL registered in the National Cancer Database between 2004–2015 was conducted. No between-group differences were observed regarding tumor size, metastasis, surgical treatment, margin status, and radiation. Children received chemotherapy more often than adults (92.7% vs. 65.9%; p < 0.001). Children demonstrated superior overall survival vs. adults (log-rank, p < 0.001) with 5-year rates of 84.4% vs. 48.2%, respectively. In multivariable Cox regression for all patients, adults demonstrated an increased risk of mortality compared to children (p < 0.001), while metastasis was associated with an increased (p = 0.02) and surgical treatment with a decreased (p = 0.001) risk of mortality. In multivariable Cox regression for surgically-treated patients, adulthood (p = 0.004) and margin-positive resection (p = 0.03) were independently associated with an increased risk of mortality. Multimodal treatment including complete surgical resection and chemotherapy results in long-term survival in most children with UESL. However, adults with UESL have poorer long-term survival that may reflect differences in disease biology and an opportunity to further refine currently available treatment schemas.

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Section: Discussionmentioning

confidence: 78%

Section: Introductionmentioning

confidence: 99%

Undifferentiated Embryonal Sarcoma of the Liver in Children Versus Adults: A National Cancer Database Analysis

Ziogas

Zamora

Lovvorn

et al. 2021

Cancers

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“…The initial diagnosis of hydatid cyst may lead to the treatment delay, which was the case in three of our patients. Due to the cystic nature of some UESL, percutaneous biopsy may not always provide representative tissue and may be inconclusive 13,31 . Wildhaber et al.…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated sarcoma of the liver: Multicenter international experience of the Cooperative Soft‐Tissue Sarcoma Group and Polish Paediatric Solid Tumor Group

Murawski

Scheer

Leuschner

et al. 2020

Pediatric Blood & Cancer

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Background: Undifferentiated embryonal sarcomas of the liver (UESL) are extremely rare and continue to pose a diagnostic and therapeutic challenge. The aim of the study was to present a multicenter experience of the German CWS and Polish PPSTG groups in the treatment of UESL in children. Procedure: Twenty-five patients were treated according to the CWS-96, CWS-2002, and CYVADIC protocols. Distant metastases were observed in four cases (16%). In four cases, an initial disease presentation mimicked other entities. A pure cystic appearance of liver mass led to misdiagnosis of hydatid cyst in three cases. In one case, laparotomy was performed due to the signs of appendicitis, and bleeding from ruptured liver tumor was found. All these patients were finally diagnosed as UESL. Results: Thirteen patients received preoperative chemotherapy. Partial response was observed in 10 cases. Tumor resection was performed in 20 patients (primary resections, 12; delayed resections-, 8). In five patients, the primary tumor never became operable. The macroscopically complete resection rate was 95% (19/20). Postoperative chemotherapy was given to 20 children. Local radiotherapy was used in three children. After a median follow-up time of 136 months, 17 patients (68%) were alive with no evidence of disease. All children with unresectable tumor and three out of four patients with distant metastases died. The five-year overall survival (OS) rate was 72%. Conclusions: In summary, a complete tumor excision plays the central role in the treatment of UESL. A cystic presentation of the liver lesion on imaging does not exclude the diagnosis of malignant tumor.

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“…European hospitals prefer VAC regimen or chemotherapy with a modified VAC backbone, but in China, we tend to use chemotherapy regimen (vincristin, cisplatin, cyclophosphamide and adriamycin) [24-29]. Merli et al [27] treated 13 undifferentiated liver sarcoma patients with surgery and postoperative chemotherapy according to the European protocol (7 patients with neoadjuvant chemotherapy). A review of the literature found 26 patients who were treated with VAC or VAC-based chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment and Chemotherapy of Adult Primary Liver Sarcoma: Experiences from a Single Hospital in China

Zhou¹,

Huang²,

Zhou³

et al. 2018

Dig Surg

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Background: The aim of this study is to improve the preoperative diagnostic accuracy and treatment results by investigating the clinical features and prognosis of primary liver sarcoma (PLS). Methods: Clinical data, surgical treatments, adjuvant chemotherapy, and prognosis of 17 PLS patients whose diseases were pathologically confirmed were retrospectively analyzed. Results: The main clinical symptoms included epigastric pain in 9 patients, epigastric distention in 7, and loss of appetite in 4; these symptoms were detected during the postoperative follow-up for gastric carcinoma in 1. The resection rate was 64.7% (12/17), including R0 resection in 10 patients and R1 resection in 2, and laparotomy with biopsy in 5. Five patients accepted an adjuvant selective hepatic artery infusion chemotherapy (mitomycin C 16–20 mg+ 5-fluorouracil 5.0 g+ epirubicin 40–50 mg), and 4 accepted adjuvant systemic chemotherapy (vincristin, cisplatin, cyclophosphamide, and adriamycin). All 5 patients with simple laparotomy died within 1 year, and the overall 1-, 3-, and 5-year survival rates for all patients were 58.8% (10/17), 29.4% (5/17) and 11.7% (2/17), respectively, whereas those were 100.0% (10/10), 50.0% (5/10), and 20.0% (2/10) for R0 resected patients respectively. Conclusions: The diagnosis of PLS is difficult before operation due to its nonspecific manifestations, and the high survival rate can be achieved by radical resection with adjuvant chemotherapy.

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Pitfalls in the Surgical Management of Undifferentiated Sarcoma of the Liver and Benefits of Preoperative Chemotherapy

Abstract: USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.

Cited by 16 publications

References 12 publications

Undifferentiated Embryonal Sarcoma of the Liver in Children Versus Adults: A National Cancer Database Analysis

Undifferentiated Embryonal Sarcoma of the Liver in Children Versus Adults: A National Cancer Database Analysis

Undifferentiated sarcoma of the liver: Multicenter international experience of the Cooperative Soft‐Tissue Sarcoma Group and Polish Paediatric Solid Tumor Group

Surgical Treatment and Chemotherapy of Adult Primary Liver Sarcoma: Experiences from a Single Hospital in China

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