Adult Embryonal Sarcoma of the Liver: Management of a Massive Liver Tumor

Treitl, Daniela; Roudenko, Alexandra; Hussein, Siba El; Rizer, Magda; Bao, Philip

doi:10.1155/2016/5625762

Cited by 6 publications

(5 citation statements)

References 18 publications

(32 reference statements)

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“…On average, their survival duration is about 29 months [ 15 ]. Within the first year, the survival rate stands at 61%, dropping slightly to 55% in the second year [ 16 ]. Survival diminishes significantly, to just 12 months, for those who have only partial tumor removal and do not undergo chemotherapy [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

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An Adult With Undifferentiated Embryonal Sarcoma of the Liver: A Case Report of a Rare Encounter

Rammohan,

Joy,

Magam

et al. 2023

Cureus

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Undifferentiated embryonal sarcoma of the liver (UESL) is a rare, aggressive tumor mainly found in children but can also appear in adults. Its diagnosis in adults remains a conundrum; it is often identified late due to its non-specific symptoms and resemblance to benign lesions. A comprehensive treatment regimen involving surgical intervention, chemotherapy, and possibly radiation significantly boosts survival rates. Imaging often yields inconclusive outcomes, further complicating the diagnostic process. Here, we report the case of a 28-year-old female diagnosed with UESL, emphasizing the need for timely intervention. Undifferentiated embryonal sarcoma of the liver requires differentiation from a variety of hepatic tumors in adults. Though there are no distinctive characteristics to differentiate UESL from other hepatic masses, its morphology and immunohistochemical profiles significantly vary. The staging often reveals UESL as a large, well-defined mass with the potential for diverse differentiation. Its prognosis has been considerably improved with the advent of multidisciplinary treatment. Surgical resection remains a cornerstone, often combined with chemotherapy. While pediatric cases exhibit better overall survival rates than adults, outcomes heavily depend on the chosen treatment regimen. A combination of chemotherapy and complete tumor removal has been found to significantly elevate survival chances. Disease recurrence remains a challenge and is influenced by treatment strategy. In conclusion, the diagnosis and treatment of UESL are fraught with challenges, particularly in adults. A multidimensional approach, combining various therapies, is paramount for better outcomes. Continuous research and enhanced awareness are crucial for improving diagnostic precision and treatment outcomes for UESL patients.

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Section: Discussionmentioning

confidence: 99%

“…However, with the addition of subsequent chemotherapy, recurrence dropped to 23%, with an average recurrence period of 28 months post-treatment [ 15 ]. Finally, the role of interventional radiology in UESL treatment is growing, aiming to lessen the side effects of systemic chemotherapy and shrink tumors before surgery [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

An Adult With Undifferentiated Embryonal Sarcoma of the Liver: A Case Report of a Rare Encounter

Rammohan,

Joy,

Magam

et al. 2023

Cureus

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“…There is variable staining for desmin, smooth-muscle actin, CD68, CD56, BCL2, and CD10. No immunoreactivity has been described for HepPar-1, myogenin, CD34, CD117, S-100, Alk-1, or AFP [ 22 – 25 ]. Glypican 3 (GPC3) and paranuclear dot-like staining for cytokeratin has been reported [ 14 , 27 ].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Metastases from an Undifferentiated Embryonal Sarcoma of the Liver: A Case Report and Review

Shi

Sangster

et al. 2018

Case Reports in Oncological Medicine

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Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor that occurs primarily in children. Only a limited number of cases have been reported in the literature due to low incidence of one per million, and reports of metastatic lesion of UESL are even rarer. We hereby describe the case of a 13-year-old male who presented with a palpable mass with imaging findings suggestive of a large complex tumor in the right lobe of the liver. He underwent extended right hepatectomy followed by adjuvant chemotherapy. The tumor was confirmed to be UESL by postoperative pathology and immunohistochemical staining analysis. Four years later, surveillance imaging revealed a small lung nodule in the left lower lobe. Complete removal of the lung tumor by wedge resection was performed, and a histological diagnosis of metastatic UESL was made. The patient also received postoperative adjuvant chemotherapy and is currently in a good general condition and tumor-free in the present eight-month period. This case is presented with emphasis on clinicopathological and immunohistochemical findings of the primary UESL and lung metastases with the aim of collecting more data and expanding our understanding of this rare malignancy.

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“…There is a higher recurrence rate in patients who do not receive chemotherapy even if they undergo a complete resection. 2 -5…”

Section: Commentmentioning

confidence: 99%

“…There is a higher recurrence rate in patients who do not receive chemotherapy even if they undergo a complete resection. [2][3][4][5] Cardiac metastases are found in from 10% to 25% of patients who died with these malignancies. There are four pathways by which metastases may reach the heart: through lymphatic or hematogeneous spread and by direct or transvenous extension.…”

Section: Commentmentioning

confidence: 99%

Treatment of Hepatic Embryonal Undifferentiated Sarcoma With Cardiothoracic Involvement

Domínguez-Massa

Serrano-Martínez

Blanco-Herrera

et al. 2018

World J Pediatr Congenit Heart Surg

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Thorough study is required to decide the appropriate management of hepatic tumors in children. We present a case report of a hepatic embryonal undifferentiated sarcoma with unfavorable prognosis in a nine-year-old girl. After undergoing a detailed cancer characteristics and extension study, a two-stage surgery approach was decided. The hepatic tumor resection was the first procedure to be performed. One week later, under cardiopulmonary bypass, deep hypothermia, and circulatory arrest, thrombectomy of the inferior vena cava and right atrium was accomplished, plus thromboendarterectomy of the right pulmonary artery. During a four-year follow-up, the patient continues to be disease-free.

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Adult Embryonal Sarcoma of the Liver: Management of a Massive Liver Tumor

Cited by 6 publications

References 18 publications

An Adult With Undifferentiated Embryonal Sarcoma of the Liver: A Case Report of a Rare Encounter

An Adult With Undifferentiated Embryonal Sarcoma of the Liver: A Case Report of a Rare Encounter

Pulmonary Metastases from an Undifferentiated Embryonal Sarcoma of the Liver: A Case Report and Review

Treatment of Hepatic Embryonal Undifferentiated Sarcoma With Cardiothoracic Involvement

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