Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis. Case report and review of the literature

Sakellaridis, Timothy; Panagiotou, Ioannis; Georgantas, Themistoclis; Micros, George; Rontogianni, Dimitra; Antiochos, Christos

doi:10.1186/1477-7819-4-9

Cited by 30 publications

(17 citation statements)

References 13 publications

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“…The true histology of recurrence was not known. In previous literature, the prognosis of undifferentiated sarcoma was poor, but recent evidence shows that long-term survival is possible after radical resection with or without adjuvant chemotherapy [ 34 ]. The 5-year survival of patients with undifferentiated sarcoma was 60% in our series.…”

Section: Discussionmentioning

confidence: 99%

Surgical experience of adult primary hepatic sarcomas

Lin

Concejero

et al. 2015

World J Surg Onc

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BackgroundPrimary hepatic sarcoma (PHS) is a rare primary liver malignancy. The histological types of PHS are diverse, and the clinical outcomes and management mainly depend on the histopathology. This study aims to evaluate the results of surgical intervention.MethodsBetween January 2003 and June 2009, 13 adult patients with pathologically proven PHS were identified by record review. The patients’ demographic profile, tumor characteristics, treatment modalities, and outcomes were reviewed and analyzed. The end of follow-up was December 2014.ResultsNine (69%) underwent curative liver resection and two underwent liver transplantation; the others received non-operative treatments. The pathologic findings were six (46%) angiosarcomas, four (30.7%) undifferentiated sarcomas, one (7.6%) leiomyosarcoma, one (7.6%) malignant mesenchymoma, and one (7.6%) hepatic epithelioid hemangioendothelioma. The median follow-up was 31.4 (2.8 ~ 142.5) months. The 1-, 2-, and 5-year survival of surgical patients were 72.7%, 63.6%, and 36.4%, respectively. Importantly, the 1-, 2-, and 5-year survival rates of non-angiosarcoma patients were superior to those of angiosarcoma (85.7% vs. 33.3%, 71.4% vs. 16.7%, and 57.1% vs. 0%, respectively, P = 0.023).ConclusionsSurgical intervention provides the possibility of long-term survival from PHS. Angiosarcoma is associated with a more dismal outcome than non-angiosarcoma.

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Section: Discussionmentioning

confidence: 99%

Surgical experience of adult primary hepatic sarcomas

Lin

Concejero

et al. 2015

World J Surg Onc

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“…Mild leukocytosis or leukopenia, low albumin, anemia, and slightly elevated transaminase levels and erythrocyte sedimentation rates may be seen. Evaluation of some tumor markers including AFP, cancer antigen 19-9, and carcinoembryonic antigen often yields normal results, but rare cases with increased levels of AFP and cancer antigen 125 have been reported [ 16 ]. There is one reported case of UESL that secretes erythropoietin, which was used as a marker of the tumor recurrence [ 17 ].…”

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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“…Other symptoms may include nausea, vomiting, constipation, and respiratory distress secondary to compression caused by the tumor 5. Spontaneous rupture of the neoplasm has also been documented 6. Liver test results and neoplastic markers are usually normal.…”

Section: Discussionmentioning

confidence: 99%

“…Macroscopically, UESL are single and well-circumscribed lesions with both cystic and solid components of gray-white gelatinous appearance and a fibrous pseudocapsule formed by compressed liver parenchyma. Frequently, dark-brown areas of hemorrhage and yellow-softer areas of necrosis are found 69. Microscopically, UESL shows undifferentiated spindle cells with ill-defined borders, inconspicuous nucleoli, hyperchromatic nuclei, and eosinophilic cytoplasm surrounded by a myxoid matrix showing undifferentiated and highly proliferative phenotype.…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated embryonal sarcoma of the liver in adult patient: A report of two cases

Sánchez-Morales

Emilio

Alvarez-Bautista

et al. 2018

Ann Hepatobiliary Pancreat Surg

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Primary sarcomas of the liver are unusual neoplasms developing in adults. They constitute a heterogeneous group of neoplasms including undifferentiated embryonal sarcoma. Patients usually present with an abdominal mass and abdominal pain. Case 1: A 53-year-old woman presented with abdominal pain. Computed tomography showed an occupying mass in the right lobule and an intra-auricular multi-lobulated mass suggestive of a secondary deposit. Biopsy of the hepatic lesion revealed undifferentiated embryonal sarcoma. Despite radiotherapy and supportive measures, her overall status progressively worsened until cardiac arrest. Case 2: A 41-year-old woman presented with hepatomegaly. Abdominal imaging showed cystic lesions in the right hepatic lobule with multiple septae. The patient underwent extended right hepatectomy and a histopathological study reported high-grade undifferentiated embryonal sarcoma. Two years after surgery, a new cystic lesion in the surgical site was recorded and chemotherapy was scheduled. The lesion remained stable for three years when disease progression was observed and second-line chemotherapy was initiated. Although undifferentiated embryonal sarcoma of the liver has poor prognosis, early diagnosis is essential to increase the chances of survival. Currently, surgical resection and chemotherapy are the primary treatment modalities.

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Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis. Case report and review of the literature

Abstract: It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy.

Cited by 30 publications

References 13 publications

Surgical experience of adult primary hepatic sarcomas

Surgical experience of adult primary hepatic sarcomas

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

Undifferentiated embryonal sarcoma of the liver in adult patient: A report of two cases

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