Undifferentiated Embryonal Sarcoma of the Liver in an Adult Patient

Beksaç, Kemal; Mammadov, Rustam; Çiftçi, Türkmen; Güner, Güneş; Akyol, Aytekin; Kaynaroğlu, Volkan

doi:10.7759/cureus.3037

Cited by 7 publications

(8 citation statements)

References 20 publications

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“…Despite the low overall incidence, UESL is the most common sarcoma and the third most common hepatic malignancy in the pediatric population after hepatoblastoma and hepatocellular carcinoma (HCC) [42,43]. Most cases of UESL are diagnosed in the first decade of life, between 6 and 10 years of age, but several case reports have been described in adults and even elderly patients [36,41,[44][45][46][47][48]. UESL shows no sex predilection in children and a slight female predominance in adults [41,45].…”

Section: Undifferentiated Embryonal Sarcomamentioning

confidence: 99%

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Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Martins-Filho

Putra

2020

Hepat. Oncol.

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This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

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Section: Undifferentiated Embryonal Sarcomamentioning

confidence: 99%

“…Most cases of UESL are positive for Vimentin, alpha-1 antytrypsin and CD68 [69][70][71][72]. Other common -but slightly less prevalent -markers include BCL2, CD10 and CD56 [36,47,69,70,72,73]. UESL is usually negative for HepPar1, AFP, alpha smooth muscle actin, myogenin, synaptophysin, CD117, HMB-45 and CD34 [69,70,72].…”

Section: Pathologic Findingsmentioning

confidence: 99%

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Martins-Filho

Putra

2020

Hepat. Oncol.

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“…Patients usually experience non-specific gastrointestinal symptoms such as abdominal pain, nausea, and vomiting. In some cases, weight loss as well as a palpable tumor in the upper abdomen are described [4][5][6]. In addition, the radiological properties of a UESL are rather unspecific.…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of this disease is very low at 0.6 to 1.2 cases per 1 million patients, and adults are affected by this tumor in only 10% of all cases [4,5]. Originally, UESL was considered to be a very aggressive tumor with a poor prognosis, but due to advanced treatment options (advanced liver resection techniques and highly effective chemotherapeutic agents) prognosis has markedly improved [6].…”

Section: Introductionmentioning

confidence: 99%

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Perl¹,

Häring

Horger³

et al. 2020

EJNMMI Res

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Background: The undifferentiated embryonic sarcoma of the liver (UESL) is a rare, aggressive tumor mainly affecting children. Since UESL has no specific clinical symptoms or imaging characteristics, many cases of UESL are diagnosed late. The paraneoplastic leukemoid reaction (PLR) is a very rare concomitant of oncological patients associated with poor prognosis. This report describes the clinical course of a patient combining these two rare entities and describes the diagnostic challenges and dynamics of paraneoplastic syndrome. Case presentation: We report a case of UESL in a 46-year-old male who became clinically conspicuous due to pronounced B symptoms. CT and MRI showed a suspicious unifocal liver lesion. As the histological analysis of a tissue sample did not reveal a clear result, an 18F-FDG-PET-CT examination was performed. In addition to a high glucose metabolism of the liver lesion, an increased glucose metabolism in the entire bone marrow was observed. This finding was considered as pronounced paraneoplasia and laparotomy with liver segment resection followed. Immediately after resection of the tumor the paraneoplastic symptoms completely declined and the patient showed no signs of recurrence in the 1-year follow-up. Conclusions: Although UESL is rare and predominantly affects children, this diagnosis should always be considered for unclear unifocal cystic liver lesions, regardless of the patient's age, as appropriate treatment has a good prognosis.

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“…Undifferentiated embryonal sarcoma of the liver (UESL) is highly malignant with a poor prognosis. It often occurs in children aged 6-10 years and it tends to be uncommon in adults (2). An early diagnosis of UESL is often difficult because clinical symptoms and imaging findings do not manifest until the later stages of the neoplasm.…”

Section: Introductionmentioning

confidence: 99%

Undifferentiated Embryonal Sarcoma of the Liver Identified after the Initial Diagnosis of a Hepatic Cyst

et al. 2020

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Abdominal ultrasound in a 50-year-old Japanese man revealed a cystic lesion on the caudate lobe of the liver. Four-month follow-up imaging showed a rapid increase in the size of the cystic lesion. The patient underwent laparoscopic partial hepatectomy because of a suspicion and perceived risk that the lesion might be malignant. The initial histological diagnosis was a hepatic cyst. Eleven months later, computed tomography showed a giant cystic lesion in the abdominal cavity and multiple liver metastases. The patient underwent excision of the giant cystic lesion and a partial hepatectomy. Immunohistochemistry for the recurring lesion revealed undifferentiated embryonal sarcoma of the liver.

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Undifferentiated Embryonal Sarcoma of the Liver in an Adult Patient

Cited by 7 publications

References 20 publications

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Undifferentiated Embryonal Sarcoma of the Liver Identified after the Initial Diagnosis of a Hepatic Cyst

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