Undifferentiated embryonal sarcoma of the liver mimicking venolymphatic malformation

Kamrani, Kambiz; Patel, Anshu; Guerrieri, Claudio; Bethel, Colin; Phatak, Tej

doi:10.1016/j.radcr.2019.04.005

Cited by 10 publications

(13 citation statements)

References 7 publications

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“…Immediately postoperative both inflammatory values dropped sharply and leucocytes reached their normal values on the third postoperative day (8770/μl) and CRP on the 12th postoperative day (0.43 mg/dl) rapid tumor growth can be assumed. Some case reports describe tumors measuring up to 25 × 19 × 14 cm [5], and there are case reports in which the patients became clinically conspicuous only after the tumor had ruptured [9][10][11]. Due to the pronounced paraneoplastic syndrome in this case, the patient became clinically noticeable relatively early, so that the diagnosis could be made at a comparatively small tumor diameter of 5.3 × 6 cm.…”

Section: Discussionmentioning

confidence: 92%

“…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%

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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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Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Perl¹,

Häring

Horger³

et al. 2020

EJNMMI Res

View full text Add to dashboard Cite

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“…On CT, UESL will be a hypodense solid and cystic mass (Figure 1A) (2). On MRI, the cysts within the mass may show multiple fluid-fluid levels (Figure1B) (9). Due to their cystic character radiologically, UESLs have been known to be mistaken for benign cystic entities such as biliary cystadenomas, hydatid cyst disease, or venolymphatic malformations (2,8,9).…”

Section: Radiologymentioning

confidence: 99%

“…On MRI, the cysts within the mass may show multiple fluid-fluid levels (Figure1B) (9). Due to their cystic character radiologically, UESLs have been known to be mistaken for benign cystic entities such as biliary cystadenomas, hydatid cyst disease, or venolymphatic malformations (2,8,9). In fact, the radiological appearance of USEL can be quite misleading to a point where delays in diagnosis or even the initiation of the wrong treatment have occurred (4,10).…”

Section: Radiologymentioning

confidence: 99%

Undifferentiated Embryonal Sarcoma of the Liver in Adults

Huang

2021

Liver Cancer

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Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignancy that most commonly affects the pediatric age group. This tumor very rarely occurs in adults and, in such instances, can pose a considerable diagnostic challenge for the clinicians, radiologists, and pathologists involved. The clinical presentation is most often non-specific, and the radiology shows a solid and cystic liver mass which has a considerable differential on imaging. Especially in adult patients, UESL is a diagnosis of exclusion meaning that all other diagnostic possibilities must be excluded before the diagnosis of UESL can be confidently made. From a pathology perspective, this means careful examination of the histology along with a comprehensive panel of immunohistochemistry for almost all cases of newly diagnosed UESL. The prognosis of UESL used to be dismal, but with advances in treatment and the introduction of a multimodality approach, there has been considerable progress in improving outcomes and survival for patients with this aggressive tumor. This chapter outlines the clinical, radiological, and pathological features of UESL. An in-depth discussion is undertaken to describe the diagnostic approach and the differential diagnosis for this rare and challenging tumor.

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Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332

Spunt,

Xue,

Gao

et al. 2024

Cancer

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BackgroundEmbryonal sarcoma of the liver (ESL) is a rare mesenchymal tumor most common in childhood; the optimal treatment approach is uncertain. The clinical features and outcomes of patients with ESL enrolled in a Children’s Oncology Group (COG) clinical trial that evaluated a risk‐based strategy for treating soft tissue sarcomas in patients aged <30 years were evaluated.MethodsThis subset analysis included patients with ESL enrolled in COG study ARST0332. Central review of records, pathology, and imaging confirmed the diagnosis, presenting features, and surgery extent and complications. All patients received dose‐intensive ifosfamide/doxorubicin chemotherapy, with cycle timing dependent on surgery and radiotherapy. Tumor resection occurred before study entry or after four cycles of chemotherapy; radiotherapy for residual tumor was optional.ResultsThirty‐nine eligible/evaluable patients with ESL were analyzed. All tumors were >10 cm in diameter; four were metastatic. Tumor resection was performed upfront in 23 and delayed in 16. Positive surgical margins (n = 6) and intraoperative tumor rupture (n = 6) occurred only in upfront resections. Eight patients received radiotherapy. Estimated 5‐year event‐free and overall survival were 79% (95% confidence interval [CI], 65%–93%) and 95% (95% CI, 87%–100%), respectively. Positive margins increased the local recurrence risk. One of 13 patients with documented hemorrhagic ascites and/or tumor rupture developed extrahepatic intra‐abdominal tumor recurrence.ConclusionsThe treatment strategy used in ARST0332 achieved favorable outcomes for patients with ESL despite a substantial proportion having high‐risk disease features. Deferring tumor resection until after neoadjuvant chemotherapy may decrease the risk of intraoperative tumor rupture and improve the likelihood of adequate surgical margins.

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Undifferentiated embryonal sarcoma of the liver mimicking venolymphatic malformation

Cited by 10 publications

References 7 publications

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Undifferentiated Embryonal Sarcoma of the Liver in Adults

Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332

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