Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children

Gabor, Flaviu; Franchi‐Abella, Stéphanie; Merli, Laura; Adamsbaum, C.; Pariente, D.

doi:10.1007/s00247-016-3670-3

Cited by 36 publications

(62 citation statements)

References 21 publications

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“…Hemorrhage also played a role in the cystic appearance, which was reported more frequently seen in PHSs than some other rare liver malignant tumors and attributed by rupture of the tumor for the serpiginous vessels (10,11). In previous studies, there was often extensive hemorrhage in PHS creating a huge cyst mass so that the underlying tumor was obscured and misdiagnosed as a hematoma, abscess or cystic tumor (28), which also occurred in our study.…”

Section: Discussionsupporting

confidence: 63%

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Clinical and imaging characteristics of primary hepatic sarcomatoid carcinoma and sarcoma: a comparative study

Shi

Sun

et al. 2020

Preprint

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Abstract Background：Primary hepatic sarcomatous carcinoma (PHSC) and primary hepatic sarcoma (PHS) are rare malignancies with frequent overlap in both the clinic and radiology. No comparative study of these tumors for the restricted cases has previously been undertaken. The purpose of our study was to analyze the clinical and imaging features of PHSCs and PHSs, with an emphasis on particularities and similarities through a comparison of the two tumors.Methods: We retrospectively analyzed the clinical and imaging features of 39 patients with pathologically proven PHSCs (n=23) and PHSs (n=16) from four university centers over a 9-year period from 2010-2019. Univariate analyses were performed to determine the consistent and distinctive features. Results：The background of chronic hepatitis or cirrhosis was observed with a high frequency in both of PHSCs (73.7%) and PHSs (62.5%). Tumors with a diameter greater than 10cm were significantly more common in PHSs than PHSCs (p=0.043) and cystic masses were more detected in PHSs (P=0.041). Both PHSCs and PHSs mainly presented hypovascularity (78.3% vs 81.3%). The ring hyper enhancement on the arterial phase (AP) and wash out were more frequently seen in PHSCs and the iso-hypo enhancement on the AP followed persistent or progressive enhancement was more commonly detected in PHSs (all, p<0.05). Conclusion：PHSC and PHS generally present as mass lesions with hypovascularity. The ring hyper enhancement on the AP and wash out favor the diagnosis of PHSC. The large size greater than 10cm, cystic lesion, iso-hypo persistent or progressive enhancement pattern might suggest the possibility of PHSs.

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Section: Discussionsupporting

confidence: 63%

“…Clinically, these tumors are usually asymptomatic until they become signi cantly large by the time of diagnosis, and most tumor markers are not sensitive (8)(9)(10). The ne needle biopsy usually failed to determine the nature of the mass due to its large size and insu cient samples.…”

Section: Introductionmentioning

confidence: 99%

Clinical and imaging characteristics of primary hepatic sarcomatoid carcinoma and sarcoma: a comparative study

Shi

Sun

et al. 2020

Preprint

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“…1 The initial CT abdomen during the portal venous phase in axial reconstruction (upper left) shows a hypodense lesion (*) in liver segment VII. In the MRI examination, the lesion had hypointense signal in the T1 vibe Dixon sequence (lower row on the left) and a hyperintensive signal in the T2 blade fs (bottom row on the right) hepatic lesion with a cystic component that represents areas of necrosis and hemorrhages [7,8]. In MRI studies, UESL are predominantly described as inhomogeneous T1-hypo-and T2-hyperintensity lesions with cystic parts [4,7].…”

Section: Discussionmentioning

confidence: 99%

“…In the MRI examination, the lesion had hypointense signal in the T1 vibe Dixon sequence (lower row on the left) and a hyperintensive signal in the T2 blade fs (bottom row on the right) hepatic lesion with a cystic component that represents areas of necrosis and hemorrhages [7,8]. In MRI studies, UESL are predominantly described as inhomogeneous T1-hypo-and T2-hyperintensity lesions with cystic parts [4,7]. The interaction of missing clinical symptoms and unspecific radiological characteristics contributes to the fact that most patients are diagnosed late so the tumors are already very large at the time of diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…The interaction of missing clinical symptoms and unspecific radiological characteristics contributes to the fact that most patients are diagnosed late so the tumors are already very large at the time of diagnosis. Gabor et al [7] described a case series of 15 patients with UESL with a mean tumor diameter of 13.5 cm and rapid growth in 2 of these patients. In our case, the tumor showed a size increase of approx.…”

Section: Discussionmentioning

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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Liver Ultrasound in the Paediatric Population

Panayiotou,

Sellars,

Deganello

2023

Liver Ultrasound

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Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children

Abstract: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.

Cited by 36 publications

References 21 publications

Clinical and imaging characteristics of primary hepatic sarcomatoid carcinoma and sarcoma: a comparative study

Clinical and imaging characteristics of primary hepatic sarcomatoid carcinoma and sarcoma: a comparative study

Paraneoplastic syndrome in undifferentiated embryonic sarcoma of the liver

Liver Ultrasound in the Paediatric Population

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