Undifferentiated (Embryonal) Sarcoma of the Liver: An Intriguing Diagnosis in a Child

Boybeyi, Özlem; Karnak, İbrahìm; Orhan, Dıclehan; Akçören, Zühal; Tanyel, Feridun Cahit

doi:10.1055/s-2008-1039051

Cited by 8 publications

(12 citation statements)

References 15 publications

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“…UES of the liver is a rare, malignant hepatic neoplasm of mesenchymal origin [ 93 ] and is also known as fi bromyxoid sarcoma, malignant mesenchymoma, and embryonal sarcoma [ 94 ].…”

Section: Undifferentiated Embryonal Sarcoma (Ues)mentioning

confidence: 99%

“…Males and females are equally affected [ 93 ]. Patients present with an abdominal mass, abdominal pain, nausea, anorexia, intermittent fever, or headache [ 93 , 96 ].…”

Section: Clinical Features and Epidemiologymentioning

confidence: 99%

“…9.25 and 9.26 ) UES usually arises in the right lobe of the liver [ 93 ]. Metastases typically involve the lungs, pleura, and peritoneum [ 64 ].…”

Section: Clinical Features and Epidemiologymentioning

confidence: 99%

“…9.27a ). The cellular regions frequently feature multinucleated giant cells and marked pleomorphism [ 93 ]. Intracellular PAS-positive, diastase-resistant hyaline globules occur commonly (Fig.…”

Section: Pathologymentioning

confidence: 99%

“…Hyaline globules may show reactivity with alpha-1 antitrypsin antibodies, and they stain positively with PASD. Ki-67 stains demonstrate an elevated proliferation index [ 93 ] Molecular diagnostic features and cytogenetics : Both mesenchymal hamartomas and their associated UES frequently harbor rearrangements of band 19q13.4, including the translocation t(11;19)(q13;q13.4). The breakpoint at 11q13 occurs in the MALAT1 gene, also known as ALPHA , which is rearranged in renal tumors harboring the t(6;11)(p21;q13) translocation.…”

Section: Pathologymentioning

confidence: 99%

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Gastrointestinal, Pancreatic and Hepatic Malignancies in Children

Towbin

Rowland

Parham

2014

Pediatric Malignancies: Pathology and Imaging

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The gastrointestinal tract, liver, and pancreas are uncommon sites for primary malignant neoplasms in the pediatric population. None of the individual malignant neoplasms of these sites represented more than 2 % of cancer diagnoses in children between 0 and 19 years of age in the SEER database [ 1 ]. This is in marked contrast to the frequency of malignant neoplasms of these sites in adulthood. Gastrointestinal TractLymphoma is the most common pediatric gastrointestinal tract malignancy, accounting for approximately 75 % of malignancies in most series. This is followed by neuroendocrine tumors (NET), adenocarcinoma, and sarcoma [ 2 -4 ]. Carcinomas and a variety of rare neoplasms can occur in the gastrointestinal tract but are not addressed in this chapter. Lymphoma is discussed in another chapter.

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“…UES of the liver is a rare, malignant hepatic neoplasm of mesenchymal origin [ 93 ] and is also known as fi bromyxoid sarcoma, malignant mesenchymoma, and embryonal sarcoma [ 94 ].…”

Section: Undifferentiated Embryonal Sarcoma (Ues)mentioning

confidence: 99%

“…Males and females are equally affected [ 93 ]. Patients present with an abdominal mass, abdominal pain, nausea, anorexia, intermittent fever, or headache [ 93 , 96 ].…”

Section: Clinical Features and Epidemiologymentioning

confidence: 99%

“…9.25 and 9.26 ) UES usually arises in the right lobe of the liver [ 93 ]. Metastases typically involve the lungs, pleura, and peritoneum [ 64 ].…”

Section: Clinical Features and Epidemiologymentioning

confidence: 99%

Section: Pathologymentioning

confidence: 99%

Section: Pathologymentioning

confidence: 99%

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Gastrointestinal, Pancreatic and Hepatic Malignancies in Children

Towbin

Rowland

Parham

2014

Pediatric Malignancies: Pathology and Imaging

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Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma

et al. 2013

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The outcomes of hepatic undifferentiated embryonal sarcoma (HUES) have historically been limited by persistent, unresectable disease and the subsequent development of disease resistance and dissemination. We present our institutional experience with HUES and assess current treatment trends and outcomes in the era of liver transplantation. We conducted a retrospective chart review of cases presenting with HUES at our institution over the past 10 years. The collected data included age, sex, presenting symptoms, imaging and the associated Pretreatment Extent of Disease (PRETEXT) score, pathology, chemotherapy, surgical interventions, and outcomes. Approval was obtained from the institutional review board of the Cincinnati Children's Hospital Medical Center. HUES was identified in 6 patients (4 males and 2 females) with a median age at diagnosis of 11 years (range 5 7-13 years). Initial imaging was available for all but 1 patient. The PRETEXT stage for these patients ranged from II to III. One patient was diagnosed with lung metastases. Two patients underwent upfront resection, and 1 patient received neoadjuvant therapy and then conventional resection. Three patients were treated with orthotopic liver transplantation (OLT) after neoadjuvant chemotherapy (primary OLT in 2 cases and salvage OLT for local recurrence in 1 case). Two patients received posttransplant adjuvant chemotherapy. All 6 patients remained in clinical remission with a mean follow-up of 35 months (range 5 12-84 months). In conclusion, OLT has rarely been reported as a treatment option for HUES. The addition of liver transplantation as a surgical option for treating patients with HUES can result in improved survival for patients whose tumors are initially unresectable or recur.

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Undifferentiated sarcoma of the liver: Multicenter international experience of the Cooperative Soft‐Tissue Sarcoma Group and Polish Paediatric Solid Tumor Group

Murawski

Scheer

Leuschner

et al. 2020

Pediatric Blood & Cancer

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Background: Undifferentiated embryonal sarcomas of the liver (UESL) are extremely rare and continue to pose a diagnostic and therapeutic challenge. The aim of the study was to present a multicenter experience of the German CWS and Polish PPSTG groups in the treatment of UESL in children. Procedure: Twenty-five patients were treated according to the CWS-96, CWS-2002, and CYVADIC protocols. Distant metastases were observed in four cases (16%). In four cases, an initial disease presentation mimicked other entities. A pure cystic appearance of liver mass led to misdiagnosis of hydatid cyst in three cases. In one case, laparotomy was performed due to the signs of appendicitis, and bleeding from ruptured liver tumor was found. All these patients were finally diagnosed as UESL. Results: Thirteen patients received preoperative chemotherapy. Partial response was observed in 10 cases. Tumor resection was performed in 20 patients (primary resections, 12; delayed resections-, 8). In five patients, the primary tumor never became operable. The macroscopically complete resection rate was 95% (19/20). Postoperative chemotherapy was given to 20 children. Local radiotherapy was used in three children. After a median follow-up time of 136 months, 17 patients (68%) were alive with no evidence of disease. All children with unresectable tumor and three out of four patients with distant metastases died. The five-year overall survival (OS) rate was 72%. Conclusions: In summary, a complete tumor excision plays the central role in the treatment of UESL. A cystic presentation of the liver lesion on imaging does not exclude the diagnosis of malignant tumor.

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Undifferentiated (Embryonal) Sarcoma of the Liver: An Intriguing Diagnosis in a Child

Cited by 8 publications

References 15 publications

Gastrointestinal, Pancreatic and Hepatic Malignancies in Children

Gastrointestinal, Pancreatic and Hepatic Malignancies in Children

Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma

Undifferentiated sarcoma of the liver: Multicenter international experience of the Cooperative Soft‐Tissue Sarcoma Group and Polish Paediatric Solid Tumor Group

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