Undifferentiated embryonal sarcoma of the liver in adults: Retrospective analysis of a case series and systematic review

Abstract

Adult undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignant tumor. As UESL is rare, the literature predominantly includes case reports, with a limited number of small case series. The aim of the present study was to investigate the presentation, treatment modalities and outcomes of this rare tumor. The present study includes a case series of adult UESL and a systematic review. A single-institution case series of adult UESL were retrospectively analyzed, and a systematic review of adult UESL was performed by searching MEDLINE, Web of Science, EMBASE, the Google Scholar database and the Cochrane Library. For all identified adult UESL cases, the demographic variables, treatments and survival were analyzed. Three female adult patients with UESL (median age, 21 years) were successfully treated by complete tumor resection, with or without adjuvant chemotherapy, at Beijing Tsinghua Changgung Hospital between 2015 and 2018. Of these patients, two are currently alive (follow-up, 9 and 41 months), and one died after pulmonary recurrence 17 months post-diagnosis. The present systematic review identified 108 cases of adult UESL. Among all 111 analyzed cases, the median overall and disease-free survival rates were as follows: 1-year, 72 and 67%; 3-year, 56 and 40%; and 5-year, 47 and 35%, respectively. Treatment strategies combining complete tumor resection and chemotherapy promoted improved overall and disease free survival time compared with radical tumor resection alone. The present analysis included one of the largest case series of UESL in adults, and is the first such study to present survival rates. The results of the present study confirmed that survival was improved by treatment strategies combining complete tumor resection and chemotherapy.

Adult versus paediatric undifferentiated embryonal sarcoma of the liver: a SEER database analysis

“…9,10 In 1978, Stocker and Ishak first used the term UESL to refer to this group of malignant mesenchymal neoplasms. 5,8 Because of its rarity, there are few reports on the prognosis of UESL, especially in adult patients. This study compared the characteristics and outcomes of 32 adult and 81 paediatric patients with UESL in the SEER database.…”

“…UESL was first described as a mesenchymoma in 1946 and a malignant mesenchymoma in 1948 9,10 . In 1978, Stocker and Ishak first used the term UESL to refer to this group of malignant mesenchymal neoplasms 5,8 . Because of its rarity, there are few reports on the prognosis of UESL, especially in adult patients.…”

“…In the literature, ages ranging from 15 to 22 were used to divide paediatric and adult groups. 3,[6][7][8]11,12 In the present study, after referring to more relevant literature aligning with our purpose, we defined an adult as being ≥18 years old. 6 Although rare in adults, UESL can occur at any stage of life, with a peak onset of 6-10 years of age.…”

“…In 2019, upon combining data from 130 studies and one case from their institute, Wu et al 7 performed a pooled individual data analysis of 308 patients with UESL, which included 89 patients aged ≥18 years. In 2020, Shu et al 8 analysed 111 patients aged >15 years, of whom 108 were identified through a literature review and three cases were identified from their institute. However, these studies were affected by intrinsic biases due to their specific design nature, and their findings were not externally validated.…”

New insights in gastrointestinal “pediatric” neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups

“…It accounts approximately for 6-13% of all hepatic pediatric malignancies, representing the third most frequent malignant tumor of the liver after hepatoblastoma and hepatocellular carcinoma in this age group 1 , 54-60 . Although ESL is classically considered a pediatric neoplasm, some cases have also been reported in adult patients with a predilection for women 58 , 61 , 62 . The etiology of ESL is largely unknown; based on the evidence that some cases of ESL may contain areas with mesenchymal hamartoma (MH)-like histology and/or share with this benign tumor the same chromosome translocation t(11;19) (q13;q13.4), a molecular continuum between these two entities has been suggested 63 , 64 .…”