Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

The embryonal sarcoma of the liver is an extremely malignant, aggressive and infrequent condition of the liver, predominantly affecting children in the age groups of four to ten. The disease manifests with fever, abdominal pain or mass, nausea and laboratory findings suggestive of liver cell injury. The diagnosis can be made using tumor markers, imaging, immunohistochemistry and histopathology. The multimodal management protocol focuses the use of surgical resection, chemotherapy and radiation to significantly improved survival rates and quality of life in these patients

Extrahepatic undifferentiated embryonal sarcoma, cytology as an accurate method for early diagnosis: an extremely rare case report in an Indian child

Undifferentiated embryonal sarcoma has been described in the liver, a rare malignant mesenchymal neoplasm, that occurs primarily in children and teenagers. Approximately 260 cases have been reported arising in the liver since 1978 when this disease was first described. Its pathogenesis is still obscure. Authors presented a case of extrahepatic undifferentiated embryonal sarcoma in a 9-year-old female presenting with upper abdominal dull pain. Ultrasound and CT Scan showed normal liver architecture, with liver pushed upwards due to compression by tumor arising in the retroperitoneum. To the best of our knowledge, this is first case of extrahepatic undifferentiated embryonal sarcoma diagnosed on cytomorphology and confirmed by histopathology and immunohistochemistry markers.

CT and MRI features of undifferentiated embryonal sarcoma of liver in adults / 中华肝胆外科杂志

Objective@#To analyze the CT and MRI manifestations of undifferentiated hepatic embryonal sarcoma in adults with an aim to improve the preoperative diagnosis of this disease.@*Methods@#A retrospective analysis was carried out on the clinical data of 11 patients with undifferentiated hepatic embryonal sarcoma treated in Wenzhou People’s Hospital of Zhejiang and Wenzhou Central Hospital of Zhejiang between January 2008 and December 2018. There were 5 males and 6 females. The age ranged from 46 to 76 years (median 64 years). The site, size, morphology, margin, and pattern and degree of enhancement of the tumors were analyzed.@*Results@#Among the 11 patients, 10 patients had a single tumor, only 1 patient had multiple tumors. A total of seven patients' tumors were located in the right liver, 2 in the left liver, and 2 involved both the left and right livers. The maximum diameter of the tumors ranged from 5.5 cm to 21.2 cm, the maximum diameter (10.2±4.8 cm). The edge of tumors was clear in 9 patients and unclear in 2 patients. The tumors were elliptical in 4 patients or irregular in 7 patients. Plain CT scan showed 4 patients' tumors had mainly a cystic component, while 5 patients had both cystic and solid components. Plain MRI scan showed that five tumors were cystic and solid, with mixed internal signals. T1 weighted imaging (T1WI) showed slightly lower and lower signals; T2 weighted imaging (T2WI) showed slightly higher and higher signals. There was a low signal stripe separating inside; Diffusion-weighted imaging showed a high signal and apparent diffusion coefficient showed a low signal. The enhancement was characterized by "fast in and fast out" (marked enhancement in the arterial phase and fast clearance in the venous phase), and "delayed enhancement" (mild to moderate enhancement in the arterial phase and even more obvious enhancement in the venous or delayed phase).@*Conclusion@#Adult undifferentiated embryonic sarcoma has certain CT and MRI features. Its shape is elliptical or irregular, and mostly with a clear boundary. There were intratumoral cystic or cystic solid components, with mixed density/signal on CT and MRI, being characterized by "fast in and fast out" and "delayed enhancement" on intravenous contrast enhanced scans.

Undifferentiated embryonal sarcoma of the liver in an adult patient

Undifferentiated embryonal sarcoma of the liver (UESL) is rare primary hepatic sarcoma and is known to occur in pediatric patients. This case is the UESL occurred in a 51-year old male patient. Multilocular cystic lesion was composed of primitive spindle cells without specific differentiation. This rare case would help to review differential diagnosis of primary sarcoma in liver and cystic neoplasm of the liver.

Undifferentiated embryonal sarcoma of the liver in a young female: treatment with portal vein embolization and liver trisectonectomy

Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a tumor highly malignant, of mesenchymal origin. It is a rare finding in adults, though less rare in children. The strategy to be followed and the therapeutic targets to be reached for this tumor, in adult cases, remain ambiguous and controversial. Herein we report the case of a 29 year old female patient with a massive UESL and we describe our therapeutic approach. A 29 year-old female patient was referred to our center with severe intermittent epigastric pain and fever due to a voluminous liver tumor: Needle biopsy was of no specific findings and surgical excision was decided. Right portal vein embolization and selective embolization of the segment's IV branch was performed in order to achieve adequate future liver remnant (FLR). Right trisectonectomy was then performed, with uneventful post operative period and the patient was discharged at the 11th post operative day. UESL is a rare tumor that needs aggressive surgical approach and multidisciplinary team management is of paramount importance.

A Case of Embryonal Sarcoma of the Liver Mimicking a Hydatid Cyst in an Adult

An undifferentiated (embryonal) liver sarcoma (ULS) originates from a primitive mesenchymal cell, with a predilection for childhood and very rare occurrence in adults. We report a case of a ULS that was incidentally found in a 53-year-old female. Our case was initially interpreted as a large hydatid cyst, which was later suspected to be a neoplastic lesion because its size was increasing and a solid portion was newly detected after shrinkage of the cyst following drainage. The patient underwent successful right hepatic lobectomy with complete resection, and is currently disease-free without adjuvant therapy. Although it is difficult to diagnose a hepatic cyst as a ULS due to its rare occurrence in adulthood and lack of specific findings, its possibility should be considered, especially when its size is increasing, because early diagnosis and curative resection are necessary for a favorable outcome.

Embryonal Sarcoma of the Liver in an Adult

Undifferentiated (embryonal) sarcoma of the liver is a rare and highly malignant hepatic neoplasm of a mesenchymal origin. This tumor almost exclusively affects pediatric patients, and the prognosis is poor. Although there has been controversy as to the most appropriate treatment, the previous studies have reported that long term survival is possible after complete surgical resection with or without perioperative chemotherapy. We report here on a case of undifferentiated (embryonal) sarcoma of the liver, which showed cystic formation, in a 37-year-old woman with no prior history of any hepatitis or liver cirrhosis. She was admitted with right abdominal pain. The physical examination findings were unremarkable except for tenderness in the right abdomen.

A Case of Long-Term Survivor after Surgical Resection for Undifferentiated Embryonal Sarcoma of the Liver in Adult / 한국간담췌외과학회지

An undifferentiated (embryonal) sarcoma of the liver (USL) is a rare and highly malignant hepatic neoplasm of mesenchymal origin. This tumor almost exclusively affects pediatric patients, with a poor prognosis. The highest incidence is noted in pediatric patients, usually from 5~10 years of age, but only 43 cases of USL have been reported in adults worldwide since it was first as a clinicopathological entity. With a USL in adults, most patients are known to have died within 1 year of diagnosis. The absence of specific symptoms, rapid tumor growth, normality of common tumor markers and the consequential delay in diagnosis often result in significant enlargement of the tumor, with a poor prognosis. Various therapeutic modalities have been attempted in adult patients, but only a few long-term survivors have been reported. Herein, our experience of a 42-year-old female patient who is still alive, 55 months after the first resection for a USL is reported.

Undifferentiated (Embryonal) Sarcoma of the Liver in Children

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1years (range, 7 - 13years). Six patients presented with an abdominal mass and/or pain, and one with weight loss. Tumor size ranged from 8.0x8.0cm to 15.0x15.0cm. Four tumors were located in right lobe, two in left lobe and one in both. One patient died during preparation for chemotherapy. Initial complete resection was performed in three patients. Two patients received the complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferentiated (embryonal) sarcoma of the liver is highly malignant, we think that the combination therapy of surgery, chemotherapy and radiotherapy can get good results.

Undifferentiated Sarcoma of the Liver in Adult: A Case Report and Review of the Literature / 대한간학회지

Undifferentiated sarcoma is one of the rare primary malignant tumor of the liver typically occur ring in later childhood. It has also been referred to embryonal sarcoma or malignant mesenchymoma. In Korea, three child and two adult cases have been reported to date in liter ature. We experienced a case of undifferentiated sarcoma in a 65- year- old female who presented with abdominal pain and right upper quadrant mass . Contrast-enhanced abdominal CT scan revealed a well demarcated mass with low at tenuation and sept ation at left lobe of the liver and the mass showed hypovas cular pattern on hepatic angiography. On cross section, the tumor was well demarcated from the adjacent liver by afibr ouspseudocapsule and showed cystic degener ation. Pathologically, the tumor was consistent with an undifferentiated sarcoma of the liver.

Undifferentiated ( Embryonal ) Sarcoma of the Liver: A case report including immunohistochemical, electronmicroscopic and flow-cytometric study

Undifferentiated(embryonal) sarcoma of the liver developing predominantly in the late childhood is extremely rare in malignant hepatic tumors. It has been described by a variety of terms such as malignant mesenchymoma, embryonal sarcoma, fibromyxosarcoma, embryonal rhabdomyosarcoma or primary sarcoma of the liver. The tumor usually presents a huge mass containing solid and sometimes cystic components, and is cmposed of large stellate and spindle cells in an abundant myxoid matrix. The treatment of choice is surgical resection with or without adjuvant chemotherapy and/or radiotherapy, but the prognosis of this disease has been reported extremly poor. We experienced a case of undifferentiated(embryonal) sarcoma of the liver recently which was confirmed by pathological examinations in a 18-year-old boy. He visited our hospital for examination of the progressive growing RUQ abdominal mass detected incidentally. On physical examination, a firm, non-tender, hard, fixed, huge, abdominal mass was palpated in the right upper quadrant of abdomen. Abdominal ultrasonography, computerized tomography and magnetic resonance imaging showed a 10x15cm sized mass confined to the left lobe of the liver, and it was hypovascular mass on angiography. We performed left lobectomy including the tumor and it was confirmed as undifferentiated (embryonal) sarcoma of the liver by pathologic examination. We herein report this case including histologic, electronmicroscopic and flow cytometric results with the review of literatures.

Embryonal Sarcoma of the Liver with Chondrosarcomatous Differentiation: A case report

Embryonal sarcomas of the liver, often called undifferentiated sarcomas or malignant mesenchymomas, are extremely rare tumors that occur chiefly in children with poor prognosis and uncertain histogenesis. Histologically, tumor cells are stellate or spindle shaped, loosely arranged in edematous or myxoid pattern alternating with highly cellular zones and do not show evidence of differentiation. Ultrastructural and immunohistochemical studies by previous investigators indicate that the neoplastic cells are mostly primitive mesenchymal cell which occasionally differentiate to fibroblasts, lipoblasts, histiocytes and smooth muscle cells via intermediate cell types. The authors experienced a case of 6-year old boy who presented with an enlarging abdominal mass of the liver which revealed typical histological and ultrastructural features of embryonal sarcoma with focal areas of unusual chondrosarcomatous differentiation.