Sarcoma Of Liver Research Articles

Clinicopathologic observation of pediatric undifferentiated embryonal sarcoma of liver

Clinicopathologic observation of pediatric undifferentiated embryonal sarcoma of liver

Clinicopathologic features and prognosis of inflammatory pseudotumor-like follicular dendritic cell sarcomas in liver and spleen: an analysis of seven cases

Objective: To investigate the clinicopathological features and prognostic parameters of the inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) of liver and spleen. Methods: Ninteen cases of inflammatory pseudotumor (IPT) and 5 cases of IPT-like FDCS of the liver and spleen were collected at the First Affiliated Hospital, Army Medical University from 2006 to 2016. HE sections, immunohistochemical staining, and Epstein-Barr virus encoded nuclear RNA (EBER) in situ hybridization were reviewed along with a summary of the literature. Results: Among the previously diagnosed 19 cases of IPT of the liver and spleen, 2 cases were misdiagnosed (the ratio of 2/19). Among 7 new cases including 3 males and 4 females, 3 cases involved the liver and 4 cases involved the spleen. The age range was 37-64 years (mean 53 years). The maximum tumor diameter ranged from 3.0 to 11.0 cm (mean 6.5 cm). Surgical resections were performed in all patients with follow-up time ranging from 3 to 84 months.All patients were disease-free.7 new cases were all positive for EBER, and showed the expression of at least one of the FDC markers, including CD21, CD23, and CD35. The rest of 17 cases of IPT were all negative for EBER and essentially negative for FDC markers, but were all positive for SMA. Conclusions: IPT-like FDCS of the liver and spleen is a rare low-grade malignant tumor morphologically mimicking inflammatory pseudotumor, and is easy to be misdiagnosis due to under-recognition. EBER in situ hybridization and FDC markers are indispensable for confirming the diagnosis.

Primary liver sarcomas in the modern era: Resection or transplantation?

Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients. We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS. Of 237 patients identified, the majority were female (60.8%), with median age of 52 years. Histologies were: epithelioid hemangioendothelioma (n = 67), angiosarcoma (n = 64), leiomyosarcoma (n = 33), embryonal rhabdomyosarcoma (n = 31), carcinosarcoma (n = 16), giant cell sarcoma (n = 14), spindle cell sarcoma (n = 12). Ninety-seven (40.9%) patients underwent lobectomies or extended lobectomies, 41 patients (17.3%) underwent transplantation. Surgical margins were negative in 82.9%. Tumors were well differentiated in 11.3%. Histology type correlated with outcome with the best prognosis for epithelioid hemangioendothelioma (OS: not reached, similar for resection and transplantation) and the worst for angiosarcoma (OS:16.6 mo with resection; 6 mo with transplantation; P = 0.04). Resections with microscopically negative margins were associated with improved survival (58.7 vs 11.3 mo for positive margins; P < 0.001). Chemotherapy and radiation therapy were used in a minority of patients (32.9% and 4.3% respectively) with no improvement in outcomes. Both hepatic resection and liver transplantation can be associated with long term survival for selected primary liver sarcomas such as epitheliod hemangioendotheliomas. Histology type and the ability to resect the tumor with negative margins correlate with outcomes and the decision to operate should be carefully weighed for subtypes with particularly dismal prognosis such as angiosarcomas.

Surgical Treatment and Chemotherapy of Adult Primary Liver Sarcoma: Experiences from a Single Hospital in China

Background: The aim of this study is to improve the preoperative diagnostic accuracy and treatment results by investigating the clinical features and prognosis of primary liver sarcoma (PLS). Methods: Clinical data, surgical treatments, adjuvant chemotherapy, and prognosis of 17 PLS patients whose diseases were pathologically confirmed were retrospectively analyzed. Results: The main clinical symptoms included epigastric pain in 9 patients, epigastric distention in 7, and loss of appetite in 4; these symptoms were detected during the postoperative follow-up for gastric carcinoma in 1. The resection rate was 64.7% (12/17), including R0 resection in 10 patients and R1 resection in 2, and laparotomy with biopsy in 5. Five patients accepted an adjuvant selective hepatic artery infusion chemotherapy (mitomycin C 16–20 mg+ 5-fluorouracil 5.0 g+ epirubicin 40–50 mg), and 4 accepted adjuvant systemic chemotherapy (vincristin, cisplatin, cyclophosphamide, and adriamycin). All 5 patients with simple laparotomy died within 1 year, and the overall 1-, 3-, and 5-year survival rates for all patients were 58.8% (10/17), 29.4% (5/17) and 11.7% (2/17), respectively, whereas those were 100.0% (10/10), 50.0% (5/10), and 20.0% (2/10) for R0 resected patients respectively. Conclusions: The diagnosis of PLS is difficult before operation due to its nonspecific manifestations, and the high survival rate can be achieved by radical resection with adjuvant chemotherapy.

Undifferentiated Embryonal Sarcoma of Liver- A Rare Case Report

Undifferentiated embryonal sarcoma of liver (USEL) is a rare primary hepatic malignancy principally affecting patients of pediatric age group. We present a case undifferentiated embryonal sarcoma of liver in a 1-year old female. She presented with complaints of abdominal distension and vomiting for 2 weeks. Ultrasonography showed grossly enlarged liver with multiple heterogenous lesions. Computed tomography revealed presence of large exophytic lesion of size 11.3 × 7 cm involving left lobe of liver. Ultrasound guided biopsy was performed, and specimen was sent for histopathological examination. On Microscopic examination undifferentiated round to spindle cells in myxoid background were seen. Immunohistochemistry revealed positivity for vimentin and AE1 thus proving undifferentiated embryonal sarcoma of liver. Diagnosis is often challenging because of overlapping epidemiological, clinical, radiological findings with those of other liver tumors.

Undifferentiated Embryonal Sarcoma Of Liver- A Rare Entity At Tertiary Cancer Centre

Undifferentiated Embryonal Sarcoma Of Liver- A Rare Entity At Tertiary Cancer Centre

Safety and Efficacy Outcomes of Embolization in Hepatic Sarcomas.

The outcome for patients with unresectable hepatic sarcoma is poor with a median survival period of 12-16 months. The purpose of this study was to evaluate liver-directed transcatheter therapies for the treatment of hepatic sarcomas. In a retrospective study, the cases of patients with primary and metastatic hepatic sarcoma treated by transcatheter embolization, chemoembolization, and 90Y radioembolization between 2004 and 2015 were identified. Response Evaluation Criteria in Solid Tumors version 1.1 response was assessed for the target tumor. Survival was assessed by means of Kaplan-Meier analysis. Twenty-eight patients (17 [61%] men, 11 [39%] women; median age, 47 years) were included. Eighteen patients were treated electively. Two of the electively treated patients underwent embolization; eight, chemoembolization; six, radioembolization; and two, a combination of transcatheter treatments. Treatment was well tolerated; only one patient had grade 3 hepatic toxicity. The objective response rate of the index tumor was 61%, and the median overall survival period was 26.7 months. Ten patients underwent emergency embolization to control acute hemorrhage from tumor rupture. The median overall survival periods were 611 days for the patients with ruptured gastrointestinal stromal tumors (GIST) (n = 3) and 19 days for the patients with ruptured angiosarcoma (n = 7). Liver-directed transcatheter therapies are safe and may have a role in the elective management of unresectable primary and metastatic liver sarcomas. Emergency embolization for ruptured GIST may be effective for stabilizing the patient's condition and allowing more definitive therapy in the future. However, emergency embolization has limited efficacy in treating patients with ruptured angiosarcoma, likely because of substantial venous bleeding at rupture and the aggressive behavior of this lesion.

Gram negative rod meningitis due to Strongyloides stercoralis

A 57-year-old male Mexican immigrant living in Dallas presented with altered mental status, progressive confusion, mild headache and fevers. He was diagnosed with embryonal liver sarcoma one year prior to admission and had recently started dexamethasone therapy for metastatic spinal lesions. Blood and cerebrospinal fluid cultures from a lumbar puncture were both positive for Escherichia coli. He was diagnosed with spontaneous gram negative rod (GNR) meningitis. Given his travel history, immunosuppression and GNR meningitis, a stool ova and parasite sample was obtained to screen for Strongyloides stercoralis. His stool was markedly positive for Strongyloides stercoralis larvae and he was further diagnosed with Strongyloides hyperinfection syndrome. Strongyloides is capable of chronically re-infecting human hosts without an external life cycle via autoinfection. In chronic infections, hyperinfection can be triggered with immunosuppressive medications, especially steroids. Disseminated Strongyloides should be considered as the source for unexplained GNR bacteremia or meningitis especially in immunosuppressed patients. Our patient likely had a chronic asymptomatic Strongyloides infection acquired in Mexico that became a hyperinfection resulting in GNR meningitis after starting high doses of dexamethasone.

A Case of Undifferentiated Liver Sarcoma in an Adult

A Case of Undifferentiated Liver Sarcoma in an Adult

Primary liver sarcoma: diagnosis and surgical treatment

Objective To study the diagnosis and surgical treatment of primary sarcoma of liver in order to obtain a better understanding of this disease and to improve its clinical treatment. Methods The clinical data on the clinicopathological features, surgical treatments and prognosis of 17 patients with primary liver sarcoma who were treated from January 2001 to May 2016 were retrospectively analyzed. Results Of 17 patients with primary sarcoma of liver, elevation of preoperative serum AFP was detected in one patient (5.9%), HBsAg positivity in 3 patients (17.6%), elevation of CEA in 2 patients (11.8%) and abnormal liver function in 3 patients (17.6%). The main clinical symptoms included epigastric pain in 9 patients, epigastric distention in 7 patients, loss of appetite in 5 patients (including in one patient after resection of gastric carcinoma). Twelve of these 17 patients underwent resection (resection rate 64.7%). Five patients underwent laparotomy and biopsy. Among patients who were treated with surgical resection, 10 patients had R0 resection and 2 patients had R1 resection. Postoperatively, 5 of these patients underwent adjuvant selective hepatic arterial infusion chemotherapy (mitomycin+ fluorouracil+ epirubicin), and 4 patients were treated with adjuvant systemic chemotherapy (vincristin, cisplatin, cyclophosphamide and Doxorubicin). The postoperative 1, 3 and 5-year overall survival rates for all the patients were 58.8% (10/17), 29.4% (5/17) and 11.7% (2/17), respectively. In patients with liver resection, the survival rates were 83.3% (10/12), 41.6% (5/12) and 16.7% (2/12), and for R0 resection, 100.0% (10/10), 50.0% (5/10) and 20.0% (2/10), respectively. Conclusions The diagnosis of primary sarcoma of liver was difficult before operation. High survival rate could be achieved by radical resection and adjuvant chemotherapy. Key words: Primary liver sarcoma; Liver tumor; Hepatectomy; Chemotherapy

Retroperitoneal Sarcoma Presenting as a Liver Mass; A Diagnostic Dilemma

Primary sarcoma of the liver is very rare and accounts for less than 1% of all liver malignancies. Retroperitoneal sarcomas, on the other hand, account for approximately 15% of all soft tissue sarcomas. Here we present a case where a retroperitoneal sarcoma presented like a primary liver mass. A 64-year-old man presented for evaluation of RUQ abdominal pain for 3 weeks. He admitted to a weight loss of 50 lb. along with night sweats, fevers, chills and one episode of black stool five days prior to presentation. His past medical history was remarkable only for HTN and gout. His vitals were normal. Abdominal exam elicited tenderness over the RUQ with voluntary guarding. He had a palpable enlarged liver, with no stigmata of chronic liver disease. His AST, ALT, bilirubin, alkaline phosphatase and GGT were elevated. Abdominal CT revealed large necrotic right liver mass measuring 8.3 x 7 x 9.3 cm. The mass extended beyond the liver capsule and was compressing the duodenum. Initial differential included HCC or Gall bladder carcinoma. The AFP was normal but the CA19-9 was mildly elevated. Workup for viral hepatitis was negative. Core biopsies of the liver mass were taken. Pathologic analysis revealed atypical spindle cells that were strongly positive for Vimentin. GIST was excluded as CD117 and DOG1 were negative. Markers for primary liver sarcomas like CD31 and CD34 were negative. Review of imaging showed the actual epicenter of the tumor in the retroperitoneum. MDM2 by FISH was negative which ruled out dedifferentiated liposarcoma. Markers for other retroperitoneal sarcomas were also negative. The final diagnosis was felt to be Undifferentiated Pleomorphic Sarcoma which is usually a diagnosis of exclusion. Patient's clinical course was complicated by upper GI bleeding, intestinal obstruction by the rapidly growing mass, which had more than doubled in size during this admission, and sepsis. Palliative radiation was begun. His family eventually decided to pursue comfort care & the patient expired. This case highlights the importance of liver biopsy, histopathology and immunohistochemistry in the diagnosis of atypical liver lesions. Early diagnosis and treatment is vital as sarcomas can sometimes rapidly progress as evidenced in this case. There has not been much literature regarding the local invasion of the liver by retroperitoneal sarcomas and this case can help shed light on that phenomenon.Figure 1Figure 2

肝切除後短期間で再発し，その後急速に進行して死亡した成人発症の肝未分化肉腫の1例

肝切除後短期間で再発し，その後急速に進行して死亡した成人発症の肝未分化肉腫の1例

Undifferentiated (embryonal) sarcoma of liver.

A 7-year-old male was brought to our out-patient department by his father with complaint of pain in the abdomen for 10 days. The parents noticed a swelling in the right upper abdomen a day after the pain started. He denied any past history of fever, jaundice, gastrointestinal blood loss or trauma. Examination revealed a non-tender swelling in the right hypochondrium which moved with respiration. Blood work, including tumor markers, was within normal limits. Contrast-enhanced computed tomographic (CECT) scan of abdomen was carried out which showed a large, hypodense, peripherally enhancing lesion involving the right lobe of liver (Panels A,B). The child underwent right hepatectomy which revealed a 15 cm × 12 cm sized solid exophytic tumor adhered to anterior abdominal wall and diaphragm focally. Grossly, the tumor showed a variegated appearance (Panel C). Histopathological examination revealed malignant nuclear features with cellular polymorphism (Panel D). Intracytoplasmic hyaline globules were also seen (Panel E) which were periodic acid-schiff (PAS) positive and diastase resistant. In addition, positivity for vimentin (Panel F), alpha-1-antitrypsin (Panel G) and Pan-CK (Panel H) was seen consistent with the diagnosis of undifferentiated (embryonal) sarcoma. The child had an uneventful recovery, being discharged 7 days after the surgery.

成人発症の肝未分化肉腫の1例

成人発症の肝未分化肉腫の1例

Embryonal sarcoma of the liver in a child patient: a report of one case

1 临床资料患儿女,6岁.因间断腹痛2个月于2012年12月12日入院.2012年10月患儿无明显诱因出现腹痛,以脐周为著,呈间歇性钝痛,与进食无关,无恶心、呕吐,无发热等不适,在当地医院诊断为肝囊肿并进行局部麻醉B超引导下肝囊肿穿刺术,引流出酱油色液体约450 mL,并注入平阳霉素5 mg,患儿腹痛症状缓解后出院.为求进一步诊断与治疗来我科就诊.入院后体格检查:右季肋区肋缘下可触及直径约5 cm质地坚硬包块,压痛阳性.既往无肝炎等特殊疾病史. 关键词：胚胎性肉瘤,肝脏;诊断;治疗

Undifferentiated embryonal liver sarcoma in childhood: A case report.

In order to improve the diagnosis and therapy of undifferentiated embryonal liver sarcoma (UELS), the present study presents the case of a 9-year-old female with UELS and discusses UELS in childhood. The patient presented with abdominal pain and fever. The laboratory tests, radiographic examination and pathological features presented by the female were similar to those of typical cases of UELS reported in childhood. The patient initially received surgical treatment and the immunohistochemical findings suggested that the patient had UELS. The patient’s parents refused adjuvant chemotherapy and demonstrated a right prerenal mass 6 months post-surgery. Microscopic examination revealed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. Previous studies have shown that patients who receive combined therapy with complete tumor resection and adjuvant chemotherapy have a longer survival time than those who undergo surgical therapy alone. Complete tumor resection combined with adjuvant chemotherapy may reduce the risk of recurrence and enhance the survival time in patients with UELS.

成人に発症した肝原発未分化肉腫の1例

成人に発症した肝原発未分化肉腫の1例

Undifferentiated embryonal sarcoma in adult liver.

Undifferentiated embryonal sarcoma (UES) is the third most common primary malignant liver tumor in children, following hepatoblastoma and hepatocellular carcinoma. UES of the liver was first named by Stocker and Ishak in 1978,1 and the majority of patients at the time ranged from 6 to 10 years of age. A recent review of the literature showed only 70 cases of UES in adults reported worldwide in 2008.2 This rare presentation is especially true for adults aged >60 years with only 14 cases of UES reported in this population until now.3,4,5,6,7 In Korea, eight cases of UES in adults have been reported: five cases were in women and four cases were in people older than 60 years.5,6,7,8 We present a new case of adult UES of the liver arising in a 67-year-old man. Like pediatric UES, the adult tumor has an unclear pathogenesis and poor prognosis.

Proton pump inhibitors as anti vacuolar-ATPases drugs: Preliminary findings in companion animals with spontaneous neoplasms treated with metronomic chemotherapy

Event Abstract Back to Event Proton pump inhibitors as anti vacuolar-ATPases drugs: Preliminary findings in companion animals with spontaneous neoplasms treated with metronomic chemotherapy Enrico Spugnini1* and Stefano Fais2 1 Regina Elena Cancer Institute, Italy 2 Istituto Supriore di Sanita, Italy Background and aim. The vacuolar ATPases are ATP-dependent proton pumps whose functions include the acidification of intracellular compartments and the extrusion of protons through the cell cytoplasmic membrane. These pumps play a pivotal role in the regulation of cell pH in normal cells and, to a much greater extent, in tumor cells. In fact, the glucose metabolism in hypoxic conditions by the neoplasms lead to an intercellular pH drift towards acidity. The acid microenvironment is modulated through the over-expression of H+ transporters that are also involved in tumor progression, invasiveness, distant spread and chemoresistance. Aim of the study was the evaluation of tolerability and efficacy of high dose proton pump inhibitors in dogs carrying relapsing or chemoresistant neoplasms. Methods. Dogs and cats with down/staged spontaneous neoplasms or with chemoresistant tumors were treated with omeprazole at the dose of 5 mg/kg from Monday through Wednesday and 1 mg/kg from Thursday to Saturday, combine with metronomic chemotherapy and a water alkalizer. Gastro-intestinal toxocity was scored accordingly to the Veterinary Comparative Oncology Group guidelines. Response to treatment was assessed on the basis of clinical evalutation and confirmatory biopsies. Results. Fourteen pets (thirteen dogs and one cat) have been recruited over a 18 months period. Age ranged from 8 to 15 years. Tumor types included: 1) anal sac carcinoma with sublumbar metastases T3N1M0; 2) thyroid carcinoma T4N0M1; 3) Liver sarcoma T3N1Mx; 4) Liver carcinoma T4N0M0; 5) Neck soft tissue grade III sarcoma TiN0M0; 6) Splenic HSA T3N1Mx; 7) Splenic HSA T3N0Mx; 8) inflammatory mammary carcinoma T3N1M0; 9) Metastatic mammary carcinoma T3N1M0; 10) Mammary carcinoma grade III T0NxMx (adjuvant therapy); 11) Soft tissue limb sarcoma T3NxM1; 12) Adenocarcinoma of nasal sinuses (T2N0M0); 13) Foreleg grade III anaplastic sarcoma T0NxMx (adjuvant therapy); 14) The cat had nasal chondrosarcoma T2N0M0. The treatment was overall well tolerated, one dog had mild diarrhea but continued the therapy albeit at a decreased dose. The responses were: Three complete remissions, 7 partial responses, 6 no evidence of disease (adjuvant therapy group). Conclusions / Discussion. The pump inhibitor lansoprazolo, administered at high dose has proven to enhance chemosensitivity and to prolong survival time in a spontaneous pet tumor modes and could be a useful addition to the current options for refractory neoplasms in consideration of its low cost, limited toxicity, and ease of administration.

A Single-institution Retrospective Cases Series of Childhood Undifferentiated Embryonal Liver Sarcoma (UELS)

Undifferentiated embryonal liver sarcoma (UELS) makes up 9% to 15% of all malignant liver tumors in children. UELS is characteristically diagnosed between the ages of 6 and 10 years and presents with abdominal pain, vomiting, and an abdominal mass. There is currently no standardized treatment for UELS except attempt at complete surgical resection. There have been only about 150 cases of UELS reported in the literature all with historically poor overall survival of <37.5% at 5 years. This report is one of the largest single-institution reports of UELS consisting of 5 patients over 2 decades. The purpose of this study is to characterize presentation and to report treatment success in UELS in children, adolescents, and young adults and the use of liver transplantation and, lastly, to suggest a use of positron emission tomography/computed tomography (PET/CT) in monitoring of this disease process. We conducted an Institutional Review Board-approved retrospective chart review. Data were collected from UELS patients younger than 21 years seen at the University of California Los Angeles over the past 20 years (January 2001 to September 2011). Descriptive analysis was conducted including multiple parameters of patient demographics, tumor characteristics, treatment modalities, and morbidity and mortality. Five patients with UELS were identified. Patients initially presented with fever, abdominal pain, or nausea. Ages ranged from 10 to 19 years old (median age 13 y old), and there was a 4:1 male-to-female predominance. Tumor size ranged from 6 to 22 cm in largest diameter. One patient presented with metastatic disease to the lungs and heart and 1 patient recurred 2 years from diagnosis with bilateral paraspinal masses. Treatment included local control surgery with neoadjuvant and adjuvant chemotherapy with an anthracycline/alkylating agent combination. One patient with recurrent and refractory disease achieved local control with an orthotopic liver transplantation (OLT). Metastatic disease was controlled with surgery and radiation therapy. 18-Fluorodeoxyglucose PET/CT was a useful imaging tool for judging response to therapy with complete loss of metabolic activity in tumor after neoadjuvant chemotherapy in 2 representative cases. Although follow-up is short for some patients, overall survival in these 5 patients was 100% with follow-up ranging from 21 to 68 months. Disease-free survival ranged from 8 to 46 months with no patients with residual disease. UELS is an aggressive high-grade primary liver sarcoma with high metastatic potential. This report represents one of the largest single-institution studies of UELS. Using multimodality therapy, patients have achieved 100% overall survival even in the setting of extensive disease, metastases, and recurrence. In cases of unresectable primary tumor or recurrent and refractory disease isolated to the liver, OLT is a potential therapeutic option. We report success with adjuvant chemotherapy and complete surgical resection with OLT as an alternative in unresectable or refractory cases. We also suggest a possible utility of PET/CT in monitoring treatment response in this disease.