• Liver angiosarcoma
• Population-based study
• SEER
• Survival analysis

• Humans
• Hemangiosarcoma/mortality
• Hemangiosarcoma/therapy
• Hemangiosarcoma/pathology
• Male
• Female
• Middle Aged
• Liver Neoplasms/mortality
• Liver Neoplasms/therapy
• Liver Neoplasms/pathology
• Retrospective Studies
• Aged
• Prognosis
• Survival Rate
• SEER Program/statistics & numerical data
• Aged, 80 and over
• Adult
• Neoplasm Staging

• Case report
• Computed tomography scan
• Immunohistochemistry
• Liver abscess
• Magnetic resonance imaging
• Primary hepatic leiomyosarcoma

• Cox regression analyses
• SEER
• general surgery
• hepatic epithelioid hemangioendothelioma
• machine learning

• Humans
• Hemangioendothelioma, Epithelioid/therapy
• Hemangioendothelioma, Epithelioid/pathology
• Hemangioendothelioma, Epithelioid/mortality
• Hemangioendothelioma, Epithelioid/diagnosis
• Male
• Female
• Liver Neoplasms/therapy
• Liver Neoplasms/mortality
• Liver Neoplasms/pathology
• Liver Neoplasms/diagnosis
• Middle Aged
• SEER Program
• Adult
• Aged
• Prognosis
• Cohort Studies
• Treatment Outcome

• liver tumor
• malignancy
• mesenchymal neoplasms
• metastasis
• solitary fibrous tumor

• bone metastasis
• extrahepatic metastasis
• hepatic epithelioid hemangioendothelioma (HEHE)
• liver resection
• transplant surgery

• Humans
• Hemangioendothelioma, Epithelioid/pathology
• Hemangioendothelioma, Epithelioid/mortality
• Hemangioendothelioma, Epithelioid/surgery
• Female
• Male
• Liver Neoplasms/surgery
• Liver Neoplasms/pathology
• Liver Neoplasms/mortality
• Liver Neoplasms/therapy
• Retrospective Studies
• Adult
• Middle Aged
• Survival Rate
• Liver Transplantation
• Hepatectomy/mortality
• Follow-Up Studies
• Aged
• Prognosis
• Young Adult

• The authors received no specific funding for this work.

• Humans
• Female
• Leiomyosarcoma/diagnosis
• Leiomyosarcoma/pathology
• Middle Aged
• Liver Neoplasms/diagnosis
• Liver Neoplasms/pathology
• Focal Nodular Hyperplasia/diagnosis
• Focal Nodular Hyperplasia/pathology
• Diagnosis, Differential
• Liver/pathology
• Liver/diagnostic imaging
• Immunohistochemistry
• Hepatectomy
• Biomarkers, Tumor/analysis
• Biopsy
• Tomography, X-Ray Computed

• autopsy
• genetic mutation
• hepatic leiomyosarcoma

• 20K08312 JSPS KAKENHI

• Case report
• Colon polyp
• Hot snare polypectomy
• Maxillary
• Pleomorphic leiomyosarcoma
• Spindle cell tumor

• liver angiosarcoma
• liver tumour
• outcomes
• surgery
• treatment

• hepatic sarcoma
• liver cancer
• liver sarcoma
• visceral sarcoma

• Humans
• Hemangiosarcoma/pathology
• Sarcoma/surgery
• Sarcoma/pathology
• Multivariate Analysis
• Liver Neoplasms/surgery
• Soft Tissue Neoplasms/surgery
• Prognosis
• SEER Program
• Survival Rate
• Retrospective Studies

• case report
• hepatic sarcoma
• neoplasm of the liver
• surgery of de liver
• undifferentiated embryonal sarcoma of the liver

This article examines primary hepatic angiosarcoma (PHA) and fat-poor angiomyolipoma (AML), two uncommon vascular cancers. Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques. Uncommon malignant tumors of the vascular endothelium include PHA. Another diagnosis that should not be overlooked when employing contrast-enhanced MR and contrast-enhanced computed tomography (CT) imaging techniques is fat-poor AML, one of the uncommon vascular tumors of the liver. In both conditions, biopsy is the primary means of diagnosis.

In our article, besides the diagnosis of PHA, fat-poor AML, one of the other rare vascular tumors of the liver, is mentioned. In the case, a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain, weight loss, and nausea. Abdominal ultrasonography (US) revealed a hypoechoic heterogeneous lesion with occasional faint contours. In computed tomography, it was observed as a hyperdense nodular lesion in segment 4. Magnetic resonance imaging (MRI) revealed that the lesion did not contain fat. In connection with the known history of VHL Syndrome, we first evaluated the possibility of AML. Thereupon, a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5% fat content.

In conclusion, PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences. Important imaging techniques like contrast-enhanced US (CEUS), CECT, and CEMRI give us substantial advantages in both cases. However, a biopsy is used to provide the final diagnosis.

• Primary hepatic angiosarcoma
• Hepatic angiomyolipoma
• Ultrasonic diagnosis
• Imaging
• Pathology
• Case report

Primary hepatic leiomyosarcoma (PHL) is a rare tumor with a very low incidence of about 0.2%.

A 48-year-old diabetic, hypertensive, and morbidly obese female patient presented with a history of abdominal pain and weight loss for 2 mo. She had no history of fever, jaundice, or other liver disease(s). Clinical examination revealed a palpable mass in the epigastrium. Imaging evaluation with a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis revealed an ill-defined enhancing hyper vascular hepatic mass of 9.9 cm × 7.8 cm occupying the left hepatic lobe with evidence of central necrosis, compression effect on the left hepatic vein, and partial wash-out on delayed images. On further workup, the maximum standardized uptake value on positron emission computed tomography scan was 6.4, which was suggestive of malignancy. The remaining part of the liver was normal without any evidence of cirrhosis. Ultrasound-guided biopsy of the mass showed smooth muscle neoplasm suggestive of leiomyosarcoma. After optimization for co-morbidities, an extended left hepatectomy was planned in a multidisciplinary team meeting. On intraoperative ultrasound, the left hepatic lobe was entirely replaced by a large tumor extending to the caudate lobe with a compression effect on the middle and left hepatic veins. Final histopathology showed nodular and whorled white tumor comprised of spindled/fascicular cells with moderate to severe pleomorphism and focal necrosis. The mitotic index was greater than 20 mitoses per 10 high-power fields. The resection margins were free of tumor. Immunohistochemistry (IHC) depicted a desmin-positive/ caldesmon-negative/discovered on gastrointestinal stromal tumor 1-negative/ cluster of differentiation 117-negative profile, confirming the definitive diagnosis as PHL.

This case report highlights the rare malignant mesenchymal hepatic tumor. To confirm PHL diagnosis, one requires peculiar histopathological findings with ancillary IHC confirmation. Management options include adequate/complete surgical resection followed by chemotherapy and/or radiotherapy.

• Leiomyosarcoma
• Immunohistochemistry
• Hepatectomy
• Surgical resection
• Case Report

• Female
• Fluorodeoxyglucose F18
• Hemangiosarcoma/diagnostic imaging
• Humans
• Liver Failure, Acute
• Liver Neoplasms/diagnostic imaging
• Middle Aged
• Positron Emission Tomography Computed Tomography
• Radiopharmaceuticals

Hepatic angiosarcoma is a very rare disease, but it has a poor prognosis. Here, we report the case of a 77-year-old man who was referred to our hospital for suspicion of hepatocellular carcinoma and cancerous peritonitis. Based on the imaging findings, a diagnosis of spontaneously ruptured hepatic hemangioma was made. Six days later, the patient was transported to the hospital in a state of shock and an emergency transarterial embolization was performed. He underwent lateral hepatic segmentectomy 7 days later. Histopathologically, he was diagnosed with hepatic angiosarcoma. Fever was observed 21 days after surgery, and computed tomography was performed. Multiple liver masses, which ware increasing rapidly, were found, and hepatic angiosarcoma recurrence was confirmed. He requested home medical care and died at home 36 days after surgery. When a tumor diagnosed as a hepatic hemangioma by imaging has ruptured, the possibility of hepatic angiosarcoma should be considered.

• Case report
• Hepatic angiosarcoma
• Hepatic sinusoidal obstruction syndrome
• High mortality
• Outcome
• Primary cancer

Adenosarcoma is a rare type of tumor with a mixture of epithelial and stromal components and often occurs in the female reproductive system. Primary hepatic adenosarcoma (PHAS) is extremely rare, with only two cases reported so far. Both patients had poor outcomes. Here, we report the case of a 36-year-old man with pain under the xiphoid process who was diagnosed with a bile duct tumor. He was treated with adjuvant radiotherapy when surgery was performed on him. Pathologically, the tumor contained benign epithelial tissue, and the submucosa of the bile duct in the liver showed infiltrating growth of spindle cell components. The cells were dense, mildly heterotypic, and occasionally mitotic, and the patient was diagnosed with PHAS. Whole-exome sequencing results showed that a total of 12 mutations were shared by the two tissues. The patient received adjuvant radiotherapy and he was tumor-free until 31 months postoperatively. This case will provide some references of the disease to other researchers.

• Conversion surgery
• Hepatic angiosarcoma
• Preoperative chemotherapy

Sarcomas of the liver are a rare and aggressive group of malignancies for which surgery is the preferred treatment modality even though most patients are not surgical candidates and receive chemotherapy with poor outcomes. In these cases, trans-arterial liver-directed therapies are emerging as a new treatment option. Among these, radioembolization is a promising but understudied treatment option. In radioembolization, microbeads conjugated to a radioactive drug are injected into the blood vessels, nourishing the cancers and leading to cell death and tumor shrinkage. In this study, we retrospectively analyzed 35 patients with liver sarcomas receiving radioembolization at our institution. We found that those with disease control in the liver 6 months after the procedure had longer overall survival as well as patients with a liver progression-free interval post-procedure equal to or greater than 9 months. Patients with good performance status and normal liver function at baseline also had longer survival. The most common adverse reactions were nausea, fatigue, abdominal pain, and mild reversible abnormalities in liver function tests. Overall, our results suggest that radioembolization might be a safe and effective treatment option for patients with unresectable liver sarcomas.

Patients with liver-dominant metastatic or primary hepatic soft tissue sarcomas (STS) have poor prognosis. Surgery can prolong survival, but most patients are not surgical candidates, and treatment response is limited with systemic chemotherapy. Liver-directed therapies have been increasingly employed in this setting, and Yttrium-90 trans-arterial radioembolization (TARE) is an understudied yet promising treatment option. This is a retrospective analysis of 35 patients with metastatic or primary hepatic STS who underwent TARE at a single institution between 2006 and 2020. The primary outcomes that were measured were overall survival (OS), liver progression-free survival (LPFS), and radiologic tumor response. Clinical and biochemical toxicities were assessed 3 months after the procedure. Median OS was 20 months (95% CI: 13.9–26.1 months), while median LPFS was 9 months (95% CI: 6.2–11.8 months). The objective response rate was 56.7%, and the disease control rate was 80.0% by mRECIST at 3 months. The following correlated with better OS post-TARE: liver disease control (DC) at 6 months (median OS: 40 vs. 17 months, p = 0.007); LPFS ≥ 9 months (median OS: 50 vs. 8 months, p < 0.0001); ECOG status 0–1 vs. 2 (median OS: 22 vs. 6 months, p = 0.042); CTP class A vs. B (median OS: 22 vs. 6 months, p = 0.018); and TACE post-progression (median OS: 99 vs. 16 months, p = 0.003). The absence of metastases at diagnosis was correlated with higher median LPFS (7 vs. 1 months, p = 0.036). Two grade 4 (5.7%) and ten grade 3 (28.6%) laboratory toxicities were identified at 3 months. There was one case of radioembolization-induced liver disease and two cases of radiation-induced peptic ulcer disease. We concluded that TARE could be an effective and safe treatment option for patients with metastatic or primary hepatic STS with good tumor response rates, low incidence of severe toxicity, and longer survival in patients with liver disease control post-TARE.

• Yttrium-90
• liver
• soft-tissue sarcomas
• trans-arterial radioembolization

Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

•

This consensus paper provides key recommendations on the management of epithelioid hemangioendothelioma (EHE).

• diagnosis
• epithelioid hemangioendothelioma
• guidelines
• management
• sarcoma
• treatment

Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it’s rarity. However, with development of medical technology, surgical treatment may lead to better survival.

To investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute.

From August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach.

The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain (P = 0.002).

Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.

• Liver
• Angiosarcoma
• Undifferentiated sarcoma
• Operation
• Survival
• Recurrence

• Clinical characteristics
• Hepatic lobectomy
• Primary hepatic angiosarcoma
• Surgical treatments
• Transarterial chemoembolization

• Liver
• ROS1
• angiosarcoma
• cancer
• prognosis
• target therapy

With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.

• Hepatic epithelioid hemangioendothelioma
• Diagnosis
• Differential diagnosis
• Therapy
• Prognosis
• Imaging

• Body imaging
• Cholangiocarcinoma
• Hepatocellular carcinoma
• Liver imaging

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 liver undifferentiated embryonal sarcoma
• chemotherapy
• surgery
• survival

Comparisons of hepatic epithelioid hemangioendothelioma (HEHE), hepatic hemangioma, and hepatic angiosarcoma (HAS) have rarely been reported. The purpose of our study was to analyze the clinical and magnetic resonance imaging (MRI) findings of these conditions.

A total of 57 patients (25 with hemangioma, 13 with HEHE, and 19 with HAS) provided hepatic vascular endothelial cell data between June 2006 and May 2017.

The proportions of cases with circumscribed margins were 88% (22/25), 84.6% (11/13), and 31.6% (6/19) for hemangioma, HEHE, and HAS, respectively (P < 0.001). HAS lesions were less likely to have circumscribed margins. The proportions of lesions with hemorrhaging were 4% (1/25), 30.8% (4/13), and 36.8% (7/19) for hemangioma, HEHE, and HAS, respectively (P = 0.014). HEHE and HAS cases were more likely to show heterogeneous signals on T1-weighted (T1WI) MRI. HEHE and HAS cases were more likely to show heterogeneous signals on T2-weighted (T2WI) MRI. Centripetal enhancement was the most common pattern in vascular tumors, with proportions of 100, 46.2% (6/13), and 68.4% (13/19) for hemangioma, HEHE, and HAS, respectively. The difference in enhancement pattern between HEHE and HAS was not significant, but rim enhancement was more common for HEHE (46.2%, 6/13).

Our study revealed clinical and imaging differences between HEHE and HAS. The platelet count (PLT) and coagulation function of the HAS group decreased, whereas the alpha-fetoprotein (AFP) level increased. The 5-year survival rate for HAS was significantly lower than that of HEHE. A higher malignancy degree indicated a more blurred lesion margin, easier occurrence of hemorrhaging, and more heterogeneous T1WI and T2WI signals.

• Angiosarcoma
• Epithelioid hemangioendothelioma
• Hemangioma
• Liver
• Magnetic resonance imaging

A total of 185 cases (150 retrospectively and 35 prospectively) of malignant liver masses were collected. In the retrospectively collected cases hyperenhancement during wash-in was the most common feature in HCCs but there was a high percentage of cases showing no enhancement or hypo/isoenhancement. ICCs displayed a large variety of contrast enhancement patterns and, although statically significant differences between ICCs and HCCs were evident, no clear distinction between these two pathologies was possible based only on their CEUS appearance. Sarcomas displayed all the possible degrees of wash-in enhancement with non-enhancing being the most common appearance. Metastases displayed all the possible contrast-enhancement patterns, with the most common being hyperenhancement in the wash-in phase followed by hypoenhancement in the wash-out phase. A decision tree was developed based on the features of the retrospectively selected cases. Based on the developed decision tree 27/35 prospectively collected cases were correctly classified. Even if some significant differences among groups were evident, all the histotypes displayed all the possible patterns of contrast enhancement, and, therefore, the differentiation of liver masses in dogs based only on their CEUS features is not feasible and, therefore, cytology or histopathology is required.

• Biliary adenosarcoma
• Liver
• Sarcoma

Hepatic epithelioid hemangioendothelioma (EHE) is extremely rare, and preoperative diagnosis is difficult because hepatic EHE has clinicopathological features that are similar to those of angiosarcoma. However, it is important to differentiate hepatic EHE from angiosarcoma because the latter is an aggressive tumor with poor prognosis. We herein report a case of hepatic EHE that was difficult to distinguish from angiosarcoma by tumor biopsy. A 30-year-old man with Crohn's disease presented with multiple liver tumors. The tumors were preoperatively diagnosed as angiosarcoma by tumor biopsy. The patient underwent extended left hemihepatectomy with biliary reconstruction and partial resection of segments 6 and 8. Immunohistochemical staining was positive for CD34, factor VIII, and calmodulin binding transcription activator 1 (CAMTA1), and the pathological diagnosis was EHE. Two years after surgery, a recurrent tumor was found in liver segment 6, for which laparoscopic partial hepatectomy was performed. Pathological examination revealed recurrence of EHE. The patient remained well with no evidence of tumor recurrence as of 9 months after the second resection. In conclusion, we described a case of hepatic EHE that was initially diagnosed as angiosarcoma on biopsy. Immunohistochemical staining with CAMTA1 may help distinguish EHE from angiosarcoma.

• Angiosarcoma
• CAMTA1
• Differential diagnosis
• Epithelioid hemangioendothelioma
• Repeat hepatectomy

•

First Case Report about ALPPS for the treatment of an undifferentiated embryonal sarcoma of the Liver.

Embryonal sarcomas of the liver (ESL) are extremely rare solid tumors appearing mainly in children. The therapeutic standard for an ESL is a margin free resection combined with chemotherapy. The Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) procedure as a surgical therapy offers a curative approach for liver tumors of various origins where the future liver remnant (FLR) would be insufficient after a one-staged (extended) hemihepatectomy.

A 19-year-old patient was diagnosed with an undifferentiated embryonal sarcoma of the liver (UESL) in the right liver lobe with oligometastatic spread to the lungs.

After neoadjuvant chemotherapy remission was enough to plan a resection of the liver tumor. During the operation we changed our strategy from one-stage hepatectomy to ALPPS because of borderline FLR and macroscopic and histologic liver damage to avoid posthepatectomy liver failure. The interstage and postoperative course of the patient was uneventful beside postoperative bile leakage, which was treated by interventional drainage and stenting.

The ALPPS-procedure as a comparatively new surgery was considered over a portal vein ligation or embolization. ALPPS shows a faster hypertrophy compared to standard one-staged hemihepatectomy with decreased or similar proliferation, apoptosis or angiogenesis (at least for CRLM)

In experienced centers the ALPPS-procedure is evolving as the safer approach in hemihepatectomys where the FLR is critical. Additionally, ALPPS can serve as an intraoperative option when liver volume and quality seem not to be sufficient and is to be considered when facing new tumor-entities.

• Associating liver partition and portal vein ligation for staged hepatectomy
• Liver resection
• Surgery
• Undifferentiated embryonal sarcoma of the liver

• Adult
• Aged
• Aged, 80 and over
• Carcinoma, Hepatocellular/diagnostic imaging
• Carcinoma, Hepatocellular/pathology
• Electronic Health Records
• Female
• Hemangiosarcoma/diagnostic imaging
• Hemangiosarcoma/pathology
• Humans
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/pathology
• Male
• Middle Aged
• Neoplasm Staging
• Prognosis
• Radiographic Image Interpretation, Computer-Assisted/methods
• Retrospective Studies
• Survival Analysis
• Tomography, X-Ray Computed

Solitary fibrous tumor of the liver (SFTL) is a rare occurrence with a low number of cases reported in literature. SFTL is usually benign but, 10%-20% cases are reported to be malignant with a tendency to metastasize. The majority of malignant SFTL cases are associated with a paraneoplastic hypoglycaemia defined as Doege-Potter syndrome. Surgery is the best therapeutic treatment, however, long- life follow-up is recommended.

A 74-year-old man, was admitted to the emergency department after a syncopal episode with detection of hypoglycaemia resistant to medical treatment. The computed tomography revealed a solid mass measuring 15 cm of the left liver. An open left hepatectomy was performed with complete resection of tumor. Histopathological analyses confirmed a malignant SFTL.

Large series with long-term follow-up have not been published neither have clinical trials been undertaken. Consequently, the methodical long-term follow-up of surgically treated SFTLs is strongly recommended.

• Solitary fibrous tumor
• Malignant solitary fibrous tumor of the liver
• Mesenchymal tumor
• Hepatic tumor
• Doege-Potter syndrome

Primary hepatic angiosarcoma is a rare tumor with poor prognosis. The aim of this study was to generate a new angiosarcoma model to improve research on hepatic angiosarcoma.

Pigs sus scrofa were treated with different regimens of diethylnitrosamine (DENA). Tissues were analyzed by histology and immunohistochemistry. Serum parameters were determined. Angiosarcoma tissue was investigated for chromosomal aberrations by aCGH analysis.

Animals of almost all different treatment regimens developed a multitude of variable liver lesions. Different tumor types such as granulation tissue type, cellular-like, hyalinization necrosis-like, angiosarcoma-like, dysplastic nodule-like, hepatocellular-like, glandular structure-like, and leiomyoma-like lesions were observed. Weekly treatment with 15 mg/kg for up to 52 weeks or a single shot of 200 mg/kg DENA led to the development of hepatic angiosarcomas. aCGH analysis of angiosarcoma tissue revealed increased alterations in tumors compared to non-tumorous tissue. Most of the chromosomal alterations were found on chromosomes 6, 7, 12, and 14.

In this preliminary study treatment of sus scrofa with weekly injections of 15 mg/kg DENA results in a new model for primary hepatic angiosarcoma. This model may help to shed light on the pathomechanisms of primary hepatic angiosarcoma and might therefore open new treatment options.

• Gastrointestinal stromal tumor
• Hepatectomy
• Pleomorphic hepatic leiomyosarcoma

• Liver malignancies
• Liver resection
• Surgical oncology

• abscess
• imaging
• leiomyosarcoma
• liver

• Adult
• Diagnostic Errors
• Female
• Hepatectomy/methods
• Humans
• Leiomyosarcoma/diagnostic imaging
• Leiomyosarcoma/pathology
• Leiomyosarcoma/surgery
• Liver Abscess/diagnostic imaging
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/pathology
• Liver Neoplasms/surgery
• Neoplasm Recurrence, Local/therapy
• Tomography, X-Ray Computed

• ex situ hepatectomy
• liver autotransplantation
• prognosis
• solitary fibrous tumors