Malignant vascular lesions of bone: radiologic and pathologic features

The malignant vascular tumors of bone represent an uncommon diverse group of tumors with widely variable clinical and radiographic presentations. Although the radiographic imaging features of the lytic osseous lesions typically seen with this group of tumors are relatively nonspecific, the propensity to develop multifocal disease in an anatomic region is a feature that can be helpful in suggesting the diagnosis of a vascular tumor. The differential diagnosis varies according to the age of the patient and presence of solitary or multifocal disease. The histologic features are variable and range from tumors with vasoformative features to those that mimic mesenchymal neoplasm or metastatic carcinoma. Familiarity with the radiographic and pathologic spectrum of disease is essential for making an accurate diagnosis in this diverse group of neoplasms. This paper will provide a review of the nomenclature for the malignant vascular tumors of bone and discuss the radiographic and pathologic differential diagnosis. Received: 17 December 1999 Revision requested: 8 February 2000 Revision received: 19 May 2000 Accepted: 9 June 2000     

Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation

AIM: To investigate the indications and outcomes of liver transplantation for hepatic epithelioid hemangioendothelioma (HEHE).METHODS: Between 1989 and August 2013, in the Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, 1306 orthotopic liver transplantations (OLTx) were performed, including 72 retransplantations. Unresectable HEHE was an indication for OLTx in 10 patients (0.8% of primary OLTx), the mean age of the patients was 40.5 ± 13.3 years (range 23-65 years), and the male-to-female ratio was 2:8. Kaplan-Meier survival analysis in HEHE, hepatocellular carcinoma (HCC), and other OLTx recipients groups was performed. The differences in mortality were compared using the χ² test. A P-value < 0.05 indicated statistical significance.RESULTS: No concomitant liver disease was found in any patient. There was no neoadjuvant chemotherapy or radiotherapy. Liver function test results were normal in most of the patients. The levels of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. In immunohistochemical staining, the neoplastic cells were positive for factor VIII-related antigen, CD31, and CD34, which are endothelial cell markers, and negative for cytokeratin 19, cytokeratin 7, and HepPar-1. Nine patients were alive without tumor recurrence. One patient died 2 mo after OLTx due to septic complications. No morbidity was observed. Maximum follow-up was 11.4 years, with a minimum of 1 mo. The cumulative survival rate at the end of follow-up in HEHE patients was 87.5% compared with 54.3% in the HCC group and 76.3% in the other OLTx recipients group (χ² test = 1.784, df = 2, P = 0.409).CONCLUSION: Unresectable HEHE, without extrahepatic metastases is an excellent indication for liver transplantation. Long-term survival is very good and much better than in HCC patients and the entire group of OLTx patients.     

Dedifferentiated liposarcoma of the spermatic cord with a hemangioendothelioma-like component: A case report and review of the literature

Atypical lipomatous tumor or well-differentiated liposarcoma/dedifferentiated liposarcoma (DDLPS) is the most frequent subtype of malignant adipocytic tumor. This tumor typically presents in late adult life, most commonly in the retroperitoneum, extremities, or spermatic cord. It has been reported that the dedifferentiated component consists mainly of high-grade sarcoma, including undifferentiated pleomorphic sarcoma, fibrosarcoma, and myxofibrosarcoma, and it has been recently reported that the dedifferentiated component can be also made up of a low-grade sarcomatous component. Therefore, the dedifferentiated areas exhibit a wide morphological spectrum that commonly includes fibroblastic/myofibroblastic and fibrohistiocytic tumors but very rarely includes vascular tumors. We present here the first reported case of DDLPS with a hemangioendothelioma-like component in the spermatic cord.     

Infantile hemangioendothelioma: A case report and discussion

Infantile hepatic hamangioendothelioma type II is similar to angiosarcoma in terms of histomorphology and behavior. Various presentations of this lesion have been reported in the literature, e.g. cases with a hepatic mass, cutaneous hemangiomas, heart failure, etc. We report on a patient, male/2 years, who had two jejunal masses and a hepatic mass accompanied by lower GI bleeding and intestinal obstruction. The two jejunal masses and the hepatic lesion were diagnosed as angiosarcoma histomorphologically (IHHE type II), and were positive for vascular markers (CD31 and CD34) on immunohistochemistry. The patient had no skin lesions. We report this case and provide a literature review because of the unusual presentation and the overall rarity of this entity.     

Interventional treatment of hepatic arterial and venous pathology: A commentary

Hepatic aneurysms, pseudoaneurysms and fistulas (arterial biliary and arterial portal) causing blecding or portal hypertension, and arteriovenous malformations causing high output cardiac failure in adults can be successfully managed by embolization techniques. Results of embolization in infantile hemangioendotheliomas are less uniform and tumors with massive arteriovenous shunting are difficult to manage. Transjugular intrahepatic portal systemic shunts using expendable stents have been successfully created in patients and have effectively controlled portal hypertension and variceal bleeding.     

Kasabach-Merritt综合征1例

患儿女,53天.体检发现血小板减少1天.无牙龈出血、无鼻衄、无呕血、便血.查体:发育中等,轻度贫血貌,皮肤黏膜无出血点,全身浅表淋巴结不大,心肺正常,腹平、软,肝肋下1.5 cm,质软,边缘光滑,脾不大.     

气压法治疗小儿Kasabach-Merritt现象17例分析

目的 总结气压法治疗小儿 Kasabach-Merritt现象(KMP)的疗效和安全性.方法 回顾性分析1997年10月至2008年5月接受气压法治疗的17例KMP患儿的临床资料.17例中男11例,女6例;年龄3 d～3岁,中位年龄3.5个月;病变位于躯干8例,下肢5例,上肢4例;瘤体最大直径均>8 cm;血小板均<100×109/L,血红蛋白均<110 g/L,血清纤维蛋白原均<2.0 g/L.气压法治疗应用自行设计的"气压式血管瘤治疗器"(专利号:ZL97232266.3).瘤体局部取材活检或手术切除组织病理检查以明确KMP的病理性质.结果 2例患儿分别接受4和6个月的气压法治疗后瘤体消失,血液指标恢复正常,随访11个月和5年无复发,疗效评定为治愈;11例接受4～6个月气压法治疗,随访6～24个月病情继续好转,未再治疗,疗效评定为显效;2例接受6个月气压法治疗后随访5个月和2年,病情无进展,疗效评定为好转;2例接受气压法治疗6个月无效,改行手术切除治疗.组织病理学检查证实17例KMP中卡波西形血管内皮瘤14例,丛状血管瘤3例.结论 气压法对四肢、躯干部位的KMP疗效确切,无明显不良反应.     

Infantile Hepatic Hemangioendothelioma in Comparison with Hepatoblastoma in Children: Clinical and Ultrasound Features

Background

Infantile hepatic hemangioendothelioma (IHH) and hepatoblastoma (HBL) are respectively the most common benign and malignant liver tumors in children.

Objectives

To study the clinical manifestations and the ultrasound features of the pediatric patients for distinguishing IHH from HBL.

Patients and Methods

Between 2002 and 2012, thirteen children with IHH and 38 children with HBL under the age of 10 years were included. We retrospectively reviewed the clinical and the ultrasound features of the two groups, especially including parameters as follows: age at diagnosis, gender, alpha-fetoprotein (AFP) elevation, venous involvement and Doppler ultrasound.

Results

Compared with HBL group, the age of IHH group was much younger (5.8 months vs. 35.1 months, P = 0.000), the AFP elevation was less likely to be detected in IHH group (23.1% vs. 89.5%, P = 0.000). Although the color flow were the same commonly observed (61.5% vs. 52.6%, P > 0.05), the spectral Doppler showed IHH was less likely to appear as arterial flow with resistance index (RI) > 0.7(12.5% vs. 75.0%, P < 0.05), characterized by arterial flow with RI < 0.7 and/or venous flow. Combined the clinical features including age (< 6 months) and normal AFP level yielded high capability in differential diagnosis, with sensitivity, specificity and Youden index of 77% (10/13), 95% (36/38), and 0.72, respectively. When combined clinical features (age and AFP) and spectral Doppler as the diagnostic criterion for distinguishing these cases with positive color flow signals, the sensitivity, specificity, accuracy and Youden Index were 88%, 95%, 89% and 0.83, respectively.

Conclusions

The clinical features are effective indicators for distinguishing IHH from HBL, and the spectral Doppler may be a useful adjunct parameter for differential diagnosis.