Epithelioid hemangioendothelioma of the foot

Epithelioid hemangioendothelioma is a rare bone tumor. The authors report a case of a grade 1 multifocal epithelioid hemangioendothelioma of the foot in a 24-year-old man treated by large surgical resection of the two lesions.     

Subperiosteal hemangioendothelioma of the femur

Primary neoplastic vascular lesions of bone are rare, and include haemangiomas, haemangioendothelioma, epithelioid haemangioendothelioma and angiosarcoma. These lesions may be multicentric, and when they involve bone are typically intraosseous and lytic. Radiological findings are not always specific. We report a case of haemangioendothelioma localised to the subperiosteum of the femur, a site not previously described for this lesion. The nomenclature for vascular neoplasms in bone is discussed.     

Epithelioid hemangioendothelioma of bone

 Neoplasms of bone can arise from any of the cellular elements that constitute osseous tissues. Although tumors of vascular origin are not uncommon, the vast majority are benign. A rare malignant vascular tumor – epithelioid hemangioendothelioma of bone – classifically affects young males and produces osteolytic lesions involving the cortex and cancellous bone of the lower extremities. We present a case with these findings, as well as such unusual findings as cervical spine instability and lesions affecting no fewer than 45 different bones. We conclude that epithelioid hemangioendo-thelioma should be investigated by skeletal survey because (1) osteolytic lesions involving more that 50% of the cortex present a serious risk for pathologic fracture and (2) the natural history of multicentric epithelioid hemangioendothelioma is more indolent than its solitary counterparts     

Hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare, malignant neoplasm of vascular origin arising in soft tissues, lung, and liver. Four cases of hepatic EHE are reported, and 49 previously reported cases are reviewed. The tumor occurs in adults of all ages; 66% of patients are women. The prognosis is variable. Computed tomographic findings suggest the tumor begins as multiple hepatic nodules that grow and coalesce, forming large confluent masses preferentially involving the liver periphery. Extensive hepatic involvement is associated with enlargement of uninvolved portions of the liver and splenomegaly. Awareness of the radiologic features is helpful because clinical findings are nonspecific, and biopsy specimens may be misinterpreted as carcinoma or venoocclusive disease.     

Intramuscular spindle cell hemangioendothelioma

Spindle cell hemangioendothelioma occurring in skeletal muscle is extremely rare. No reported studies have performed an imaging evaluation of intramuscular spindle cell hemangioendothelioma. We report on such a tumor arising in an unusual site, the right extensor digiti minimi, in a 46-year-old woman. An en bloc resection was performed and the patient has been disease free for 8 years. Radiologic imaging in the present case showed similar findings to those described in intramuscular hemangioma. Received: 11 January 1999 Revision requested: 18 March 1999 Revision received: 22 April 1999 Accepted: 23 April 1999     

Multifocal epithelioid hemangioendothelioma of the phalanges of the hand

Epithelioid hemangioendothelioma (EH) of bone is a rare vascular neoplasm characterized by epithelioid endothelial cells and a variable biologic behaviour. The principal sites of occurrence of this osteolytic tumor are the lower extremity and the axial skeleton. Approximately half of the cases present with multifocal disease. The latter feature can be helpful in suggesting the diagnosis of a vascular tumor; on the other hand, it strengthens the need for a skeletal survey or whole-body MRI/CT. We report on the clinical, histologic and radiologic features—including CT and MRI findings—of EH in a case of multifocal disease of the phalanges of the hand, a very uncommon anatomic site of affliction.     

Imaging findings in epithelioid hemangioendothelioma

Purpose-objectiveEpithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE.MethodsRetrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE.ResultsWe evaluated 29 patients (17 F) with median age of 16 years (range 2–76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a “lollipop” sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton.ConclusionEHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and “lollipop” sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.     

FDG PET in epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EH) is an uncommon tumor of endothelial origin. It can develop in any tissue and can be multicentric or metastatic. The usual course is a slow progression. Imaging techniques are generally useful in determining the extent of the disease. A case of EH involving bone marrow and mediastinum is described. We discuss the use of FDG PET scanning in EH, showing its use in detecting bone marrow involvement and determining the extent of the disease.     

Scintigraphic findings in infantile hemangioendothelioma

The scintigraphic findings on sulfur colloid liver-spleen imaging, Tc-99m labeled RBC blood pool imaging, and Tc-99m MDP bone imaging in four patients with infantile hemangioendothelioma are described. Thirteen radionuclide studies were performed, with serial sulfur colloid images obtained in three patients, allowing interval assessment of liver size and tumor involvement. Findings of Tc-99m MDP uptake in the livers of two patients with hemangioendothelioma and diffuse increase in hepatic RBC labeled blood pool activity in one patient also are reported.     

肝脏上皮样血管内皮瘤的影像学特征

目的：提高对肝脏上皮样血管内皮瘤（EHE）的认识和影像诊断水平。方法：搜集经手术病理证实的5例肝脏上皮样血管内皮瘤患者的CT、MRI和病理学资料,分析和总结其影像学征象。结果：CT平扫示肝脏多个类圆形低密度灶,2例病灶边缘可见条状钙化灶。增强后动脉期主要表现为肿瘤周边轻度强化,延迟后肿瘤实质部分无明显对比剂进入,而中央更低密度区无强化。MRI显示肝内多个类圆形病灶T1WI呈低信号,T2WI呈高信号,病灶边缘有低信号晕环。结论：EHE具有一些特征性的影像学特点,CT和MRI检查能为临床诊断及鉴别诊断提供有价值的信息。     

Scintigraphic features of malignant epithelioid hemangioendothelioma

The scintigraphic features of malignant epithelioid hemangioendothelioma of the liver have not been previously documented. In particular, there are no reports on the nature of gallium uptake in these rare tumors. The case reported is a young woman with primary hepatic epithelioid hemangioendothelioma. Metastatic disease in the skull was detected on a Tc-99m MDP bone scan. A Ga-67 study demonstrated that this tumor was not gallium avid.     

Kaposiform hemangioendothelioma arising from the internal auditory canal

Kaposiform hemangioendothelioma is a rare vascular tumor and locally aggressive endothelial-derived spindle cell neoplasm, which occurs almost exclusively in infants and adolescents. Radiologically, hemangioendothelioma, including Kaposiform hemangioendothelioma, is seen as a highly vascularized well-enhancing tumor, but no characteristic findings differentiate Kaposiform hemangioendothelioma from other soft-tissue tumors, particularly when the tumor is too small to have any locally aggressive features or identifiable large vessels. We present a case of Kaposiform hemangioendothelioma in the internal auditory canal that had no differential features on initial MR images and rapidly grew into a huge mass in a few months.