Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 36-year-old woman presented with a 5-month history of a growing left thigh mass, causing difficulty walking. Biopsy revealed a malignant epithelioid hemangioendothelioma (EHE), and the patient was referred for a staging F-18 FDG PET/CT that showed intense FDG uptake in the thigh mass, but no FDG-avid local lymph nodes or distant metastases. A few reports have noted the usefulness of F-18 FDG PET/CT imaging in the staging of EHEs in the lung, liver, and bone marrow. This rare study highlights the usefulness of F-18 FDG PET/CT in the staging of malignant EHE of the soft tissues of the extremities.
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PMID:Malignant epithelioid hemangioendothelioma of the lower extremity: staging with F-18 FDG PET/CT. 2163 69

Epithelioid hemangioma is a rare and benign vascular tumor, most often occurring in the skin. Numerous other localizations, including bones, have been reported. The overall favorable clinical outcome of cutaneous epithelioid hemangioma is now well documented, but it still remains debated in osseous localization, as local recurrences and metastases have been described. We report a case of epithelioid hemangioma of the spine occurring in a 25-year-old male, and discuss main differential diagnoses. Recent studies tend to demonstrate that osseous epithelioid hemangioma, as cutaneous epithelioid hemangioma, should be considered as a benign tumor. Histologically, epithelioid hemangioma consists of a vascular proliferation with diffuse or lobular pattern and features vascular spaces lined by epitheliod endothelial cells with numerous lymphocytes and eosinophils. The main differential diagnosis is represented by epithelioid hemangioendothelioma, the surgical treatment of which must be more aggressive. Precise histological diagnosis is essential for accurate clinical management and to avoid overtreatment.
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PMID:[A case of epithelioid hemangioma of the spine]. 2183 59

Vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification systems. None of the classification schemes have been accepted due to lack of consistent terminology, accepted histologic criteria, and limited correlation with clinical outcome. It is acknowledged that vascular tumors of bone originate from endothelial cells, resulting in variable expression of endothelial markers. None of these markers are useful to discriminate between benign and malignant lesions. Although radiologic appearance is not specific, radiologic multifocality should trigger to include a vascular neoplasm in the differential diagnosis. This review gives an overview of current literature by describing all different histologic subtypes in correspondence with clinical, radiologic and genetic data. We propose the classification of vascular tumors of bone according to the three-tiered World Health Organization classification scheme for soft tissue tumors dividing entities into a benign, intermediate and malignant category. Hemangioma is the most often and commonly recognized benign lesion. Epithelioid hemangioma has been better defined over the past few years. Based on its locally aggressive behavior and occurrence of lymph node metastases, classification within the intermediate category could be considered. Angiosarcoma is the only accepted term for high-grade malignant vascular tumor of bone and so far, epithelioid hemangioendothelioma is the only accepted low-grade malignant vascular tumor of bone. It is still unclear whether other low-grade malignant vascular tumors of bone (e.g. hemangioendothelioma) truly exist. Unfortunately, molecular / genetic studies of vascular tumors of bone which might support the proposed classification are very sparse.
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PMID:Primary vascular tumors of bone: a spectrum of entities? 2190 30

A 60 year-old woman was admitted to our hospital because of management of multiple liver tumors. According to image findings and liver biopsy, she was diagnosed as having epithelioid hemangioendothelioma of the liver accompanied by metastases in the spleen, lungs and bones. Based on the spread of the liver tumors and the extensive systemic metastases, she was considered inoperable. Instead, she received hepatic arterial infusion therapy using recombinant interleukin-2. However, she died due to liver failure about two months after admission. Autopsy revealed that the liver tumor was angiosarcoma. It is difficult to differentiate angiosarcoma from epithelioid hemangioendothelioma based on the image findings and pathological findings of percutaneous liver biopsy. Many cases are diagnosed as angiosarcoma at autopsy. There is no established effective treatment for hepatic angiosarcoma, because the tumor stage at the time of diagnosis is often progressive. To date, immunotherapy with recombinant interleukin-2 has been reported to be effective clinically for cutaneous angiosarcoma, such as of the scalp and facial skin. To our knowledge, there have been no reported cases of hepatic angiosarcoma treated with recombinant interleukin-2. Our case is important should recombinant interleukin-2 be considered effective for hepatic angiosarcoma in the future.
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PMID:A first case of hepatic angiosarcoma treated with recombinant interleukin-2. 2238 54

Epithelioid hemangioendothelioma is an extremely rare intracranial tumor and is regarded as a low-proliferation tumor. We present two cases of primary intracranial epithelioid hemangioendothelioma and give an overview of the English literature pertaining to this disease. We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words "epithelioid hemangioendothelioma". Only cases in the English language that were intracranially located and contained clinical information pertinent to the analysis were included. The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles. The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium. Both tumors were well vascularized. The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss. A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature. The tumor was typically diagnosed in young adults and infants. There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1). Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy. Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %. Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.
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PMID:Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior. 2288 11

We report a case of multiple hepatic epithelioid hemangioendothelioma( EHE) mimicking hepatic metastasis of colon cancer. A 71-year-old man was referred to our hospital for further evaluation of multiple hepatic tumors. Enhanced CT scan was not performed because of renal dysfunction. Total colonoscopy revealed a type 1 cancer at the rectosigmoid junction. We therefore diagnosed the hepatic tumors as metastases from rectal cancer. Anterior resection was performed and postoperative chemotherapy targeting the hepatic tumors was carried out. Unfortunately, the size of the hepatic tumors increased. We therefore performed lateral segmentectomy and partial hepatectomies (11 lesions). The macroscopic findings indicated basically localized tumor without diffuse infiltration. Microscopically, spindle and oval-shaped cells with abundant eosinophilic cytoplasm were observed; immunohistochemical staining was positive for factor VIII and CD34. These data were conclusive for EHE. EHE of the liver is a rare neoplasm of which little is currently known. We herein report a case of EHE in the liver, misdiagnosed as colorectal metastatic tumor.
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PMID:[A case of multiple hepatic epithelioid hemangioendothelioma mimicking metastatic hepatic tumor]. 2326 60

: Pseudomyogenic hemangioendothelioma is a recently described vascular tumor that occurs predominantly in the distal extremities of young adults. Because of multifocal presentation, epithelioid morphology, and strong cytokeratin expression, the tumor was frequently misdiagnosed as epithelioid sarcoma. However, substantial immunohistochemical studies revealed an endothelial nature. It has been currently considered a tumor of intermediate malignancy with frequent local recurrence but low risk of distant metastasis. In this report, we describe a case of pseudomyogenic hemangioendothelioma occurring in a 22-year-old man who presented with multifocal disease in the lower extremity and developed bilateral pulmonary metastases within a short period.
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PMID:Pseudomyogenic hemangioendothelioma: report of an additional case with aggressive clinical course. 2347 48

Hemangioendotheliomas are poorly differentiated neoplasms built from strands of endothelial cells and have a very narrow lumen filled with blood cells. Hemangioendotheliomas are commonly detected during the initial six months of life, concerning mainly soft tissues and skin. It is the most common bening liver cancer in children. In adults, hemangioendotheliomas are very rare findings, being mostly described in the liver, lungs, brain and bones. Hemangioendotheliomas of the large intestine are extremely rare in adults. Cancer developing there is of high degree of malignancy. The authors present the case of 68 year old patient in whom preoperative benign tumor with ulcers were diagnosed. Intraoperative macroscopic picture suggested malignant process. The patient underwent anterior rectal resection with regional lymphadenectomy. The postoperative histopathological evaluation with immunohistochemical studies angiosarcoma was diagnosed. A four year survival free of cancer was obtained, and the patient died due to myocardial infarct. Literature describes the short periods of survival of patients with vascular sarcoma of the colon. The most common reason for decease are metastases with massive haemorrhages. Bleeding tendency results from growing Kasabach-Merritt syndrome. It is characterized by profound thrombocytopenia and coagulopathy. The presented patient is a unique example of angiosarcoma with long period of survival.
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PMID:Angiosarcoma of the large intestine - a case report. 2378 50

The paper describes a rare case of mediastinal epithelioid hemangioendothelioma characterized by a population of fusiform cells, metaplastic osteogenesis, and aggressive behavior. A 5.5x4-cm encapsulated mass was found in the anterior mediastinum of a 19-year-old female patient. A bone density tumor was sealed with lung tissue and it occluded the lumen of the left subclavian vein. Microscopically, the tumor was composed of foci of the so-called "blister cells" typical of epithelioid hemangioendothelioma, anastomosing chains of epithelioid cells in the microhyaline stroma, diffusely located bone trabeculae, and hemorrhagic stroma. Fusiform cells were present in considerable quantities. A moderate cellular and nuclear polymorphism occurred when mitotic figures were absent. Tumor cells expressed Flil, vimentin, CD31, and CD34. Multiple metastases were detected in the liver and lung. They had the similar morphology.
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PMID:[Epithelioid hemangioendothelioma of the mediastinum]. 2380 70

Hemangioendotheliomas are vascular tumors which have an intermediate clinical behavior between hemangiomas and angiosarcomas. The epithelioid subtype of hemangioendothelioma, which is rarely seen in bone, has the potential to metastasize and may be confused radiologically with benign or malignant lesions. A metacarpal origin of this tumor is extremely rare. In this article, we present a 42-year-old female case with an hemangioendothelioma in the second metacarpal which was initially managed as a benign lesion before the patient was referred to our center. The potential malignant nature of epithelioid hemangioendothelioma has been ill-defined. We recommend excision with a wide surgical margin. Amputation may be necessary to perform excision in bones such as the metacarpals or phalanges.
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PMID:Solitary epithelioid hemangioendothelioma of the metacarpal. 2419 86


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