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 case of epithelioid hemangioendothelioma of the cauda equina is reported. The patient presented with rapidly worsening low back pain. Magnetic resonance imaging revealed a sharply demarcated intradural lumbar lesion. A bluish-red lesion, attached to the filum terminale, was removed. The patient is alive without evidence of recurrence 18 months after surgery. The tumor was composed of variously sized vessels lined by epithelioid endothelial cells with clear cytoplasm and centrally located, moderately atypical nuclei. These cells were immunoreactive for CD31 and factor VIII antibodies. Cytogenetic analysis disclosed two clones: 44-45X, - Y [cp3]/46XY[11]. Epithelioid hemangioendothelioma may arise in several sites, the most common being soft tissues. It is a borderline tumor that may recur, may metastasize, and rarely causes death. The present case appears to be the first example of epithelioid hemangioendothelioma of the spinal cord.
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PMID:Epithelioid hemangioendothelioma of the spinal cord: Description of a case with cytogenetic analysis. 1704 Dec 4

Epithelioid hemangioendothelioma (EH) of the liver is a rare tumor, generally considered to have low-grade malignancy. Little is known about its clinical behavior and the therapeutic strategy is not established. We report the case of a 36-year-old woman who underwent living donor liver transplantation for EH with splenic metastases and died of recurrence 8 months later. To determine if transplantation improves the prognosis of patients with EH, we must re-evaluate its indications.
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PMID:Living donor liver transplantation for epithelioid hemangioendothelioma: Report of a case. 1707 29

Epithelioid hemangioendothelioma (EHE) arising in the skin is extremely rare, and the majority of documented cases have developed in soft tissues or parenchymatous organs. We report on a 9-year-old boy who presented with a painful erythematous plaque on the nose. Histopathological findings showed a dermal tumor composed of epithelioid cells with moderate cellular atypia and characteristic intracytoplasmic vacuoles. Immunohistochemical stainings confirmed the vascular nature of the tumor. On the basis of these findings, the diagnosis of an EHE was made. Then, two cervical lymph nodes were excised and adjuvant therapy with interferon alpha 2b was initiated. Eighteen months after diagnosis, there is no evidence of local tumor recurrence or metastases. To our knowledge, this is the first report of a metastasizing cutaneous EHE on the nose in childhood. As our case shows, this rare tumor entity has to be considered as a differential diagnosis in neoplasms of the skin, even in childhood.
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PMID:Metastasizing epithelioid hemangioendothelioma of the nose in childhood. 1854 51

Epithelioid hemangioendothelioma (EHE) of soft tissue is a distinctive vascular tumor that has been variously considered a tumor of borderline malignancy and low-grade angiosarcoma. The majority of cases are associated with low mortality, but some metastasize and cause patient death. We analyzed 49 cases of EHE to determine if a method for stratifying risk for mortality could be developed. Fifty-one cases of EHE diagnosed during the period of 1989 and 2005 were retrieved. Tumors were evaluated with respect to location, size, cytologic atypia, mitotic activity, tumor cell spindling, and necrosis. Follow-up information was obtained for all cases. For actuarial analysis, disease-specific overall survival was evaluated using univariate and multivariable analysis. Most tumors occurred in adults (range, 9 to 93 y) and affected women predominantly (21 M:28 F). They developed in the head and neck (6), extremities (32), mediastinum (4), trunk (4), genitals (2), and retroperitoneum (1) and ranged in size from 0.5 to 18 cm. Clinical follow-up was obtained for all patients and ranged from 1.5 to 170 months (mean, 57.9 mo); 31 patients were alive without disease, 5 patients were alive with disease, 9 patients died of disease and 4 died of other causes. Overall 5-year disease-specific survival was 81%. Eleven patients (22%) had metastatic disease affecting lung (6), lymph node (4), liver (2), and bone, retroperitoneum, and soft tissue (1 each). Two patients had metastases to multiple sites. Treatment modality was known for 46 patients: 31 were treated surgically and 15 were treated with surgery and chemotherapy and/or radiation therapy. By univariate analysis, mitotic activity and size were associated with higher mortality (P=0.007 and 0.004, respectively). By multivariable analysis, increasing mitotic activity (P=0.00827, hazard ratio 10.03) and size (P=0.01, hazard ratio 2.26) were associated with decreased survival. Tumor site, cytologic atypia, the presence of necrosis, and tumor spindling were not significant. Those tumors with >3 mitotic figures/50 high power fields and size >3.0 cm had the worst prognosis (P=0.0002). Patients with high-risk tumors had a 5-year disease-specific survival of 59%; no patients with low-risk tumors died. In conclusion, we report that EHE can be stratified into 2 risk groups with markedly different clinical courses.
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PMID:Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. 1855 49

Epithelioid hemangioendothelioma is a rare vascular neoplasm which exhibits the potential for malignancy and recurrence as well as the ability to metastasize. Although numerous sites of involvement are possible, these tumors most commonly arise in soft tissues, lung, liver, bone, and lymph nodes. In this report, we describe a case of oral epithelioid hemangioendothelioma in a child. This tumor appeared as exophytic ulcerated painless masses in the maxillary and mandibular gingiva. Histologically, the tumor was composed of a proliferation of tumor cells arranged in nests, cords, and short strands. Epithelioid cells exhibited abundant eosinophilic cytoplasm with nuclear and cellular pleomorphism and intra-cytoplasmic vacuoles.
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PMID:Epithelioid hemangioendothelioma of the oral cavity: a case report. 1858 15

Liver transplantation for hepatic malignancies has emerged as a well-documented and proven treatment modality. However, early unsatisfactory results emphasized that only a highly selected patient population would benefit from transplantation. Currently, 15% of all liver transplants performed are for hepatocellular carcinoma (HCC). There is no controversy about the fact that liver transplantation for HCC in the adult population yields good results for patients whose tumour masses do not exceed the Milan criteria. It remains to be determined whether patients with more extensive tumours can be reliably selected to benefit from the procedure. In patients with small HCC at an early stage and preserved liver function, liver resection provides an alternative to transplant. Liver resection may offer similar survival results to orthotopic liver transplantation (OLT) in the short term, and does not carry the long-term effects of immunosuppression; however, long-term and disease-free survival favours liver transplantation. Very promising results have been obtained for cholangiocarcinoma treated by aggressive combination therapies, including chemo- and radiotherapy followed by OLT. Survival rate in these selected patients can approach that of patients with cholestatic liver disease, and the role of transplantation now requires re-evaluation. Similarly, hepatoblastoma is an excellent indication in paediatric patients with unresectable or recurrent tumours. Epithelioid hemangioendothelioma is also an appropriate indication for liver transplantation, even in the presence of extrahepatic metastases, unlike angiosarcoma which is associated with a very poor survival and considered as a contraindication. And finally for metastatic liver disease from neuroendocrine tumours, liver transplantation can result in long-term survival and even cure in well selected patients. Conversely, the value of transplantation for colorectal liver metastases (currently a contraindication) requires further evaluation by well-designed trials.
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PMID:Liver transplantation for primary and metastatic liver cancers. 1871 48

Epithelioid hemangioendothelioma is a neoplasm of vascular origin with a low-to-intermediate malignant potential and is one of the rare sarcomas arising from the liver. Its etiology is unknown and its clinical outcome is unpredictable. There is no generally accepted therapeutic strategy because of its rarity and the variable natural course between hemangioma and angiosarcoma. We report a case of a 64-year old woman who underwent hepatic resection due to epithelioid hemangioendothelioma in the right lobe that progressed to extrahepatic metastases of the bone, pleura, and peritoneum 22 months later. However, after resection there was no primary hepatic recurrence.
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PMID:[A case of hepatic epithelioid hemangioendothelioma that caused extrahepatic metastases without intrahepatic recurrence after hepatic resection]. 1911 48

Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular tumor that most commonly presents within the liver. Patients with hepatic EHE are often candidates for liver transplantation as the disease is usually multifocal at diagnosis. Although these patients achieve excellent early outcomes post-transplant, there are very few data regarding tumor markers that can further direct chemotherapy in hepatic EHE to prevent recurrent disease. The purpose of this study was to analyze the expression of the angiogenic factor vascular endothelial growth factor (VEGF) and its receptors in hepatic EHE. Six patients with hepatic EHE were assessed for liver transplantation at our center. Pathology specimens of primary and recurrent EHE were analyzed by hematoxylin and eosin staining and by immunofluorescence for VEGF, fetal liver kinase 1 (Flk-1), and fms-related tyrosine kinase 1 (Flt-1) expression. Five patients underwent liver transplantation, and 1 patient underwent liver resection. Biopsy-proven recurrent EHE occurred in 3 patients. VEGF expression was present in 100% of the EHE specimens examined, whereas Flt-1 expression was present in only 1 sample, and Flk-1 was not observed in any of the specimens. In 1 patient with recurrent hepatic EHE post-liver transplantation, a progressive increase in the VEGF fluorescence intensity and distribution was observed. In conclusion, in this series, VEGF expression was observed in all hepatic EHE specimens analyzed. These data suggest that anti-VEGF chemotherapeutic agents will be of use in patients with hepatic EHE, particularly as a means of reducing the tumor volume prior to resection, as a means of treating unresectable or metastatic disease, or as an adjuvant therapy in the setting of liver transplantation.
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PMID:Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management. 2010 92

Epithelioid hemangioendothelioma of the liver is a very rare vascular tumour in children with intermediate malignant potential. We present a case in which the typical imaging appearances of coalescent peripheral hepatic masses with capsular retraction contributed to the diagnosis. A positron emission tomography-CT (PET-CT) procedure was performed in staging the disease with a strong suspicion of coeliac nodal involvement confirmed after laparotomy and histological analysis. Our case is unique because of the rarity of the disease, the young age of the child, and proven nodal metastases at initial diagnosis. The use of PET-CT allows better staging at initial diagnosis and thus better management with improved follow-up in these patients.
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PMID:Epithelioid hemangioendothelioma of the liver with metastatic coeliac lymph nodes in an 11-year-old boy. 2011 13

Epithelioid hemangioendothelioma is an uncommon vascular tumor of soft tissue and bone that may rarely occur in the liver, lung and the head and neck. We present five new cases of epithelioid hemangioendothelioma of the head and neck region diagnosed and managed in one institution in order to define the phenotypic characteristics, podoplanin immunohistochemical staining and the biological outcome. Podoplanin is a transmembrane mucoprotein selectively expressed in lymphatic endothelium and recently in some vascular neoplasms. The patients were comprised of two male and three female patients ranging in age from 4 to 71 years. The lesions were found in the gingiva, submandibular region soft tissue, nasal cavity and tongue, and ranged in size from 0.7 to 2.5 cm. All tumors manifested infiltrative cords and nests of epithelioid cells with occasional spindle morphology in a myxoid stroma. Immunohistochemical analysis of vascular and epithelial markers showed strong and uniform cytoplasmic reactivity for podoplanin and variable intensity and staining of CD31 and lack of cytokeratin staining in tumor cells. Surgical treatment included simple and wide local excisions. Of the three patients with follow-up, one developed lymph node metastasis and one had no evidence of disease 10 months after surgery. The patient with multiple recurrences and LN metastases was additionally treated with chemotherapy and is under consideration for radiation therapy. Hemangioendothelioma of the head and neck is: (1) a low-grade malignancy with a tendency for local recurrence and regional lymph node metastasis, (2) complete excision with negative margins is the treatment of choice for localized disease and (3) podoplanin may be useful in differentiating epithelioid hemangioendothelioma from non-vascular tumors.
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PMID:Epithelioid hemangioendothelioma of the head and neck: role of podoplanin in the differential diagnosis. 2061 38


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