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)

Ehlers-Danlos syndrome (EDS) is a collection of inherited connective tissue disorders with at least 10 types, differentiated on clinical and genetic grounds. Malignancy has been described only rarely in association with the syndrome. Epithelioid hemangioendothelioma (EH) is a rare endothelial tumor, which displays clinical behavior intermediate between that of hemangioma and angiosarcoma. A case report of a 50-year-old man with type IV EDS who was extensively investigated for several years for multiple mediastinal nerve palsies and chest pain. Magnetic resonance imaging (MRI) demonstrated an anterior mediastinal mass, which at biopsy showed EH. Subsequent metastatic spread to liver and lungs is unique among reported cases of mediastinal EH. The patient experienced significant symptomatic improvement from external beam radiotherapy (RT) to the mediastinum. After metastatic disease developed, multiagent chemotherapy was administered, but without response. The literature is reviewed regarding treatment of EH and the potential problems associated with EDS. Although there appears to be no etiological association between EDS and EH, the connective tissue disease clearly contributed to a delay in diagnosis and raised concerns regarding RT tolerance. The potential predisposition to aggressive tumor invasion remains a possibility. In addition, mediastinal EH has the potential to metastasize, and in this case demonstrated resistance to a broad range of chemotherapy agents.
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PMID:Mediastinal epithelioid hemangioendothelioma in a patient with type IV Ehlers-Danlos syndrome: a case report and review of the literature. 925 1

In this third and last part of our review of cutaneous vascular proliferations we include malignant vascular neoplasms and a group of heterogeneous cutaneous neoplasms characterized by a significant vascular component. We also review some disorders that, in our opinion, have been erroneously considered as vascular neoplasms. We review the epidemiologic, histogenetic, clinical, and histopathologic aspects of Kaposi's sarcoma in its four distinctive variants (classic, African-endemic, immunosuppressive drug-associated, and AIDS-associated Kaposi's sarcoma). There is still controversy about whether Kaposi's sarcoma represents a reactive vascular proliferation or a true neoplastic proliferation. In any event, most authors believe that Kaposi's sarcoma does not produce metastatic disease, but rather develops in multifocal fashion. However, Kaposi's sarcoma may cause death, especially in immunosuppressed patients. Epithelioid hemangioendothelioma, Dabska's tumor, and retiform hemangioendothelioma are examples of low-grade angiosarcoma. In contrast, cutaneous angiosarcomas, including the clinical variants of angiosarcoma of face and scalp in elderly patients, angiosarcoma associated with lymphedema, and radiation-induced angiosarcoma are highly aggressive neoplasms with poor prognosis and most patients die within a short period after presentation. A group of benign and relatively frequent cutaneous neoplasms, including multinucleate cell angiohistiocytoma, angiofibroma, angioleiomyoma, angiolipoma, cutaneous angiolipoleiomyoma, and cutaneous angiomyxoma are here covered because of their significant vascular component. Finally, we review briefly a series of cutaneous disorders that have been erroneously considered as vascular neoplasms. Kimura's disease is an inflammatory reactive condition of unknown origin, "benign" angioendotheliomatosis is a reactive intravascular proliferation of endothelial cells that occurs in the skin as a response to a variety of stimuli, "malignant" angioendotheliomatosis is an intravascular lymphoma, and acral pseudolymphomatous angiokeratoma of children (APACHE) is better interpreted as a pseudolymphoma.
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PMID:Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. 948 70

Epithelioid hemangioendothelioma arising in the skin is extremely rare, and the majority of documented cases have developed in association with an underlying bone tumor. We report eight patients with an age range of 29-84 years (mean 53), who presented with primary cutaneous tumors at a variety of sites including the palm, shin, neck, knee, nose, back, and penis with a duration of between 6 and 12 months. Histologically, all eight cases presented as circumscribed nodules with an overlying acanthotic epidermis, three showing striking acrosyringeal proliferation, reminiscent of eccrine syringofibroadenoma. The tumors were composed of an admixture of slightly pleomorphic spindle and epithelioid cells with abundant, sharply defined eosinophilic cytoplasm and vesicular nuclei containing single nucleoli. Mitoses were generally sparse. All tumors showed intracytoplasmic lumina and intraluminal erythrocytes were occasionally apparent. The tumor cells were embedded in a myxoid or hyaline matrix. In contrast to visceral lesions, a vascular origin was not evident in any of our cases. The tumor cells variably expressed CD31, CD34, factor VIII-Rag, and smooth-muscle actin but not pankeratin or epithelial membrane antigen. Follow-up ranged from 4 months to 3 years. None of the lesions has thus far recurred and there have been no metastases.
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PMID:Epithelioid hemangioendothelioma presenting in the skin: a clinicopathologic study of eight cases. 985 48

Epithelioid hemangioendothelioma (EH) is a rare, low-grade, malignant neoplasm of vascular origin that may develop at different sites, such as in the soft tissue, lungs, or liver. We report the case of a 21-year-old female with primary EH of the liver treated by liver transplantation and review the available literature on EH. This patient developed symptomatic recurrence of the tumor in the pelvis 2 months posttransplant. Treatment with interferon alpha-2b resulted in substantial regression of the pelvic metastases and alleviation of symptoms, but the patient developed graft rejection and died of associated complications 16 months posttransplant. This report is the first showing the efficacy of interferon in this setting.
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PMID:Epithelioid hemangioendothelioma of the liver disseminated to the peritoneum treated with liver transplantation and interferon alpha-2B. 1213 11

Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in Kasabach-Merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.
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PMID:Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy. 1239 62

Epithelioid hemangioendothelioma of the liver is a rare vascular tumor with intermediate malignant potential. On imaging studies, the lesion has a solid appearance and may mimic metastatic disease. We present a case in which the morphologic features (multifocal aspect, peripheral location, and capsular retraction) and the clinical history aided in including this entity in the differential diagnosis.
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PMID:Epithelioid hemangioendothelioma of the liver: radiologic-pathologic correlation. 1529 Sep 49

Major progress has been achieved during the last decades in the treatment of malignant liver tumors in children, both in chemotherapy and surgical management. Chemosensitivity varies between tumor types, and radical resection remains essential to effect a cure. In tumors extensively involving a normal liver, in a diffuse or multifocal manner, radical resection cannot be accomplished with a partial hepatectomy. This has been the case for some instances of advanced hepatoblastoma and epithelioid hemangioendothelioma. In hepatoblastoma, current experience shows that results of primary liver transplantation with neoadjuvant chemotherapy are excellent with around an 80% 5-to-10-year disease-free survival rate. Epithelioid hemangioendothelioma is very rarely seen in children and may have a more malignant behavior than in adult patients, and liver transplantation may not be the best management option. In nonresectable hepatocellular carcinoma (HCC) developed on an otherwise normal liver, the results of liver transplantation are similarly poor to those obtained in adult patients, except in a few highly selected series fulfilling the Milano criteria. The experience with HCC is still very scarce in children. Incidental HCC associated with chronic liver disease does not seem to impact posttransplant survival. When they are symptomatic, however, indications for transplantation should be very selective regarding tumor size, multi-focality, vascular invasion and distant metastases.
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PMID:The contribution of transplantation to the treatment of liver tumors in children. 1622 98

Epithelioid hemangioendothelioma is a very rare tumor of vascular origin. It can develop in different tissues such as soft tissue, lung, or liver. Hepatic epithelioid hemangioendothelioma (HEH) mostly affects females. The malignant potential of HEH often remains unclear in the individual patient. It can range from benign hemangioma to malignant hemangioendotheliosarcoma. Here we present our experience with five patients with primary HEH, who were treated with curative intention in our department. All patients in our series with confirmed histological HEH did not show extrahepatic extension and consequently underwent surgical treatment. In three patients, liver transplantation (LTx) was performed (two cadaveric and one living related). In one patient, a right-sided hemihepatectomy with partial resection of the diaphragm was performed. One patient died while she was on the waiting list for LTx due to rapid tumor progression. Postoperative follow-up ranged from 1 to 13 years. No adjuvant chemotherapy was applied. Until now, no recurrence of local tumor or distant metastases could be observed during follow-up in our series. Early detection and surgical intervention in case of HEH can potentially offer curative treatment. The treatment of first choice appears to be radical liver resection. In our view, LTx represents a potentially important option for patients with a nonresectable tumor. Despite the long waiting time, its often unclear dignity, and a proven progressive growth pattern, living related LTx also plays a potentially important role. The 5-year overall survival rate of patients with HEH in the literature varies from 43% to 55%. Long-term survival of patients with HEH is significantly higher compared to other hepatic malignancies. The role of adjuvant therapy currently remains unclear.
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PMID:Surgical treatment of primary hepatic epithelioid hemangioendothelioma. 1628 86

Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. The authors describe two cases of spinal EH, one involving the T-10 vertebra and the second involving the upper cervical spine. In the first case the patient underwent resection of the tumor; this case represents the longest reported follow-up period for spinal EH. In the second case, extensive involvement of C-2, C-3, and C-4 as well as encasement of both vertebral arteries precluded safe tumor resection, and posterior occipitocervical stabilization was performed. The patient subsequently died of metastatic disease. The findings in these two cases underscore the difficulty in predicting the clinical behavior of spinal EH based solely on histological and clinical features as well as the uncertainty of the roles of surgery, chemotherapy, and radiotherapy in the oncological management of a spinal tumor for which clinical data are very limited.
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PMID:Epithelioid hemangioendothelioma of the spine. Report of two cases. 1630 36

Epithelioid hemangioendothelioma (EHE) of the lungs (syn.: intravascular bronchioloalveolar tumor/IVBAT) is a neoplasm with a vascular line of differentiation. This study provides a clinicopathological analysis of 11 cases of this tumor. There were 7 female and 4 male patients. Patients' age ranged from 33 to 83 years (mean: 49.8, median: 48 years) showing 2 peaks (third/fourth and sixth/eighth decades). The typical radiological appearance revealed multiple bilateral lung nodules from 0.5 to 2 cm. In 2 cases, a striking perivascular and intravascular growth pattern associated to pulmonary veins was evident. All tumors were characterized by epithelioid tumor cells with nests, cords and strands embedded in a sclerosing, hyaline matrix with alveolar, bronchiolar and vascular extension. Necrosis was identified in 8 tumors and ossification in 2 tumors. The mitotic index was below one mitosis in 10 HPF. By immunohistochemistry, all tumors were positive for CD 31, CD 34, and vimentin, 60% positive for factor VIII, and negative for pancytokeratin and calretinin. Follow-up was available for 8 patients with a range of 6 to 105 months (mean: 51.5 months, median: 52 months): 3 patients died (2 certainly from their tumors), 4 are alive disease-free, and one is alive with metastases. EHE of the lungs (IVBAT) represents an intermediate-grade, malignant, mesenchymal neoplasm with a characteristic histological appearance and immunohistochemical profile.
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PMID:[Epithelioid hemangioendothelioma of the lung (IVBAT)--clinicopathological and immunohistochemical analysis of 11 cases]. 1642 4


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