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)

We report eight cases of epithelioid angiosarcoma arising in deep, usually intramuscular soft tissue. All the patients were men (mean age, 58). All the lesions arose in a limb or limb girdle. Cardinal morphologic features were the diffuse, sheetlike growth pattern, with only focally apparent vascular differentiation, and epithelioid tumor cells with a degree of intracytoplasmic vacuolation/lumen formation. Immunohistochemically, all eight cases coexpressed keratin as well as endothelial markers. In three cases, endothelial differentiation was confirmed ultrastructurally. Clinically, deep-seated epithelioid angiosarcomas are high-grade neoplasms that rapidly develop metastases. These findings expand the range of recognized epithelioid endothelial tumors and provide further evidence of keratin expression by such lesions. The presence of intracytoplasmic lumina/vacuoles (sometimes containing red blood cells) combined with the characteristic reticulin pattern and striking positivity for Factor VIII-RAg provide the clearest means of distinction from an epithelial metastasis.
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PMID:Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm. 171 76

Thirteen patients with soft tissue sarcomas were treated with a combination of intra-arterial Adriamycin, conventionally fractionated radiotherapy (2 Gy per day), and conservative surgery (trimodal therapy). Severe acute complications occurred in 10 patients: 3 brachial artery thromboses, 6 delayed wound healing, 4 wound infections, and 3 cases of necrosis of the skin plus subcutaneous tissues. Three patients have developed local recurrence. Five patients are alive, 4 of whom are disease-free, and the median follow up time of surviving patients is 56 months. One has significant impairment of limb function due to joint ankylosis. An additional 2 patients were treated with intra-arterial Adriamycin and conservative surgery for local recurrence after previous surgery and radiotherapy; both died of subsequent metastatic disease, one having a further local recurrence. One patient with multifocal angiosarcoma was treated with intra-arterial Adriamycin and radiotherapy but no surgery, and is alive free of disease 49 months later. The combination of radiotherapy and intra-arterial Adriamycin with surgery resulted in significant acute toxicity. This small study has not demonstrated any improvement in local control compared with that expected with conservative surgery and radiotherapy alone.
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PMID:Intra-arterial adriamycin, conventionally fractionated radiotherapy and conservative surgery for soft tissue sarcomas. 173 76

A 66-yr-old woman with cutaneous angiosarcoma of the face presented with thrombocytopenia and metastases to the skeleton. Scintigraphic imaging with 111In-oxine-labeled autologous platelets demonstrated localization of radiolabeled platelets at sites of metastatic tumor. This imaging study suggests intratumoral destruction of platelets by the metastases of the malignant vascular tumor as the cause of the patient's thrombocytopenia.
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PMID:Metastatic angiosarcoma with thrombocytopenia and intratumoral indium-111-platelet deposition. 174 15

Angiosarcoma of the face and scalp developed in 12 patients. The patients were five women and seven men with an average age of 71 years. Initial features were solitary or multiple violaceous vascular nodules or plaques. The clinical course was complicated by ulceration, secondary infection, bleeding, anemia, infiltration into the underlying bones, tumor cachexia, and death. Metastases were not observed. Histologically, seemingly benign hemangiomatous capillary-like structures were found in some areas of the tumors, with richly cellular, solid sarcomatous proliferations in other areas. Early and extensive surgical excision is the therapy of choice, but generally it does not alter the relentless course of the disease. Neither palliative radiation therapy nor polychemotherapy is capable of interfering with tumor progression.
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PMID:Angiosarcoma of the face and scalp. 181 27

Between 1982 and 1990, 69 adult patients were admitted to Memorial Sloan-Kettering Cancer Center with a diagnosis of angiosarcoma, lymphangiosarcoma, or malignant hemangiopericytoma. The existing literature regarding sarcomas focuses on individual histologic conditions or site-specific activity and includes pediatric patients, which makes estimates of survival difficult. We describe the clinical course of all vascular sarcomas, the survival without distant recurrence of patients with vascular sarcomas, and the overall survival of patients with vascular sarcomas. Using Cox's stepwise regression model, histologic characteristics of the tumors, tumor grade, tumor size, and other factors were assessed to determine their prognostic significance. Noncurative treatment and the presence of metastases were the only two factors influencing survival. Tumor grade approached significance. Disease recurrence was common among curatively treated patients (37% of such patients), and more than half of these recurrences involved distant sites. The survival of these patients depends on complete surgical resection and is independent of individual histologic characteristics of the tumor.
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PMID:Vascular soft-tissue sarcomas. An analysis of tumor-related mortality. 184 76

Seventeen cases of soft tissue sarcoma (STS) developing in the pleural cavity were collected from Japanese hospitals, and their clinical and pathologic findings summarized. Eight of the 17 patients had a 15-year to 50-year (mean, 28.8) history of chronic pleural inflammatory disease (pleuritis, pyothorax, and pulmonary tuberculosis) before the onset of the pleural sarcoma. Histologically, malignant fibrous histiocytoma was the most common tumor type (11 cases), followed by angiosarcoma (four). The age at diagnosis of the sarcoma ranged from 15 to 74 years (mean, 58); the male-to-female ratio was 3.3:1. In the eight cases of sarcoma associated with chronic pleural inflammatory disease, male preponderance was more marked (7:1). The commonest presenting symptom was chest pain. A mass could be detected by chest roentgenograms in 13 patients and computed tomographic scans in 15 patients. No patient had distant metastases at first admission. Thirteen patients were treated by surgery, chemotherapy, and/or radiation therapy. Thirteen of the 17 patients died 1 to 87 months (mean, 14.2) after therapy for STS. The actuarial 1-year survival rate was 38.5%. These findings suggest that long-standing pleural inflammation might be an etiologic factor for development of pleural STS.
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PMID:Soft tissue sarcoma of the pleural cavity. 189 56

Epithelioid sarcoma (ES) is a rare malignant tumour of young adults, usually presenting as a skin ulcer or subcutaneous nodule in the distal portion of the upper limb. Multiple recurrences and late metastases are typical, leading to fatality in a third to one-half of all cases. The slow evolution of the tumour is one reason for its delayed recognition. The other is its frequent histological misinterpretation, in particular, as a peculiar granulomatous reaction. In our case, the primary tumour presented a variant morphological pattern so closely mimicking a cavernous angiosarcoma as to mislead several reputable opinions. Later recurrences and metastases were typical of ES, while a focal angiomatoid pattern was maintained. The morphology and immunoreactivity to a wide spectrum of tumour markers is compared with that of six file cases of classical ES. Retrospectively, all neoplastic lesions in our patient were ES. In young adults, lesions of the upper extremity, even when angiomatoid or haemorrhagic, should raise a suspicion of ES. Once epithelioid sarcoma is suspected, the differential diagnosis can be elucidated on immunohistochemical grounds. Early diagnosis provides the best opportunity for radical surgery at a stage when the tumour has not spread locally or disseminated systemically.
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PMID:Epithelioid sarcoma mimicking angiosarcoma: the value of immunohistochemistry in the differential diagnosis. 190 Sep 74

Primary neoplasms of the heart are rare and difficult to diagnose prior to surgery, even with modern imaging techniques. Often, the tumors are diagnosed only at autopsy. Angiosarcoma is the most common malignant neoplasm. This disease is most commonly found in middle-aged men, and the tumor is most often located in the right atrium. It commonly causes blood flow abnormalities, extensively infiltrates cardiac structures, and may extend through the heart wall to involve adjacent structures. Metastatic spread at the time of diagnosis is common, and surgical mortality is high. We present a case of primary angiosarcoma involving the right ventricle of the heart. This tumor developed 6 months after the patient had undergone coronary artery bypass surgery. The patient was initially thought to have a massive thrombus within the right ventricle but at surgery was found to have a malignant neoplasm invading the myocardium. Subsequently, he was found to have pulmonary metastases. A debulking procedure was performed, and the patient was started on chemotherapy. Rather prompt improvement occurred after the debulking procedure, but subsequent studies have indicated progression of the pulmonary metastases despite ongoing chemotherapy.
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PMID:Cardiac angiosarcoma: a case report. 194 Jun 96

The authors report the case of a 2-year-old boy with a recurrent and locally metastasizing, spindle cell, vascular tumor with histiocytoid cells involving the skin, subcutaneous tissue, and muscle of the right forearm, the right distal radius and ulna, and multiple lymph nodes of the right axilla. Diagnoses of hemangioma, hemangiopericytoma, angiomatosis, spindle cell hemangioendothelioma, and malignant hemangioendothelioma were made on successive excision specimens. The soft tissue of the right arm became diffusely enlarged, and a severe syndrome developed that was similar to that described by Kasabach and Merritt. The limb was amputated above the elbow, the axillary lymph nodes were cleared, and a total dose of 6,000 centigrays axillary radiation was given. After operation, the Kasabach-Merritt syndrome resolved. Despite the lymph node metastases and multiple tissues involved, the patient has remained well 6 years after surgery. Although the tumor exhibited some of the histologic features of a spindle cell hemangioendothelioma, the low-grade aggressive behavior resembled that of an epithelioid hemangioendothelioma. The reported case cannot be classified into any of the recognized categories or subdivisions of vascular tumors.
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PMID:Locally metastasizing vascular tumor. Spindle cell, epithelioid, or unclassified hemangioendothelioma? 195 Nov 88

We report a case of angiosarcoma of the large bowel in a 66 year old man with histochemical, immuno histochemical and ultra structural study. The patient subsequently completed non surgical staging, showing no extension of the tumor. No etiologic factor was found. Despite complete surgical exeresis, the clinical course is rapidly fatal with occurrence of numerous metastases. Primary angiosarcoma of the gastro intestinal tract are very uncommon. According to the degree of differentiation the diagnosis of large bowel's primary angiosarcoma can be difficult with others large bowel sarcoma, a benign blood vessel tumor or an undifferentiated carcinoma. The immuno histochemical and ultra structural study are useful to perform the diagnosis of angiosarcoma.
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PMID:[Primary angiosarcoma of the colon. Anatomo-clinical study of a case]. 203 31


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