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 42-year-old man presented with a progressively painful mass of the penile shaft that clinically resembled superficial penile vein thrombosis. The patient's symptoms were refractory to conservative therapy, and surgical excision revealed multifocal penile epithelioid hemangioendothelioma. He had no evidence of metastatic disease and was well 1 year after excision. Vascular neoplasms should be included in the differential diagnosis of painful penile masses and penile thrombophlebitis. To our knowledge, this is only the second reported case of multifocal penile epithelioid hemangioendothelioma.
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PMID:Multifocal penile epithelioid hemangioendothelioma masquerading as superficial penile vein thrombosis. 1697 10

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

We report an unusual case of primary cardiac epithelioid hemangioendothelioma (EHE) with atypical features, which was treated by orthoptic transplantation with a good outcome for 10 years despite recurrent pulmonary and nodal metastases. EHE is a rare vascular tumor that belongs to the group of malignant proliferations from the new World Health Organization classification of soft tissue tumors. EHE may harbor atypical features that confer a more aggressive course, albeit better than that of conventional angiosarcomas. Histological examination of the primary cardiac tumor revealed a proliferation of large epithelioid tumor cells presenting atypical features and a mitotic index of 3 mitoses per 10 high power fields. In contrast, pulmonary metastases exhibited typical features of EHE, and CD 34 and CD 31 immunostainings strongly stained cytoplasmic vascular lumen. In this report, we illustrate the potential aggressiveness of the atypical variant of EHE and suggest that transplantation might be considered as an alternative therapy in the treatment of EHE of the heart.
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PMID:Atypical primary epithelioid hemangioendothelioma of the heart. 1740 53

Primary hepatic epithelioid hemangioendothelioma (HEH) is a rare, low-grade malignant neoplasm of endothelial origin, with an unpredictable clinical course and prognosis. No standard therapeutic strategies are still available for HEH, due to the infrequency of the disease and to its variable natural history that limit the identification of the most effective treatment. In the absence of metastatic disease, surgical resection or liver transplantation represent the treatment of choice for HEH, while several antineoplastic agents have been proposed in the presence of metastatic nonresectable disesase. Herein, we describe the biological characterization and the clinical course of a primary HEH progressively responsive to treatment with intermediate doses of interferon-alpha (IFN)-alpha2a. Furthermore, based on the newly-identified expression of endoglin (CD105) on HEH, we discuss the clinical potential of novel anti-angiogenetic approaches to the disease.
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PMID:Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel anti-angiogenetic treatments? 1755 Jan 44

The clinicopathologic features of 46 patients with germ cell tumors with sarcomatous components (GCTSC) involving either the primary site or their metastases were studied. There were 43 men and 3 women aged 17 to 74 years. Twenty-three tumors arose in the mediastinum, 2 in the retroperitoneum, and 21 in the gonads. The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1). The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1). Immunohistochemical studies were carried out in 34 cases with appropriate results supporting the diagnoses. Metastases containing both GCT and SC were observed in 6 cases, metastases of SC alone in 4, and metastases containing only GCT elements in 3. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. Clinical follow-up was available in 40 patients (1 to 96 mo; mean=24 mo). Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo). Comparison of these patients with an age-matched and stage-matched control group of patients with GCT without SC showed statistically significant differences in survival between the 2 cohorts (P <or=0.001). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.
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PMID:Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. 1772 Nov 91

There is controversy regarding whether lymphatic vessels are present or absent in bone. Although lymphangiomas have been described in bone, lymphatic vessels have not been identified morphologically with certainty in any other benign or malignant bone tumors or in normal human bone. In this study, we determined by immunohistochemistry, using 2 specific lymphatic endothelial cell markers, LYVE-1 and podoplanin, whether lymphatics are present in normal bone and a wide range of primary and secondary bone neoplasms. In normal bone, LYVE-1+/podoplanin+ lymphatic vessels were not identified in cortical or cancellous bone but were seen in connective tissue overlying the periosteum. With the exception of lymphangioma, Gorham-Stout disease, and hemangioendothelioma, primary benign and malignant bone tumors (as well as secondary carcinomas) that were confined to bone did not contain lymphatic vessels. Primary and secondary bone tumors that had extended through the bone cortex contained LYVE-1+/podoplanin+ lymphatic vessels that seemed to extend for a short distance from surrounding soft tissues into the tumor. Three cases of osteosarcoma that had extended through the bone cortex and had lymph node metastases were all found to contain lymphatic vessels within the tumor. These results indicate that the lymphatic circulation is unlikely to play a role in bone fluid transport in normal bone and that lymphatic vessels are absent from most primary and secondary tumors confined to bone. These findings also suggest that lymphangiogenesis is not involved in the disease progression of most primary bone tumors and that carcinomatous metastasis to bone does not occur via lymphatics.
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PMID:Lymphatics and bone. 1902 56

Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Subsequent surgery and pathologic assessment revealed a papillary angioendothelioma (Dabska tumor) within lymphatic spaces. The child has no evidence of recurrence or metastases 1 year postoperatively.
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PMID:Splenic papillary angioendothelioma in a 6-year-old girl. 1809 Sep 26

The term hemangioendothelioma has been used in recent years to name a heterogeneous group of vascular neoplasms, intermediate in both biological behavior and histopathologic appearance between benign tumors (hemangiomas) and frankly malignant tumors (angiosarcomas). Thus, within the spectrum of hemangioendothelioma have been successively included epithelioid hemangioendothelioma, spindle cell hemangioendothelioma, retiform hemangioendothelioma, kaposiform hemangioendothelioma, polymorphous hemagioendothelioma of the lymph nodes, papillary intralymphatic angioendothelioma (PILA) and composite hemangioendothelioma. The latter is a vascular neoplasm showing varying combinations of benign, low-grade malignant and malignant vascular components. We herein report a case of composite hemangioendothelioma showing a combination of retiform hemangioendothelioma, epithelioid hemangioendothelioma, spindle cell hemangioma and PILA. The neoplasm showed a more aggressive behavior than other reported cases of composite hemangioendothelioma and it developed satellitosis and metastases to the inguinal lymph nodes. Neoplastic cells expressed immunoreactivity for Prox-1, supporting a lymphatic line of differentiation.
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PMID:Cutaneous composite hemangioendothelioma with satellitosis and lymph node metastases. 1819 Apr 50

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

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of low-grade malignancy, the prognosis of which remains unpredictable. Surgical and/or chemotherapeutic options have to be evaluated depending on intrathoracic tumor spread and systemic metastases. Three cases of PEH with both clinical and pathological findings are herein summarized and the relevant current literature discussed.
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PMID:Pulmonary epithelioid hemangioendothelioma: report of three cases. 1875 52


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