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)

Kaposiform hemangioendothelioma (KH) is a rare tumor of childhood often associated with Kasabach-Merritt phenomenon (KMP) and occasionally lymphangiomatosis. Although generally considered distinct from other vascular neoplasms, its rarity has precluded a thorough study of its immunophenotypic profile and long-term behavior. Thirty-three cases of KH were reviewed and immunostained for alpha-smooth muscle actin, various endothelial markers (CD31, CD34, vWf, FLI1), a platelet marker (CD61), and the juvenile hemangioma-associated markers GLUT-1 and Lewis Y antigen (LeY). In addition, the presence of HHV-8 was evaluated by RT-PCR. The patients (20 males and 13 females) ranged in age from 2 weeks to 20 years (mean 3 years 9 months). Tumors developed on the extremities (17 cases), head/neck (8 cases), and other sites (8 cases) and affected both superficial and deep soft tissue. Those in the skin presented as slightly raised blue-red lesions. More than half of the patients presented with KMP (14 of 25). Tumors consisted of irregular, infiltrating nodules of compressed vessels, which modulated between areas resembling a capillary hemangioma and Kaposi sarcoma (KS). Endothelial cells in nodules were CD31, CD34, and FLI1 positive but negative for GLUT1 and LeY. Scattered "epithelioid" or glomeruloid islands featuring endothelium associated with clusters of plump alpha-smooth muscle actin-positive pericytes, stippled hemosiderin, and CD61-positive fibrin thrombi likely represent the morphologic sites of platelet consumption. Small and large lymphatic channels occurred in 22 of 33 cases and were typically seen peripheral or deep to the main tumor mass. HHV-8 transcripts were not identified (0 of 3 cases). Follow-up information was available in 22 patients (range 8 months to 15 years; mean 2 years) and indicated that 3 died of disease, 8 were alive with disease, and 10 were alive without residual disease. Two patients developed regional perinodal soft tissue involvement, but none developed distant metastases. KH is a lesion having both a vascular and lymphatic component. Its common association with KMP probably relates in part to unique architectural features that favor turbulent blood flow and platelet activation. KH can also be reliably separated from JH by GLUT-1 and LeY immunostaining, indicating differences in the morphologic and functional attributes of the endothelium between the two lesions. The absence of HHV-8 in KH underscores a different pathogenesis from Kaposi sarcoma. Our study, the largest to date, emphasizes that mortality is due to KMP and not metastatic disease, which appears limited to regional perinodal soft tissue. Given this behavior, its continued classification as a vascular tumor of intermediate malignancy is warranted.
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PMID:Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. 1510 42

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that mainly occurs during childhood. It generally originates on the skin, usually affecting deeper tissue by infiltrative growth. It appears as one or multiple masses, and in most cases is associated to consumptive coagulopathy (Kasabach-Merritt syndrome), and lymphangiomatosis. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. These tumors tend to be locally invasive, but are not known to produce distant metastases. The development of KHE in adolescents or in adults is very rare, but cases have also been described. Several factors are associated with the outcome of patients with KHE: accessibility to surgical excision, location (cutaneous versus visceral), size of tumoral mass, clinical response to interferon and glucocorticoids, and the absence of lymphangiomatosis and Kasabach-Merritt syndrome, may result in partial remissions. On the other hand, bulk visceral masses lead to a 40-50% mortality rate, mainly due to progressive failure of the infiltrated organ(s), in spite of interferon, glucocorticoids, and combined chemotherapy. In conclusion, the onset of a consumptive coagulopathy following the presence of a vascular tumor, in children as well as in older patients, should spark suspicion of KHE, among other entities.
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PMID:Kaposiform hemangioendothelioma. 1932 97

We report a case of a neonate presenting with the rare vascular tumor, Kaposiform hemangioendothelioma. She had a lesion arising from the left ovary with multiple intraperitoneal metastases causing small bowel obstruction. We managed this case with primary surgical resection followed by laparoscopic surveillance of the peritoneal cavity and metastectomy. The child is fit and well and free of tumor after 32 months. Neither this presentation of this tumor nor this management approach has been previously described in the literature.
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PMID:Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance. 2243 Dec 47

Kaposiform haemangioendothelioma is a rare, locally aggressive vascular tumour, which is mainly found in children. Typically, it occurs in the skin. The tumour is borderline malignant due to its rapid infiltrative growth. Metastasis has not been described. Since 1993, when this tumour was first defined, less than 160 cases have been reported. To our knowledge, this is the first case of kaposiform haemangioendothelioma in the gastrointestinal tract. We report a case of a three year-old boy, who presented with small bowel obstruction due to invagination of a kaposiform haemangioendothelioma.
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PMID:[Kaposiform haemangioendothelioma in the small intestine of a three year-old boy]. 2268 97

The homeobox gene PROX1 is critical for organ development during embryogenesis. The Drosophila homologue, known as prospero has been shown to act as a tumor suppressor by controlling asymmetric cell division of neuroblasts. Likewise, alterations in PROX1 expression and function are associated with a number of human cancers including hematological malignancies, carcinomas of the pancreas, liver and the biliary system, sporadic breast cancer, Kaposiform hemangioendothelioma, colon cancer, and brain tumors. PROX1 is involved in cancer development and progression and has been ascribed both tumor suppressive and oncogenic properties in a variety of different cancer types. However, the exact mechanisms through which PROX1 regulates proliferation, migration, and invasion of cancer cells are by large unknown. This review provides an update on the role of PROX1 in organ development and on its emerging functions in cancer, with special emphasis on the central nervous system and glial brain tumors.
Cancer Metastasis Rev 2012 Dec
PMID:Transcription factor PROX1: its role in development and cancer. 2273 8

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that does not metastasize. We report two unique cases of KHE involving the right hemithorax and the upper ipsilateral extremity. Kasabach-Merrit phenomenon and osteolytic lesions in the scapula were observed in both cases.
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PMID:Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma. 2838 16

Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.
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PMID:Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review. 3046 40