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Hemangiosarcoma
Hemangioendothelioma
Abstract Background Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis. Case presentation We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years. Conclusions Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.
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Kaposi's sarcoma
Hemangiosarcoma
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Hemangioendotheliomas are vascular neoplasms occupying a spectrum of biological potential ranging from benign to low-grade malignancy. Composite hemangioendothelioma (CH) is one of the less commonly encountered variants exhibiting a mixture of elements of other hemangioendothelioma subtypes, such as epithelioid, retiform, and spindle cell. Some authors have identified areas histopathologically equivalent to angiosarcoma within CH, raising the question of the true nature of this neoplasm. Although CH recurs locally, there are only 3 reported cases which metastasized. To date, 26 cases (including the present case) have been described in the literature. Herein, we describe a unique case of CH arising in the background of previous radiation therapy and long-standing lymphedema (classically associated with the development of angiosarcoma-Stewart-Treves syndrome) that harbored higher grade areas but behaved as a low-grade malignant neoplasm. This, in conjunction with the many reported cases of CH-harboring angiosarcoma-like areas, and the occasional association with a history of lymphedema, raises the question of whether this variant of hemangioendothelioma may actually be an angiosarcoma that behaves prognostically better than the conventional type. After careful study of the natural disease progression of the current case and review of the literature, we discuss justification for the continued classification of CH as a low-grade malignancy.
Hemangioendothelioma
Hemangiosarcoma
Epithelioid Hemangioendothelioma
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Four cases of childhood hepatic angiosarcoma (HAS), representing the malignant form of infantile hemangioendothelioma, are described. The morphologic appearance of childhood HAS differs from the adult form in the following features: the associated presence of benign infantile hemangioendothelioma; the presence of dysontogenetic features; and an altered appearance of the angiosarcoma itself. It is postulated for these cases that the benign infantile hemangioendothelioma progressed to the malignant angiosarcoma. One of the four cases had exposure to elevated levels of arsenic in the environment that may have contributed to this progression. This latter case adds to published reports associating arsenic exposure with increased risk for hepatic angiosarcoma.
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We describe the clinical and pathologic features of an hepatic angiosarcoma in a 5-year-old child. The neoplasm manifested as a multicentric vascular tumor and was initially treated by lobectomy. Histopathologically, the lesion showed the features of infantile hemangioendothelioma, in which foci of cytologic atypia ("type 2") were present. A recurrence with rapid growth appeared that suggested a malignant tumor, but no metastases developed. A literature review confirmed the rarity of angiosarcomas in children and underscored the difficulties of formulating a prognostic estimate about infantile hemangioendotheliomas based on histology alone. Mitotic activity and cytologic atypism are regular features of angiosarcoma in children and cannot be ignored. This report also contrasts certain features of the biology of malignant vascular tumors of the liver in childhood with the counterpart in adults.
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The transformation of a benign hemangioma into a malignant angiosarcoma has been rarely reported, with only 11 cases reported in the literature. There have been no reports of malignant transformation of hemangioma into angiosarcoma in association with epithelioid hemangioendothelioma, to our knowledge. The existence of precursor malignancies in the tumorigenesis of sarcomas is still not clearly defined. We describe the case of a 40-year-old woman with a preceding history of a suspected hemangioma for ten years, who upon resection was found on histology to have evidence of a hemangioma with an associated area of epithelioid hemangioendothelioma as well as areas of overt high grade epithelioid angiosarcoma. These findings raise the possibility of the evolution of hemangioma to epithelioid hemangioendothelioma, and the latter to overt angiosarcoma. The patient was managed as having a high grade sarcoma with wide resection and radiation. She declined systemic adjuvant chemotherapy after a thorough discussion about the risks and benefits of chemotherapy, and she currently remains disease free one year after the surgery.
Epithelioid Hemangioendothelioma
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A 14-month-old white male was diagnosed as having angiosarcoma of both the liver and spleen. At 17 months he developed pulmonary metastases and died. There was no apparent environmental or hormonal exposure either prenatally or during infancy. The malignant tumor probably arose from its benign counterpart (hemangioendothelioma), which was found in the spleen. This is the fifth case reported of splenic angiosarcoma in the pediatric age group (18 years or younger).
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A peculiar vascular tumor of the pericardial cavity affecting a 21-year-old man is reported. The tumor was formed by spindle cells and narrow vascular channels. It was difficult to decide between primary pericardial Kaposi's sarcoma or angiosarcoma. The patient was HIV negative and no other tumors were revealed in the course of the disease. Immunohistochemical investigations showed positive endothelial markers-FVIII, CD 31, CD 34 in the tumor cells. Due to an inconclusive diagnosis of the tumor six cases of Kaposi's sarcoma and six cases of angiosarcomas were retrieved from our files, the same spectrum of immunohistochemistry as in above case applied, and the results compared. We found no significant differences in the positivity of endothelial markers among these cases and when compared with the case of pericardial tumor. The reactivity of CD 34 was, however, strong in cases of Kaposi's sarcoma whereas in angiosarcomas it yielded a weak and focal reaction only. In spite of the latter finding we feel that in individual cases, like the described tumor of the pericardial cavity, the immunohistochemical investigation cannot discriminate angiosarcoma from Kaposi's sarcoma. The reported tumor of the pericardial cavity was considered an unusual form of a low grade angiosarcoma with immunophenotypic features of Kaposi's sarcoma.
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