1/4. Multifocal cerebral hemangio-endothelioma. Case report and review of the literature.BACKGROUND: Intracranial hemangio-endotheliomas (HE) are rare vascular tumours that present histological features and biological behaviour of an intermediate malignancy. We report the first case of primary HE presenting as multiple intracranial masses. CASE DESCRIPTION: The patient is a 20-year-old female who presented with paresis of the left arm. magnetic resonance imaging (MRI) showed multiple lesions in both frontal lobes. Total surgical excision of the tumours was obtained; a histological diagnosis of hemangio-endothelioma was made. Five months later, MRI scan showed new intradiploic skull lesions whereas a scintigraphic study revealed multicentric extracranial bone involvement. Treatment based on interferon-alpha (IFN-alpha) administration was undertaken; this produced partial regression of the lesions. Approximately three years after the first operation, the patient is in good clinical condition. CONCLUSIONS: Surgery continues to play the leading role in the treatment of intracranial HE. However, adjuvant therapy with IFN represents a new and well-tolerated therapeutic method for residual intracranial lesions as well as multicentric extracranial disease.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/4. Vascular tumors simulating occlusive disease.Two cases of vascular tumors of large vessels with intraluminal growth simulating venous thrombosis and arterial occlusive disease are reported. One was a borderline malignant epithelioid hemangioendothelioma of the femoral vein and the other a malignant epithelioid angiosarcoma of the carotid artery. Immunohistochemical studies permitted to classify the tumors. Treatment consisted in surgical resection. No recurrence and no metastasis are noted at 24 months. uncertainty regarding biological behaviour of vascular tumors and treatment persists.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/4. Splenic littoral cell haemangioendothelioma: a new low-grade variant of malignant littoral cell tumour.AIMS: Littoral cell angioma is a recently described splenic vascular tumour of splenic sinus lining cells. Almost all cases of splenic littoral cell tumours hitherto described were benign. methods AND RESULTS: A splenic littoral cell tumour recurred 8 years after splenectomy, with an abdominal mass and multiple liver metastases, resulting in the patient's death. Histologically, the original splenic tumour showed solid areas with small necrotic foci in addition to large areas of typical littoral cell angioma. The recurrent tumours showed increased solid architecture and slightly increased nuclear atypia. The tumours showed an immunohistochemical profile positive for factor viii, CD31, CD68, cathepsin d, and CD21 and negative for CD34 and CD8, consistent with the immunophenotype of classic littoral cell angioma. Ki67 index in the recurrent tumours was higher than in the primary tumour. CONCLUSIONS: The mildly atypical, but not frankly malignant, histological features as well as the protracted clinical course support definition of the tumour as 'littoral cell haemangioendothelioma'. Low rate of Ki67 staining and diploid dna histogram with low S-phase fraction of the tumours are in accordance with a low-grade malignancy. literature review revealed two other cases of littoral cell tumours with disseminated disease that may be other examples of littoral cell haemangioendothelioma. Littoral cell haemangioendothelioma should be distinguished from the overtly malignant splenic angiosarcomas, of which a few may show splenic lining cell differentiation with some immunohistochemical features of littoral cells. Due to difficulties in predicting biological behaviour based on histological features of splenic littoral cell tumours, a long-term follow-up for these patients, especially for those with atypical histology, is recommended.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/4. Composite cutaneous haemangioendothelioma: case report and review of the literature.Composite cutaneous haemangioendothelioma is a recently characterized rare tumour of vascular origin. As there are only a few reported cases, the biological behaviour of the tumour and appropriate therapeutic approaches are not yet clear. We report a new case of composite cutaneous haemangioendothelioma and discuss prognostic and therapeutic aspects of this neoplasm.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |