1/86. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases.AIMS: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association. methods AND RESULTS: Four cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor. CONCLUSIONS: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.- - - - - - - - - - ranking = 1keywords = neurofibromatosis, peripheral, nerve (Clic here for more details about this article) |
2/86. Epithelioid angiosarcoma associated with a Dacron vascular graft.Angiosarcoma developed at the site of a Dacron vascular prosthesis 8 years after an aortobifemoral bypass graft insertion. The tumor was composed of epithelioid cells, which showed positive staining for cytokeratin and expression of the common endothelial markers CD31, CD34, and von willebrand factor. Ultrastructural examination showed aggregates of large cells with intercellular lumina and focal perinuclear whorls of intermediate filaments. The patient, who had abdominal pain and weight loss, died of disseminated pelvic and abdominal disease 6 months after diagnosis. Sarcomas associated with vascular Dacron grafts and angiosarcomas associated with metal or polymer foreign bodies are rare. Their development is probably analogous to the common experimental development of foreign body-associated sarcomas in rodents. physicians caring for patients with vascular grafts or metal foreign bodies should be aware of this complication.- - - - - - - - - - ranking = 0.18193927729091keywords = von (Clic here for more details about this article) |
3/86. Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma.Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma are rare sarcomas constituting less than 0.2% of lung cancers. The typical imaging appearance is a large, well-circumscribed, non-cavitating, non-calcified, peripheral lung mass without hilar or mediastinal lymphadenopathy. bronchoscopy and percutaneous needle aspiration are of limited value in differential diagnosis, and thoracotomy is warranted for definitive diagnosis.- - - - - - - - - - ranking = 0.0073254440955756keywords = peripheral (Clic here for more details about this article) |
4/86. Establishment and characterization of a spontaneously immortalized myofibroblast cell line derived from a human liver angiosarcoma.BACKGROUND/AIM: fibrosis and/or cirrhosis are present in the precursor stages of most liver cancers. However, little is known about the reciprocal interactions of fibroblasts, mainly responsible for fibrosis, and the other liver cells. We report here the isolation of a new liver myofibroblast cell line from a human liver angiosarcoma and its characterization. methods: The cells were isolated by the explant technique and characterization was performed, on one hand, using immunohistochemical and ultrastructural analysis and, in the other hand, by determining their karyotype, ras and p53 status and their tumorigenic properties. RESULTS: To date, the cells have undergone approximately 170 population doublings and are still proliferating. Immunohistochemically, they were negative for desmin, smooth muscle myosin, cytokeratin 19 and von willebrand factor, positive for vimentin and alpha-smooth muscle actin, with an important deposition of fibronectin around the cells. Ultrastructure showed particularly cytoplasmic microfilament bundles. Their chromosome number ranged from 38 to 168 with a bimodal population, near diploid and hypotetraploid. No mutations were found in codons 12, 13 or 61 of Ha-, Ki- and N-ras genes but a homozygous missense mutation in codon 179 (CAT-->CTT) was detected in the p53 gene. They were unable to form foci in soft agar or tumors in nude mice. CONCLUSIONS: Taken together, these results show that these cells, called BM 2.2.1, exhibited typical myofibroblast-like features. Although they contained a karyotype suggestive of tumoral cells and a homozygous mutated p53 gene, they were not tumorigenic. The nature of these cells and the abnormalities of the p53 gene and the karyotype, suggest that: i) they were a component of the tumor stroma, and ii) they could have been involved in angiosarcoma development. Thus, this cell line may be valuable for the study of cellular interactions in liver carcinogenesis.- - - - - - - - - - ranking = 0.18193927729091keywords = von (Clic here for more details about this article) |
5/86. Schwannoma with angiosarcoma. Report of a case and comparison with other types of nerve tumors with angiosarcoma.BACKGROUND: Schwannoma with angiosarcomatous change is a rare tumor, the clinical characteristics of which have not been analyzed. methods: A patient with schwannoma with angiosarcoma arising in the midneck and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of their clinical features with those of schwannoma with conventional malignant transformation and cases of neurofibroma and malignant peripheral nerve sheath tumor (MPNST) with angiosarcoma. RESULTS: There are four reported cases, including the present case. Schwannoma with angiosarcoma affects older adults, mainly men. Three tumors arose from the vagus nerve in the neck. Three of the four angiosarcomas were epithelioid in type. Treatment in all cases was surgical resection followed by radiation and chemotherapy in one case and by radiation alone in another. One patient died with residual local angiosarcoma 5 months after the diagnosis. The remaining three patients were alive and disease free at 27 months, 43 months, and 90 months, with distant metastasis (after 15 months) reported only in the patient described in this case report. CONCLUSIONS: Schwannoma with angiosarcoma should be included in the differential diagnosis of presumed carotid body paragangliomas. Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence. Schwannoma with angiosarcoma is capable of local spread with a fatal outcome and of distant metastasis, but follow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma. Recommended treatment is attempted complete surgical resection followed by radiation therapy and chemotherapy, if it can be tolerated by the patient.- - - - - - - - - - ranking = 0.020647982942115keywords = peripheral, nerve (Clic here for more details about this article) |
6/86. mast cells in an angiosarcoma complicating xeroderma pigmentosum in a 13-year-old girl.A cutaneous angiosarcoma, a rare tumor that occurs almost exclusively in sun-exposed skin of individuals older than 50 years, developed in an adolescent with xeroderma pigmentosum (XP). As this is the second report of a child with angiosarcoma and XP, ultraviolet-induced dna damage may be involved in the pathogenesis of this tumor. Strongly increased numbers of mast cells were found, particularly in the peripheral tumor area, which may reflect with the requirement of mast cells for the growth of vascular structures or a role for mast cells in the antitumor immune response.- - - - - - - - - - ranking = 0.0073254440955756keywords = peripheral (Clic here for more details about this article) |
7/86. Intimal angiosarcoma of the aorta with tumour embolisation causing mesenteric ischaemia. Report of a case diagnosed using CD31 immunohistochemistry in an intestinal resection specimen.Primary intimal angiosarcomas of the aorta (i.e. mostly intraluminal sarcomas with evidence of endothelial differentiation) are extraordinarily rare. We report a case in which the diagnosis was accurately made using immunohistochemistry in an intestinal resection specimen and confirmed during autopsy. The patient was a 64-year-old woman with mesenteric ischaemia and a "thrombus" in the abdominal aorta. Two segments of the ileum and the right colon were surgically removed. Histological examination showed multiple tumour emboli in small arteries of the submucosa, serosa and mesentery. The highly atypical cells comprising these emboli were positive immunohistochemically with antibodies to ulex Europaeus, von willebrand factor and CD31 and negative for CD34. During post-mortem examination, the intraaortic mass was located around the orifices of the coeliac and the superior mesenteric arteries, and gross tumour thrombi were found in the left renal and splenic arteries. This case emphasises the need for a wide panel of immunohistochemical antibodies when tumour emboli of unknown origin are under study.- - - - - - - - - - ranking = 0.18193927729091keywords = von (Clic here for more details about this article) |
8/86. Hepatic angiosarcoma mimicking cavernous hemangioma on angiography.A 60-year-old woman was admitted to our department for evaluation of a hepatic mass. The mass was diagnosed as a hemangioma of the liver by abdominal angiography because of typical cotton wool appearance and stretched arterial vessels and no peripheral staining. However, one month later, the mass was surgically removed because of extravasation. Histological findings of a specimen of the mass revealed that it entirely contained abundant necrotic tissue, and a small residual part after transcatheter arterial embolization was consistent with hemangioma. However, she complained of hemoptysis and thigh pain after several weeks. Computed tomography revealed multiple lung masses and a mass of right musculus gluteus medius. Reexamined histological findings of the liver tumor showed hemangiosarcoma. We should pay attention to the fact that it is sometimes difficult to differentiate cavernous hemangioma from angiosarcoma by angiography.- - - - - - - - - - ranking = 0.0073254440955756keywords = peripheral (Clic here for more details about this article) |
9/86. iridium-192 brachytherapy for hemorrhagic angiosarcoma of the scalp: a case report.A 60-year-old woman presented with multicentric skin tumors of the head. The histologically proven hemangioendothelioma was bleeding as a result of disseminated coagulopathy. In addition to immunotherapy, 6 MeV electron beam radiotherapy was used for the purpose of hemostasis with a single portal, 20 x 20 cm in size, covering the whole scalp from the top of the head. The radiotherapy was discontinued after 39 Gy/13 fractions/20 days because of the progress in size of a peripheral tumor and the stability of coagulopathy. After another electron boost delivery by two portals focused to exophytic parietal and temporal tumors of 20 Gy/10 fractions, high dose rate brachytherapy with a surface mold technique was performed, 3 Gy/fraction, four fractions/week, for a total of 36 Gy. It induced partial regression of the tumor and complete recovery of platelet counts from 2.5 x 10(4) to 18.2 x 10(4). The tumor disappeared in 3 months. No late side effects occurred, except for permanent alopecia. The patient developed a cervical lymph node metastasis 1 year after and marginal recurrence 2 years after the initial treatment. Both recurrent tumors were successfully treated by 4 MV external photons of 60 Gy/20 fractions/46 days and electron beam irradiation of 60 Gy/20 fractions/29 days, respectively. She has remained disease free for 3 years after the initial presentation.- - - - - - - - - - ranking = 0.0073254440955756keywords = peripheral (Clic here for more details about this article) |
10/86. Metastasis of a renal carcinoma to a cerebellar haemangioblastoma in a case of von hippel-lindau disease.A patient with von hippel-lindau disease had a long-standing cerebellar cyst which recurred for the fifth time. At operation there was evidence of a renal carcinoma metastasis in the wall of the cyst which was probably a haemangioblastoma.- - - - - - - - - - ranking = 0.72775710916363keywords = von (Clic here for more details about this article) |
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