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At least 19 recordsLinked to original sources
PubMed2000

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.

Carotid Body Tumor↗
PubMed1998

Verrucous angiosarcoma of the skin: a distinct variant of cutaneous angiosarcoma.

AIMS: Cutaneous angiosarcoma is a malignant vascular neoplasm with well established clinical and histopathological features. Epidermal changes in cutaneous angiosarcoma other than atrophy and necrosis have not been described. In this study, we report three cases of a peculiar variant of angiosarcoma with striking verrucous changes of the overlying epidermis. METHODS AND RESULTS: On light microscopy, verrucous changes characterized by marked pseudocarcinomatous epithelial hyperplasia with hypergranulosis, compact orthokeratosis, and papillomatosis were present overlying an angiosarcoma in the dermis. Clinically the lesions presented as violaceous to brown scaly verrucous nodules or plaques. All lesions developed on an extremity. CONCLUSIONS: Verrucous epidermal changes seem to be a manifestation of pseudocarcinomatous hyperplasia, presumably caused by lymphoedema and the superficial setting of the tumour in the papillary dermis. It is important to be aware of this presentation of angiosarcoma because verrucous epidermal changes may be seen in a wide variety of skin diseases. Furthermore, a biopsy that includes the dermis is necessary to ensure that the angiosarcoma underlying the hyperplastic epidermis is correctly identified. The descriptive appellation verrucous angiosarcoma of the skin is proposed to designate a newly recognized variant of angiosarcoma.

Adult↗
PubMed1999

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.

Adult↗
PubMed2003

Two cases of epithelioid angiosarcoma involving the thyroid and a brief review of non-Alpine epithelioid angiosarcoma of the thyroid.

Epithelioid angiosarcoma involving the thyroid is a rare entity, more often described in the Alpine region. Two cases of epithelioid angiosarcoma that affected the thyroid in patients from a non-Alpine location were diagnosed during a 10-year period in our department. The first case occurred in an 89-year-old Chinese man with a history of longstanding goiter, whereas the second case involved a 74-year-old Chinese man with a history of angiosarcoma of the scalp. On histologic examination, both thyroid tumors were composed of plump epithelioid cells with vesicular chromatin and prominent nucleoli, forming vascular structures and solid sheets. Positive staining for CD31 and factor VIII-related antigen confirmed endothelial differentiation in both cases. Both patients died within 5 months following the diagnosis of thyroid disease. The relationship of the scalp angiosarcoma and thyroid disease of the second patient is unclear. A brief review of non-Alpine primary thyroid epithelioid angiosarcoma is presented.

Aged↗
PubMed1983

Angiosarcoma of the vagina: a clinicopathologic report. A reappraisal of the radiation treatment of angiosarcomas of the female genital tract.

A first case of angiosarcoma of the vagina is described. Review of medical literature fails to show any documented cases of primary angiosarcoma of the vagina. Because of its rarity, no definite treatment could be suggested. The guideline for the management of this disease can only be drawn from several previously reported angiosarcomas of the uterus, cervix, and pelvis. It is recommended that a combined form of external irradiation and intracavitary brachytherapy be used after removal of the tumor.

Brachytherapy↗
PubMed2000

Cutaneous angiosarcoma arising in a gouty tophus: report of a unique case and a review of foreign material-associated angiosarcomas.

Rare cases of angiosarcoma have been reported to arise in the setting of retained foreign material or in association with arteriovenous fistulae. No previous case of angiosarcoma, or any other malignancy, has been reported to arise with a gouty tophus. We present a case of an 86-year-old man with a high-grade angiosarcoma that arose within a long-standing tophus.

Aged↗
PubMedDate not supplied

Two new cases of liver angiosarcoma: history and perspectives of liver angiosarcoma among plastic industry workers.

In this report of two new cases of liver angiosarcoma (ASL) among plastic industry workers, the authors present the history and perspectives of this problem. The first cases of ASL have been registered since 1974, and in 1984, the European register of angiosarcoma was founded. In this register, 11 cases of ASL and one case of haemangiopericytoma have been registered from Croatia, all from a single plastics plant near Split. Two new cases of ASL (in retired autoclave cleaners, who were exposed to a concentration of 500-1000 ppm vinyl chloride monomer (VCM) during the working process) in the same plant are represented. They were detected with combined techniques of ASL detection, and both are still alive. The diagnoses have been histologically confirmed: one of them was surgically treated with segmental liver resection. The appearance of new cases of ASL confirms the perspective presented in the last report by the same authors.

Adult↗
PubMed2003

Oral and salivary gland angiosarcoma: a clinicopathologic study of 29 cases.

Angiosarcomas of the oral and salivary gland area are extremely rare, mostly presented as case reports. We wanted to study the clinicopathologic features of a series of oral and salivary gland angiosarcomas. Cases coded as "angiosarcoma" were retrieved from the Oral and Maxillofacial Pathology Department of the Armed Forces Institute of Pathology. Patient folders and pathology were reviewed and recorded; immunohistochemistry and follow-up were obtained. Inclusion required oral or salivary gland location, vasoformative growth, cytologic atypia, mitoses, and vascular markers. Skin, bone, and subcutaneous angiosarcomas were excluded. Primary and secondary (metastatic) oral angiosarcomas were included. The 22 primary angiosarcomas involved tongue (n = 9), parotid (n = 4), lip (n = 4), submandibular gland (n = 3), and 1 each of soft and hard palate. The 7 secondary angiosarcomas involved the gingiva (n = 4) and parotid gland (n = 3). Overall, patient ages ranged from 6-90 years (mean, 55 years). There were 15 males and 14 females. Symptoms included a mass with recent enlargement and bleeding. Tumor sizes ranged from 0.8-7.0 cm (mean, 2.6 cm). Histologically, all tumors were vasoformative; 86% had solid and 17% had distinctive papillary areas. Eight (28%) were classified as the epithelioid subtype. Immunohistochemical stains showed that the tumor cells were positive for Factor VIIIrag in 19/21, CD31 in 16/19, CD34 in 7/12, and Ulex in 1/1. Primary tumors were classified as low grade (n = 7, in all locations except salivary gland), intermediate (n = 7), and high grade (n = 8); all secondary tumors were high grade. Follow-up was available on 14/22 primary and 7/7 secondary angiosarcomas. Of primary tumors, two tongue angiosarcoma patients died at 1 and 9 years, but 4 were alive without disease over a mean of 7.3 years (range, 1-13 years). Four primary salivary gland angiosarcoma patients were alive without disease over a mean of 5.8 years (range, 1-14 years), and 1 had only a late (15 years) metastasis and death (at 20 years). Three primary lip angiosarcoma patients were without disease over a mean of 14.3 years (range, 13-16 years). Of secondary tumors, three salivary gland angiosarcoma patients died within 1 year, and all four secondary gingival angiosarcoma patients died of disease within 3 years. Assessing follow-up of primary oral and salivary gland angiosarcoma patients by grade, 5 patients with high-grade tumors had no evidence of disease over a mean of 7.6 years (range, 1-16 years), 3 patients with intermediate-grade tumors had no evidence of disease over a mean of 12.7 years (range, 11-14 years), 2 patients with intermediate-grade tumors died of disease at 9 and 20 years, 3 patients with low-grade tumors had no evidence of disease over a mean of 6.3 years (range, 1-14 years), and 1 patient with low-grade tumor died of disease at 1 year. Primary oral and salivary gland angiosarcomas, albeit rare, mostly involve the tongue, parotid gland, and lip of adults, often with relatively good outcome. Although the most common angiosarcoma morphology in this area is spindled vasoformative and solid, almost one third of oral and salivary gland angiosarcomas are the rare epithelioid angiosarcoma variant. Most gingival and few parotid angiosarcomas appear to be metastases from other locations, with many patients succumbing to death within 3 years. Despite predominantly high- or intermediate-grade morphology, patients with primary angiosarcoma of the tongue, salivary gland, and lip have a better prognosis than do patients with primary cutaneous or deep soft tissue angiosarcoma, including those patients with secondary oral and salivary gland involvement.

Adolescent↗
PubMed1999

Nine breast angiosarcomas after conservative treatment for breast carcinoma: a survey from French comprehensive Cancer Centers.

OBJECTIVES: To conduct a survey of the angiosarcomas developing after breast conservation for carcinoma in the French Cancer Centers, to study the evolution of these cases in detail, and to review literature in an attempt to propose an optimal treatment scheme. MATERIAL AND METHODS: Eleven of the 20 French Cancer Centers agreed to research and retrospectively analyze all angiosarcomas discovered in patients previously treated by conservative treatment. The majority of the patients were node negative, T1N0M0. The mean age of the patients at the time of primary breast cancer treatment was 62.5 years, and 69 years at the diagnosis of the angiosarcoma. RESULTS: During the last two decades, nearly 20,000 patients have been treated conservatively in these 11 centers, and only 9 cases of angiosarcoma were found. The median latency period between the treatment of the breast carcinoma and the diagnosis of the breast angiosarcoma was approximately 74 months, with a range of 57-108 months. Mastectomy was performed as the main treatment of this angiosarcoma. All recurrences after mastectomy for the angiosarcoma appeared within 16 months after the mastectomy. A median time of recurrence was found to be 7.5 months, regardless of the treatment. The angiosarcomas appeared to be very aggressive, and chemotherapy, radiotherapy, and sometimes hyperthermia could only palliate the condition for a short time. After the diagnosis of angiosarcoma, the median survival was 15.5 months, showing a particularly poor prognosis. Only 1 patient of 9 is alive without progressive disease at 32 months after salvage mastectomy for the recurrence of the angiosarcoma. Precise data obtained from 11 centers show that, of 18115 breast carcinomas treated conservatively, only 9 breast angiosarcomas are reported, which represents a prevalence of 5 cases of angiosarcoma per 10,000, which is the same prevalence for primary breast angiosarcomas occurring in healthy breasts. CONCLUSION: Angiosarcoma developing after breast conserving therapy for carcinoma is a rare event, and induction of it by treatment is controversial. However, early diagnosis is essential and it appears that radical mastectomy gives the highest chance of cure and the best long-term survival.

Age of Onset↗
PubMed1994

Cutaneous angiosarcoma and atypical vascular lesions of the skin and breast after radiation therapy for breast carcinoma.

Despite the widespread use of radiation therapy to treat breast carcinoma, angiosarcomas arising in the field of radiation therapy are rare. The authors studied three patients with cutaneous angiosarcoma and four patients with atypical vascular lesions (AVL). All had breast conserving surgery, axillary lymph node dissection, and radiation therapy for breast carcinoma. Six patients received conventional high energy postoperative doses of external beam radiation therapy to the breast. Details of radiation therapy were not available for one angiosarcoma patient. Angiosarcoma was diagnosed 3.5 years, 3.7 years, and 5.25 years after radiotherapy. The three angiosarcomas were multifocal or diffuse and high grade, with solid cellular foci located mainly in the dermis. In three patients AVL presented as discrete skin nodules (2 unifocal and 1 multifocal) and in one patient as a breast mass. The four AVL consisted of focal proliferation of dilated vascular spaces lined predominantly by a single layer of plump and sometimes hyperchromatic endothelial cells. After two angiosarcoma patients underwent mastectomy, one died 10 months after diagnosis with recurrent local angiosarcoma and the other is alive without angiosarcoma 2 months after diagnosis. One angiosarcoma patient died of unrelated causes 2 weeks after diagnosis. One AVL patient developed a second cutaneous AVL in the axillary region 17 months after excision of an AVL from the same area. None of the AVL patients has developed angiosarcoma or recurrent mammary carcinoma. They remain well 10 months, 18 months, 7 years and 7 months, and 10 years, respectively, after initial local excision. The authors conclude that cutaneous angiosarcoma and unusual benign cutaneous vascular lesions can develop within the field of radiation therapy for breast cancer. Unlike other radiation therapy-induced sarcomas, cutaneous angiosarcoma often occurs within a short time interval after radiotherapy. It is important to distinguish AVL from angiosarcoma. Longer follow-up will be necessary to fully characterize the prognostic importance of atypical vascular lesions, but currently there is no evidence that they represent a precursor to radiation-induced angiosarcoma.

Aged↗
PubMedDate not supplied

Immunohistological distribution of stem cell factor and kit receptor in angiosarcoma.

Angiosarcoma is a rare neoplasm, originating in the endothelium, which has a poor prognosis because of a high potential for metastasis. Although little is known about the pathogenesis of angiosarcoma, angiogenic cytokines are suggested to play an important role in tumor progression in a paracrine/autocrine fashion. Mast cells contain several mediators or cytokines influencing vascularization. To clarify the role of mast cells in angiosarcoma, mast cells were counted in primary lesions of angiosarcoma (n=7). The results showed that the number of mast cells in the lesional skin of angiosarcoma (91.2+/-14.6/mm2) was significantly increased compared to that in normal skin (30.1+/-4.6/mm2) (p < 0.001). Immunohistological localization of stem cell factor, a mast cell growth factor, demonstrated that stem cell factor-positive cells occurred in the tumors forming the vascular lumen in nodular-type angiosarcoma. In macular angiosarcoma, stem cell factor was also detected in the tumor vascular endothelial cells. Infiltrating mast cells were positive for the kit receptor in both types of angiosarcoma. These results suggest that tumor cell-derived stem cell factor may play a role in the increased number of mast cells, via the kit receptor, which may contribute to the proliferation of tumor cells, leading to the progression of angiosarcoma.

Aged↗
PubMed1992

Immunohistochemical studies of blood group antigens ABH in cutaneous angiosarcoma.

Eight angiosarcomas were clinicopathologically and immunohistochemically investigated. Four of the angiosarcomas originated in the scalp of the elderly, and four cases were angiosarcomas associated with chronic lymphedema after hysterectomy for uterine carcinoma or after mastectomy for breast carcinoma. Six of the angiosarcomas showed conventional histopathology, whereas the remaining two cases were considered cutaneous epithelioid angiosarcoma. Immunohistochemical studies for blood group antigens ABH were carried out. Three of the conventional angiosarcomas were weakly positive and three were moderately positive with antigens ABH. The antigens were strongly positive in the two epithelioid angiosarcomas. The biologic course of the two epithelioid angiosarcomas was longer than that of the other six cases. This study suggested that the strong positivity of blood group antigens may be one of the characteristics of epithelioid angiosarcoma.

ABO Blood-Group System↗
PubMed1979

The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature.

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.

Adult↗
PubMed1997

Angiosarcoma of the liver in Great Britain in proximity to vinyl chloride sites.

OBJECTIVES: To study the incidence of angiosarcoma of the liver in England and Wales 1979-86 and Scotland 1975-87. To investigate whether any non-occupational neighbourhood cases occurred near a vinyl chloride site. METHODS: This is a geographical study of incident cases among the general population of Great Britain. Diagnosis of angiosarcoma of the liver was based mainly on the national cancer registry, the world register of cases among vinyl chloride workers, and the register of cases (including histological review) maintained by the Health and Safety Executive. Proximity (< 10 km) of residence to a vinyl chloride site was based on postcode of address at the time of diagnosis. RESULTS: 55 cases were ascribed to angiosarcoma of the liver in England and Wales with a further six cases in Scotland (annual incidence in Great Britain from all sources of around 1.4 cases per 10 million population). There were two cases with documented exposure to Thorotrast, and 10 cases among vinyl chloride workers. There were no vinyl chloride sites in Scotland. Among the 25 cases in England and Wales with histological diagnosis after review by a panel of pathologists, only 15 were confirmed as angiosarcoma, and one of the two Scottish cases after histological review was also confirmed. Overall, 11 cases ascribed to angiosarcoma were resident within 10 km of a vinyl chloride site; nine were vinyl chloride workers, one further case on histological review was not considered to have been correctly diagnosed as angiosarcoma, and the remaining case, confirmed as angiosarcoma, was employed at a vinyl chloride factory during the late 1950s, although not as a vinyl chloride worker. CONCLUSION: The incidence of angiosarcoma of the liver in Great Britain remains extremely rare. The one confirmed case in a non-vinyl chloride worker within 10 km of a site must nevertheless be presumed to have been exposed to vinyl chloride in the workplace. In the period of study, there were no confirmed non-occupationally exposed cases of angiosarcoma among residents living near a vinyl chloride site in Great Britain.

Adult↗
PubMed1995

Use of immunohistochemical procedures in diagnosing angiosarcoma. Evaluation of 98 cases.

BACKGROUND: Differential diagnosis of angiosarcoma, predominantly showing a non- or poorly vasoformative proliferation from other types of sarcomas, poorly differentiated carcinomas, and amelanotic melanoma, is often problematic. METHODS: The use of antibodies directed against Factor VIII-related antigen (FVIIIRA), Ulex europaeus lectin type 1 (UEA-1), CD31, and vascular endothelial growth factor (VEGF) in the diagnosis of angiosarcoma was examined in 98 cases of autopsy-proven angiosarcoma diagnosed during 1974-1990 in a survey of 178 Japanese hospitals. Reactivity of angiosarcoma cells for epithelial membrane antigen, cytokeratin, and melanoma cell antigen (HMB45) also was examined. RESULTS: Histologic specimens were formed exclusively by vasoformative areas in 32 cases and combined vasoformative and varying extents of non- or poorly vasoformative areas in another 66 cases. In vasoformative areas, the proliferating cells showed a diffuse positive reaction in the cytoplasm and/or cell surface for anti-FVII-IRA in 82 (84%) of 98 cases, for anti-CD31 in 78 (80%), and for UEA-1 in 69 (70%). In non- or poorly vasoformative areas, the positivity rate for FVIIIRA, CD31, and UEA-1 was 29%, 62%, and 46%, respectively. A positive reaction was found for either one of three endothelial markers in the non- or poorly vasoformative areas of 57 cases (86%). Epithelial membrane antigen and anticytokeratin antibody were positive in 4 and 11 cases, respectively, in the vasoformative areas and in 3 and 14 cases, respectively, in non- or poorly vasoformative areas with a simultaneous positive reaction for either one of three endothelial cell markers. None of the proliferating cells showed a positive reactivity for HMB45. The positivity rates of the angiosarcoma cells for each marker were different according to the primary tumor sites. The angiosarcoma cells in non- or poorly vasoformative areas showed the lowest positivity rate for anti-FVIIIRA in the heart (9%) and for anti-CD31 in the extremities (17%) and the highest positivity rate for anticytokeratin in the trunk (60%). Ulex europaeus lectin type 1 had almost the same reactivity rate (30-56%) in every organ. Angiosarcoma cells in 13 (36%) of 36 biopsy specimens and 8 (14%) of 56 autopsy specimens were positive for the anti-VEGF antibody. CONCLUSION: These findings suggest that the combined use of endothelial cell markers including FVIIIRA, UEA-1, and CD31 is useful in the diagnosis of angiosarcoma, especially in cases exclusively with a non- or poorly vasoformative pattern.

Adolescent↗