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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↗
PubMed2005

Paclitaxel and pegylated-liposomal doxorubicin are both active in angiosarcoma.

BACKGROUND: Paclitaxel has unique activity in angiosarcomas of the face and scalp, but its activity in angiosarcomas originating at other sites is less well defined. Paclitaxel and pegylated-liposomal doxorubicin (PLD) are highly effective in Kaposi sarcoma (KS). Because of the efficacy of PLD in soft tissue sarcoma in general, and in KS in particular, coupled with potential similarities in KS and angiosarcoma, and the apparent activity of paclitaxel in angiosarcomas, the authors treated patients with angiosarcoma with either paclitaxel or PLD as initial chemotherapy. METHODS: To better define the efficacy of these agents in angiosarcoma, the authors reviewed their experience with paclitaxel and PLD in patients with angiosarcoma treated between 1994 and 2004. RESULTS: They identified seven patients with angiosarcoma treated with paclitaxel, and six treated with PLD. Only one patient in the series had an angiosarcoma of the scalp. Two patients receiving paclitaxel had received previous therapy with PLD, and four of six patients treated with PLD had previously received paclitaxel. Of the eight patients treated with paclitaxel, five had major responses (three had partial responses [PR] and two had complete disease remission [CR]) and three had progressive disease (PD). Of the 6 patients who received PLD, 3 had a PR for 6, 19, and >20 months, respectively, 2 had stable disease for 7 and 11 months, respectively, and 1 had PD. CONCLUSIONS: The current study demonstrated the activity of PLD (five of six patients experienced clinical benefit) and extended the data on paclitaxel in angiosarcoma, both of the face and scalp, as well as angiosarcoma originating at other sites.

Aged↗
PubMed2005

Angiosarcoma of the breast.

BACKGROUND: Angiosarcoma of the breast is a rare entity. The objectives of this study were to evaluate prognostic factors and determine outcomes in a large contemporary series of patients. METHODS: Clinical and pathologic factors were analyzed in all patients with angiosarcoma of the breast treated between 1990 and 2003. Overall survival (OS) and disease-free survival (DFS) were estimated using the methods of Kaplan and Meier. Multivariate analyses were performed to evaluate prognostic factors. RESULTS: Fifty-five women with angiosarcoma of the breast were identified. The median age was 49.1 years, and the median follow-up time was 3.7 years. The median OS and DFS were 2.96 years and 2.26 years, respectively. For the 32 patients with primary angiosarcoma of the breast 5-year OS was 59%. Twenty-three (42%) patients had received prior radiation therapy for the treatment of breast cancer. These patients with radiation therapy-associated angiosarcoma were on average 30 years older and less likely to present with distant metastatic disease than patients presenting with radiation-naive angiosarcoma of the breast. Although radiation-naive patients appeared to have had better early DFS and OS, the Kaplan-Meier curves were not statistically different between patients with radiation therapy-associated disease and radiation therapy-naive patients. On multivariate analysis, tumor recurrence (P = 0.006) was the only significant adverse prognostic factor noted for OS. CONCLUSIONS: In this series of 55 patients with angiosarcoma of the breast, radiation therapy-naive angiosarcomas occurred in younger patients, but they behaved similarly to radiation therapy-associated angiosarcomas.

Adult↗
PubMed2006

Angiosarcoma after conservation treatment for breast carcinoma: our experience and a review of the literature.

The development of angiosarcoma of the breast is a recognized complication of breast conservation therapy (BCT), but the evolution, prevalence, and outcome have not been accurately established. We sought to evaluate and review the clinicopathologic, prognostic, and treatment attributes of angiosarcoma arising in the irradiated breast after BCT. We conducted a retrospective chart and slide review of 8 patients seen between 1996 and 2004 with a diagnosis of secondary angiosarcoma. All were treated with mastectomy. Clinical and histopathologic findings were studied and previously reported cases were reviewed. Primary surgery-related breast edema and cellulitis was observed in 7 and 5 patients of the 8 patients studied, respectively. Postirradiation breast edema and grade 2/3 fibrosis occurred in 5 and 8 patients, respectively. The mean age of the patients at onset of the breast cancer and angiosarcoma was 65 and 72 years, respectively. The mean latency period between the treatment of the breast cancer and the diagnosis of angiosarcoma was 75 months. The actuarial rate of 2-year survival for patients presented with single (n = 4) compared with multiple (n = 4) skin lesions was 50% and 0%, respectively (P = .0233). The estimated incidence of angiosarcoma after BCT was found to be 0.14 %. BCT-associated angiosarcoma arises after a relatively brief interval, and breast edema-fibrosis can possibly contribute to its development. Special attention should be paid to skin changes occurring after BCT. The extent of skin lesions is predictive of survival. As shown by a review of the literature, angiosarcomas are often resistant to surgery, chemotherapy, and radiotherapy, and targeted therapy against tumor biological properties may be a new approach to angiosarcoma treatment.

Aged↗
PubMed2005

Comparative genomic hybridization in four angiosarcomas of the female breast.

OBJECTIVE: Angiosarcomas represent a heterogeneous group of malignant vascular tumors occurring in different anatomic sites. In the female breast, they account for less than 1% of all malignant tumors and mainly develop as secondary angiosarcomas after prior irradiation. Data on cytogenetic findings in angiosarcomas are scarce and up to now no characteristic primary aberrations have been established. We herein add molecular cytogenetic findings in another 4 angiosarcomas to 11 previously reported cases. METHODS: We investigated four angiosarcomas of the female breast (three primary angiosarcomas and one secondary tumor after irradiation for breast cancer) for DNA copy number changes using comparative genomic hybridization (CGH). RESULTS: All angiosarcomas revealed aberrant karyotypes including multiple DNA copy number changes involving various chromosomes. Recurrent DNA copy number changes that occurred in at least two cases included gains at 1q, 7p, 7q, and 8q, as well as losses at 5q and 16q. CONCLUSIONS: Angiosarcoma of the female breast represents a genetically heterogeneous tumor entity without a readily identifiable pattern of common chromosomal alterations. However, reviewing the cytogenetic literature on angiosarcomas of different sites, gains at 8q and 20p appear to emerge as the most frequent aberrations in at least a subset of these tumors.

Adult↗
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↗