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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↗

PubMed2006

Primary angiosarcoma of the breast: observations in Asian Indian women.

BACKGROUND: Primary angiosarcomas of breast are rare tumors, with a fatal outcome. MATERIAL AND METHODS: We studied histological prognostic factors and c-kit expression by immunohistochemistry (IHC) in 12 angiosarcomas accessioned at a cancer referral center in India. RESULTS: All patients had primary angiosarcoma; no case of secondary angiosarcoma was accessioned during the study period. Median age of patients was 24.5 years. Nine patients had intermediate grade tumors, one a well differentiated tumor and three patients had high-grade tumors. Interesting cases encountered included an epithelioid angiosarcoma and an angiosarcoma arising on the background of a biphasic tumor. Eight patients had lumpectomy, four mastectomy and two patients were given radiotherapy. Of the nine patients (seven type I/II and two high grade) with follow up, eight patients developed disseminated metastases within a year of presentation. The patient with well-differentiated angiosarcoma also died of metastasis albeit after a longer time. On IHC c-kit staining was weakly seen in two cases. CONCLUSION: Primary angiosarcoma was fatal in young Indian women even in lower grade tumors. The low expression of c-kit on IHC suggests that targeting this protein for therapy may not be successful in treating these tumors.

Adult↗

PubMed2006

[Clinical and immunohistochemical findings of intra- and extraoral angiosarcomas].

PURPOSE: A clinico-pathologic study of typical symptoms of intra- and extraoral angiosarcomas and clinical course under therapy is presented as well as an analysis of the immunohistochemical differential diagnosis of the tumour specific formed spaces. PATIENTS AND METHODS: Four male patients aged 63-78 years suffered from angiosarcomas of the maxillary sinus, the bucca (two patients) and the alveolar ridge of the lower jaw. HISTOPATHOLOGY: For comparative analysis paraffin embedded tissue of the initial biopsies was available. The slides were stained with standardized H&E, PAS and Gömörri. For standardized immunohistochemistry following primary antibodies were applied: monoclonal antibodies to pancytoceratin clones AE1/AE3, alpha-smooth-muscle-actin clone 1A4, CD31 clone JC/70A, factor-VIII-related antigen clone F/86, Fli-1 (polyclonal, Zymed, USA), tenascin-C: BC4 (Prof. L. Zardi), oncofetal glucosylated fibronectin clone FDC6 (ACCR), laminin-5: D4B5. Detection using AP-ChemMate and Autostainer (Dako, Denmark). RESULTS: While the benign appearance of the lesions resulted primarily in wrong diagnoses the histopathologic examination of the biopsies revealed the characteristic pattern of angiosarcomas. Wide surgical excision, radiotherapy and/or antiangiogenic chemotherapy could not prevent tumour progression and death within two and a half years after primary diagnosis. All angiosarcomas reacted partially positive for factor-VIII-related antigen and CD31. The tumour associated structural defect of vascular lamina with partial loss of pericytes/vascular smooth muscle cells was identified immunohistochemically by alpha-smooth-muscle-actin and for the first time by tenascin-C. CONCLUSIONS: (1.) The variable presentation and the benign appearance of oral and perioral angiosarcomas may often delay diagnosis. Oral and perioral angiosarcomas show poor prognosis despite of multimodal therapy. (2.) Cytoceratin and laminin-5-positivity as typical epithelial antigens don't exclude angiosarcoma. Factor-VIII-related antigen, CD31 as well as Fli-1 identify angiosarcoma. (3.) alpha-smooth-muscle-actin and the loss of the tenascin-C-matrix indicate immunohistochemically the characteristic sarcomatous defect of differentiation.

Aged↗

PubMed2002

Angiosarcoma treated with radiotherapy: impact of tumor type and size on outcome.

PURPOSE: Angiosarcoma is a rare and highly malignant vascular neoplasm. The purpose of this study was to elucidate the tumor characteristics and evaluate the efficacy of radiotherapy (RT) for angiosarcoma. MATERIALS AND METHODS: Thirty patients with angiosarcoma (20 males and 10 females, age range 4-89 years, median 66) who received RT from 1986 to 1999 were enrolled in the study. Twenty-four patients had angiosarcoma of the face and scalp (AFS), and 6 patients had angiosarcomas at other sites. AFS was classified into two categories (according to the macroscopic features): nodular AFS (14 patients) and endophytic AFS (10 patients). The median prescribed irradiation dose was 68 Gy. Surgery had been previously performed in 9 patients, and adjuvant immunotherapy using recombinant interleukin-2 (rIL-2) was combined during and after RT in 20 patients. Univariate analyses and calculation of survival by Kaplan-Meier methods were performed. RESULTS: Local tumor control was obtained in 17 patients (57%). However, 7 (47%) of them developed distant metastases. The median survival time for all patients was 8 months (7 months for AFS), and the 13-year overall survival rate was 25% (20% for AFS). Twenty-one patients died of angiosarcoma, with the cause of death local failure in 7 patients, distant failure in 7, and both in 7. Tumor type and size were found to be significant prognostic factors (p = 0.004 and p = 0.007, respectively), and age, total amount of rIL-2, gender, radiation dose, and surgery were not. Six patients (4 with nodular AFS and 2 with angiosarcoma in other parts) survived >2 years. No patient with endophytic AFS survived >2 years. Ten patients (33%) died of respiratory failure secondary to pulmonary metastases. High-dose rIL-2 administration suppressed the occurrence of distant metastases (p = 0.006). Two patients developed radiation dermatitis (Radiation Therapy Oncology Group Grade 4). CONCLUSION: RT, combined with complete resection or adjuvant rIL-2 immunotherapy, could be a promising treatment strategy, leading to prolonged survival in patients with angiosarcoma.

Adolescent↗

PubMedDate not supplied

A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy.

PURPOSE: Angiosarcoma is a rare vascular malignancy, and there are few published data to guide chemotherapy treatment decisions. We present a retrospective analysis of angiosarcoma encompassing all anatomic sites of disease presenting to a single institution over a 14-year period. Characteristics at presentation and prognostic factors are reviewed. For patients with unresectable disease, progression-free survival with various chemotherapy regimens is described. PATIENTS AND METHODS: Pathological confirmation of all cases was performed before they were included in this analysis. One hundred twenty-five patients with angiosarcoma were seen and treated between January 1, 1990 and December 31, 2003. RESULTS: Angiosarcoma showed marked variation by anatomic site regarding gender ratio, median age at diagnosis, overall survival, and response to chemotherapy. Overall 5-year survival was 31% for angiosarcoma. Superficial depth and negative microscopic surgical margins correlated with longer overall survival, but tumor size did not reach significance as a prognostic factor. For unresectable angiosarcoma, doxorubicin based regimens yielded progression-free survival of 3.7-5.4 months. Paclitaxel achieved a progression-free survival of 6.8 months for scalp angiosarcoma and 2.8 months for sites below the clavicle. DISCUSSION: Angiosarcoma is an aggressive malignancy characterized by biologic heterogeneity at different anatomic sites and relative sensitivity to paclitaxel and doxorubicin.

Adult↗

PubMedDate not supplied

Cytomorphologic features of angiosarcoma on fine needle aspiration biopsy.

OBJECTIVE: To present the cytomorphologic features of angiosarcomas identified on fine needle aspiration biopsy, review the literature, and discuss the differential diagnosis and pitfalls involved in such cases. STUDY DESIGN: Fine needle aspirate smears from 11 cases (1 hepatic, 3 breast and 7 subcutaneous/soft tissue lesions of angiosarcomas from eight patients were reviewed. All cases had histologic confirmation of angiosarcoma. RESULTS: All aspirates were hypocellular, with predominantly single cells in a background of moderate to abundant amounts of blood. Nine cases had scattered inflammatory cells, primarily neutrophils, in the background. Six of the cases had rare small clusters of cells. The cells were oval, round or spindled, with eccentric, round to spindle-shaped nuclei and moderate to abundant amounts of pale blue-gray, vacuolated cytoplasm. The cells ranged from two to nine times the size of the background red blood cells. In four cases, malignant cells demonstrated intracytoplasmic hemosiderin deposits. Small nucleoli were identified in five cases, large nucleoli in one case and hyperchromasia in seven cases. Mitotic figures, erythrophagocytosis, acinarlike or vascular structures, and necrosis were not identified in any of the studied cases. In four cases, a definitive diagnosis of angiosarcoma was rendered on the fine needle aspiration specimen. In three other cases, the differential diagnosis remained between angiosarcoma and radiation change. CONCLUSION: The presence of scarce single pleomorphic cells in a bloody background should raise the diagnostic possibility of angiosarcoma. A definitive diagnosis of angiosarcoma is often difficult to render due to the paucity of diagnostic cells unless intracytoplasmic hemosiderin deposits can be identified. Multiple aspirations are often needed in order to obtain diagnostic material. In the setting of radiotherapy, it may be impossible to distinguish angiosarcoma from radiation change, and biopsy should be recommended.

Aged↗

PubMed1991

Flow cytometric DNA content in Kaposi's sarcoma by histologic stage. Comparison with angiosarcoma.

Kaposi's sarcoma occurs as a multicentric proliferation of endothelial cells. A lesion may progress through several histologic stages, culminating in a lesion consisting of spindle cells with marked nuclear atypia that may be indistinguishable from angiosarcoma. To assess the relationship between the nuclear DNA content and the stage, 29 paraffin-embedded biopsy specimens from 25 cases of Kaposi's sarcoma were classified according to their histologic stage and flow cytometric DNA ploidy status. The findings were compared with those in 14 angiosarcomas (5 postmastectomy angiosarcomas, 6 other cutaneous angiosarcomas and 3 angiosarcomas of deep tissues). The Kaposi's sarcoma specimens studied included samples with irregular lymphatic-like channels (stage 1), transition to spindle cells (stage 1t2), nodular spindle-cell aggregates (stage 2), scattered atypical spindle cells (stage 2t3) and histologic features indistinguishable from those of angiosarcoma (stage 3). Of the 25 Kaposi's sarcoma specimens of stage 2 or less, 17 had a diploid DNA distribution while an additional 8 had broad diploid G0G1 peaks (peridiploid, with a coefficient of variation greater than 7.5%, present in similar proportions in stages 1, 1t2 and 2). One of three stage 2t3 lesions showed tetraploidy while the single stage 3 specimen (from the leg) was aneuploid, with a DNA index (DI = 1.16) similar to that of four of the five postmastectomy angiosarcomas (DI = 1.14 to 1.20). An additional three angiosarcomas also showed nondiploid distributions (DI = 1.16, 1.98 and 2.13, respectively); the remainder were diploid or peridiploid. These results support previous cytogenetic data suggesting a normal karyotype in Kaposi's sarcoma up to stage 2, with atypia beginning as cells acquire numerical and structural chromosomal aberrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1979

Angiosarcoma following therapeutic irradiation.

This paper reports a case of angiosarcoma of the terminal ileum in a 66-year-old female. This angiosarcoma developed 8 years after postoperative irradiation for ovarian carcinoma. This case appears to be the sixth case of angiosarcoma arising at the site of therapeutic irradiation thus far reported in the literature. The fact that the angiosarcomas in these cases developed in anatomic sites, which are unusual for angiosarcomas in general, suggests that there is a cause-and-effect relationship between irradiation and angiosarcoma. Apart from the direct carcinogenic effect of irradiation, prolonged stimulation for tissue repair resulting from tissue damage secondary to irradiation-induced vascular changes may also play an etiologic role in the development of angiosarcomas in these cases.

Adenocarcinoma↗

PubMed1995

Angiosarcoma in Japan. A review of 99 cases.

BACKGROUND: Angiosarcoma is rare, and information about its clinical features are limited. Therefore, a large scale study of angiosarcoma was performed in Japan. METHODS: Through a nationwide Japanese study, 99 cases of angiosarcoma were collected and their clinicopathologic findings were summarized relative to predisposing risk factors. RESULTS: The patient age at diagnosis was 3-92 years, (mean, 62 years), with a two to one male to female ratio. The head and face were the most common primary site (29 cases); other sites were liver (17); trunk (13): pleural cavity (6), chest wall (2), abdominal wall (2), buttock (2), inguinal region (1); heart (12); and extremities (7). The proven predisposing risk factors included chronic pyothorax for angiosarcoma in the pleural cavity (six), thorotrast in the liver (five), radiotherapy to the abdominal wall and buttock (four), and chronic limb edema of the forearm (one). Irrespective of primary sites, the majority of cases had metastases to lung in 72 cases, bone in 42, liver in 36, regional lymph nodes in 30, and adrenal gland in 24. The 2-year survival rate was 17%. CONCLUSIONS: This study describes a different etiology in the development of angiosarcoma in patients from Japan compared with that of patients from Western countries, though the frequency of angiosarcoma among all soft-tissue sarcomas was similar in both areas. In Japan, chronic pyothorax, radiotherapy, and thorotrast proved to be distinctive causative factors of angiosarcoma.

Adolescent↗

PubMed2000

[Cutaneous angiosarcoma].

BACKGROUND AND OBJECTIVES: Angiosarcomas of the skin arise almost exclusively in the following clinical settings: 1. the face and scalp, usually in elderly individuals, 2. lymphedematous regions (lymphedema-associated angiosarcomas), and 3. skin that has been previously irradiated (post-radiation angiosarcomas). Clinical and histopathologic diagnosis of angiosarcoma is difficult often resulting in great delay that obviates against early and possibly successful treatment of these very aggressive neoplasms. Diagnostic problems are described, and prognostic factors as well as the effect of different forms of treatment on the outcome are discussed. PATIENTS/METHODS: Retrospective study of 11 patients with cutaneous angiosarcomas. Clinical presentation, histopathology, therapy and survival time are analysed. RESULTS: Only 1 of 11 cases cutaneous angiosarcoma was clinically identified. Survival time was 1-24 months. Three patients who received radical surgery have not developed metastases and are still alive. CONCLUSIONS: Clinical and histopathologic diagnosis of cutaneous angiosarcomas is often very difficult. Prognosis is very bad; radical surgery seems to be the best therapeutical option.

Adult↗

PubMed1999

Intravascular ("intimal") epithelioid angiosarcoma: clinicopathological and immunohistochemical analysis of three cases.

Angiosarcomas are rare malignant mesenchymal tumours, characterized morphologically by anastomosing vascular channels lined by atypical and proliferative active endothelial cells. An epithelioid cytomorphology of tumour cells is often seen focally in angiosarcoma, whereas purely epithelioid angiosarcomas are rare. Although angiosarcomas show a vascular differentiation they are almost never confined to pre-existing blood vessels. We describe three cases of intravascular epithelioid angiosarcoma arising in the carotid artery of a 60-year-old man, in the infrarenal part of the abdominal aorta and both renal arteries of a 69-year-old woman, and in the abdominal aorta of a 68-year-old man. In all cases malignant tumour tissue was found incidentally after disobliteration of thrombosed vessels. Histologically, purely epithelioid angiosarcoma composed of solid sheets of epithelioid tumour cells was seen; immunohistochemistry confirmed the endothelial differentiation of neoplastic cells. The reported cases show that angiosarcoma can occasionally arise within a pre-existing vessel.

Aged↗

PubMed1998

MDM-2 oncoprotein overexpression, p53 gene mutation, and VEGF up-regulation in angiosarcomas.

The endothelium is one of the largest cellular compartments of the human body and has a high proliferative potential. However, angiosarcomas are among the rarest malignancies. Despite this interesting contradiction, data on growth and angiogenesis control mechanisms of angiosarcomas are scarce. In this study of 19 angiosarcomas and 10 benign vascular control lesions we investigated the sequence and expression of the p53 tumor suppressor gene and the expression of the mdm-2 proto-oncogene, which is a negative regulator of p53 activity and of the vascular endothelial growth factor (VEGF), whose expression, among other factors, is regulated by the p53/MDM-2 pathway. Ten sarcomas (53%) exhibited clear nuclear p53 protein accumulation. Two of these cases revealed mutations in the sequence-specific DNA binding domain of the p53 gene. Thirteen angiosarcomas (68%) showed an increased amount of MDM-2 protein. Elevated expression of p53 and MDM-2 protein correlated with increased VEGF expression, which was found in nearly 80% of the angiosarcoma cases. Negative or clearly lower immunostaining was obtained in cases from the benign control collective. Only one case of a juvenile hemangioma reached the cutoff value of p53 positivity coincidentally with high VEGF expression. Our data suggest that the p53/ MDM-2 pathway is impaired in about two-thirds (14/ 19) of the angiosarcomas. This may be a key event in the pathogenesis of human angiosarcomas. The increased VEGF expression observed supports this hypothesis.

Adult↗

PubMed1998

Cytogenetic analysis of four angiosarcomas from deep and superficial soft tissue.

Angiosarcomas are rare malignant vascular tumors, most commonly found in the skin or superficial soft tissue. We found clonal chromosome aberrations in four short-term cultured angiosarcomas. Two cases were diagnosed as epithelioid angiosarcomas, and one as postmastectomy angiosarcoma. Two of the tumors were deep-seated and two were superficial. Angiosarcomas from deep, soft tissue are extremely rare and have never been cytogenetically investigated before. The chromosome number ranged from hypodiploid to hypertriploid. When the results from the present study were combined with data on the four previously reported cytogenetically aberrant angiosarcomas, the most frequently rearranged chromosomes were 5, 7, 8, 13, 15, 20, 22, and the Y chromosome. Recurrent changes, each found in three of these eight angiosarcomas, were gains of 5pter-p11, 8p12-qter, and 20pter-q12, losses of 7pter-p15 and 22q13-qter, and -Y in two of three men.

Adult↗

PubMed2002

Angiosarcoma after breast-conserving therapy: experience with hyperfractionated radiotherapy.

PURPOSE: To report our promising results of hyperfractionated radiotherapy (RT) in conjunction with surgery for angiosarcoma occurring after breast-conserving therapy for early-stage breast cancer. METHODS AND MATERIALS: Since 1997, 3 cases of angiosarcoma after breast-conserving therapy have been managed at the University of Florida. The histologic specimens in each case were reviewed and graded by one of us (J.D.R.). RESULTS: Explosive growth of discolored skin lesions coincident with histologic evidence of angiosarcoma characterized all 3 cases but was preceded by a fairly indolent period (almost 2 years) of atypical vascular hyperplasia in 2 patients. All 3 patients were treated initially with radical surgery for the angiosarcoma, but extensive recurrences were noted within 1 to 2 months of surgery. Because of the extremely rapid growth noted before and after surgery, hyperfractionated RT was used. Two of the patients underwent planned resection after RT, and neither specimen demonstrated any evidence of high-grade angiosarcoma. All 3 patients were alive without any recurrent disease 22, 38, and 39 months after treatment. CONCLUSIONS: Hyperfractionated irradiation appears to be effective treatment for rapidly proliferating angiosarcoma. For previously untreated angiosarcoma, we now recommend hyperfractionated RT followed by surgery to enhance disease control and remove as much reirradiated tissue as possible.

Aged↗

PubMed2006

Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposi's sarcoma: report of a case and review of the literature.

BACKGROUND: Despite common endothelial origins, angiosarcoma and Kaposi's sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdominal pannus is extremely uncommon. Similarly, tumors with the combined histologic features of angiosarcoma and Kaposi's sarcoma have rarely been described. METHODS: We reviewed the literature on angiosarcoma arising in a lymphedematous abdominal pannus and evaluated an 81-year-old morbidly obese woman who had profound, long-standing edema of the lower abdominal wall in which an aggressive vascular tumor developed. RESULTS: Three clinically similar cases were identified in the literature. All patients were women who generally experienced rapid disease progression. In addition, in our patient, sequential cutaneous sampling from different lesional sites demonstrated disparate histologic changes, ranging from those of classic Kaposi's sarcoma to high-grade angiosarcoma, to areas with combined features of the two tumors. A polymerase chain reaction performed on lesional tissue was negative for human herpesvirus-8 DNA. CONCLUSION: It is important to note that angiosarcoma may develop in the abdomen in association with chronic lymphedema, as demonstrated by the cases noted in this report. In addition, our case highlights the difficulty in differentiating histologically angiosarcoma from Kaposi's sarcoma in some situations, and demonstrates the value of close clinicopathologic correlation and sequential tissue sampling in evaluating problematic cases.

Abdomen↗