Short German guidelines:Angiosarcoma and Kaposi sarcoma (original) (raw)
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Pediatric angiosarcoma of soft tissue: a rare clinicopathologic entity
Archives of pathology & laboratory medicine, 2010
Angiosarcomas are rare tumors that predominantly affect adults and elderly patients. Although angiosarcomas are well described in a variety of clinical settings, they have been poorly studied in children and little is known about their biology, natural history, or optimal treatment. Childhood angiosarcomas are exceedingly rare. The head and neck region and mediastinum seem to be the preferred locations. Most tumors are high-grade tumors. Vasoformative architecture is not always obvious on light microscopy requiring the benefit of immunohistochemical study. The differential diagnosis includes Kaposi sarcoma, epithelioid hemangioendothelioma, hemangiopericytoma, and spindle cell hemangioendothelioma whose prognosis is different. Complete resection is required for patients with localized tumors. Malignant vascular tumors are rare in children in the first 2 decades of life and when they do occur they seem to be more aggressive than in adults. Pathologic diagnosis is difficult particular...
Angiosarcoma—Report of an unusual case
British Journal of Plastic Surgery, 1974
ANGIOSARCOMAS are relatively rare malignant tumours of endothelial origin which may be divided histogenetically into haemangiosarcomas and lymphangiosarcomas; lymphangiosarcomas are angiosarcomas developing in post-mastectomy lymphoedematous arms (Stewart and Treves, 1948; Treswell et al., 1962). Angiosarcomas arising primarily on the extremities of young adults have been described (McCarthy and Pack, 1950) and several authors have reported angiosarcomas in the head and neck (Reed et al., 1966; Bardwill et al., 1968). Most agree that the prognosis is poor. The patient presented here survived for 5 years and his angiosarcoma behaved as if it were multicentric in origin rather than a primary with metastases.
Cytologic features of angiosarcoma: A review of 26 cases diagnosed on FNA
Cancer Cytopathology, 2016
BACKGROUND: This study describes the cytologic features of 26 angiosarcomas diagnosed on fine-needle aspiration. METHODS: Twenty-six angiosarcomas from 20 patients were confirmed by cytomorphology and immunocytochemical (immunohistochemistry) positivity for at least 2 of 3 vascular markers. Specimens were examined for spindled/epithelioid/plasmacytoid single cells, 3-dimensional clusters, multiple prominent/bar-shaped nucleoli (5 times longer than their width), chromatin strands, abnormal mitoses, necrosis, and vasoformative features. RESULTS: Eight males and 12 females with a mean age of 52 years (range, 2-94 years) underwent aspiration of tumors in the following: soft tissue or skin/subcutis (n 5 10), bone (n 5 4), nodes (n 5 5), lung (n 5 2), liver (n 5 2), heart (n 5 1), parotid gland (n 5 1), and pleural fluid (n 5 1). An angiosarcoma diagnosis was rendered for 24 of the 26 cases (92%); 1 was diagnosed as "atypical cells, cannot exclude angiosarcoma," and another was diagnosed as a malignant vascular neoplasm. Abnormal mitoses were most frequent (85%), and they were followed by single malignant cells (81%: epithelioid [69%], spindled [62%], and plasmacytoid [19%]), 3-dimensional clusters (54%), multiple prominent (62%) or bar-shaped nucleoli (54%), and chromatin strands (31%). Vasoformative features, including hemophagocytosis (54%), cytoplasmic lumina/vacuoles (69%) containing red blood cells (54%)/neutrophils (31%), and endothelial wrapping (69%), were seen in 88%; 23% had all vasoformative features, 88% had at least 1, and 12% had none. CONCLUSIONS: Angiosarcomas show a range of cytomorphologic features that make them potentially recognizable on cytology. Although vasoformative features are highly suggestive, they are not specific for angiosarcoma and may be seen in some nonvascular neoplasms. Immunohistochemistry and a high index of suspicion are required for an accurate diagnosis.
Sarcoma, 2013
Angiosarcomas are aggressive tumors of vascular endothelial origin, occurring sporadically or in association with prior radiotherapy. We compared clinicopathologic and biologic features of sporadic angiosarcomas (SA) and radiation-associated angiosarcomas (RAA). Methods. From a University of Michigan institutional database, 37 SA and 11 RAA were identified. Tissue microarrays were stained for p53, Ki-67, and hTERT. DNA was evaluated for TP53 and ATM mutations. Results. Mean latency between radiotherapy and diagnosis of RAA was 11.9 years: 6.7 years for breast RAA versus 20.9 years for nonbreast RAA ( = 0.148). Survival after diagnosis did not significantly differ between SA and RAA ( = 0.590). Patients with nonbreast RAA had shorter overall survival than patients with breast RAA ( = 0.03). The majority of SA (86.5%) and RAA (77.8%) were classified as high-grade sarcomas ( = 0.609). RAA were more likely to have well-defined vasoformative areas (55.6% versus 27%, = 0.127). Most breast SA were parenchymal in origin (80%), while most breast RAA were cutaneous in origin (80%). TMA analysis showed p53 overexpression in 25.7% of SA and 0% RAA, high Ki-67 in 35.3% of SA and 44.4% RAA, and hTERT expression in 100% of SA and RAA. TP53 mutations were detected in 13.5% of SA and 11.1% RAA. ATM mutations were not detected in either SA or RAA. Conclusions. SA and RAA are similar in histology, immunohistochemical markers, and DNA mutation profiles and share similar prognosis. Breast RAA have a shorter latency period compared to nonbreast RAA and a significantly longer survival.
Congenital Angiosarcoma of the Arm in a Pediatric Patient: A Therapeutic Dilemma
Journal of Clinical Oncology, 2012
A 17-day-old girl presented with a rapidly growing swelling in her left arm that had been present since birth. Her mother had an uneventful pregnancy and a full-term, uncomplicated vaginal delivery. Both parents were laborers by profession. Their first child was a healthy 6-year-old boy without any disease or disability. There was no history of any significant illness or debility in any of the other family members. The parents also did not give a history of exposure to any known carcinogens. Three days after birth, the infant's arm swelling increased significantly and was seen to be compromising the distal blood supply; to correct this, an incision biopsy and decompression were performed at a primary care center elsewhere. Subsequently, the patient received a blood transfusion with a course of antibiotics and anti-inflammatory medications. On examination, she had a 6 ϫ 6-cm-sized swelling in her left arm with an overlying wound through which necrotic slough was visible. A few axillary lymph nodes were palpable. There was no distal neurovascular deficit. A complete clinical examination did not reveal any other lesion. Magnetic resonance imaging of the arm revealed a large soft tissue swelling that encased the axillary and brachial neurovascular bundle (Figs 1A and 1B). The medial cortex of the humerus was eroded with adjacent marrow edema and periosteal reaction. In addition to several enlarged axillary nodes, a discrete nodule was noted that was anterosuperior to the main mass (Fig 2). The lungs and liver were normal on a whole-body imaging survey. Laboratory investigations revealed elevated liver enzymes (ALT, 146 U/L; AST, 44 U/L [normal range, 10 to 37 U/L]), total bilirubin of 8.84 mg/dL (normal range, 0.2 to 1.9 mg/dL), and serum lactate dehydrogenase levels of 453 U/L (normal range, Ϫ100 to 190 U/L). Histopathologic review of the biopsy revealed a cellular tumor that comprised predominantly polygonal to oval cells that were arranged in a solid/diffuse pattern, including vascular formations amid conspicuous areas of hemorrhage, interspersed red blood corpuscles, and discrete tumor necrosis (Figs 3A to 3D). Individual tumor cells exhibited moderate to focally marked nuclear pleomorphism with a moderate amount of eosinophilic cytoplasm and pleomorphic nuclei, including oval to short spindle-shaped forms with prominent nucleoli in several cells (Fig 3D inset). There were numerous mitotic figures, ranging from 4 to 5 per high power field within the tumor (Figs 3B to 3D, arrows). There were no myxoid areas (Figs 3A to 3D). According to immunohistochemistry analysis performed on paraffin-embedded tissue microsections, tumor cells were diffusely positive for CD34 (Fig 4A) and Fli1 (Friend leukemia integration 1; Fig 4B). Cells were also positive for CD31 (Fig 4C). MIB1 (mindbomb homolog 1; proliferation marker)
A Case of Primary Breast Angiosarcoma
Radiology Case Reports, 2013
A 45-year-old woman with a history of systemic lupus erythematosus presented for workup of a palpable abnormality of the left breast. The patient identified this abnormality on self-examination three months earlier. In the lower inner quadrant of the left breast, there was reportedly a lobulated smooth mobile mass. No lymphadenopathy was evident on clinical examination. Since the patient recently had a screening mammogram with benign findings (Fig. 1), she underwent a breast sonogram. No sonographic correlate was found in the area of the palpable abnormality. Thus, the patient was followed clinically, and the diagnosis of lupus mastopathy was considered. Due to persistence of the palpable abnormality, the patient underwent a contrast-enhanced breast MRI (Fig. 2). In the left lower inner breast, a lobulated irregular mass measuring 2.7 x 2.2 cm, hypointense on T1-weighted images and hyperintense on T2-weighted images, was seen. The mass had kinetics demonstrating a rapid rise to peak with washout. The mass also had increased circumferential signal on STIR images, suggesting possible edema. A second mass demonstrating similar kinetics was seen in the supra-areolar region. Scattered enhancing nodules were incidentally noted in the right breast. Excisional biopsy of the area of the MRI abnormality was performed. On pathology, multifocal angiosarcoma of intermediate to high grade was identified. The largest focus measured 1.6 cm. Histologic sections showed neoplastic blood vessels lined by endothelial cells with marked cytologic atypia as well as frequent associated mitoses. Intermediate-to high-grade areas consisted of solid growth or prominent endothelial tufting with papillary formations as