Angiosarcoma Presenting with Minor Erythema and Swelling (original) (raw)
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.
Angiosarcomas are ubiquitous neoplasms involving both cutaneous and soft tissue and visceral locations. Accumulating biomolecular evidences suggest that cutaneous angiosarcomas are distinctive entities with molecular, clinical and pathological peculiarities. Despite several ongoing clinical trials with promising therapeutic agents, the prognosis of cutaneous angiosarcomas is dismal and survival still rely on early diagnosis and surgery. An accurate diagnosis and the knowledge of the underlying molecular landscape are therefore essential to improve the prognosis. We detail the molecular, clinical, dermoscopic, morphological and prognostic features of cutaneous angiosarcoma. Although the molecular landscape of cutaneous angiosarcoma is not completely understood, accumulating evidences suggest that there are characteristic molecular alterations including dysregulation of angiogenesis and several complex molecular pathways. Secondary cutaneous angiosarcomas, arising in correlation with chronic lymphedema and ionizing radiation, have different molecular hallmarks, which are also leading to the first diagnostic applications. The diagnosis of cutaneous angiosarcoma may be challenging, as well-differentiated forms can be hard to distinguish from benign and low-grade vascular neoplasms, while poorly differentiated forms can be easily confounded with other non-vascular high-grade neoplasms. An accurate and early diagnosis, which is mandatory to ensure the best survival for the patients, is mainly based on morphological hallmarks.
Anais brasileiros de dermatologia
Angiosarcoma is a rare and aggressive tumor with high rates of metastasis and relapse. It shows a particular predilection for the skin and superficial soft tissues. We report three distinct and typical cases of angiosarcoma that were diagnosed in a single dermatology clinic over the course of less than a year: i) Angiosarcoma in lower limb affected by chronic lymphedema, featuring Stewart-Treves syndrome; ii) a case of the most common type of angiosarcoma loated in the scalp and face of elderly man and; iii) a skin Angiosarcoma in previously irradiated breast. All lesions presented characteristic histopathological findings: irregular vascular proliferation that dissects the collagen bundles with atypical endothelial nuclei projection toward the lumen.
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.
Challenges in the treatment of angiosarcoma: a single institution experience
The American Journal of Surgery, 2014
Angiosarcomas are rare tumors that carry poor prognosis. Because of insidious growth rate, the diagnosis is often difficult and delayed. Between 1990 and 2011, 72 (41 female, 31 male) patients were treated at our institution. Pathologic confirmation was obtained and multiple prognostic factors were evaluated for survival. Forty-four cases were sporadic and 28 cases were secondary. In the sporadic group, 16 (36%) patients had increased sun exposure, while in the secondary group, the majority (n = 23, 82%) of patients had prior exposure to radiation. The latent period between radiation exposure and diagnosis was predictive of survival (P = .037). Presentation was delayed by more than 3 months in 41% of patients. The majority of men developed head and neck angiosarcomas (n = 15, 48.5%), while women developed breast angiosarcomas (n = 21, 51%). Median survival was prolonged in patients treated initially with surgery. A delay in the diagnosis of angiosarcoma can affect survival. Clinical suspicion and prompt diagnosis are essential for successful multimodal therapy. Initial surgical resection with adjuvant chemotherapy provides survival advantage.
Clinical and Molecular Biology of Angiosarcoma
‘Skepticism is a healthy response to diagnosis of any tumor as angiosarcoma. ‘ (Lane, 1952) Angiosarcoma (AS) is an aggressive malignancy of vascular tissue or vessel forming cells (Requena & Sangueza 1998). AS is rare in humans, making up 1-2 % of soft-tissue sarcomas (Young et al. 2010) and having an estimated incidence of 0.2/100,000 persons per year. Although AS can present anywhere in the body, in humans they typically arise in the skin or superficial soft tissues. It is most frequently noted on the face and scalp of elderly men where their persistent growth causes ulceration and infection, as well as on breasts, and extremities (Brennan et al. 2001; Fayette et al. 2007; Glazebrook et al. 2008). Less frequently AS arises in liver, heart, and spleen (Young et al. 2010). The literature is replete with retrospective analyses and case studies on AS but the rarity of patients diagnosed with this disease makes it difficult to perform more than a superficial investigation on the biolo...
An Immunocytochemical Assessment of 19 Cases of Cutaneous Angiosarcoma
Histopathology, 1996
Four endothelial cell markers, two selective cytokeratin markers and a monoclonal smooth muscle antibody (SMA) were employed in the assessment of 19 cases of cutaneous angiosarcoma classified according to their degree of tumour differentiation. No labelling was seen for SMA or with cytokeratin markers MNF116 and CBL170. Expression of factor VIII-related antigen was seen in two tumours and positivity for CD34 (QBend 10 antibody) was found in four tumours. By contrast the pan-endothelial cell marker Ulex europeaus agglutinin 1 (UEA-1) and the CD31 marker JC70A labelled all cases of cutaneous angiosarcoma with the exception of one poorly differentiated tumour. These data confirm the endothelial cell origin of angiosarcoma, they demonstrate that CD31 and UEA1 are reliable markers in routinely processed tissue, and they suggest a lymphatic derivation for the tumour. This finding is in marked contrast to Kaposi's sarcoma where CD34 is the most reliable marker.
Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
European Journal of Case Reports in Internal Medicine, 2020
Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing the importance of biopsy with immunohistochemistry confirmation. There are few reports of extremity involvement in patients with pre-existing chronic lymphoedema, or exposure to radiation therapy. We report the case of an 82-year-old woman with lower limb extensive cutaneous involvement, distant metastatic disease, and poor therapy response. Its rare location without predisposing factors highlights the need to raise awareness about this disease. LEARNING POINTS Extremity involvement of cutaneous angiosarcoma has been rarely described. The marked heterogeneity in presentation leads to a delay in diagnosis and poor prognosis, so the index of suspicion should be high. The cases reported in ...
A Case of a Cutaneous Angiosarcoma that Demonstrated a Late but Comprehensive Response to Treatment
Clinical Medicine Reviews in Oncology, 2016
IntroductIon: A cutaneous angiosarcoma case with visible illness was treated with a modified lateral electron-photon technique (m-LEPT) that demonstrated a late but successful response to treatment. cAse presentAtIon: A 74-year-old man presented after surgery with remnants of the tumor at the angiosarcoma bed, which did not stop growing. MAnAgeMent And outcoMe: m-LEPT technique was developed and applied to optimize dosimetry of the scalp. Late complete response was achieved after a disheartening posttreatment clinical state. dIscussIon: This is a rare and difficult-to-treat pathology, and hence case studies like this should encourage treatment despite poor early results.