Clinical presentation of the patient with angiosarcoma prior to treatment.

Clinical presentation of the patient with angiosarcoma prior to treatment.

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An unusual case of cutaneous angiosarcoma clinically mimicking eczema is described. A 98-year-old Caucasian male presented with a 6-month history of a flesh-colored, subcutaneous nodule on his left forehead with contralateral facial erythema and scaling that had been previously diagnosed as eczema. Despite treatments with topical steroids and moist...

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... The usual clinical presentation of cAS is rapidly growing erythematous-to-purplish plaques or nodules with ulcerative or necrotic lesions in the progressive stages. However, cAS may mimic eczema, rosacea, hemangioma, or cellulitis, leading to delay in diagnosis [3,4]. Although case reports of eyelid and periocular lesions, as in our patient, have been reported, they are rare [5]. ...
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Cutaneous angiosarcoma is a rare and highly aggressive neoplasm with poor prognosis. Owing to its variable presentation, it may be a challenging clinical and histological diagnosis. Herein, we describe a particular case of cutaneous angiosarcoma of the head and neck in a 71-year-old male with skin type V who presented with solid, progressive eyelid edema. A histological examination of skin biopsies first concluded cutaneous metastasis of an undifferentiated carcinomatous tumor. Immunostaining was essential to reach the correct diagnosis. The treatment of cutaneous angiosarcoma remains unsatisfactory. We sincerely hope that the prognosis of cutaneous angiosarcoma will be improved with the use of targeted therapies based on current genetic studies as it has been for melanoma.
... 4 Predisposing factors for angiosarcoma include trauma, chronic lymphoedema, irradiation and age; however, most cases present with no obvious etiology. 5 Angiosarcomas has been classified into lymphoedema associated, radiation induced, primary breast angiosarcoma, sporadic cutaneous angiosarcoma (age related) and angiosarcoma of the soft tissue. 6 It was first described by Jones in 1964 as malignant angioendothelioma of skin. ...
... Regional lymph node involvement occurs in 20-30% cases, which is higher than most other sarcomas. 2,5 The most common site for distant metastasis is lungs followed by liver, spleen and bone. Prognosis of angiosarcoma is poor because of its high potential for metastasis. ...
... Cutaneous angiosarcoma is a rare and aggressive malignant vascular tumor that mostly develops in the head and neck region of the elderly. 1 Cutaneous angiosarcoma may initially appear as mild erythema or ill-defined bruise-like patches and plaques. 2 ...
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Band‐shaped presentation of cutaneous angiosarcoma has not been reported. Cutaneous angiosarcoma should be included in the differential diagnosis when a persistent erythematous lesion is observed in the head and neck region of an elderly patient without a clear explanation, but skin biopsy is required for an accurate diagnosis. Cutaneous angiosarcoma should be included in the differential diagnosis when a persistent erythematous lesion is observed in the head and neck region of an elderly patient, but skin biopsy is required for an accurate diagnosis.
... 1 Cutaneous angiosarcoma (CA) accounts for ve percent of all malignant skin tumors. 2 CA mainly arises in three distinct clinical scenarios: 1) Sporadic idiopathic (primary) CA involving the head and neck; 2) CA secondary to long-standing lymphedema (Stewart-Treves syndrome); and 3) CA secondary to irradiation. 1,3 The prognosis of CA is poor relative to that of other skin malignancies. Therefore, early diagnosis and appropriate management are vital. ...
... 1,4 This is typically an insidious tumor found in elderly men with a median age of 70 to 75 years, arising de novo and showing a predilection for the scalp and central face. [1][2][3] Patients with fair skin tones are more commonly a ected than those with darker skin. 6 Contrary to secondary subtypes of CA, no etiological factor has been proven to take part in primary CA of the head and neck. 2 Clinically, idiopathic CA of the head and neck presents with erythematous, violaceous or dark blue macules, patches, papules, nodules, or plaques. ...
... 2,6 These are lined by atypical, pleomorphic, hyperchromatic endothelial cells with pleomorphic nuclei exhibiting a di use epithelioid or spindle-cell proliferation with variable mitotic activity. 3 The tumor can display a spectrum from well to poorly di erentiated forms. [10][11][12] Immunohistochemical staining for the endothelial markers von Willebrand factor, CD31, CD34, Ulex europaeus agglutinin 1, vascular endothelial growth factor receptor, Factor VIII-related antigen and podoplanin (D2-40) can con rm the clinical and histological diagnosis. ...
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Idiopathic cutaneous angiosarcoma (CA) of the head and neck is a distinct subtype of angiosarcoma most commonly presenting as a single or multiple purple, bruise-like patches that arise de novo and enlarge over several months. In clinical practice, both misdiagnosis and delayed diagnosis are frequently encountered. Here, we present a case of idiopathic CA on the scalp with invasion to the cranium in a patient with breast cancer metastatic to the brain. The patient was initially misdiagnosed and mistreated with herpes zoster and breast cancer metastatic to the skin, which led to a delayed diagnosis by two months until dermatologic evaluation. The diagnosis was then firmly established as CA based on consistent clinical and histological features. Since the tumor was inoperable, radiotherapy and chemotherapy were been considered as the appropriate adjuvant modes of therapy. Despite an initial favorable response, the disease demonstrated a rapidly progressive course and the patient succumbed to the disease within six months. This report briefly reviews the clinical and histological portrait and management options for this aggressive tumor.
... Clinical features of cASs are extremely variable, since the neoplasm may mimic both other cutaneous neoplasms and non-neoplastic skin diseases. For example, cases of cASs clinically simulating rosacea, eczema, haemangioma, xanthelasma, cellulitis, and facial and eyelid angioedema, squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, atypical fibroxanthoma and malignant melanoma have been reported in literature [27][28][29]. However, at least in early stages, the most common presentation is that of a haematoma-like lesion (Fig. 3). ...
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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.
... [21][22][23] Although single-agent therapy was successful for many patients but patients with advanced age and significant clinical comorbidities may not qualify for therapy. [24] Angiosarcoma response to VEGF-targeted agents is limited, and biomarkers are required to identify patients most likely to respond to treatment. [25] Researchers reported that flavonoids and iso-flavonoids possess multiple bioactivities and can either kill or resensitise conventional chemotherapeutics to resistant cancer cells. ...
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Objectives This research aimed to evaluate the antiangiogenic activity of isolated flavonoid 4a,5,8,8a‐tetrahydro‐5‐hydroxy‐3,7,8‐trimethoxy‐2‐(3,4‐dimethoxyphenyl) chromen‐4‐one (TMF) from Tabebuia chrysantha. STAT3‐MMP9 signalling is a signal transduction mechanism that promotes angiogenesis in various cancers. Methods The tumour xenografting chicken embryo chorioallantoic membrane (CAM) model‐based ex vivo assay was used to evaluate the activity of TMF. The Western blot, densitometric analysis and quantitative real‐time polymerase chain reaction (qRT‐PCR) were performed to evaluate the activity of the MMP9. Zebrafish embryos were used to evaluate embryotoxicity, and in vitro free radical scavenging activity of flavonoid was also elucidated. Key findings This research assessed the high level of STAT3, p‐ERK, VEGF‐R and MMP9 in the tissue extract of the control group, and also, the suppression of angiogenesis in the treatment groups was due to scavenged ROS and RNS, dephosphorylation of STAT3 and ERK, and suppression of MMP9 gene expression. Conclusion The isolated flavonoid named TMF from T. chrysantha functions as specific regulators of target proteins of angiosarcoma. The STAT3‐MMP9 signalling may be used as an effective prognostic marker of angiosarcoma.
... Cutaneous angiosarcoma has been described to be clinically variable in literature. Previously published case reports have described lesions as raised purplish-red papules, which can be misdiagnosed as rosacea, eczema, and hematoma [3][4][5][6]. Due to this variability in presentation, the correct diagnosis can be delayed, often drastically altering the outcome and treatment options. Prognosis is poor with a reported survival Figure 4: The neoplastic cells also express CD31, a relatively sensitive and specific marker for endothelial cells that is also positive in vascular tumors, including angiosarcoma. ...
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Angiosarcoma is a cancer that is derived from endothelial cells that line blood vessels and lymphatic channels. Cutaneous angiosarcoma can appear anywhere on the skin and the clinical presentation is highly variable. Most cases appear on the scalp and face de novo. Our case describes a 91-year-old female with cutaneous scalp angiosarcoma. Our case serves to remind physicians that an abnormal skin finding in older adults should raise their index of suspicion for angiosarcoma and an early biopsy should be performed.
... Clinically, AS presents asymptomatically as a haematoma-like lesion, but may also resemble rosacea, eczema, haemangioma, violaceous plaques or nodules, xanthelasma, cellulitis, and facial and eyelid angioedema. [37][38][39] Nodules, papules, plaques and exophytic tumours develop on the surface of progressing lesions. Not uncommonly, advanced lesions can mimic epithelial neoplasms including squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, AFX and malignant melanoma. ...
Article
Cutaneous angiosarcoma (cAS) is a rare malignant neoplasm with variable clinical presentation. Although a distinct vascular tumour, cAS shares many overlapping histopathological features with other vasoformative and epithelioid tumours or ‘mimickers’. cAS shows aggressive behaviour and carries a grave prognosis, thus early diagnosis is of paramount importance to achieve the best possible outcomes. Recently, several genetic studies were conducted leading to the identification of novel molecular targets in the treatment of cAS. Herein, we present a comprehensive review of cAS with discussion of its clinical, histopathological and molecular aspects, the differential diagnosis, as well as current therapies including ongoing clinical trials.
... Due to its wide range of plausible etiologies, it has been categorized into lymphedema-associated, radiation-induced, primary breast angiosarcoma, sporadic cutaneous angiosarcoma (age related), commonly located on the head and neck region, [13] and angiosarcoma of the soft tissue. [17] Although chronic lymphedema is the most widely recognized predisposing factor in angiosarcoma of the skin and soft tissue, other common predisposing factors may be trauma, irradiation, and age per se. [17] Our case had a short clinical history of 3 months of stony hard progressively increasing plaque in the scalp with cervical lymphadenopathy. ...
... [17] Although chronic lymphedema is the most widely recognized predisposing factor in angiosarcoma of the skin and soft tissue, other common predisposing factors may be trauma, irradiation, and age per se. [17] Our case had a short clinical history of 3 months of stony hard progressively increasing plaque in the scalp with cervical lymphadenopathy. This only stands to reason that these findings suggest that lymphedema had occurred secondary to the infiltrating tumor mass rather than the tumor secondarily developing in lymphedema, as has been described earlier. ...
... Cutaneous angiosarcomas are known to be great mimickers and may present as single or multiple, bluish or violaceous nodules, plaques, or flat infiltrating lesions. [2,17] Occasionally, they may ulcerate or bleed. They have often being described in literature to mimic rosacea, [18] local bruise, [8] eczema, [17] Kaposi sarcoma, [19] scarring alopecia, [20] sebaceous cysts, [21] and rhinophyma [22] and thus delaying the diagnosis [ Table 2]. ...
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We report a case of a 70-year-old male, with slowly widening induration, ulceration, and oozing for 3 months on the scalp and face. The diagnosis of aggressive cutaneous angiosarcoma was made on histopathology and immunochemistry from the biopsy material from the involved area of the skin.
... Angiosarcoma is a rare, malignant neoplasm comprising 1-3% of adult soft tissue sarcomas [1][2][3][4]. This is typically a tumor of older individuals with a median age of 70-75 years and a male predominance, having a predilection for the scalp and central area of the face. ...
... The neoplasm tends to invade tissue more widely than is clinically apparent and is thus prone to incomplete excision. Majority of patients present with locally advanced disease, regional lymph node involvement, or distant metastases at the time of initial diagnosis, all of which are associated with a poor prognosis [4]. The present study describes a case of cutaneous angiosarcoma of the foot in the setting of chronic lymphedema which was treated with definitive radiation alone. ...
... As regards clinical appearance, the appearance of cutaneous angiosarcoma can be variable [4] and it can manifest as bruise-like lesions [8], raised purplish-red papules [9], and rosacea-like lesions [10]. Due to the variability in the appearance of cutaneous angiosarcoma, the correct diagnosis can often be delayed. ...
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Primary Angiosarcoma of the skin of the foot is very rare. Angiosarcoma is typically treated with resection and wide-field postoperative radiation therapy. Chemotherapy and radiation therapy have also been used. Regardless of the treatment, the risk of local and distant relapse remains high for this disease. We present a case of an elderly patient who developed cutaneous angiosarcoma of the foot. It posed as a diagnostic dilemma at presentation. Chronic lymphedema was a possible predisposing factor. Given his age, preexisting renal dysfunction, refusal of surgery, and preference not to receive chemotherapy, the patient was ultimately treated with definitive radiotherapy. We present this case because of its rare site, unique presentation and delay in diagnosis of the condition, and attainment of an excellent response to radiation at the time of follow-up. We also review the current literature on this topic.