Regardless of the treatment, the risk of local and distant relapse remains high for this disease. Histopathology was required to make the diagnosis in this case and found the typical features of angiosarcoma, which range from infiltrating irregular vasculature to poorly formed vasculature with concurrent poorly differentiated cells. Pdf a cutaneous angiosarcoma is a rare malignant tumour of vascular. Connor j, doppalapudi s, he w, benson m, esposito m, et al 21 primary renal angiosarcoma with cutaneous metastasis clin ncol case rep 2. Atypical fibroxanthoma and pleomorphic dermal sarcoma may be difficult to separate from cutaneous angiosarcoma. Angiosarcomas of the skin are uncommon and constitute less than 1% of all sarcomas. Case report fatal poorly differentiated angiosarcoma of the scalp. Cutaneous angiosarcoma cas is a rare malignant neoplasm with variable clinical presentation. The setting is an academic teaching hospital skin cancer center.
Basal cell carcinoma bcc is the most common of the nonmelanoma skin cancers and is a highly curable skin growth. Cutaneous angiosarcoma is a rare aggressive tumor of capillary and lymphatic endothelial cell origin. Webpathology is a free educational resource with 10297 high quality pathology images of benign and malignant neoplasms and related entities. Apr 29, 2020 angiosarcoma is cancer that forms in the lining of blood vessels and lymph vessels. The disease most commonly occurs in the skin, breast, liver, spleen, and deep tissue. Cutaneous angiosarcoma is a rare malignant tumor of vascular endothelial cells that has the. There are 3 main variants of cutaneous angiosarcoma. Nov 18, 20 angiosarcomas of bone are very rare and account for less than 1% of all angiosarcomas in any organ and less than 1% of primary bone tumors. An angiosarcoma as is an uncommon malignant neoplasm characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular bloodfilled spaces. Pathology of epithelioid angiosarcoma dr sampurna roy md. We aim to study the morphological spectrum of pseudoangiomatous features in these. Hemangiosarcoma hsa is a type of cancer that develops from the cells that normally create blood vessels endothelial cells. The immunophenotypic profile using four endothelial markers showed positive staining in all cases for factor viii related antigen in a predominantly paranuclear dot.
Angiosarcomas are rare malignancies of endothelial origin. Radiologic findings are often non specific and may appear completely normal in onethird of cases with primary angiosarcoma. The 5year survival rate is between 12% and 34% according to most studies, 1,2 but it can be as high as 62%. Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy, most commonly arising in the deep soft tissues, but a variety of primary sites, including the adrenals, thyroid, skin, and bone, are encountered.
Chemotherapy and radiation therapy have also been used. Apr 22, 20 primary angiosarcoma of the breast is extremely rare. This is the most common pure malignant stromal tumour of the breast. Cutaneous angiosarcoma has one of the worst prognoses among all cutaneous tumors.
All lesions presented characteristic histopathological findings. With increasing tumour size, tissue infiltration, oedema, tumour. Angiosarcoma is a malignant tumor of vascular endothelial cells that arises in the head and neck. Angiosarcoma as is a rare and highly aggressive malignant neoplasm derived from vascular endo thelial cells that has predilection for the skin. Angiosarcoma is a rare malignant tumor, which most often occurs in the skin and soft tissue. The best outcomes are reported with surgery followed by radiotherapy.
Eight caucasian patients could be identified, 5 men and 3 women. It presents as multiple purple and red papules, and nodules on the head and neck or the extremities. Cutaneous angiosarcoma can initially resemble a bruise, or a raised purplishred papule, is typically multifocal and can be mistaken for a simple benign lesion leading to delayed presentation and diagnosis. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy andor radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary. Angiosarcoma is typically treated with resection and widefield postoperative radiation therapy. Cprasb differs from stewarttreves angiosarcoma by its shorter latency period and lack of association with lymphedema. Cutaneous hemangiosarcoma general information hemangiosarcoma is a malignant, aggressive type of softtissue tumor that comes from the epithelial cells that line the walls of blood vessels. A 70yearold turkish woman was admitted to our hospital complaining of red mass of the scalp the size was 4x4x5 cm. Update on cutaneous epithelioid vascular tumours diagnostic.
We present a case of an elderly patient who developed cutaneous angiosarcoma of the foot. Poor prognosis with 3 year survival of 41% associated with lymphedema 10 years after radical mastectomy for breast cancer, arising from dilated lymphatics lymphangiosarcomas, also called stewarttreves syndrome, not associated with radiation therapy. In addition, the tumor cells showed weak focal positivity for. On hematoxylineosinstained sections, the pathologist encounters sheets of large, mildly to moderately pleomorphic. Cutaneous angiosarcoma of the breast after segmental mastectomy and radiation therapy scott c. The cause of hemangiosarcoma in most cases is unknown. Expression of erg, an ets family transcription factor. The prognosis is usually poor because of the high rates of local recurrence and early development of metastasis. Infiltrating, freely anastomosing channels lined by spindled to epithelioid endothelial cells with variable atypia, surrounding adnexae and dissecting dermal collagen. Angiosarcoma is a cancer of the endothelial cells that line the walls of blood vessels or lymphatic vessels.
Primary angiosarcoma of the skin of the foot is very rare. The disease is primarily located on the head and neck of elderly persons and is also known as wilsonjones angiosarcoma, senile angiosarcoma or malignant angioendothelioma. Surgical removal followed by adjuvant chemotherapy seems improve the prognosis. Cutaneous angiosarcoma of the head and neck region is a subtype of cutaneous angiosarcoma with an unfavourable prognosis. Cutaneous angiosarcoma of head and neck of the elderly is a. We report the case of an elderly man whose histopathologic diagnosis. Cutaneous cd30positive epithelioid angiosarcoma following b.
Vascular, homogeneous, spongy mass with hemangiomalike cut surface. Angiosarcoma of the skin, or cutaneous angiosarcoma, makes up the majority of angiosarcoma cases, and it is usually found on the scalp and face. It is very aggressive and has high local recurrence rates. Bone and soft tissue angiosarcoma are also reported to be more frequent in males bone angiosarcoma appears most often in adults second to seventh. Cutaneous angiosarcoma journal of clinical pathology. Postoperative histopathology and immuno histochemistry of the wide local excision specimen proved the tumor to be cutaneous angiosarcoma and the patient completed adjuvant radiotherapy. Of these markers, cd31 is known to be the most sensitive and specific marker of angiosarcoma. Cutaneous form of angiosarcoma initially presents as a bruise or a raised purplishred papule on the central face. Expression of erg, an ets family transcription factor, distinguishes cutaneous angiosarcoma from histological mimics. The histopathology of angiosarcoma depends on the areas of tumour differentiation. Cutaneous angiosarcoma of the scalp masquerading as a. Endoglin cd105 and claudin5 expression in cutaneous.
Epithelioid angiosarcoma is a rare rapidly growing malignant vascular tumour with an aggressive course. Cutaneous postradiation angiosarcoma of the breast cprasb may occur in patients who have undergone breastconserving therapy combined with adjuvant radiation. Its occurrence in the head and neck region, and the intraoral region in particular, is uncommon. Primary cutaneous angiosarcoma is one of the most aggressive skin tumors and carries a very poor prognosis. Oct 20, 2014 our findings outline the morphological spectrum of cytoplasmic change in cutaneous angiosarcoma. However, we do know that sunlight can cause this cancer on the belly skin. Known as stewarttreves syndrome in its original association with postmastectomy lymphedema association with radiation breast, urinary bladder. Its initially indolent clinical presentation explains. The differential diagnosis of cd30 epithelioid angiosarcoma is discussed. Because these cancers are carried by the blood flow or lymphatic flow, they can more easily metastasize to distant sites, particularly the liver and lungs. Early detection and multidisciplinary approach may improve survival.
A case of cutaneous epithelioid angiosarcoma with anomalous cd30 expression, occurring after breastconserving surgery and adjuvant irradiation, is reported. Angiosarcoma of the head and neck is a rare malignant neoplasm. Cutaneous angiosarcoma of the head, neck, and face of the. Cutaneous angiosarcoma is more frequent in males than in females, with a maletofemale ratio of 2. Cutaneous angiosarcoma of head and neck of the elderly is a great mimicker with many clinical presentations. We report a case of cutaneous angiosarcoma that developed. These neoplasms are classified into cutaneous, visceral, and soft tissue subtypes. The epithelioid variant of angiosarcoma was first described in cases of cutaneous angiosarcoma by rosai et al in 1976 and later documented in deep soft tissue by weiss et al. He is now on regular followup for 1 year without recurrence.
Cutaneous angiosarcoma is a cutaneous neoplasm with a very poor prognosis. Cutaneous angiosarcoma fulltext case reports in dermatology. Although a distinct vascular tumour, cas shares many overlapping histopathological features with other vasoformative and epithelioid tumours or mimickers. African americans in the united states are rarely affected by cutaneous angiosarcoma 12. The spectrum of rare morphological variants of cutaneous. We report the case of an elderly man whose histopathologic diagnosis confirmed the clinical suspicion of cutaneous angiosarcoma. Angiosarcoma of the breast is an uncommon entity, and the development of cutaneous angiosarcoma of the breast after segmental mastectomy and irradiation therapy is even less common. Microscopically, diagnosis of cutaneous angiosarcoma can present a challenge, as the histology varies between a. Surgical resection with negative margins chemotherapy is occasionally used clinical images. Analysis of 434 cases from the surveillance, epidemiology, and end results program, 19732007. Delay to diagnosis was between 12 to 4 months mean 7.
Awareness and a high degree of suspicion in the context of tumours affecting sun. Cutaneous angiosarcoma for skin and superficial soft tissue, accounting. An excisional biopsy was performed, and histopathology and immunohistochemical studies were consistent with cutaneous angiosarcoma. Rathod et al cutaneous angiosarcoma of face a case report int j cur res rev, jan 20 vol 05 01. Your lymph vessels, which are part of your immune system, collect. A case report of angiosarcoma of maxillary gingiva. An elderly woman presented with an aggressive hemi facial. It is predominantly seen in the whiteskinned people and rarely dealt with among the colored races.
A previous study of angiosarcoma demonstrated the diagnostic value of cd31, cd34, and uea1 immunostaining. Most tumors of visceral blood and lymphatic vessel walls are cancerous malignant. Cutaneous angiosarcoma is the most common form of angiosarcoma not associated with chronic lymphoedema. Cutaneous epitheiloid vascular tumours are heterogeneous groups of vascular proliferations sharing in common the epithelioid morphology of tumour cells.
Cutaneous angiosarcoma is a rare, highly malignant vascular tumor with three clinical types. Four cases of cutaneous epithelioid angiosarcoma are described together with the potential diagnostic trap of mistaking these tumours for poorly differentiated carcinoma or malignant melanoma. It carries a high probability of local relapse and has a 10% to 15% survival rate at 5 years. Jul 06, 2011 here, we report a case of poorly differentiated angiosarcoma of the scalp. Pdf cutaneous angiosarcoma is a rare malignant tumor showing blood or lymphatic. The aim of this case report is to highlight the importance of histopathological examination and to underscore the characteristic features of angiosarcoma. Cutaneous angiosarcoma of the scalp and face is the most common form and appears as an enlarging bruise, dark blue to black nodule, or unhealed ulceration.
Cutaneous angiosarcoma is a very rare but aggressive tumor. Cutaneous angiosarcoma of the head and neck regions seems to be a distinctive neoplasm with characteristic clinicopathologic features that differ from angiosarcoma in other anatomic locations. Angiosarcoma imitating a morpheaform basal cell carcinoma. The role of chemotherapy in cutaneous angiosarcoma has not yet been established, although for patients with metastasis or tumors deemed unresectable, doxorubicin intraarterial or systemic is indicated. Angiosarcoma can be classified as cutaneous angiosarcoma, lymphoedemaassociated angiosarcoma stewarttreves syndrome, radiationinduced angiosarcoma, primary breast angiosarcoma and soft tissue angiosarcoma. Here, we report a case of poorly differentiated angiosarcoma of the scalp. Cutaneous angiosarcoma of the breast after segmental.
The 5year survival rate is between 12% and 34% according to most studies,1, 2 but it can be as high as 62%. Histopathologic examination of the specimen diagnosed a cutaneous angiosarcoma. After biopsy and staging, treatment remains challenging with a combination of surgery and radiation therapy most successful. Smink, jr, md \sb\angiosarcoma of the breast is an uncommon entity, and the development of cutaneous angiosarcoma of the breast after segmental mastectomy and irradiation therapy is even less common. Cutaneous angiosarcoma of head and neck a singlecentre. Department of pathology, shizuoka city shimizu hospital, shizuoka. Cutaneous metastases secondary to internal malignancies present in less than 10% of overall cases.
Angiosarcoma causes, symptoms, life expectancy, treatment. Angiosarcoma is a cancer of the inner lining of blood vessels, and it can occur in any area of the body. Atypical fibroxanthoma with pseudoangiomatous features. Approximately 50% of angiosarcomas occur in the head and neck. Based on the who classification epithelioid vascular tumours are classified on the basis of their biological behavior into benign tumours epithelioid haemangioma, epithelioid angiomatous nodule and malignant tumours epithelioid. Stewarttreves syndrome, refers to a lymphangiosarcoma, a rare complication that forms as a result of chronic, longstanding lymphedema. We present a case of an elderly patient who developed cutaneous angiosarcoma of the. Grave prognosis is expected in this rare but aggressive neoplasm. Our findings outline the morphological spectrum of cytoplasmic change in cutaneous angiosarcoma. With increasing tumour size, tissue infiltration, oedema, tumour fungation, ulceration, and haemorrhage can develop. Specialists apply the term angiosarcoma to a wide range of malignant endothelial vascular neoplasms that affect a variety of sites.
Histopathological exams revealed, in the purple macule lesion, proliferation of irregular vascular clefts, interposed by the dermal collagen fibers, formed by. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy andor radiotherapy for breast cancer, it may also describe. Cutaneous metastases from esophageal adenocarcinoma on the. Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. Primary renal angiosarcoma with cutaneous metastasis. It often affects the skin and may appear as a bruiselike lesion that grows over time. Liver angiosarcomas are associated with vinyl chloride exposure cutaneous angiosarcomas are classically seen on the head. Primary angiosarcoma of the breast is extremely rare. Oncology fact sheet american college of veterinary. Histopathology from the biopsy of the nodules in both patients confirmed cutaneous angiosarcoma.
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