Cutaneous tumours of vascular origin: two rare entities

Skin and subcutaneous tissue are the most common location of benign and malignant vascular tumors. The benign or malignant nature of the lesion may not always be identified clinically because of the varying presentations. The histology of the lesion, especially in malignancies may not exhibit definite vascular pattern.  In such cases, clinical presentation has to be correlated with histopathological picture as well as immunohistochemical characteristics to reach a specific diagnosis. Judicious use of immunohistochemical markers proves to be invaluable.  Two rare cases of cutaneous vascular tumors which presented at our institution are cited, the diagnosis of which involved correlation of these modalities.

Clinicopathological Analysis of Cutaneous Angiosarcoma / 대한피부과학회지

BACKGROUND: Cutaneous angiosarcoma (AS) is a rare malignant tumor of endothelial origin with a very poor prognosis, frequent recurrences, and high metastatic potential. Clinical suspicion is often raised too late, but histological findings and immunohistochemical assays have proved to be very helpful in the diagnostic process. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathologic features of angiosarcoma. METHODS: We retrospectively analyzed 12 patients with angiosarcoma who had visited our department of dermatology in Gyeongsang National University Hospital between 1998 and 2013. The clinical features, evolution, treatment, and outcome were examined, and biopsy specimens were reviewed by a trained dermatologist, with subsequent immunohistochemical assessment. RESULTS: Cutaneous AS was clinically diagnosed in 3 of 12 patients. There were many clinical misdiagnoses including lupus erythematosus, metastatic cancer, and infectious granuloma. The male to female ratio was 1:0.71 and the mean age of the patients was 66.25 years. The most frequent site of angiosarcoma was the scalp, in 8 of 12 patients. Histopathologically, well-differentiated angiosarcoma was composed of vascular channels, lined by flattened atypical endothelial cells that could be distinguished from their benign counterparts. Variable differentiation may be observed even in the same tumor. Immunohistopathologic analysis showed that the tumor cells were positive for CD31, CD34, Ki 67, and Factor VIII-related antigen. CONCLUSION: This study provides useful data on the clinical and histopathologic characteristics of angiosarcoma in the Korean population.

Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study.

Primary malignant vascular tumors of the bone are exceedingly rare and represent <1% of primary malignant bone tumors. Angiosarcoma is a malignant mesenchymal neoplasm in which the neoplastic cells demonstrate endothelial differentiation. Epithelioid angiosarcoma (EA) is a rare variant of angiosarcoma that is characterized by large cells with an epithelioid morphology. EA is an aggressive tumor with poor prognosis. Here, we present a case of a 62-year-old man who had primary EA of the left tibia. He was treated with amputation and chemotherapy. After 1 month of chemotherapy, he developed pleural effusion and died.

Interdisciplinary approach to trating patients with epithelioid angiosarcoma: a case report / Conduta interdisciplinar para tratar paciente com angiossarcoma epitelióide: relato de um caso-clínico

Epithelioid angiosarcoma is a rare malignant neoplasm found in vascular endothelial cells. The present study aims to describe the radiological, clinicopathological, and immunohistochemical findings of a case of epithelioid angiosarcoma located in the posterior mediastinum of a 65-year-old male patient who underwent surgery and clinical treatment (Doxorubicin-based chemoterapy). It could be observed that the patient presented poor oral hygiene, severe cervical erosions in his teeth, chronic caries, periodontal disease, and periapical abscess. For this reason, the participation of a dentist in an interdisciplinary team is of utmost importance. Oral support treatment also becomes essential in an attempt to eliminate all foci of oral infections, as well as minimize morbidity and improve the general condition of the patient before, during, and after oncological treatment. In addition, the dentist will work toward the education and motivation of the patient to take on an appropriate oral hygiene so as to minimize the oral complications of chemotherapy, in turn providing better conditions for the patient to eat, speak, socialize, and maintain a high quality of life.

A Case of Epithelioid Angiosarcoma on the Back / 대한피부과학회지

Epithelioid angiosarcoma has recently been described as a variant of angiosarcoma, based on its pathologic feature which is characterized by epithelioid or histiocytoid morphology of the malignant tumor cells. We report a case of epithelioid angiosarcoma on the lower back of a 65-year-old man. The patient had several, variable-sized, pedunculated, fungating masses. On histopathologic examination, the tumor was chiefly composed of solid sheets of atypical epithelioid cells with prominent eosinophilic cytoplasm, a large vesicular nuclei, and occasional intracytoplasmic vacuoles. Primitive vascular spaces, and a cleft with malignant cells and proliferating vessels were also found in some areas. The reticulum stain and immunohistochemical stain using factor VII-related antigen and CD 31 were focally positive in the tumor. He was treated by wide surgical excision.

A Case of Epithelioid Angiosarcoma of the Scalp / 대한피부과학회지

Angiosarcoma is a rare malignant tumor of endothelial cell origin. Histopathologically, varied differentiation may be observed even within the same tumor. Epithelioid angiosarcoma is one subset of poorly differentiated angiosarcoma which is a rare variant. We report a case of epithelioid angiosarcoma of the scalp in a 59-year-old man. The patient had a painful solitary, 2x3 cm sized, dome-shaped, firm, erythematous to purplish nodule with crust on the vertex of the scalp. Histopathologically, the tumor was chiefly composed of solid sheets of atypical epithelioid cells with prominent eosinophilic cytoplasm, a large vesicular nuclei, and occasional intracytoplasmic vacuoles. Well to moderately differentiated areas with irregular vascular channel-like structures lined by atypical hyperchromatic endothelial cells were also seen in the peripheral areas of the tumor. Immunohistochemically, the tumor cells were positive with CD31 which is sensitive marker of endothelial cell.

A Case of Cutaneous Epithelioid Angiosarcoma / 대한피부과학회지

The epithelioid angiosarcoma is recently described ns a variant of ang-osarcoma based on its pathological feature, which is characterized by the epithelioid or histiocytoid morphology af the malignant tumor cells. The existence of vascular endothelial tumor with an epithelioid feature has been accepted for many years, most notablyn the forms of epithelioid hemangioma and epithelioid hemangioendo thelioma. In recent years, cutaneous angiosarcoma with epitheloid morphology have been reported as a cutaneos epithelioid angiosarcoma in the literatures. A 60-year-old man presente with a mild tender erythematous 2 x 2 x 2.5cm sized soft fluctuating single nodule with dark hemorrhagic crust on the left forehead for 3 months. He had had an abrasion wound on the lesion site of the forehead by an accidental trauma 5 month before the lesion appeared. Histopathological findings revealed that the tumor mass was chiefly composed of solid sheets of poorly differentiated malignant cells with an epithelioid feature. The characteristics of classical angiosarcoma such as primitive vascular spaces and clefts with malignat cells, and proliferating vessels were also found in some areas. The immunohistochemical stain wi.h the factor VIII related antigen ivas focally reactive in the tumor cells. Unfortunately he refused further treatment and expired 7 months after discharge at home without knowing the direct cause of his health. We report herein an interesting and rare case of the cutaneous epitheioid angiosarcoma which had typical clinical and histopathological findings and suggested as a case with a very aggressive course.