Cardiac epithelioid angiosarcoma presenting as cutaneous metastases

BACKGROUND: Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases. METHODS AND RESULTS: We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now. CONCLUSION: Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.     

Cutaneous breast angiosarcoma after conserving treatment of breast cancer.

Cutaneous angiosarcoma is a rare malignancy that sometimes occurs as a late sequela of breast conservation therapy. We report on a 79-year-old female who developed well-differentiated angiosarcoma in a lymphedematous left breast 5.5 years after surgery and radiotherapy for early invasive ductal breast cancer. The initial appearance was very similar to late radiation dermatitis, and histologically interpreted as scar tissue with atypical vascular lesion. The lesion progressed further, and was clinically suspicious for angiosarcoma. Thus, a second biopsy was taken which confirmed the diagnosis. A complete mastectomy removed all the tumor with clear margins. However, within a period of 16 months she presented four local recurrences which were treated by wide local excision. At present, the patient is free of locally recurrent tumour for 7 months. The few cases of breast angiosarcoma after breast conservation therapy reported so far demonstrate that these lesions are difficult to diagnose due to their rarity and their highly variable and benign appearance, which sometimes may mimic radiation-induced cutaneous changes. Since chronic lymphedema possibly contributes to the development of angiosarcoma, long-term clinical surveillance of these patients is recommended. Biopsies should be taken if new skin lesions occur.     

Complete remission of a radio-resistant cutaneous angiosarcoma of the scalp by systemic treatment with liposomal doxorubicin

We report an 80-year-old man suffering from an angiosarcoma of the scalp. Because of the wide extent of the lesions, surgery was not performed. Instead, the patient was treated with electron-beam radiation. Later, the patient failed to benefit from radiotherapy demonstrated by a local relapse and new malignant lesions. Additionally, a cervical lymph node metastasis appeared for the first time. Subsequently, we successfully administered liposomal doxorubicin (Caelyx(R)). Shortly after administration of two cycles the scalp angiosarcoma showed a clear regression. Following six cycles, the patient clinically showed a complete remission of all skin lesions and the cervical lymph node; metastasis was confirmed by histology and fine needle aspiration, respectively. Liposomal and pegylated doxorubicin, a cytostatic drug belonging to the anthracyclines, has already shown to be effective and mostly well tolerated in the therapy of acquired immune deficiency syndrome-related Kaposi's sarcoma and very recently in cutaneous T-cell lymphoma, too. Caelyx(R) appears to be a promising alternative to conventional treatment of cutaneous angiosarcoma.     

Case of Rapidly Progressing Angiosarcoma after Total Hip Arthroplasty

The occurrence of malignant tumor in proximity to an arthroplasty prosthesis has been a matter of debate since it was first reported in 1978. Upon considering the number of orthopedic implants used, the occurrence of malignancy is rare. Especially in case of angiosarcoma, only a few cases have been reported worldwide. In this case, we report an extremely rare case of angiosarcoma arising at the site of a revision total hip arthroplasty. A 69-year-old female had received total hip replacement on her left hip due to osteoarthritis 8 months ago. Four months later, she complained pain on her operated area, X-ray showed loosening of implanted cup on her left hip. Thereafter, erythematous and purpuric papules and nodules were developed and spread around on her left hip. Through the skin biopsy she was diagnosed with angiosarcoma, and then she died of a sharp deterioration. Herein, we report a rare case of angiosarcoma occurred after total hip replacement with a review of the literature.     

Angiosarcoma in postsurgical lymphedema. An unusual occurrence in a man

A 63-year-old man underwent partial left axillary node dissection for metastatic melanoma of unknown primary. Lymphedema of his left upper extremity developed after surgery. Eight years later, extensive cutaneous angiosarcoma developed in the edematous limb. The occurrence of cutaneous angiosarcoma in postsurgical lymphedema is extremely rare in men; to our knowledge, our case is the fifth such case thus far reported.     

Distant cutaneous metastasis of pleural malignant mesothelioma

We report a facial tumor that was proven to be a metastatic mesothelioma. The diagnosis was not established pre-mortem. The patient died shortly after the facial biopsy, and an autopsy revealed a large pleural-based mass which had the gross appearance typical of a mesothelioma. Electron microscopic examination of tissue from the pleural tumor was diagnostic for mesothelioma. The patient had extensive visceral metastatic disease. Inclusion of this entity in the differential diagnosis of certain cutaneous tumors is important, in part because this lesion may be confused with angiosarcoma, particularly when it occurs in the skin of the face or head in older patients.     

Angiosarcoma in an irradiated breast: a case description

Post-radiotherapy cutaneous angiosarcomas have been described in different locations, including the breast. We present a case of cutaneous angiosarcoma of the breast, diagnosed 6 years after a carcinoma of the breast had been treated with radiation. The patient was a 44-year-old female with a history of ductal carcinoma (CA) of the right breast treated with tumorectomy, axillary lymphadenectomy, chemotherapy (FEC) and radiotherapy, who 6 years later presented with a violaceous, indurated plaque with a satellite nodule on the same breast. The histology of the skin biopsies showed angiomatous proliferation throughout the entire dermis, with no cellular atypia. Considering the patient's symptoms, history and the changes observed via mammography, it was decided to completely excise the lesion followed by a simple mastectomy, with the diagnosis of angiosarcoma being confirmed. The patient was later treated with paclitaxel, and the disease was apparently controlled. Despite this fact, two years and one month later, the angiosarcoma recurred on the internal area of the mastectomy scar. The patient was treated with paclitaxel, and surgical excision of the lesion area was once again performed. This complication usually appears 5-10 years after treatment with radiotherapy, so angiosarcoma should be ruled out if any angiomatous lesions later appear on skin that had been irradiated.     

Superinfected cutaneous angiosarcoma: a highly malignant neoplasm simulating an inflammatory process

This report describes a patient with a poorly differentiated cutaneous angiosarcoma (CA) of the face superinfected with pseudomonas aeruginosa. Neoplastic cells were positive for CD-34, CD-31 and vimentin, whereas they failed to express other vascular markers such as Factor VIII and Ulex europeaus lectin. The tumor spread rapidly through the skin and the superficial soft tissue before metastasizing. The patient died of disease 6 months after histopathological diagnosis. An autopsy revealed widespread metastases in the lung and the liver. The aim of this report is to call attention to some circumstances in which CA may masquerade as an inflammatory process, delaying the right diagnosis with serious consequences for the patient.     

Primary cutaneous anaplastic large cell lymphoma with intralymphatic involvement associated with chronic lymphedema

Chronic lymphedema predisposes to develop malignant cutaneous tumors, including angiosarcoma, Kaposi's sarcoma and B‐cell lymphoma. T‐cell malignancy has rarely been associated with chronic lymph stasis. Here, we report a case of primary cutaneous anaplastic large cell lymphoma (pcALCL) with lymphatic spread associated with chronic lymphedema. The patient is a 56‐year‐old man who received orchiectomy and right inguinal lymphadenectomy for malignant seminoma 10 years ago, which led to prominent lymphedema of the right leg. He developed extensive skin nodules on the lymphedematous area for 3 months. Histopathology findings confirmed a diagnosis of pcALCL, which is a subtype of cutaneous T‐cell lymphoma characterized by the presence of CD30+ T cells. Intralymphatic infiltration of malignant cells is prominent. The pathogenesis of intralymphatic cutaneous anaplastic large cell lymphoma is largely unknown. Our case suggests that chronic lymphedema resulted in persistent CD4+ T‐cell inflammation, which then may contribute to the development of pcALCL.     

Cutaneous angiosarcoma of the face

Cutaneous angiosarcoma is a rare tumour of vascular origin, which has a poor prognosis because of its high potential for metastasis. We report the case of a 57-year-old man with an 8-month history of a progressively enlarging, asymptomatic red patch over the left periorbital region of the face, previously diagnosed as angiolupoid leishmaniasis, insect-bites, 'cellulitis' and treated with several topical antibiotic and steroid therapy, without any improvement. A skin biopsy of the lesion was performed and histological and immunohistochemical examination revealed a pattern of poorly differentiated angiosarcoma. The peculiarity of the localization at the periorbital area and the particular clinical presentation are emphasized.     

Cutaneous angiosarcoma with skin metastases and persistent bloody pleural effusions

Cutaneous angiosarcoma is a rare aggressive malignancy of vascular origin that usually arises in the scalp or face of elderly men. We describe a case of primary cutaneous angiosarcoma with skin metastases and presumed metastases to the lung in a 58-year-old man who presented with persistent bloody pleural effusions, an asymptomatic nontraumatic red patch on the forehead of 2 to 3 months' duration, and a pair of purpuric papules on his left mid back of unknown duration. Cutaneous metastases of angiosarcoma are uncommon. Spontaneous persistent bloody effusions without hemoptysis are distinctly uncommon, and pleural fluid cytology is repeatedly negative in lung or pleural angiosarcoma, making it difficult to diagnose without tissue biopsy.     

Angiosarcoma complicating systemic sclerosis: a case report

Cutaneous angiosarcoma is a rare malignant vascular tumor with a poor prognosis, most commonly affecting elderly white men. Diagnosis usually is delayed because the tumor has a highly variable and often innocuous presentation. Cutaneous angiosarcoma has been associated with a number of conditions and factors, including chronic lymphedema, prior radiation therapy, exposure to chemicals, and vascular malformations. We report the case of a 40-year-old black man with systemic sclerosis (SSc) who developed a cutaneous angiosarcoma in an area of sclerodermatous scalp. We propose that vascular endothelial growth factor (VEGF) overexpression in sclerodermatous skin may predispose a patient to the development of vascular tumors, such as angiosarcoma. Because early diagnosis and treatment positively impact survival outcome in patients with angiosarcoma, it is essential that physicians recognize the association of angiosarcoma and SSc and maintain a low threshold for performing a biopsy when suspicious lesions are present on sclerodermatous skin.     

Angiosarcoma of the thigh with thrombosis in the femoral artery.

We report a case of angiosarcoma with some peculiar clinical features developing on the left thigh of a 63-year-old man. The early primary lesion was erythematous with necrotic areas. The initial biopsy specimen of the lesion indicated a benign angioproliferative process. However, the necrotic area enlarged rapidly, ulcerated with severe pain, and thus was widely excised. The excised specimen had a malignant histologic appearance, particularly in the fascia, and was diagnosed as angiosarcoma. Following local recurrence, the lesion was complicated by thrombosis of the femoral artery at the affected site. A similar cutaneous lesion subsequently arose on the right lower leg, and thrombosis developed in the right femoral artery. Both legs had to be amputated. There were no distant metastases.     

Recurrent and metastatic cutaneous neuroendocrine (Merkel cell) carcinoma mimicking angiosarcoma

An elderly man presented with bright red-to-purple confluent nodules and plaques of the scalp that appeared clinically consistent with angiosarcoma. He had undergone surgical removal of a similar lesion from his left temple seven months before this presentation. Although routine histologic examination was not sufficient for diagnosis, Merkel cell carcinoma was diagnosed by immunohistochemical studies and electron microscopy. In addition, oat cell carcinoma of the lung was excluded with a computed tomographic scan of his chest. With chemotherapy, the recurrent and metastatic lesions of Merkel cell carcinoma completely resolved, rapidly recurred, and resolved a second time with chemotherapy. Eight months following the fourth course of chemotherapy, the patient again developed widespread cutaneous lesions. He committed suicide within one month of this last recurrence. An autopsy revealed no primary tumor other than of the skin.     

Metastasis of Mediastinal Epithelioid Angiosarcoma to the Finger

Epithelioid angiosarcoma (EA) is a rare malignant, vascular tumor that is usually observed in middle-aged and elderly males. Cutaneous metastasis of EA is extremely rare. We report the case of a 41-year-old woman presenting with a painful bluish, bulla-like lesion on the distal extent of the left third finger. The patient had recently been diagnosed with mediastinal EA with disseminated metastases. The skin biopsy specimen revealed metastatic EA. This is thought to be the first reported metastasis of EA to the finger. Whenever a patient has metastatic disease, acrometastases should be considered in the differential diagnosis of inflammatory lesions of the digits and a skin biopsy should be performed.     

Angiosarcoma arising in a lymphedematous abdominal pannus with histologic features reminiscent of Kaposi's sarcoma: report of a case and review of the literature

BACKGROUND: Despite common endothelial origins, angiosarcoma and Kaposi's sarcoma are clinically and histologically distinct vascular proliferations. The development of angiosarcoma in a chronically edematous abdominal pannus is extremely uncommon. Similarly, tumors with the combined histologic features of angiosarcoma and Kaposi's sarcoma have rarely been described. METHODS: We reviewed the literature on angiosarcoma arising in a lymphedematous abdominal pannus and evaluated an 81-year-old morbidly obese woman who had profound, long-standing edema of the lower abdominal wall in which an aggressive vascular tumor developed. RESULTS: Three clinically similar cases were identified in the literature. All patients were women who generally experienced rapid disease progression. In addition, in our patient, sequential cutaneous sampling from different lesional sites demonstrated disparate histologic changes, ranging from those of classic Kaposi's sarcoma to high-grade angiosarcoma, to areas with combined features of the two tumors. A polymerase chain reaction performed on lesional tissue was negative for human herpesvirus-8 DNA. CONCLUSION: It is important to note that angiosarcoma may develop in the abdomen in association with chronic lymphedema, as demonstrated by the cases noted in this report. In addition, our case highlights the difficulty in differentiating histologically angiosarcoma from Kaposi's sarcoma in some situations, and demonstrates the value of close clinicopathologic correlation and sequential tissue sampling in evaluating problematic cases.     

Scleromyxoedema with associated peripheral neuropathy: successful treatment with thalidomide

We report a patient with scleromyxoedema and peripheral neuropathy treated successfully with thalidomide. An objective evaluation was carried out using histopathology, cutaneous ultrasonography and magnetic resonance imaging (MRI). A 67-year-old woman presented with a leonine face, generalized thickened skin, an underlying peripheral neuropathy and a monoclonal gammopathy. She was treated with thalidomide 100 mg/day. After 20 months of therapy, there was a dramatic clinical improvement in the skin lesions, and the neuropathy also improved. Cutaneous ultrasonography showed a reduction in dermal thickness, whereas the results of the cutaneous MRI were inconclusive. Thalidomide appears to be effective in scleromyxoedema. Its specific effect on the underlying monoclonal gammopathy might have contributed to the improvement in the skin and neurological symptoms. In this case, assessment of cutaneous improvement with cutaneous ultrasonography was superior to that of cutaneous MRI. Thalidomide should be considered for the treatment of scleromyxoedema despite the presence of an underlying peripheral neuropathy.     

Cutaneous angiosarcoma of the leg showing radiation sensitivity

We report a case of cutaneous angiosarcoma occurring on the leg of a 97-year-old Japanese woman. Considering the patient's age and general condition, she was treated with electron beam irradiation, which led to the almost complete disappearance of the tumour. Because cutaneous angiosarcoma is an aggressive tumour with a high propensity for local recurrence and distant metastases, therapy preferably involves a multimodal approach. However, monotherapy with radiation may be effective in some cases of cutaneous angiosarcoma.     

Granulocyte colony-stimulating factor-producing cutaneous angiosarcoma with leukaemoid reaction arising on a burn scar

We report a 46-year-old man with a giant tumour in a burn scar on his buttock. Pathological examination revealed that the dermis was filled with anastomosing vascular channels and round- or spindle-type atypical cells, which were compatible with the diagnosis of cutaneous angiosarcoma. Based on prominent leucocytosis (up to 113 000 microL-1), we measured serum granulocyte colony-stimulating factor (G-CSF). The highly elevated serum G-CSF of 303 ng L-1 (normal, 6.1-21.5 ng L-1) and positive immunohistochemical staining of the tumour tissue for G-CSF indicated that G-CSF was produced by the cutaneous angiosarcoma. To our knowledge, this is the first reported case of G-CSF-producing cutaneous angiosarcoma.     

Kutane Angiosarkome

BACKGROUND AND OBJECTIVES: Angiosarcomas of the skin arise almost exclusively in the following clinical settings: 1. the face and scalp, usually in elderly individuals, 2. lymphedematous regions (lymphedema-associated angiosarcomas), and 3. skin that has been previously irradiated (post-radiation angiosarcomas). Clinical and histopathologic diagnosis of angiosarcoma is difficult often resulting in great delay that obviates against early and possibly successful treatment of these very aggressive neoplasms. Diagnostic problems are described, and prognostic factors as well as the effect of different forms of treatment on the outcome are discussed. PATIENTS/METHODS: Retrospective study of 11 patients with cutaneous angiosarcomas. Clinical presentation, histopathology, therapy and survival time are analysed. RESULTS: Only 1 of 11 cases cutaneous angiosarcoma was clinically identified. Survival time was 1-24 months. Three patients who received radical surgery have not developed metastases and are still alive. CONCLUSIONS: Clinical and histopathologic diagnosis of cutaneous angiosarcomas is often very difficult. Prognosis is very bad; radical surgery seems to be the best therapeutical option.