Original Contribution
Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007

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Abstract

Cutaneous angiosarcoma is an aggressive malignant mesenchymal vasoformative neoplasm that accounts for 1% of all soft tissue sarcomas. Using data from the National Cancer Institute's Surveillance, Epidemiology, and End Results program, we analyzed the demographics and survival of cutaneous angiosarcoma. The Surveillance, Epidemiology, and End Results program recorded 434 cases of cutaneous angiosarcoma from 1973 to 2007. The incidence was nearly the same in men (222 cases) and women (212 cases). Most patients were white (88%) with a mean age of 73 years. African Americans made up only 4% of the cases. Two hundred seventy (62%) cases were tumors of the head and neck, whereas 106 (24%) cases arose in the skin of the trunk. Grade was recorded in 194 cases (45%): 28 were grade I, 44 were grade II, 60 were grade III, and 62 were grade IV. Survival rates of cutaneous angiosarcoma correlated with age, anatomical site, and stage of disease. Patients younger than 50 years had a 10-year relative survival rate of 71.7%, whereas patients 50 years and older had a 36.8% 10-year survival rate. Tumors of the scalp and neck resulted in a 13.8% 10-year relative survival rate, whereas tumors arising in the trunk resulted in a 75.3% 10-year survival rate. Tumors localized to the skin had better prognosis (53.6% 10-year relative survival rate) than those with regional or distant stage (19.0% and 6.2%). Twenty-six percent of patients with angiosarcoma had a prior primary. Cutaneous angiosarcomas arise predominantly in the head and neck of white individuals older than 60 years.

Introduction

Cutaneous sarcomas are relatively rare, accounting for only 5% of all malignant tumors of the skin [1]. Angiosarcomas are especially rare and make up less than 1% of all sarcomas [2], [3], [4], [5]. Angiosarcomas are malignant mesenchymal vasoformative neoplasms that arise in various soft tissues and visceral organs [2], [3], [4], [5]. The most common sites, in decreasing order, are the skin, breast, deep soft tissues, visceral organs, and bone [5], [6], [7], [8], [9]. Because cutaneous angiosarcomas are rare, they are usually published as case reports or small series from single or collaborating institutions. Although these tumors have been associated with multiple causes that include immunosuppression, radiotherapy, foreign material, and various clinical syndromes including xeroderma pigmentosum [1], [10], [11], [12], [13], [14], [15], [16], there is minimal information available on biological behavior, survival, and demographics.

The Internet now affords a more rapid means of access to the critical accumulation of population-based histopathologic and clinical data. Such data are available through the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute [17]. Initiated in 1973, SEER has now reached a size where clinical and descriptive characteristics of uncommon tumors can be described at a population level. In this report, we describe the population-level distribution of cutaneous angiosarcomas and their demographic features, survival, and relation to additional primary tumors.

Section snippets

Data source

Data were obtained from the SEER Program, which is a population-based registry that collects demographic, anatomic, morphologic, extent of disease, and survival information on patients with cancer. For this study, we used SEER Registry 17 to retrieve all cases of angiosarcoma of the skin from 1973 to 2007. SEER Registry 17 was established in 2000 and covers 26% of the US population [17]. Registry 17 includes 2 other registries, 9 and 13, that were established in 1973 and 1992, respectively. A

Results

From 1973 to 2007, 434 cases of angiosarcoma of the skin were recorded in SEER. Of these, 270 (62%) were angiosarcomas of the head and neck. Specifically, these angiosarcomas occurred on the skin of the lip, eyelid, external ear, scalp and neck, and unspecified parts of the face. Twenty-four percent of neoplasms were on the skin of the trunk. Other primary sites included the skin of the lower limb and hip, and upper limb and shoulder (Table 1).

Discussion

This study is based on 434 cases of cutaneous angiosarcoma collected by the SEER program during a 35-year period (1973-2007). This period provides an overview of pathologically diagnosed cutaneous angiosarcomas that exceeds those available by any single institution or even collaborative effort.

Data submitted to SEER are taken directly from hospital medical records. The SEER database does not permit a review of individual pathologic sections; however, the relatively large numbers of entry cases

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