Original ContributionCutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007
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
References (33)
- et al.
Postirradiation angiosarcoma
J Am Acad Dermatol
(1985) - et al.
Mast cells in an angiosarcoma complicating xeroderma pigmentosum in a 13 year old girl
J Am Acad Dermatol
(2000) - et al.
Cutaneous angiosarcoma: a case series with prognostic correlation
J Am Acad Dermatol
(2004) - et al.
Histopathological diagnosis of cutaneous vascular sarcomas
Rom J Morphol Embryol
(2010) Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis
J Cutan Pathol
(2009)Angiosarcoma, radiation associated-angiosarcoma, and atypical vascular lesion
Arch Pathol Lab Med
(2009)- et al.
Cutaneous soft tissue sarcoma incidence patterns in the US. An analysis of 12,114
Cancer
(2008) - et al.
Tumors of the soft tissues. Atlas of tumor pathology. Third series. Fascicle 30
(2001) - et al.
Angiosarcoma of skin and soft tissue: a study of forty-four cases
Cancer
(1981) - et al.
Cutaneous angiosarcoma of the head and neck
Cancer
(1979)
Angiosarcoma of soft tissue: a study of 80 cases
Am J Surg Pathol
Endothelin-secreting angiosarcoma occurring at the site of an arteriovenous fistula for hemodialysis in a renal transplant recipient
Br J Dermatol
Angiosarcoma associated with foreign material. A report of 3 cases
Cancer
Angiosarcoma complicating xeroderma pigmentosum
Histopathology
Cutaneous angiosarcoma in a patient with xeroderma pigmentosum
Pediatr Hematol Oncol
Cited by (137)
Cutaneous angiosarcoma of head and neck: Case report and literature review
2023, Oral Oncology ReportsManagement of Vascular Sarcoma
2022, Surgical Oncology Clinics of North AmericaManagement of Skin Sarcomas
2022, Surgical Oncology Clinics of North America