Report
Cutaneous angiosarcoma: a case series with prognostic correlation

https://doi.org/10.1016/j.jaad.2003.10.671Get rights and content

Abstract

Background

Cutaneous angiosarcoma (CA) is a rare and aggressive endothelial-derived sarcoma. Few large studies have examined the clinicopathologic and prognostic attributes of CA.

Objectives

We sought to discern the potential prognostic significance of a variety of demographic features (ie, age, sex, location), histologic attributes (ie, depth of invasion, tumor necrosis, tumor cell morphology, margin status, mitoses), and follow-up data (ie, tumor recurrence, metastases) in CA.

Methods

The statistical influence of age, sex, anatomic location, tumor depth of invasion, tumor cell morphology, presence or absence of necrosis, number of mitoses, and margin status on time to tumor recurrence and metastases were examined in a series of 47 patients with CA. Angiosarcoma arising within the breast, in a previously irradiated anatomic site, and a pre-existing vascular malformation or one associated with a lymphedematous extremity were excluded from study.

Results

Most of the patients were men (76%), with an average age of 75.1 years (range: 59-92 years). The most common location was the head and neck region (96%). The most common presentation was of a rapidly expanding erythematous patch, and the most common clinical impression was angiosarcoma. The average external diameter of the tumor was 5.3 cm (range: 1.1-8.9 cm). The most common histologic pattern was characterized by anastomosing dissecting sinusoids lined by atypical endothelial cells (64%) with 15% of cases showing a diffuse epithelioid or spindle cell proliferation and 21% showing a mixture of the 2 histologic patterns. The average depth of tumor invasion was 2.86 mm (range: 1.8->6.0 mm). Of the tumors, 78% had a mitotic rate that exceeded 3/mm2. Follow-up was available in 37 of the patients and ranged from 6 to 65 months. The 5-year local recurrence rate was 84% and the overall 5-year survival was 34%. Most patients died as a result of their disease with widespread pulmonary, cardiac, and/or brain metastases.

Conclusions

Of the gross and histologic features, external diameter (>5 cm), depth of invasion (>3 mm), mitotic rate (>3 HPF), positive surgical margins, tumor recurrence, and metastases correlated with adverse outcome by univariate analysis and, with the exception of mitotic rate, by multivariate analysis. Of the foregoing, tumor diameter, depth of invasion, positive margins, metastases, and tumor recurrence were the most robust predictors of outcome. None of the demographic factors was associated with outcome. This study confirms the poor prognosis of patients with CA. Among all demographic and histologic patterns examined for prognostic significance, tumor diameter, tumor depth of invasion, margin status, tumor recurrence, and metastases emerged as the most important determinants of outcome.

Section snippets

Materials and methods

In all, 47 cases of CA were retrieved from the files of 4 large departments of pathology located throughout the United States at institutions where the authors were affiliated. Inclusion criteria for the study included: (1) angiosarcoma arising within the dermis or subcutaneous fat; (2) initial epidemiologic data including age, location, sex, and clinical impression; (3) histologic glass slides for independent pathology review; and (4) confirmation of endothelial histogenesis by ultrastructural

Results

The clinicopathologic features of the 47 cases are summarized in Table I. The patients' age ranged from 59 to 92 years (mean: 75.1 years). The majority of the patients were men (76%). The most common anatomic location was the scalp (49%) followed by the cheek (19%). Of the cases, 95% were located on the head and neck. The most common initial clinical impression was angiosarcoma (38%), followed by lymphoma, primary carcinoma, and metastases in 10% of the cases. Other clinical diagnoses included

Discussion

The prognostic significance of histologic and clinical attributes in CA is reported herein. From this compilation of data, several important conclusions that may have meaningful prognostic and treatment implications emerge. Although these findings essentially support several previous studies, additional information, particularly as it relates to the histologic features of CA, may also have prognostic relevance.

Clinical attributes studied included patient age, sex, and anatomic location. As has

References (27)

  • A Farhood et al.

    Soft tissue sarcomas of the head and neck in adults

    Am J Surg

    (1990)
  • W Lydiatt et al.

    Angiosarcoma of the head and neck

    Am J Surg

    (1994)
  • J Abbbas et al.

    The surgical treatment and outcome of soft tissue sarcoma

    Arch Surg

    (1981)
  • K Antman et al.

    Soft tissue sarcomascurrent trends in diagnosis and management

    Curr Probl Cancer

    (1989)
  • Bardwil J, Mocega E, Butler J, et al. Angiosarcoma of the head and neck region. Am J Surg...
  • C Burgoon et al.

    Angiosarcoma

    Arch Dermatol

    (1969)
  • M Caro et al.

    Hemangioendothelioma of the skin

    Arch Dermatol Syph

    (1945)
  • L Cerroni et al.

    Angiosarcoma of the face and scalp, prognosis, and treatment

    J Dermatol Surg Oncol

    (1991)
  • P Cooper

    Angiosarcomas of the skin

    Semin Diagn Pathol

    (1987)
  • Donnell R, Rosen P, Lieberman P, et al. Angiosarcoma and other vascular tumors of the breast: pathologic analysis as a...
  • R Gerner et al.

    Soft tissue sarcomas

    Ann Surg

    (1975)
  • C Girard et al.

    Cutaneous angiosarcoma

    Cancer

    (1970)
  • U Haustein

    Angiosarcoma of the face and scalp

    Int J Dermatol

    (1991)
  • Cited by (195)

    • Incidence and outcomes of cutaneous angiosarcoma: A SEER population-based study

      2020, Journal of the American Academy of Dermatology
      Citation Excerpt :

      These findings have remained consistent with prior studies.1,6,20,32 In contrast to prior reports, there was no male predominance overall; however, those without a previous primary cancer were more likely to be male.6,14,20,32-34 Furthermore, we noted a female predominance in the group with previous primary cancer, which could be explained by the facts that breast cancer was the most common previous primary cancer and that radiation—a risk factor for CAS—is often part of breast cancer treatment.

    • Metastatic Maxillary Gingival Angiosarcoma with Aggressive Growth: A Case Report

      2024, Tokai Journal of Experimental and Clinical Medicine
    View all citing articles on Scopus

    Funding sources: None.

    Conflicts of interest: None identified.

    View full text