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Image of the Month—Diagnosis
Arch Surg. 2005;140:810.
Answer: Angiosarcoma of the Breast
At the time of mastectomy, a cutaneous nodule emerged (Figure 1). The magnetic resonance image shows enhancement of diffuse skin thickening with evidence of extension into the subareolar tissue (Figure 2). A total mastectomy with axillary lymph node dissection was performed (the latter was based on the erroneous preoperative diagnosis of ductal carcinoma). The final pathological results revealed poorly differentiated angiosarcoma both medial to the nipple and in the lower outer quadrant.
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Figure 1: Photograph of the left breast. Ruler units are centimeters.
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Figure 2: Gadolinium-enhanced magnetic resonance image of the left breast.
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Angiosarcoma of the breast that arises in the absence of known risk factors, termed primary angiosarcoma, represents less than 1% of all primary breast malignancies and 3% to 10% of all breast sarcomas.1 In general, the disease is considered to be rare.2 It is more prevalent in younger populations and is most often found in women during their third to fourth decade of life. Secondary angiosarcoma of the breast is more common and is associated with known risk factors including extremity edema and chronic lymphedema. Angiosarcoma after a breast-conserving procedure is increasingly diagnosed in a small but significant portion of breast carcinoma survivors.3 Recently, multiple studies have established an association between radiation therapy and breast angiosarcoma. Cozen et al4 reviewed 264 444 cases of women in Los Angeles County, California, with previous invasive ductal carcinoma and found a significantly increased risk of angiosarcoma of the upper extremity (odds ratio = 59) and of the breast or chest wall (odds ratio = 11.6). This increased risk is thought to be related to persistent lymphedema or the radiation therapy itself. In a review5 of 194 798 cases of breast cancer from the Surveillance, Epidemiology, and End Results Program data, patients who had undergone breast conservation and radiotherapy had a relative risk of 15.9 of developing angiosarcoma. Lesions tend to appear 4 to 7 years after therapy and may be related to persistent lymphedema.
Definitive diagnosis of angiosarcoma of the breast can be difficult. Mammography may show a nonspecific mass or thickening of the skin that may be confused with a benign lesion. In fact, approximately one third of patients with breast angiosarcoma have no abnormalities on their mammograms.6 Fine-needle aspiration may also have a low diagnostic yield because of limited specificity. In this case, the fine-needle aspiration revealed only nonspecific tumor cells. As a result of this diagnostic difficulty, magnetic resonance imaging is becoming an increasingly popular diagnostic tool. Magnetic resonance imaging often reveals low signal intensity on T1-weighted images but higher signal intensity on T2-weighted images. With the use of gadolinium, images of lesions are enhanced on low-signal T1 weighting and high-signal T2 weighting.7
Given the increasing use of radiation and breast-conserving therapy, physicians may see a rise in the incidence of secondary angiosarcoma of the breast. Furthermore, magnetic resonance imaging will likely prove useful in confirming suspicious lesions owing to the low yield of traditional imaging and the increasing incidence of angiosarcoma in these patients. However, the final diagnosis and treatment should be based on definitive pathological results. The recommended treatment for both primary and secondary angiosarcoma is simple mastectomy with an increasing role for adjuvant and neo-adjuvant therapy. High-grade angiosarcoma shows 5-year survival rates approaching 10% to 21%.8
AUTHOR INFORMATION
Correspondence: Stanley P. L. Leong, MD, Department of Surgery, University of California, San Francisco, Comprehensive Cancer Center at Mount Zion, 1600 Divisadero St, San Francisco, CA 94143-1674 (leongs{at}surgery.ucsf.edu).
Accepted for Publication: March 31, 2005.
| Submissions
The Editor welcomes contributions to the "Image of the Month." Send manuscripts to Archives of Surgery, Johns Hopkins Medical Institutions, 720 Rutland Ave, Ross 759, Baltimore, MD 21205; (443) 287-0026; e-mail: archsurg{at}jama-archives.org. Articles and photographs accepted will bear the contributor’s name. Manuscript criteria and information are per the "Instructions for Authors" for Archives of Surgery. No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.
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REFERENCES
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1. Wong RWY, Rappaport WD, Davis JR, Leong SPL. Primary angiosarcoma of the breast: a case report and review of the literature. Surg Rounds. 1996;19:454-458.
2. Georgiannos SN, Sheaff M. Angiosarcoma of the breast: a 30 year perspective with an optimistic outlook. Br J Plast Surg. 2003;56:129-134.
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3. Monroe AT, Feigenberg SJ, Mendenhall NP. Angiosarcoma after breast-conserving therapy. Cancer. 2003;97:1832-1840.
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4. Cozen W, Bernstein L, Wang F, Press MF, Mack TM. The risk of angiosarcoma following primary breast cancer. Br J Cancer. 1999;81:532-536.
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5. Huang J, Mackillop WJ. Increased risk of soft tissue sarcoma after radiotherapy in women with breast carcinoma. Cancer. 2001;92:172-180.
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6. Liberman L, Dershaw DD, Kaufman RJ, Rosen PP. Angiosarcoma of the breast. Radiology. 1992;183:649-654.
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7. Murakam S, Nagano H, Okubo K, et al. Angiosarcoma of the breast: report of a case and its findings of MRI. Breast Cancer. 2001;8:254-258.
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8. Rosen PP, Kimmel M, Ernsberger D. Mammary angiosarcoma. Cancer. 1988;62:2145-2151.
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SECTION EDITOR: GRACE S. ROZYCKI, MD
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