Atypical vascular lesions of the breast

following breast cancer

presented by

Trevor Thompson MD, (1)

Lihue, Hawaii, USA

Ron Yaar MD, PhD, (2)

Boston, MA, USA

October 26, 2010

(1) Dermatologist, Kauai Medical Group, Lihue, Kauai, HI, USA
(2) Assistant Professor, Director of Dermatopathology Training, Boston Medical Centre, Boston, MA, USA


  1. Mandrell J, Mehta S, McClure S. Atypical vascular lesion of the breast. J Am Acad Dermatol Aug 2010 Aug; 63(2):337-340

    Atypical vascular lesions (AVLs) are vascular proliferations that develop after surgery and radiation for breast carcinoma and may represent precursors to angiosarcoma. AVLs are not well-known entities and currently lack official prognostic factors and guidelines for surgical treatment.
    We report the case of a patient who developed an AVL, vascular type, 4 years after lumpectomy and radiation therapy for ductal carcinoma in situ of the breast. The patient underwent wide local excision with 1-cm margins with subsequent pathologic examination confirming complete excision of the residual atypical vascular proliferation. This case highlights the importance of close cutaneous surveillance in patients with a history of surgery and radiation for breast carcinoma, and a low threshold for biopsy. More studies are needed to further delineate the risk of AVLs progressing to angiosarcoma and to identify histologic features or immunophenotypic markers, which may be predictive of this risk. Furthermore, formal treatment recommendations for these enigmatic entities would be helpful. Full Text PDF (1MB)

  2. Gengler C, Coindre JM, Leroux A, Trassard M, Ranchère-Vince D, Valo I, Michels JJ, Guillou L. Vascular proliferations of the skin after radiation therapy for breast cancer: clinicopathologic analysis of a series in favor of a benign process: a study from the French Sarcoma Group. Cancer. 2007 Apr 15;109(8):1584-98
    Department of Pathology, University Institute of Pathology, Lausanne, Switzerland.

    BACKGROUND: Cutaneous vascular proliferations that occur in the field of prior radiotherapy include angiosarcoma and small, cutaneous lesions with a pseudosarcomatous pattern that previously were reported as atypical vascular lesions or benign lymphangiomatous papules.
    METHODS: The objective of this study was to investigate the clinicopathologic features and outcomes of 56 radiation-induced vascular proliferations that occurred in 36 patients who received previous treatment for breast carcinoma. Data from all patients were retrieved from the files of the French Sarcoma Group. Immunostaining with D2.40 antibody was performed in 24 lesions.
    RESULTS: All patients (median age, 52 years) had received external radiotherapy. Small papules developed within the field of prior radiotherapy (median latency interval, 66 months). Microscopically, the lesions were relatively well circumscribed, and they were located mostly in the superficial/middermis. They were composed of dilated or irregular-jagged vascular channels that were lined by a single layer of bland endothelial cells, and they demonstrated either a predominately lymphangioendothelioma-like or lymphangioma/lymphangioma circumscriptum-like growth pattern. Micropapillary tufts were common findings. Ten lesions showed additional cytologic and/or architectural atypia. Twenty of 24 lesions showed D2.40 positivity. Follow-up information was available for 31 patients (median follow-up, 48 months): Five women developed new cutaneous lesions, and 1 woman had spontaneous regression of her lesions. None of the patients developed cutaneous angiosarcoma. Five patients were lost to follow-up.
    CONCLUSIONS: Although vascular proliferations in irradiated skin may mimic angiosarcoma morphologically, the large majority of these lesions showed a benign clinical outcome. Despite relatively limited follow-up, the current results indicate the benign nature of these vascular proliferations. Full Text PDF (2MB)

  3. Lucas D. Angiosarcoma, Radiation-Associated Angiosarcoma, and
    Atypical Vascular Lesion Arch Pathol Lab Med. 2009;133:1804–1809

    Angiosarcoma, one of the least common sarcomas, has become increasingly important because of its association with radiation therapy, especially for breast cancer. Most are sporadic, presenting as cutaneous tumors in the scalp/ face of elderly patients. However, angiosarcoma has a wide anatomic distribution including soft tissue, visceral organ, and osseous locations. Predisposing conditions include environmental exposures to chemical or radioactive sources. Radiation-associated angiosarcoma typically presents as a cutaneous tumor several years posttherapy. The latency for radiation-associated mammary angiosarcoma is relatively short, sometimes less than 3 years. Atypical vascular lesion refers to a small, usually lymphatic- type vascular proliferation in radiated skin. Although most atypical vascular lesions pursue a benign course, they recur and very rarely progress to angiosarcoma. Distinguishing
    this lesion from well-differentiated angiosarcoma in a biopsy can be challenging, especially because areas indistinguishable from atypical vascular lesion are found adjacent to angiosarcoma. Recently, vascular-type atypical vascular lesion, which resembles hemangioma, has been described, thus expanding the definition of this entity. (Arch Pathol Lab Med. 2009;133:1804–1809)
    Full Text PDF (6MB)

  4. Luis Requena, Heinz Kutzner, Thomas Mentzel, Rafael Durán, and José Luis Rodríguez-Peralto. Benign Vascular Proliferations in Irradiated Skin.
    Am J Surg Pathol 26(3): 328–337, 2002

    Several types of cutaneous vascular proliferations have been described in areas of irradiated skin, including both benign lesions, such as benign lymphangiomatous papules, atypical vascular lesions, or benign lymphangioendothelioma, and malignant
    neoplasms such as high-grade angiosarcomas. This report describes the clinicopathologic features of 15 cases of different types of benign cutaneous vascular proliferations arisen within irradiated skin. All patients were female ranging in age from 33 to 72 years, and they had received postoperative external radiotherapy for treatment of breast carcinoma (14 cases) or ovarian carcinoma (one case). In those cases in which the latency interval period between radiotherapy and the development
    of the vascular lesion was known from the clinical records, the latency interval period elapsed between radiotherapy and diagnosis of the vascular lesion ranged from 3 to
    20 years. The most common clinical presentation of the cutaneous lesions consisted of papules, small vesicles, or erythematous plaques on the irradiated field. Histopathologically, most lesions consisted of irregular dilated vascular spaces, with a
    branching and anastomosing pattern, thin walls, and lymphatic appearance involving the superficial dermis. A discontinuous single layer of endothelial cells with flattened nuclei lined these vascular channels, and numerous small stromal papillary formations
    also lined by endothelial cells projected into the lumina of the dilated lymphatic vessels. These cases were classified as benign lymphangiomatous papules or plaques. Two cases showed different histopathologic findings because they consisted of poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis and lined by inconspicuous endothelial cells. In some areas these irregular slit-like vascular spaces dissected collagen bundles of the dermis. These cases were classified as atypical vascular proliferations mimicking benign lymphangioendothelioma or patchstage Kaposi’s sarcoma. All cases showed similar immunohistochemical findings and the endothelial cells lining the vascular spaces expressed immunoreactivity for CD31, but they stained only focally positive for CD34 or were negative for this marker. Immunohistochemical investigations for -smooth muscle actin failed to demonstrate a complete peripheral ring of actinpositive
    pericytes in most of the neoformed vascular structures. This immunohistochemical profile also supported the lymphatic nature of these vascular proliferations developed in irradiated skin. Although some of these lesions may mimic histopathologically
    patch-stage Kaposi’s sarcoma or welldifferentiated angiosarcoma, the follow-up of the patients of this series demonstrated that the vascular proliferations arisen in irradiated skin invariably showed a benign biologic behavior.
    Am J Surg Pathol 26(3): 328–337, 2002. Full Text PDF (3MB)