Case Reports

Multiple Primary Atypical Vascular Lesions Occurring in the Same Breast

Cutis. 2017 August;100(2):E13-E16

References

Patton KT, Deyrup AT, Weiss SW. Atypical vascular lesions after surgery and radiation of the breast: a clinicopathologic study of 32 cases analyzing histologic heterogeneity and association with angiosarcoma. Am J Surg Pathol. 2008;32:943-950.
Brenn T, Fletcher CD. Radiation-associated cutaneous atypical vascular lesions and angiosarcoma: clinicopathologic analysis of 42 cases. Am J Surg Pathol. 2005;29:983-996.
Gengler C, Coindre JM, Leroux A, et al. 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;109:1584-1598.
Hoda SA, Cranor ML, Rosen PP. Hemangiomas of the breast with atypical histological features: further analysis of histological subtypes confirming their benign character. Am J Surg Pathol. 1992;16:553-560.
Wagamon K, Ranchoff RE, Rosenberg AS, et al. Benign lymphangiomatous papules of the skin. J Am Acad Dermatol. 2005;52:912-913.
Diaz-Cascajo C, Borghi S, Weyers W, et al. Benign lymphangiomatous papules of the skin following radiotherapy: a report of five new cases and review of the literature. Histopathology. 1999;35:319-327.
Martín-González T, Sanz-Trelles A, Del Boz J, et al. Benign lymphangiomatous papules and plaques after radiotherapy [in Spanish]. Actas Dermosifiliogr. 2008;99:84-86.
Fineberg S, Rosen PP. Cutaneous angiosarcoma and atypical vascular lesions of the skin and breast after radiation therapy for breast carcinoma. Am J Clin Pathol. 1994;102:757-763.
Guillou L, Fletcher CD. Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi’s sarcoma: clinicopathologic analysis of a series. Am J Surg Pathol. 2000;24:1047-1057.
Rosso R, Gianelli U, Carnevali L. Acquired progressive lymphangioma of the skin following radiotherapy for breast carcinoma. J Cutan Pathol. 1995;22:164-167.
Hildebrandt G, Mittag M, Gutz U, et al. Cutaneous breast angiosarcoma after conservative treatment of breast cancer. Eur J Dermatol. 2001;11:580-583.
Di Tommaso L, Fabbri A. Cutaneous angiosarcoma arising after radiotherapy treatment of a breast carcinoma: description of a case and review of the literature [in Italian]. Pathologica. 2003;95:196-202.
Santi R, Cetica V, Franchi A, et al. Tumour suppressor gene TP53 mutations in atypical vascular lesions of breast skin following radiotherapy. Histopathology. 2011;58:455-466.
Requena L, Kutzner H, Mentzel T, et al. Benign vascular proliferations in irradiated skin. Am J Surg Pathol. 2002;26:328-337.
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Brenn T, Fletcher CD. Postradiation vascular proliferations: an increasing problem. Histopathology. 2006;48:106-114.
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Bodet D, Rodríguez-Cano L, Bartralot R, et al. Benign lymphangiomatous papules of the skin associated with ovarian fibroma. J Am Acad Dermatol. 2007;56(2 suppl):S41-S44.
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Comment

Fineberg and Rosen⁸ were the first to describe AVLs in their 1994 study of 4 women with cutaneous vascular proliferations that developed after radiation and chemotherapy for breast cancer. They concluded that these AVLs were benign lesions distinct from angiosarcomas.⁸ However, further research has challenged the benign nature of AVLs. In 2005, Brenn and Fletcher² studied 42 women diagnosed with either angiosarcoma or atypical radiation-associated cutaneous vascular lesions. They suggested that AVLs resided on the same spectrum as angiosarcomas and that AVLs may be precursor lesions to angiosarcomas.² Furthermore, Hildebrandt et al¹¹ in 2001 and Di Tommaso and Fabbri¹² in 2003 published case reports of individual patients who developed an angiosarcoma from a preexisting AVL.

The controversy continued when Patton et al¹ published a study in 2008 in which 32 cases of AVLs were reviewed. In this study, 2 histologic types of AVLs were described: vascular type and lymphatic type. Vascular-type AVLs are characterized by irregularly dispersed, pericyte-invested, capillary-sized vessels within the papillary or reticular dermis that often are associated with extravasated erythrocytes or hemosiderin. On the other hand, lymphatic-type AVLs display thin-walled, variably anastomosing, lymphatic vessels lined by attenuated or slightly protuberant endothelial cells. These subtypes have been suggested based on the antigens known to be present in certain tissues, specifically vascular and lymphatic tissue. Despite these seemingly distinct histologies, 6 lesions classified as vascular type displayed some histologic overlap with the lymphatic-type AVLs. The authors concluded that the vascular type showed greater potential to develop into an angiosarcoma based on the degree of endothelial atypia.¹

In 2011, Santi et al¹³ found that both AVLs and angiosarcomas share inactivation mutations in the tumor suppressor gene TP53, providing further evidence to suggest that AVLs may be precursors to angiosarcomas.

Although the malignant potential of AVLs remains questionable, research has shown that they do have a propensity to recur.³ In 2007, Gengler et al³ determined that 20% of patients with AVLs experienced recurrence after a biopsy or excision with varying margins; however, the group stated that these new vascular lesions might not be recurrences but rather entirely new lesions in the same irradiated field (field-effect phenomenon). Several other studies demonstrated that more than 30% of patients with 1 AVL developed more lesions within the same irradiated area.^3,14-16 Despite the high rate of recurrence documented in the literature, only 5 of more than 100 diagnosed AVLs have progressed to angiosarcoma.^1,3

Many differences can be noted when comparing the histology of AVLs versus angiosarcomas, though some are subtle (Table). Angiosarcomas display poorly circumscribed vascular infiltration into the subcutaneous tissue, multilayering of endothelial cells, prominent nucleoli, hemorrhage, mitoses, and notable aytpia. Atypical vascular lesions lack these features and tend to be wedge shaped and display chronic inflammation.^8,15,17-19 Atypical vascular lesions show superficial localized growth without destruction of adjacent adnexa, display dilated vascular spaces, and exhibit large endothelial cells.^{5,6,8,14,15,19,20} However, there is overlap between AVLs and angiosarcomas that can make diagnosis difficult.^{2,14,16,17,19} Areas within or just outside of an angiosarcoma, especially in well-differentiated angiosarcomas, can appear histologically identical to AVLs, and multiple biopsies may be required for diagnosis.^17,19,21

Conclusion

More research is needed in the arenas of classification, diagnosis, treatment, and follow-up recommendations for AVLs. In particular, more specific histologic markers may be needed to identify those AVLs that may progress to angiosarcomas. Although most AVLs are treated with excision, a consensus needs to be reached on adequate surgical margins. Lastly, due to the tendency of AVLs to recur coupled with their unknown malignant potential, recommendations are needed for consistent follow-up examinations.

Multiple Primary Atypical Vascular Lesions Occurring in the Same Breast

References

Comment

Conclusion

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