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Multiple Primary Atypical Vascular Lesions Occurring in the Same Breast

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Multiple Primary Atypical Vascular Lesions Occurring in the Same Breast CASE REPORT Multiple Primary Atypical Vascular Lesions Occurring in the Same Breast Joshua Mandrell, MD; Una Miniter, MD; Rama Vaitla, MD lymphangiomas, suggesting benign behavior.4-10 However, PRACTICE POINTS their identity as benign lesions has been a source of con- • Atypical vascular lesions (AVLs) of the breast troversy, with some investigators proposing that AVLs may can appear an average of 5 years following radia- be a precursor lesion to postirradiation angiosarcoma.2 tion therapy. Research has addressed if there are markers that can • Although the malignant potential of AVLs remains predict AVL types that are more likely to develop into debatable, excision generally is recommended, as angiosarcomas.1 Although most clinicians treat AVLs with lesions tend to recur. complete excision, there currently are no specific guide- lines to direct thiscopy practice. We report the case of a patient with a history of 1 AVL Atypical vascular lesions (AVLs) of the breast are rare cutaneous that was excised who developed 3 additional AVLs in the vascular proliferations that appear as flesh-colored or erythema- same breast over the course of 15 months. tous papules or macules in women who have undergone radiation treatment for breast carcinoma. These lesions can develop in the Casenot Report irradiated area up to 20 years after the radiation treatment but most A 55-year-old woman with a history of obesity, hyper- commonly occur within 3 to 6 years. The general consensus agrees on the benign nature of AVLs; however, their identity as benign tension, and infiltrating ductal carcinoma in situ of the lesions has been a source of controversy over the years, with Dosome right breast (grade 2, estrogen receptor and progesterone investigators proposing that AVLs may be a precursor lesion to post- receptor positive) underwent a right breast lumpectomy irradiation angiosarcomas. Currently, there are no specific guidelines and sentinel lymph node dissection. Three months later, to direct clinicians on the effective treatment of AVLs, but most AVLs she underwent re-excision for positive margins and are treated with total excision. This rare case describes the develop- started adjuvant hormonal therapy with tamoxifen. One ment of 4 AVLs within the same breast and stresses the relevance month later, she began external beam radiation therapy of the field effect in AVL development as well as the importance of field monitoring. and received a total dose of 6040 cGy over the course of Cutis. 2017;100:E13-E16. 9 weeks (34 total treatments). The patient presented to an outside dermatology CUTIS clinic 2 years after completing external beam radiation therapy for evaluation of a new pink nodule on the right typical vascular lesions (AVLs) of the breast are mid breast. The nodule was biopsied and discovered to be rare cutaneous vascular proliferations that pres - an AVL. Pathology showed an anastomosing proliferation A ent as erythematous, violaceous, or flesh-colored of thin-walled vascular channels mainly located in the papules, patches, or plaques in women who have under- superficial dermis with notable endothelial nuclear atypia gone radiation treatment for breast carcinoma.1,2 These and hyperchromasia. There were several tiny foci with the lesions most commonly develop in the irradiated area beginnings of multilayering with prominent endothelial within 3 to 6 years following radiation treatment.3 atypia (Figure 1). She underwent complete excision for Various terms have been used to describe AVLs in this AVL with negative margins. the literature, including atypical hemangiomas, benign Six months after the initial AVL diagnosis, she pre- lymphangiomatous papules, benign lymphangioendothelio- sented to our dermatology clinic with another asymptom- mas, lymphangioma circumscriptum, and acquired progressive atic red bump on the right breast. On physical examination, From the Division of Dermatology, Loyola University Medical Center, Maywood, Illinois. The authors report no conflict of interest. Correspondence: Joshua Mandrell, MD, Division of Dermatology, Loyola University Medical Center, 2160 S 1st Ave, Bldg 54, Room 101, Maywood, IL 60153 ([email protected]). WWW.CUTIS.COM VOL. 100 NO. 2 I AUGUST 2017 E13 Copyright Cutis 2017. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. ATYPICAL VASCULAR LESIONS FIGURE 1. Histopathology of an atypical vascular lesion demonstrated FIGURE 2. Linear scar at the 12-o’clock position denoted the first a relatively circumscribed, abnormal, anastomosing proliferation of excised atypical vascular lesion. Three atypical vascular lesions were thin-walled vascular channels located predominantly in the superficial subsequently found (red arrows). dermis with endothelial nuclear atypia and hyperchromasia; several tiny foci with the beginnings of multilayering with prominent endothelial atypia also were present (H&E, original magnification ×40). copy a 4-mm firm, erythematous, well-circumscribed papule was noted on the medial aspect of the right breast along with a similar-appearing 4-mm papule on the right lat- eral aspect of the right breast (Figure 2). The patient was unsure of the duration of the second lesion but felt that not it had been present at least as long as the other lesion. Both lesions clinically resembled typical capillary heman- giomas. A 6-mm punch biopsy of the right medial breast was performed and revealed enlarged vessels and capilDo- laries in the upper dermis lined by endothelial cells with focal prominent nuclei without necrosis, overt atypia, mitosis, or tufting (Figure 3). Immunostaining was posi- tive for CD34, factor VIII antigen, podoplanin (D2-40), FIGURE 3. Histopathology of an atypical vascular lesion demonstrated and CD31, and negative for cytokeratin 7 and pankeratin. enlarged vessels lined by endothelial cells in the upper dermis with This staining was compatible with a lymphatic-type AVL.1 focal prominent nuclei without necrosis, overt atypia, mitosis, or tufting A diagnosis of AVL was made and complete excision with (H&E, original magnification ×200). clear margins was performed.CUTIS At the time of this exci- sion, a biopsy of the right lateral breast was performed revealing thin-walled, dilated vascular channels in the proliferations that developed after radiation and che- superficial dermis with architecturally atypical angulated motherapy for breast cancer. They concluded that these outlines, mild endothelial nuclear atypia, and hyperchro- AVLs were benign lesions distinct from angiosarcomas.8 masia without endothelial multilayering. Clear margins However, further research has challenged the benign were noted on the biopsy, but the patient subsequently nature of AVLs. In 2005, Brenn and Fletcher2 studied declined re-excision of this third AVL. 42 women diagnosed with either angiosarcoma or atypi- During a subsequent follow-up visit 9 months later, cal radiation-associated cutaneous vascular lesions. They the patient was noted to have a 2-mm red, vascular- suggested that AVLs resided on the same spectrum as appearing papule on the right upper medial breast angiosarcomas and that AVLs may be precursor lesions (Figure 2). A 6-mm biopsy was performed and revealed to angiosarcomas.2 Furthermore, Hildebrandt et al11 in thin-walled vascular channels in the superficial dermis 2001 and Di Tommaso and Fabbri12 in 2003 published case with endothelial nuclear atypia consistent with an AVL. reports of individual patients who developed an angiosar- coma from a preexisting AVL. Comment The controversy continued when Patton et al1 pub- Fineberg and Rosen8 were the first to describe AVLs in lished a study in 2008 in which 32 cases of AVLs were their 1994 study of 4 women with cutaneous vascular reviewed. In this study, 2 histologic types of AVLs were E14 I CUTIS® WWW.CUTIS.COM Copyright Cutis 2017. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. ATYPICAL VASCULAR LESIONS Comparison of AVLs and Radiation-Associated Angiosarcomas Lesion Clinical Presentation Histology AVL1,17 Latency period, 3–6 y; Wedge-shaped, well-demarcated proliferation of dilated thin- erythematous or flesh-colored walled, interanastomosing channels without hemorrhage lined papules, patches, or plaques by attenuated endothelial cells without atypia limited to the in irradiated skin; mean lesion superficial to mid dermis; lymphatic (CD31+, D2-40+, CD34+/–) size, 0.5 cm or vascular (CD31+, CD 34+, D2-40–) staining pattern Radiation-associated Latency period, 5–7 y; Irregular interanastomosing channels that infiltrate into angiosarcoma17,18 erythematous plaques, patches, subcutaneous tissue with nuclear atypia, multilayering or nodules; +/– edema in of endothelial cells, large nucleoli, frequent mitoses, and irradiated skin; mean lesion perilesional hemorrhage; CD34+, CD31+, factor VIII +/– size, 7.5 cm Abbreviation: AVL, atypical vascular lesion. described: vascular type and lymphatic type. Vascular- hemorrhage, mitoses, and notable aytpia. Atypical vas- type AVLs are characterized by irregularly dispersed, cular lesions lack these features and tend to be wedge pericyte-invested, capillary-sized vessels within the pap- shaped and display chronic inflammation.8,15,17-19 Atypical illary or reticular dermis that often are associated with vascular lesions copyshow superficial localized growth with- extravasated erythrocytes or hemosiderin. On the other out destruction of adjacent
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