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

 
Abstract

66 yo female presented with the recent onset of asymptomatic red papules on her right breast developing over 1 week. Right sided breast cancer in 2005 treated with lumpectomy of the right breast, resection of right axillary lymph nodes, followed by radiation therapy and chemotherapy. A diagnosis of atypical vascular proliferation consistent with post-radiation atypical vascular lesion

Patient

66 yo female

Duration
2.5 months
Distribution
Right breast
History

66 yo female presented with the recent onset of asymptomatic red papules on her right breast developing over 1 week. She notes no change in any systemic symptoms.  She is currently taking rifampin, ethambutol, and azithromycin for pulmonary mycobacterium avium complex infection and recent culture positive mycobacterium abscessus. 

Past medical history includes left sided breast cancer in 1988 treated with lumpectomy of the left breast, resection of left axilary lymph nodes (all negative), and radiation therapy;

Right sided breast cancer in 2005 treated with lumpectomy of the right breast, resection of right axillary lymph nodes (with 13 positive nodes), radiation therapy (higher dose of  radiation given to right side in April 2006 than left side in 1988), and chemotherapy (docetaxel, doxorubicin, cyclophosphamide); nonischemic dilated cardiomyopathy (following chemotherapy).

Physical Examination

Initial presentation:
red and violaceous non blanching papules and macules on the Right breast with red patches on the right upper abdomen / right breast

2 months following initial visit:
violaceous non blanching papules with faint residual pigmentation of patch areas on right breast / right upper abdomen

Images

Laboratory Data

Hepatitis C, Hepatitis B, HIV - negative in 2010
RPR negative, ANA < 40 in 2008

Histopathology

2 biopsy sites showed similar processes.

Scanning view

x 200

x 400

Lesions are rather circumscribed but have dissecting periphery.  The atypia appears insufficient for diagnosis of well-differentiated angiosarcoma. Ki67 proliferative index is low.  Immunohistochemical stains show expression of CD31 but HHV-8 is not expressed.
Sections revealed an aggregate of arborizing, variably sized and dilated vascular channels lined by plump endothelials cells in the superficial dermis with extravasation of red blood cells.  In focal areas, vascular channels appear to dissect the surrounding slightly fibrotic collagen.  No apparent abnormal fibroblasts.

Diagnosis

Atypical vascular proliferation consistent with post-radiation atypical vascular lesion

Reason for presentation

Given a potential risk for malignant tranformation in atypical vascular lesions of the breast for a patient with multiple scattered papules across her right breast / chest:
Would imiquimod field therapy to the irradiated and involved area provide results and for what duration? 

Questions

Should remaining lesions be excised individually and with what margins?
Should imiquimod be utilized prior to further surgery or following further surgical interventions?
Would other medical therapies prevent possible progression of these lesions?

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

  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
    Abstract Full Text PDF (2MB)

  3. Lucas D. Angiosarcoma, Radiation-Associated Angiosarcoma, and
    Atypical Vascular Lesion Arch Pathol Lab Med. 2009;133:1804–1809
    Abstract 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.
    Abstract Full Text PDF(3 MB)
Keywords

atypical vascular lesions, breast cancer, angiosarcoma

Comments from Faculty and Members

Ron Yaar MD, PhD, Assistant Professor, Director of Dermatopathology Training, Boston Medical Centre, Department of Dermatology, Dermatopathology Section, Boston, MA, USA on October 26, 2010

We all agree that this looks like the entity described variably as atypical vascular lesion of irradiated skin or benign lymphangiomatous papules.

I've attached some literature I found regarding this diagnosis.    While relatively few cases have been described, it appears that the consensus is that these appear to behave in a benign fashion and do not represent precursors for angiosarcoma. (For further reference see presentation above )

Samuel Moschella MD, Professor of Dermatology, Emeritus, Harvard Medical School, Senior Consultant, Lahey Clinic, Burlington, MA, USA on October 28, 2010

I am aware of the disease and have heard discussions and the consensus about therapy was a trial with imiquimod (ALDARA).

Khaled El-hoshy M.D., Consultant Dermatologist, Troy, Michigan, USA on October 28, 2010

I would definitely re-biopsy another lesion in 1 month or so & keep a very close f/u. Angiosarcoma, or even metastatic breast CA, is a real possibility. I am not in favor of Imiquimod. I think it is a weak agent in such a case.

Bashir Zendah MD, Senior Registrar, Department of Dermatology, Tripoli Medical Centre, Tripoli, Libya on October 28, 2010

I agree with the opinion of Dr. Khaled because the spectrum of postradiation vascular lesions is wide and ranges from atypical vascular lesions with reportedly benign clinical behaviour to frank cutaneous angiosarcoma. There is, however, significant clinical as well as histological overlap. It is frequently difficult to classify these postradiation vascular lesions accurately and they create an emerging diagnostic and therapeutic challenge to both pathologists and clinicians. Experience with these vascular lesions is very limited.

Adel Aly MD, Dermatologist, Cairo, Egypt on October 29, 2010

I doubt imiquimod would work for a vascular lesion with malignant potential. From the review of literature it appears that cases developing into post-radiation angiosarcoma are older in age, having vascular type and larger size of the lesion (7.5 cm). Current recommendations include complete excision and f/u for any new lesion.

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