Breast

Other benign tumors

Atypical vascular proliferation


Editorial Board Member: Julie M. Jorns, M.D.
Deputy Editor-in-Chief: Gary Tozbikian, M.D.
Indu Agarwal, M.D.
Luis Blanco, Jr., M.D.

Last author update: 9 February 2023
Last staff update: 9 February 2023

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PubMed Search: Atypical vascular proliferation

Indu Agarwal, M.D.
Luis Blanco, Jr., M.D.
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Cite this page: Agarwal I, Blanco L. Atypical vascular proliferation. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/breastatypicalvascular.html. Accessed April 16th, 2024.
Definition / general
  • Atypical vascular proliferations on irradiated skin, often small and multiple
Essential features
  • Superficial dermal based growth of narrow vascular spaces
  • Develops after external radiation for breast carcinoma
  • Typically involves the skin overlying breast
  • 2 subtypes: lymphatic type and vascular type
  • Differentiated from angiosarcoma by lack of multilayering, mitosis, marked cytologic atypia, blood lakes and absence of extension into subcutaneous tissue
Terminology
  • Atypical vascular lesion term coined by Fineberg and Rosen in 1994 (Am J Clin Pathol 1994;102:757)
  • Acceptable terminology: atypical vascular proliferation
ICD coding
  • ICD-O: 9126/0 - atypical vascular lesion
  • ICD-11: XH8KN7 - atypical vascular lesion
Epidemiology
Sites
  • Skin of breast or anterior chest wall exposed to previous radiation
Pathophysiology
  • Secondary to irradiation but precise pathogenesis is unknown
Etiology
Clinical features
Diagnosis
  • Requires correlation of clinical history and physical examination with microscopic features
  • Complete excision of the lesion is required for definitive diagnosis
Prognostic factors
Case reports
  • 41 and 61 year old women with small vascular lesions of the breast diagnosed by MRI guided vacuum assisted biopsy (Radiol Case Rep 2022;17:2492)
  • 43 year old woman presented with 29 atypical vascular lesions following breast conserving surgery and radiation (Cutis 2019;103:E22)
  • 49 year old woman presented with postradiation atypical vascular proliferation mimicking angiosarcoma 8 months following breast conserving therapy for breast carcinoma (J Clin Aesthet Dermatol 2011;4:47)
Treatment
Clinical images

Contributed by Anna Purdy, M.S.N.
Upper outer quadrant erythema

Upper outer quadrant erythema

Axillary papules

Axillary papules



Images hosted on other servers:
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Postmastectomy

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Left breast is erythematous with sclerotic tissue changes

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Papules and nodules around the mastectomy scar

Gross description
  • ≥ 1 circumscribed papules, bluish purple nodules, small vesicles or erythematous plaques, median of 0.5 cm
  • Tends to be well demarcated, symmetric and wedge shaped (Semin Diagn Pathol 2017;34:410)
Microscopic (histologic) description
  • Relatively well circumscribed lesion with a somewhat wedge shaped outline, limited to the dermis and not extending into subcutis
  • 2 histologic subtypes with overlapping histologic features (Am J Surg Pathol 2008;32:943):
    • Lymphatic subtype:
      • More common subtype
      • Irregularly shaped, thin walled vascular channels with a branching and anastomosing growth
      • Lymphatic valve-like structures may be evident
      • Vessels lined by single layer of endothelial cells without atypia
      • Hobnailing and nuclear hyperchromasia may be seen
    • Vascular subtype:
      • Thin capillary channels lined by flattened to hobnail endothelium, lacking lobular growth pattern
  • Individual lesions may show composite features of both subtypes
Microscopic (histologic) images

Contributed by Indu Agarwal, M.D., Sharlene See, M.D. and Julie M. Jorns, M.D.
Wedge shaped outline

Wedge shaped outline

Flat to plump endothelial cells

Flat to plump endothelial cells

Vascular channels

Vascular channels

Excision specimen

Excision specimen


Papule

Papule

Skin punch biopsy

Skin punch biopsy

Skin punch biopsy

Skin punch biopsy (CD31)

Skin punch biopsy

Skin punch biopsy (c-MYC)

Positive stains
Negative stains
Molecular / cytogenetics description
  • Unknown
Sample pathology report
  • Left breast skin, punch biopsy:
    • Atypical vascular lesion (see comment)
    • Comment: The lesion is comprised of a vascular proliferation dissecting the collagen in the superficial dermis. There is no significant nuclear pleomorphism or mitotic activity. An immunostain performed for c-MYC is negative in the lesion. Morphologic and immunohistochemical findings support the diagnosis.
Differential diagnosis
  • Angiosarcoma, well differentiated:
    • Features of angiosarcoma which are lacking in atypical vascular lesions:
      • Large (median size is 7.5 cm) infiltrative lesion with destruction of adnexa and extension into subcutaneous tissues, multilayering of endothelial cells, prominent nucleoli, mitoses, blood lakes and solid areas
      • Unlike secondary angiosarcomas, atypical vascular lesions lack MYC amplification (by FISH) and MYC overexpression by immunohistochemistry
    • However, angiosarcoma may be part of morphologic continuum with atypical vascular lesions and may need additional biopsies to distinguish these entities (J Am Acad Dermatol 2007;57:126)
  • Hemangioma of skin:
    • No history of radiation
    • Lesions consist of dilated blood vessels in skin, filled with blood
    • Vessels have rounded contours and are nonanastomosing
  • Benign lymphangioendothelioma (Am J Surg Pathol 2000;24:1047):
    • Rare lesion with no history of radiation
    • Head and neck are more common sites, followed by limbs and trunk
    • Skin lesions may be macular or papular and pigmented
    • Anastomosing dilated vessels lined by flattened endothelium are present in superficial dermis; spaces may be empty or contain blood
    • Cytologic atypia and mitoses are absent
Board review style question #1

A brownish papular lesion of skin on the breast is excised. Histopathologic examination shows that it is comprised of a wedge shaped, relatively well circumscribed vascular proliferation, dissecting the collagen in the superficial dermis. No significant nuclear pleomorphism or mitotic activity is noted. What is the most important information you need to make the diagnosis?

  1. Family history of cancer
  2. History of cancers of other sites
  3. History of chemotherapy
  4. History of radiation to the site
Board review style answer #1
D. History of radiation to the site. Atypical vascular lesions only occur in previously irradiated areas of the breast. The histologic description matches that of an atypical vascular lesion but to make a confident diagnosis, prior history of irradiation and histologic examination of the entire lesion is essential.

Comment Here

Reference: Atypical vascular proliferation
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