#dermpathJC March 2020:

Thursday, March 26th, 9 pm EST

Article discussed: Angiosarcoma, Radiation-Associated Angiosarcoma, and Atypical Vascular Lesion

Authors: David R. Lucas

Open access courtesy of Archives of Pathology and Laboratory Medicine: https://www.archivesofpathology.org/doi/pdf/10.1043/1543-2165-133.11.1804

Summary prepared by: Cacey Peters, M.D. (@caceypeters)

 

Article Abstract:

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.

 

Journal Club Summary:

Angiosarcoma general points:

• 1% of soft tissue sarcomas
• Associated with radiation therapy, most often breast cancer
  • Also associated with chronic lymphedema, toxins (i.e. vinyl chloride), and foreign bodies (i.e. A/V fistulas)
• Usually presents as sporadic cutaneous tumor on scalp/face of elderly
• Reported in every anatomic site
• Bruise-like area that ulcerates or becomes nodular
• Often have smaller satellite lesions peripherally
• Clinically aggressive with high morbidity, regardless of grade
  • Prognosis in sporadic cutaneous angiosarcoma correlates with high- and low- risk groups on the basis of age, epitheliod histology, necrosis, and tumor depth
• Deep tumors can be hemorrhagic spongelike or microcystic with necrosis
• Well-differentiated lesions can be deceptively bland, but will have some combination of hyperchromasia, mild pleomorphism, prominent nucleoli, mitotic figures, multilayering, and/or dermal collagen entrapment forming intraluminal papillary structures
• Surgical margins may be obscured by atypical lymphatic/capillary proliferations
• Moderate/poorly differentiated types will often have heterogeneous cytoarchitectural features
  • Some combination of epithelioid, spindled, or pleomorphic cytology
  • Some combination of vasoformative, sieve-like, kaposiform, or solid architecture
• Undifferentiated angiosarcoma will need immunohistochemistry to confirm the diagnosis
  • CD31 = single best marker with high sensitivity and specificity
    • Diffuse, intense staining with membranous accentuation
    • Can be hard to interpret due to background blush, staining macrophages, and low-level expression in other tumor types
  • CD 34 and factor VIII = present in most angiosarcomas but not reliably in poorly differentiated
  • Cytokeratin pitfall = found in 35% of cases in one study (especially epithelioid angiosarcoma) although usually focal

 

Radiation-associated angiosarcoma

• Clinically and morphologically similar to sporadic angiosarcoma
  • Latency is shorter for breast angiosarcoma
    • 5-7 years on average
    • Significant occurrence in less than 3 years which questions the 3-year rule
  • More often cutaneous than sporadic angiosarcoma
    • Erythematous plaque/patch/nodules with edema
    • Diffuse, multifocal, and extensive involvement of the breast is common
    • Median size = 7.5 cm

Atypical Vascular Lesion (AVL):

• Benign course; can recur; rarely progress to angiosarcoma
• Tendency to develop further lesions, commonly AVLs
• Present as 1 or more small, flesh-colored papules or erythematous patches arising in radiated skin
• Histologically difficult to distinguish from well-differentiated angiosarcoma in a biopsy
• Often resemble lymphangiomas
  • Well-demarcated proliferation of thin walled, dilated, interanastomosing channels without erythrocytes
  • Lined by attenuated or hobnail endothelial cells without atypia
• Can resemble lymphangioendothelioma, lymphangioma circumscriptum, or both within the same lesion
• Most are limited to the superficial and mid dermis
• Current recommendations are to completely excise and follow for recurrence

Vascular-type AVL

• First described in 2005 with 3 cases that resemble lobular capillary hemangioma in mammary skin after radiation
  • All 3 cases developed angiosarcoma
• In 2008, 8 more cases were described where only one developed angiosarcoma

It is suggested that vascular-type AVL is more likely to progress to angiosarcoma than the more common lymphatic-type AVL

Memorable tweets:

 

Stay safe my friends, and we will see you next month for another dermpath journal club,

 

With love,

DermpathJC