Abstract
Background and purpose
Atypical vascular lesion (AVL) became a separate WHO diagnosis in November 2019. Due
to a possible risk of developing angiosarcoma, extensive surgery with excision of
AVL has been recommended but the benefit from this is questionable. We investigated
whether the change in WHO classification has led to an increase in the number of patients
diagnosed with AVL, thereby leading to an increase in extensive surgery.
Method
The Danish National Pathology Databank was used to identify patients diagnosed with
AVL between June 1, 2010 to June 31, 2020. The rate of AVL diagnosed before and after
change in WHO classification was compared.
Results
In total, 13 cases of AVL were identified, 3 cases diagnosed before changes in WHO
classification corresponding to 0.025 cases per month, compared to 8 cases, 1.143
cases per month, after the change in WHO classification. This corresponded to a 45-fold
increase (95%CI: 10.88-265,31) (P < .0001) in AVL diagnosis. The mean patient age at diagnosis was 67 years. Patients
received treatment varying from yearly follow up to extensive surgery. Non developed
angiosarcoma in the follow-up period of 22 months.
Conclusion
The changes in WHO classification of AVL has led to a considerable increase in the
number of patients diagnosed with the lesion. No standardized treatment exists for
this rare condition, but extensive surgery is often recommended to this frail population
despite the lack of evidence for prognostic benefit from the procedure. Prospective
follow-up studies are needed to determine the optimal treatment strategy.
Keywords
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Article info
Publication history
Published online: August 28, 2022
Accepted:
August 21,
2022
Received in revised form:
July 18,
2022
Received:
April 8,
2022
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
