A multicenter study to further explore treatment of kaposiform hemangioendotheliomas has been proposed after untreated patients did not develop Kasabach Merritt Phenomenon.
A poster presented at the 2023 Society for Pediatric Dermatology Meeting in Asheville, North Carolina, identified and described a subset of patients with kaposiform hemangioendotheliomas (KHE)/Tufted angiomas that did not require medical or surgical intervention for treatment. The poster also compared clinical characteristics of those patients receiving treatment versus those who did not and sought to understand predictors for intervention.1
“Clinical Characteristics of KHE: To Treat or Not to Treat? A retrospective cohort study” consisted of 23 patients with KHE. Investigators followed the patients for an average of 6.8 years. Of the 23 patients, 7 did not receive any treatment.
The locally aggressive tumors can cause significant hematological/coagulopathic complications including Kasabach-Merritt phenomenon (KMP), mass effect, and residual skin changes. KHE also has the potential to create long-term chronic pain and to cause compression of vital structures.
Medical treatment can include sirolimus, vincristine, and oral steroids alone or combined. Surgical intervention or embolization can be helpful in some cases of KHE, although in milder cases—no intervention is necessary.
Of the 7 patients who did not receive treatment, none developed KMP, while 69% of the patients who were treated developed KMP. The untreated patients were managed with active observation. Patients who received treatment were managed with corticosteroids and vincristine, sirolimus, beta-blockers, aspirin, and/or embolization.
Outcomes for patients not treated were residual lipoatrophy and residual discoloration. Outcomes for those patients receiving treatment were residual discoloration, chronic pain, limb discrepancy, hypertrichosis, chronic swelling, and hyperhidrosis.
The authors identified a subset of patients “who had spontaneous involution without need for medical/surgical intervention.”1 The investigators propose a multicenter retrospective study to further understand these patients who had spontaneous involution without a need for intervention. The authors also seek to further explore cosmetic and functional outcomes.
Given the changes in medical treatment, they suggest that updating clinical guidelines in treating KHE/Tufted angioma may be a possibility.