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Successful management of a retroperitoneal kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using
L Harper1, J L Michel, O Enjolras
1Department of Paediatric Surgery, Hôpital Félix Guyon, Saint-Denis de La Réunion, France. harper_luke@hotmail.com
Abstract:
It has been shown recently that Kasabach-Merritt phenomenon, the association of a vascular tumour and consumption coagulopathy, does not--as previously thought--complicate "classical" infantile hemangiomas but distinctive entities called kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), both tumours on the same neoplastic spectrum. These tumours have been found in the neck, face, thorax, abdomen, retroperitoneum and limbs and are associated with a mortality rate of as high as 30 %. Several therapeutic modalities, including alpha-interferon, vincristine, radiotherapy and surgery have been reported in the literature. We report a case of retroperitoneal kaposiform hemangioendothelioma regression using alpha-interferon and discuss the current knowledge of this entity and its treatment.
Insights
Kasabach-Merritt phenomenon is linked to kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), not infantile hemangiomas. Alpha-interferon showed promise in regressing a retroperitoneal KHE case.
Area of Science:
- Vascular oncology
- Pediatric hematology
- Coagulation disorders
Background:
- Kasabach-Merritt phenomenon (KMP) is a rare complication involving vascular tumors and coagulopathy.
- KMP is now understood to be associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), not infantile hemangiomas.
- KHE and TA are distinct vascular tumors on a neoplastic spectrum, occurring in various body locations.
Observation:
- A case of retroperitoneal kaposiform hemangioendothelioma presented with KMP.
- The patient received alpha-interferon therapy for the tumor.
- Tumor regression was observed following treatment with alpha-interferon.
Findings:
- Kaposiform hemangioendothelioma and tufted angioma are the primary entities associated with Kasabach-Merritt phenomenon.
- These vascular tumors can occur in diverse anatomical sites, including the retroperitoneum.
- Alpha-interferon demonstrated efficacy in inducing regression of a retroperitoneal KHE.
Implications:
- Reclassifies the association of KMP with specific vascular tumors, impacting diagnostic criteria.
- Highlights alpha-interferon as a potential therapeutic option for KHE and TA, especially in challenging locations.
- Emphasizes the need for further research into the pathogenesis and optimal management of KHE and TA.
