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Residual lesions after Kasabach-Merritt phenomenon in 41 patients
O Enjolras1, J B Mulliken, M Wassef
1Consultation des Angiomes, Département de Neuroradiologie et Angiographie thérapeutique, Hôpital Lariboisière, Paris, France.
Background:
Kasabach-Merritt phenomenon (KMP) is the association of a vascular tumor and thrombocytopenic coagulopathy. Vascular tumors are either kaposiform hemangioendothelioma or tufted angioma but not "true" common hemangioma of infancy. There is a conspicuous absence in the literature regarding the late outcome and possible residual lesions after apparent clinical cure of KMP.
Objective:
The purpose of the study was to analyze these residua in a large number of patients.
Methods:
Clinical data on 41 patients who had KMP were accrued in an international cooperative study. The emphasis was on the residual lesions after resolution of the thrombocytopenia and other coagulation abnormalities. Imaging studies (follow-up magnetic resonance imaging studies available for 10 patients) and histologic specimens (30 specimens available for 26 patients, 18 biopsies done during the KMP and 12 concerning the sequelae) were reviewed.
Results:
Residual lesions after "cure" of KMP were common. They exhibited 3 clinical patterns: type I lesions (n = 28) showed a cutaneous red stain, with or without associated red papules. The stain might overlap a minor fibrotic infiltration or a significant poorly delineated diffuse fibrotic infiltration. These cutaneous vascular lesions varied in size and appearance over time and were occasionally painful. Type II lesions were telangiectatic streaks and swelling (n = 5), and type III lesions showed a minor, firm, irregular, subcutaneous mass assessed by palpation or deep infiltration evidenced by computed tomography or magnetic resonance imaging (n = 8). A fourth feature was sequelae in muscles and/or joints. Histologically, tufted angioma was more common in the specimens from residual lesions, whereas kaposiform hemangioendothelioma was more common during the active phase of KMP. Imaging findings were remarkably reproducible and revealed a persistent vascular tumor.
Conclusion:
Residua of tumors associated with KMP are common after the resolution of thrombocytopenia and coagulopathy. They are (more or less) prominent dormant vascular tumors, not "scars" and, clinically as well as histologically, they differ markedly from involuted hemangioma.
Insights
Residual lesions are common after Kasabach-Merritt phenomenon (KMP) is treated. These dormant vascular tumors, not scars, persist long-term and differ from involuted hemangiomas.
Area of Science:
- Vascular tumors
- Pediatric oncology
- Coagulopathy
Background:
- Kasabach-Merritt phenomenon (KMP) involves vascular tumors and thrombocytopenic coagulopathy.
- Commonly associated vascular tumors include kaposiform hemangioendothelioma and tufted angioma.
- Limited data exists on long-term outcomes and residual lesions post-KMP treatment.
Purpose of the Study:
- To analyze residual lesions in patients with a history of KMP.
- To characterize the nature and presentation of these late sequelae.
Main Methods:
- International cooperative study of 41 patients with KMP.
- Review of clinical data, focusing on residual lesions after coagulopathy resolution.
- Analysis of imaging studies (MRI) and histological specimens (biopsies).
Main Results:
- Residual vascular tumors are common after KMP resolution, presenting in three clinical patterns.
- Type I: cutaneous red stain; Type II: telangiectatic streaks; Type III: subcutaneous masses.
- Histology revealed tufted angioma more common in residuals, while kaposiform hemangioendothelioma predominated in the active phase. Imaging confirmed persistent vascular tumors.
Conclusions:
- Post-KMP, residual vascular tumors are frequent and represent dormant lesions, not scars.
- These residuals exhibit distinct clinical and histological features compared to involuted hemangiomas.
- Long-term follow-up is crucial for understanding the nature of these persistent vascular abnormalities.