Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with rationally designed and low-intensity combination therapy of sirolimus and aspirin.
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Abdominal kaposiform hemangioendothelioma presenting as volvulus and recurring intestinal obstruction in a neonate: a case report.
J Med Case Rep
December 2024
Department of Surgery, Aga Khan University, Karachi, Pakistan.
Background: Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.
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Hematology Am Soc Hematol Educ Program
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Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences, Little Rock, AR.
The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP).
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Article Synopsis
- The understanding of vascular anomalies has progressed since the original classification by Mulliken and Glowacki, with current research focusing on distinguishing vascular tumors from malformations.
- Recent advancements in molecular diagnostics have improved the identification and management of complex vascular lesions, including Kaposiform hemangioendothelioma and intramuscular fast-flow anomalies.
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Diagnosis and differentiation of Kaposiform hemangioendothelioma based on ultrasound radiomics.
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Department of Ultrasound, Henan Provincial People's Hospital, Zhengzhou University People's Hospital Zhengzhou 450003, Henan, China.
Kaposiform hemangioendothelioma (KHE) is a rare neoplasm of the newborn, but has a very high mortality rate. In this study, we explore the application value of ultrasound radiomics in the differential diagnosis of KHE so as to provide reference for early diagnosis of KHE. We selected 194 cases of children with suspected KHE admitted to Henan Provincial People's Hospital from March 2016 to April 2024 for this retrospective analysis.
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University of California San Diego School of Medicine, La Jolla, California, USA.
Kaposiform hemangioendothelioma (KHE) is a vascular neoplasm characterized by abnormal angiogenesis and lymphangiogenesis. Here, we present a case of a 19-month-old male with KHE of the right leg with bony involvement who was initially misdiagnosed with infantile hemangioma. Due to its heterogeneous presentation and frequent occurrence of comorbidities such as Kasabach-Merritt phenomenon, clinical and pathological correlation is essential for diagnosis of KHE.
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