Treatment experience for different risk groups of Kaposiform hemangioendothelioma

BackgroundKaposiform hemangioendothelioma (KHE) is a rare vascular tumor with a high risk of mortality. Few studies with large samples of KHE have been reported. KHE may develop into the Kasabach–Merritt phenomenon (KMP), which is characterized by thrombocytopenia and consumptive coagulopathy. The features of severe symptomatic anemia and life-threatening low platelets make the management of KHE associated with KMP challenging.ObjectiveThe aim of this study was to examine the clinical characteristics of patients with KHE and discuss the treatment experience for different risk groups of KHE.MethodsThrough a retrospective review of 70 patients diagnosed with KHE between 2017 and 2022 in our center, we classify lesions into three clinicopathological stages based on the tumor involving depth, and divided the severity of KHE into three levels by estimating clinicopathological stages and severity of thrombocytopenia. Treatments of different severity groups were estimated with sufficient data.ResultsIn our cohort, 27% were neonates, and KHE lesion occurred at birth in 84% of patients. There was a slight male predominance (32 girls and 38 boys). Common clinical characteristics included associated coagulation disorder (100%), locally aggressive cutaneous blue–purple mass (89%), thrombocytopenia (78%), and local pain or joint dysfunction (20%). The lower extremities were the dominant location (35%), followed by the trunk (29%), the maxillofacial region and neck (24%), and the upper extremities (10%). Of the total cohort, 78% developed KMP; the median age at which thrombocytopenia occurred was 27.8 days. The median platelet count of patients who were associated with KMP was 24,000/µL in our cohort. Ninety-two percent of patients were given surgery treatment and 89% of these patients were given high-dose methylprednisolone (5-6 mg/kg daily) before surgery. In 55 patients with KMP, 36% were sensitive to high-dose corticosteroid therapy. Patients from the low-risk group (eight cases) underwent operation, all of whom recovered without recurrence after a maximum follow-up of 5 years. Out of 26 patients from the high-risk group, 25 underwent surgery treatment, with 1 case undergoing secondary surgery after recurrence and 1 case taking sirolimus. Out of 36 cases from the extremely high-risk group, 32 underwent surgery (including 2 cases who underwent external carotid artery ligation and catheterization), 3 of whom underwent secondary operation after recurrence, and the remaining 4 cases took medicine. The mean length of having sirolimus was 21 months; two cases stopped taking sirolimus due to severe pneumonia. Two cases died at 1 and 3 months after discharge.ConclusionsOur study describes the largest assessment of high-risk patients with KHE who have undergone an operation to date, with 5 years of follow-up to track recovery, which provides invaluable knowledge for the future treatment of patients with KHE and KMP from different risk groups: Early surgical intervention may be the most definitive treatment option for most patients with KHE; multimodality treatment is the best choice for the extremely high-risk group

Surgical treatment of a huge kaposiform hemangioendothelioma in the chest wall: A case study

Kaposiform hemangioendothelioma, a rare vascular pediatric tumor often associated with Kasabach–Merritt phenomenon, is characterized by severe thrombocytopenia and consumptive coagulopathy. Kaposiform hemangioendothelioma is a severe disease and may progress quickly, resulting in a high mortality. However, standard treatment regimens for Kasabach–Merritt phenomenon have not yet been established. We reported here an infant with a large congenital kaposiform hemangioendothelioma in his chest wall who responded extremely well to surgical excision

Clinical characteristics of infantile haemangioma in twins: a retrospective study

Abstract Background Infantile hemangioma is one of the most common benign soft tissue tumors in infants. The pathogenesis of infantile hemangioma remains unclear and twin studies regarding its incidence may help clarify disease pathogenesis. Thus, this study aimed to analyze the clinical characteristics of infantile hemangioma in twin patients and discuss its clinical incidence. Methods We retrospectively analyzed the data of 83 pairs of twins with infantile hemangioma admitted to the Guangdong Provincial Women and Children Hospital and Henan Provincial People’s Hospital between May 2016 and May 2022. Thirty-one pairs of twins among whom both developed infantile hemangioma and 52 pairs of twins among whom only one twin was affected were included. Analysis was performed using the Spearman correlation. Additionally, we analyzed the influence of factors such as sex, twin zygosity, preterm birth, birth weight, and assisted reproduction on the clinical characteristics of twins. Results We observed that disease occurrence in both twins correlated with assisted reproduction (χ2 = 13. 102, P  0.05) and total birth weight of twins (t=-3.369, P > 0.05) were not correlated with infantile hemangioma. However, among twins, the ones with lesser birth weight were more likely to develop infantile hemangioma. Conclusions The clinical characteristics of infantile hemangioma in twins were consistent with their epidemiological characteristics. Female sex, preterm birth, less birth weight, and assisted reproduction increased the probability of morbidity in both twins. Analysis of the characteristics of infantile hemangioma in twins may assist further research and clinical treatment

Surgical treatment of kaposiform hemangioendothelioma in the pelvic cavity, bladder and ureter

Kasabach-Merritt phenomenon (KMP) is a rare potentially life-threatening consumptive coagulopathy characterized by thrombocytopenia and hypofibrinogenemia occurring associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). KHE in the specific sites will also cause some non-hemorrhagic complications. We report a two-month old female infant with KHE in her pelvic cavity, bladder and ureter who responded well to surgical excision. Keywords: Kaposiform hemangioendothelioma, Kasabach-Merritt phenomenon, Pelviccavity, Bladder, Ureter, Surgical excision, Thrombocytopenia, Coagulopathy, Therap

Shape morphing of plastic films

Three-dimensional (3D) architectures have qualitatively expanded the functions of materials and flexible electronics. However, current fabrication techniques for devices constrain their substrates to 2D geometries and current post-shape transformation strategies are limited to heterogenous or responsive materials and are not amenable to free-standing inert plastic films such as polyethylene terephthalate (PET) and polyimide (PI), which are vital substrates for flexible electronics. Here, we realize the shape morphing of homogeneous plastic films for various free-standing 3D frameworks from their 2D precursors by introducing a general strategy based on programming the plastic strain in films under peeling. By modulating the peeling parameters, previously inaccessible free-standing 3D geometries ranging from millimeter to micrometer were predicted theoretically and obtained experimentally. This strategy is applicable to most materials capable of plastic deformation, including polymers, metals, and composite materials, and can even enable 4D transformation with responsive plastic films. Enhanced performance of 3D circuits and piezoelectric systems demonstrates the enormous potential of peeling-induced shape morphing for 3D devices.Agency for Science, Technology and Research (A*STAR)Ministry of Education (MOE)National Research Foundation (NRF)Published versionX.C. acknowledges the support from the National Research Foundation (NRF), Prime Minister’s Office, Singapore, under its Campus of Research Excellence and Technological Enterprise (CREATE), the Smart Grippers for Soft Robotics (SGSR) Programme, the Agency for Science, Technology and Research (A*STAR) Advanced Manufacturing and Engineering (AME) Programmatic Grant (No. A18A1b0045). S.W. and X.C. acknowledge the support from the International Partnership Program of Chinese Academy of Sciences (Grant No. 1A1111KYSB20200010). D.L. and H.G. acknowledge support from the Singapore Ministry of Educaton (MOE) AcRF Tier 1 (Grant RG120/21). H.G. acknowledges support as a Distinguished University Professorship from Nanyang Technological University and Scientific Directorship at Institute of High Performance Computing from the Agency for Science, Technology and Research (A*STAR)

Treatment experience for different risk groups of Kaposiform hemangioendothelioma

Peer reviewed: TrueBackgroundKaposiform hemangioendothelioma (KHE) is a rare vascular tumor with a high risk of mortality. Few studies with large samples of KHE have been reported. KHE may develop into the Kasabach–Merritt phenomenon (KMP), which is characterized by thrombocytopenia and consumptive coagulopathy. The features of severe symptomatic anemia and life-threatening low platelets make the management of KHE associated with KMP challenging.ObjectiveThe aim of this study was to examine the clinical characteristics of patients with KHE and discuss the treatment experience for different risk groups of KHE.MethodsThrough a retrospective review of 70 patients diagnosed with KHE between 2017 and 2022 in our center, we classify lesions into three clinicopathological stages based on the tumor involving depth, and divided the severity of KHE into three levels by estimating clinicopathological stages and severity of thrombocytopenia. Treatments of different severity groups were estimated with sufficient data.ResultsIn our cohort, 27% were neonates, and KHE lesion occurred at birth in 84% of patients. There was a slight male predominance (32 girls and 38 boys). Common clinical characteristics included associated coagulation disorder (100%), locally aggressive cutaneous blue–purple mass (89%), thrombocytopenia (78%), and local pain or joint dysfunction (20%). The lower extremities were the dominant location (35%), followed by the trunk (29%), the maxillofacial region and neck (24%), and the upper extremities (10%). Of the total cohort, 78% developed KMP; the median age at which thrombocytopenia occurred was 27.8 days. The median platelet count of patients who were associated with KMP was 24,000/µL in our cohort. Ninety-two percent of patients were given surgery treatment and 89% of these patients were given high-dose methylprednisolone (5-6 mg/kg daily) before surgery. In 55 patients with KMP, 36% were sensitive to high-dose corticosteroid therapy. Patients from the low-risk group (eight cases) underwent operation, all of whom recovered without recurrence after a maximum follow-up of 5 years. Out of 26 patients from the high-risk group, 25 underwent surgery treatment, with 1 case undergoing secondary surgery after recurrence and 1 case taking sirolimus. Out of 36 cases from the extremely high-risk group, 32 underwent surgery (including 2 cases who underwent external carotid artery ligation and catheterization), 3 of whom underwent secondary operation after recurrence, and the remaining 4 cases took medicine. The mean length of having sirolimus was 21 months; two cases stopped taking sirolimus due to severe pneumonia. Two cases died at 1 and 3 months after discharge.ConclusionsOur study describes the largest assessment of high-risk patients with KHE who have undergone an operation to date, with 5 years of follow-up to track recovery, which provides invaluable knowledge for the future treatment of patients with KHE and KMP from different risk groups: Early surgical intervention may be the most definitive treatment option for most patients with KHE; multimodality treatment is the best choice for the extremely high-risk group.</jats:sec