Hemangioendothelioma Research Articles

Analysis of ultrasound and magnetic resonance imaging characteristics of kaposiform hemangioen dothelioma

ObjectiveThe present study aims to investigate the ultrasound and magnetic resonance imaging (MRI) characteristics of kaposiform hemangioen dothelioma (KHE).MethodsA retrospective analysis was conducted on the clinical data of children diagnosed with KHE through postoperative pathology. Patients were divided into two groups: the KHE group and the KHE with Kasabach-Merritt Phenomenon (KMP) group (KMP group). Laboratory indicators, ultrasound, and MRI data were collected and analyzed statistically to summarize the imaging characteristics of the disease.ResultsThe levels of platelets and fibrinogen in the KHE group were significantly higher than those in the KMP group, while D-dimer levels, prothrombin time, and activated partial thromboplastin time were lower (P < 0.05). Ultrasound characteristics comparison revealed that lesions extending to the fat layer (42.47% vs. 54.24%) and invading the muscle layer (38.36% vs. 69.49%) were less common in the KHE group compared to the KMP group, with the lesion diameter being smaller in the KHE group (P < 0.05). The Adler grading predominantly showed Grade II (45.21%) in the KHE group, whereas Grade III (93.22%) was more prevalent in the KMP group (P < 0.05). MRI analysis indicated that the incidence of lesions invading the muscle layer and the presence of flow voids were lower in the KHE group compared to the KMP group (P < 0.05).ConclusionKHE patients with KMP exhibit lesions that are more prone to extending into the fat layer and invading the muscle layer, with larger diameters and abundant blood flow. Additionally, the MRI images of the lesions may exhibit flow voids.

Vascular Anomalies Review of the Head and Neck for Physicians in Training

A basic understanding of vascular anomalies will aid physicians-in-training as they seek to properly diagnose and determine interventions for these patient presentations. The aim of this review is to create a resource for physicians in training that encompasses the most important clinical aspects of vascular anomalies. Vascular anomalies of the head and neck are divided into two categories: vascular tumors and vascular malformations. This review will first describe vascular tumors followed by vascular malformations. This review discusses major pathology found in both categories of vascular anomaly. Vascular anomalies are frequently found in pediatric populations and can persist into adulthood, making it important for trainees to identify them on physical exam. This developing field seeks to improve form, function, and quality of life for patients with vascular anomalies and often requires a multidisciplinary approach (i.e., otolaryngology, dermatology, genetics, plastic surgery, interventional radiology). Various medical and surgical treatment options are available. A basic knowledge of these anomalies will allow for accurate, early diagnosis and appropriate intervention which can ultimately improve patient outcomes. Search Strategy and Selection Criteria: The MEDLINE/PubMed database was searched for primary research and reviews discussing various vascular anomalies which include infantile hemangioma, congenital hemangioma, pyogenic granuloma, tufted angioma, kaposiform hemangioendothelioma, capillary malformations, lymphatic malformations, venous malformations, and arteriovenous malformations. This search was conducted from 7/5/2023 to 3/21/2024.

Kaposiform haemangioendothelioma: ultrasonographic features and risk factors for the Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor with high morbidity and mortality. The aim of this study was to evaluate ultrasonographic findings associated with KHE.The clinical and ultrasonographic findings of a cohort of 64 cases with pathologically proven KHE were retrospectively reviewed and analyzed between November 2014 and February 2021. Two subtypes were divided according to the presence or absence of the Kasabach-Merritt phenomenon (KMP). The KMP risk factors in patients with KHE were analyzed statistically.Among the 64 cases of KHE, 43 (67.2%) were accompanied by KMP. There was a positive correlation between the appearance of KMP and tumor size. KHEs had an increased risk of developing KMP if the lesions measured were >6 cm and if they belonged to the deep or mixed subtype. On ultrasonography, all KHE lesions were heterogeneous, and 81.3% were hypoechoic; 93.8% of KHEs exhibited ill-defined margins, 68.7% had strands branching into the adjacent tissue, and 84.4% presented marked hypervascularity. Elastography showed that central hypoechogenic lesion areas were hard, and surrounding hyperechogenic lesion areas were soft.KHEs can occur in different parts of childrens' bodies. On ultrasonography, the main findings are heterogeneous low erosions, indistinct margins, branching strangulation into adjacent tissues, and obvious hypervascularity. Patients with lesions larger than 6 cm or belonging to deep or mixed subtypes (musculoskeletal infiltrates) are at risk for developing KMP, and clinicians should be vigilant.

Papillary Intralymphatic Angioendothelioma of the Spleen: A Rare Lymphatic Tumour in the Context of a &amp;lt;I&amp;gt;PIK3CA &amp;lt;/I&amp;gt;Mutation

Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasising lymphatic tumour that typically arises within the skin and soft tissues. We report a case of PILA arising within the spleen of a 6-year-old boy with megacephaly-capillary malformation (MCAP/MC-M) syndrome. MCAP/MC-M represents part of the phosphatidylinositol-4,5-biphosphate 3 kinase catalytic subunit alpha (&amp;lt;I&amp;gt;PIK3CA) &amp;lt;/I&amp;gt;related overgrowth spectrum (PROS). This is the 6&amp;lt;sup&amp;gt;th&amp;lt;/sup&amp;gt; reported case in the literature of a splenic PILA and the 2&amp;lt;sup&amp;gt;nd&amp;lt;/sup&amp;gt; known to have occurred in the context of a confirmed &amp;lt;I&amp;gt;PIK3CA &amp;lt;/I&amp;gt;mutation. In reporting this case we review prior reports of PILA occurring within the spleen and discuss the potential role of the &amp;lt;I&amp;gt;PIK3CA &amp;lt;/I&amp;gt;mutation in the aetiology of the lesion, and note a theoretical application of mammalian target of rapamycin (mTOR) inhibitors as a possible therapeutic strategy.

Sirolimus for kaposiform hemangioendothelioma: Potential mechanisms of action and resistance.

Kaposiform hemangioendotheliomas (KHEs) are vascular tumors that are considered borderline or locally aggressive and may lead to lethal outcomes. Traditional therapies, such as surgery and embolization, may be insufficient or technically impossible for patients with KHE. Sirolimus (or rapamycin), a specific inhibitor of mechanistic target of rapamycin, has recently been demonstrated to be very useful in the treatment of KHEs. Here, we highlight recent substantial progress regarding the effects of sirolimus on KHEs and discuss the potential mechanisms of action of sirolimus in treating this disease. The prevention of platelet activation and inflammation, along with antiangiogenic effects, the inhibition of lymphangiogenesis, the attenuation of fibrosis, or a combination of all these effects, may be responsible for the therapeutic effects of sirolimus. In addition, the mechanism of sirolimus resistance in some KHE patients is discussed. Finally, we review the somatic mutations that have recently been identified in KEH lesions, and discuss the potential of novel therapeutic targets based on these further understandings of the cellular and molecular pathogenesis of KHE.

Analysis of Risk Factors for Kasabach Merritt Phenomenom in Children With Kaposiform Hemangioendothelioma

BackgroundThis study generalized and analyzed the clinical attributes observed in patients afflicted with Kaposiform hemangioendothelioma (KHE) with the aim of elucidating the risk factors contributing to the manifestation of Kasabach-Merritt phenomenon (KMP). MethodsWe retrospectively analyzed 96 pediatric cases diagnosed with KHE at the Children's Hospital of Fudan University from January 2013 to December 2021. Among them, 62 patients (65%) showed KMP (KHE + KMP group), while 34 patients (35%) did not (KHE-KMP group). The risk factors for KMP associated with KHE were analyzed using univariate analysis and binary logistic regression analysis, comparing the differences between KHE + KMP group and KHE-KMP group. ResultsUnivariate analysis indicated no statistically significant differences between the two groups in gender, prematurity, family history, or color of involved skin. However, statistically significant differences were observed in age of onset, lesion site, and lesion depth. Multivariate analysis revealed significant associations: children with onset age ≤1 month had a 51-fold increased risk of KMP compared to those with onset age >1 month (95% CI 5.238–501.663); non-extremity lesion sites exhibited a 21-fold higher risk of KMP compared to extremity sites (95% CI 3.970–105.958); deeper lesions conferred a 5-fold higher risk of KMP compared to superficial lesions (95% CI 1.073–21.005); lesions >60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999–96.157).A comprehensive predictive model was developed using the fitting formula: Logit (P) = 3.937∗(age at onset) + 1.558∗(lesion depth) + 3.021∗(lesion site) + 2.832∗(lesion size), demonstrating an accuracy of 82.9%. Furthermore, a scoring system was established to assess the likelihood of KMP occurrence. Children diagnosed with KHE were likely to have KMP if their score exceeded 72.5, as determined by Receiver Operating Characteristic (ROC) curve analysis. ConclusionAge of onset ≤1 month, deeper lesions, non-extremity sites, and lesions ＞60 mm are independent risk factors for KMP in children with KHE. The cumulative presence of these factors escalates the likelihood of KMP development. Additionally, the identification of these factors allows for the early recognition of potential KMP cases among children with KHE, facilitating prompt therapeutic intervention. Category of the manuscriptClinical Research article. Level of evidenceLEVEL Ⅲ.

A Xenograft Model of Kaposiform Hemangioendothelioma in Nude Mice Recapitulates Kasabach-Merritt Phenomenon

BackgroundKasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors, such as Kaposiform hemangioendothelioma (KHE). The pathogenesis of KMP remains unclear and its treatment is challenging. In this study, we tried to establish an animal model of KMP, which may facilitate the research on the etiology and new treatment. MethodsA fresh sample of KHE from a one-month-old female infant with KMP was scissored into pieces and transplanted subcutaneously into the back of the nude mice. Blood routine examination was performed before the transplantation and 2, 4, 8, 12, and 16 weeks after the transplantation. Transplanted tumors were harvested 2, 4, 8, 12, and 16 weeks after the transplantation. H-E staining, immunohistochemistry staining of CD31 and α-SMA, and ultrastructural observation were performed on the plugs. ResultsBlood test showed a significant decrease in the number of platelets 2 weeks after transplantation. The number of platelets showed an overall trend of recovery from 2 weeks despite a slight decrease at 12 weeks after transplantation. There was no significant difference in the platelet count at 16 weeks after transplantation compared with the original state. H-E staining showed abundant irregular blood sinuses in the transplanted tumors with plenty of blood cells 2 weeks after the transplantation. 4, 8, and 12 weeks after transplantation, the density of blood sinuses decreased progressively. 16 weeks after transplantation, the plugs involuted into fibrous tissue. Immunohistochemistry staining showed the positive expression of CD31 in the endothelial cells and α-SMA in the perivascular cells. Ultrastructural observation also showed the features of KHE and progressive evolution of the tumors. ConclusionsWe successfully established an experimental model of KMP by the xenograft of KHE in nude mice, which manifested profound thrombocytopenia and typical pathological structure.

A case report of primary Kaposiform hemangioendothelioma of the humerus.

To examine the clinicopathological and immunohistochemical features of Kaposiform hemangioendothelioma (KHE) and discuss its differential diagnosis and prognosis. A patient with KHE was examined; the patient's clinical and histopathological features were observed, and the expression levels of CD31, CD34, ERG, D2-40, SMA, GLUT-1, and LANA-1 were assessed. The patient was a four-year-old child with primary KHE of the humerus. She was admitted to the hospital because of pain in the right elbow joint and limited movement for more than 2years. Imaging revealed Langerhans cell histiocytosis. The child was not diagnosed with Kasabach-Merritt phenomenon (KMP). The tumor consists of multiple hemangiomatous nodules with infiltrative growth separated by fibrous connective tissue. The proliferating hemangiomatoid nodules consisted of crisscrossing short spindle-shaped cell bundles and erythrocyte-containing lacunar or crescentic vessels. Immunohistochemical staining showed that the tumor cells diffusely expressed CD31, CD34, ERG, and other vascular endothelium-derived markers; further, the tumor cells expressed neither GLUT-1 nor LANA-1. The patient's general condition improved after surgical resection. There was no tumor recurrence after more than 8months of follow-up. Primary KHE of the humerus is a rare vasculogenic tumor. It presents with morphological features that require an accurate differential diagnosis.

Surgical and wound management for hand-localized kaposiform hemangioendothelioma: a case report

Surgical and wound management for hand-localized kaposiform hemangioendothelioma: a case report

Composite hemangioendothelioma of breast: A case report with review of literature

Composite hemangioendothelioma (CHE) is an intermediate grade neoplastic tumor of vascular origin. It consists of a varied mixture of benign, intermediate and malignantvascular components. Only few cases of CHE occurring in different soft tissue areas of the body and rarely in internal organs have been reported in literature till now. We report a case of CHE presenting as lump in the breast in a 42 year old female. A review of literature is also presented to create awareness of this entity which can be misleading in breast where angiosarcoma is also known to occur.

Soft tissue vascular tumor-like lesions in adults: imaging and pathological analysis pitfalls per ISSVA classification

ObjectivesTo compare the magnetic resonance imaging (MRI) and Doppler ultrasound (DUS) findings with the pathological findings of soft tissue vascular tumors (STVTs) according to the 2018 ISSVA (International Society for the Study of Vascular Anomalies) classification to differentiate vascular tumors from vascular malformations.MethodsThis retrospective study included patients with STVTs who underwent contrast-enhanced MRI and pathological analysis at our hospital between 2010 and 2020. The presumptive diagnosis based on the on-site imaging and histological analysis was compared with imaging and histological analysis conducted off-site utilizing the ISSVA criteria.ResultsThis study included 31 patients with 31 vascular tumors located in the head and neck (n = 3), trunk (n = 2), and extremities (n = 26). The off-site pathological analysis confirmed benign vascular tumors in 54.8% of cases (non-involuting congenital hemangioma: 35.5%; epithelioid hemangioma: 13%; pyogenic granuloma: 3%; and spindle cell hemangioma: 3%). Based on the off-site histological analysis, 25.8% were reclassified as having a vascular malformation whereas three had other benign lesions. Only phleboliths were associated with a vascular malformation (p = 0.03). The concordance between off-site MRI and pathological findings was fair (k = 0.3902 (0.0531–0.7274)), whereas that between on-site and off-site pathological analyses was poor (k = −0.0949 (−0.4661 to 0.2763)).ConclusionBenign vascular tumors have non-specific imaging features on imaging with some overlap with atypical vascular malformations. Therefore, histological analysis is recommended. Imaging and pathological analyses should be performed in accordance with the ISSVA classification to minimize inter-observer discrepancies.Critical relevance statementImaging features of benign vascular tumors on MRI are non-specific, leading to discrepancies with pathological findings and potential overlap with atypical vascular malformations. Imaging and histological analyses should be performed in accordance with ISSVA guidelines to improve patient management.Key PointsThe imaging features of benign vascular tumors are non-specific.Histological analysis is recommended for soft tissue vascular tumors in adults.Analyses of soft tissue vascular tumors should be performed in accordance with ISSVA guidelines.Graphical

Treatment experience for different risk groups of Kaposiform hemangioendothelioma.

Kaposiform 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. The 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. Through 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. In 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. Our 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.

Endoscopic Management of Recurrent Epistaxis Caused by Retiform Hemangioendothelioma in a Child: A Case Report.

Retiform hemangioendothelioma (RH) is a rare intermediate (locally aggressive) vascular tumor that mostly affects the dermis of the trunk and limbs, but has never been reported in the inferior turbinate. A 10-year-old Chinese boy presented with recurrent epistaxis in his left nasal cavity and anemia for more than 2 years. Radiographic and electronic video laryngoscopic images showed an expansile mass in the left inferior turbinate. Endoscopic surgery and electrocautery were performed to resect the tumor beyond the macroscopic border. Histopathologically, the tissues were infiltrated by hyperplastic blood vessels arranged in a retiform pattern, and endothelial cells proliferate significantly in some areas. Immunohistochemistry showed a positive result for CD31, CD34, Fli-1, and ERG. No epistaxis, tumor recurrence, or metastasis was found on reexamination over 18 months after surgery.

NRASQ61R mutation drives elevated angiopoietin-2 expression in human endothelial cells and a genetic mouse model.

Angiopoietin-2 (Ang-2) is increased in the blood of patients with kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE). While the genetic causes of KHE are not clear, a somatic activating NRASQ61R mutation has been found in the lesions of KLA patients. Our study tested the hypothesis that the NRASQ61R mutation drives elevated Ang-2 expression in endothelial cells. Ang-2 was measured in human endothelial progenitor cells (EPC) expressing NRASQ61R and a genetic mouse model with endothelial targeted NRASQ61R. To determine the signaling pathways driving Ang-2, NRASQ61R EPC were treated with signaling pathway inhibitors. Ang-2 levels were increased in EPC expressing NRASQ61R compared to NRASWT by Western blot analysis of cell lysates and ELISA of the cell culture media. Ang-2 levels were elevated in the blood of NRASQ61R mutant mice. NRASQ61R mutant mice also had reduced platelet counts and splenomegaly with hypervascular lesions, like some KLA patients. mTOR inhibitor rapamycin attenuated Ang-2 expression by NRASQ61R EPC. However, MEK1/2 inhibitor trametinib was more effective blocking increases in Ang-2. Our studies show that the NRASQ61R mutation in endothelial cells induces Ang-2 expression in vitro and in vivo. In cultured human endothelial cells, NRASQ61R drives elevated Ang-2 through MAP kinase and mTOR-dependent signaling pathways.

Right iliopsoas kaposiform hemangioendothelioma: a case report

BackgroundKaposiform hemangioendothelioma (KHE) is a rare neoplasm of vascular origin. It may involve skin, bone, connective tissue, and parenchymal organs, and it is difficult to make a definite diagnosis in the early stage because Kasabach-Merritt phenomenon may occur in some cases.Case presentationOn June 12, 2022, a 19-month boy was admitted to our department with the chief complaint of flexion of the right hip joint for over 1 year. An exploratory laparotomy and pelvic lesion resection were conducted and the resection of the iliopsoas muscle mass was performed. The intraoperative resection of the neoplasm confirmed the pathological diagnosis of kaposiform hemangioendothelioma. The vital signs of the child were stable and recovered well after surgery, and the platelet and coagulation indices were normal.ConclusionKHE is a rare disease in clinics, the number of confirmed cases in the world is still relatively few, and the etiology and mechanism of the disease are still unknown, which could result in misdiagnose and delayed treatment in the disease early stages. It is necessary to continue to collect relevant cases, to unify the standard treatment guidelines as soon as possible, to reduce the mortality rate, and to improve the quality of life of the cases.

Euphorbia helioscopia L. exhibits promising therapeutic effects on hemangioendothelioma and melanoma through angiogenesis inhibition

BackgroundEuphorbia helioscopia L (EHL), a widely used medicinal plant in traditional Chinese medicine, has shown promising effects on certain cancers. However, previous studies on EHL did not elucidate the underlying molecular mechanisms. Herein, for the first time, we present the strong therapeutic potential of EHL extracts on malignant hemangioendothelioma, a rare type of vascular tumor. PurposeTo investigate the potential anti-tumor mechanism of extracts of EHL on hemangioendothelioma and melanoma. MethodsThe dried stems and leaves of EHL were extracted with Ethyl Acetate and n-Butyl alcohol, yielding two crude extracts Ethyl Acetate fraction (EA) and n-Butyl alcohol fraction (Bu). EA and Bu were prepared to assess the potential mechanism by assays for cell proliferation, cell cycle, apoptosis, colony formation, tube formation, cellular metabolic activity, reactive oxygen species (ROS), N-Acetylcysteine (NAC) antagonism, RNA expression and western blot. To further confirm the anti-tumor effect of EHL in vivo, we established hemangioendothelioma and melanoma tumor-bearing mouse model using node mice and administered with EA and Bu, tracked alterations in tumor volume and survival rate. Furthermore, tissue samples were obtained for histological, protein, and genetic investigations. ResultsWe demonstrate that the injection of EA and Bu, significantly inhibits tumor growth and prolongs the lifespan of tumor-bearing mice. Bu treatment exhibited a remarkable 33 % healing effect on the primary hemangioendothelioma tumor, bringing the survival rate to a level comparable to that of healthy mice. Mechanically, both EA and Bu impair respiratory chain complexes, leading to mitochondrial dysfunction and accumulation of reactive oxygen species (ROS), resulting in DNA damage, cell apoptosis, and finally blocked angiogenesis. While EA demonstrates robust inhibitory effects on cancer cell growth and a broader impact on metabolism in vitro, the in vivo effect of Bu surpasses that of EA in terms of strength. EA and Bu also exhibit potent anti-tumor effects on a primary melanoma model by inhibiting angiogenesis. Importantly, when compared to other compounds used in the treatment of hemangioendothelioma, EA and Bu demonstrate more profound anti-tumor effects. ConclusionFor the first time, our findings reveal that EHL extracts, especially the high polarity compounds, exhibit potent anti-tumor effects by targeting cellular metabolism, specifically through the inhibition of mitochondria-related metabolic activities. This leads to the accumulation of ROS and effectively suppresses abnormal angiogenesis.

Primary intraosseous papillary intralymphatic angioendothelioma of the distal femoral epiphysis: a case report with literature review.

Papillary intralymphatic angioendothelioma (PILA) is an exceptionally rare metastasizing soft tissue tumor. It tends to arise in the subcutaneous tissues of distal extremities in children. Only four intraosseous PILA cases have been reported until now in English language literature. We present a case of PILA arising in the distal femoral epiphysis of a 50-year-old female patient. It started as a relentless pain in her left knee. A plain radiography revealed a radiolucent area in the left internal femoral condyle. Computerized tomography revealed a 1-cm lytic lesion with a sclerotic rim. Magnetic resonance images showed a significant bone marrow edema signal focused on a 1-cm subchondral lesion suggestive of an intraarticular osteoid osteoma. Histologically, the tumor contained vascular channels covered by a single endothelial layer with intraluminal papillary endothelial structures lined with hobnail cells. Immunohistochemically, the cells were positive for ERG, CD31, and D2-40. The tumor underwent cryoablation and 6months later, after local recurrence or tumor persistence, a wide tumor resection was referred. After 7years of follow-up, the patient displayed neither local recurrence nor distant metastases. Primary intraosseous PILAs are exceedingly rare tumors that should be considered in the differential diagnosis of vascular bone tumors.

Complex therapy of kaposiform hemangioendothelioma complicated by Kasabach-Merritt syndrome. A case report and review of the literature

Background: Kasabach-Merritt syndrome is an infrequent complication of large hemangiomas that could be life-threatening. Ninety percent of this phenomenon is associated with kaposiform hemangioendothelioma. Aim of the study: To present the therapeutic process of a child with kaposiform hemangioendothelioma complicated by this rare syndrome. Case report: The patient was admitted to the hospital during the first month of life due to a lesion on the head and neck. After many attempts with pharmacological treatment, surgery was the only effective treatment.Conclusions: We want to emphasize that operative management of these tumors should be considered in cases of treatment resistance or rapidly progressive growth of the hemangioendothelioma.

Ocular retiform haemangioendothelioma in a Hallikar bullock and its surgical treatment

Ocular retiform haemangioendothelioma in a Hallikar bullock and its surgical treatment

Construction and applications of the EOMA spheroid model of Kaposiform hemangioendothelioma

BackgroundKaposiform hemangioendothelioma (KHE) is a rare intermediate vascular tumor with unclear pathogenesis. Recently, three dimensional (3D) cell spheroids and organoids have played an indispensable role in the study of many diseases, such as infantile hemangioma and non-involuting congenital hemangiomas. However, few research on KHE are based on the 3D model. This study aims to evaluate the 3D superiority, the similarity with KHE and the ability of drug evaluation of EOMA spheroids as an in vitro 3D KHE model.ResultsAfter two days, relatively uniform morphology and high viability of EOMA spheroids were generated by the rotating cell culture system (RCCS). Through transcriptome analysis, compared with 2D EOMA cells, focal adhesion-related genes such as Itgb4, Flt1, VEGFC, TNXB, LAMA3, VWF, and VEGFD were upregulated in EOMA spheroids. Meanwhile, the EOMA spheroids injected into the subcutaneous showed more obvious KMP than 2D EOMA cells. Furthermore, EOMA spheroids possessed the similar characteristics to the KHE tissues and subcutaneous tumors, such as diagnostic markers (CD31 and LYVE-1), cell proliferation (Ki67), hypoxia (HIF-1α) and cell adhesion (E-cadherin and N-cadherin). Based on the EOMA spheroid model, we discovered that sirolimus, the first-line drug for treating KHE, could inhibit EOMA cell proliferation and downregulate the VEGFC expression. Through the extra addition of VEGFC, the effect of sirolimus on EOMA spheroid could be weakened.ConclusionWith a high degree of similarity of the KHE, 3D EOMA spheroids generated by the RCCS can be used as a in vitro model for basic researches of KHE, generating subcutaneous tumors and drug screening.