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ABSTRACT Infantile hemangiomas (IH) are the most common benign tumors of infancy and progress through recognized stages of evolution including early proliferation, plateau, and involution. Ulceration is a common complication of IHs typically observed during the early proliferative stage characterized by rapid growth. In rare cases, ulceration is the primary clinical manifestation of IHs. We present the case of a newborn with an IH manifesting as an aggressive, early‐onset ulcerative lesion on the lower extremity associated with transient thrombocytopenia and coagulation abnormalities. [ABSTRACT FROM AUTHOR]

Abstract Objective The present study aims to investigate the ultrasound and magnetic resonance imaging (MRI) characteristics of kaposiform hemangioen dothelioma (KHE). Methods A 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. Results The 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

Infantile hemangiomas are the most common type of vascular tumors, affecting ~5% of infants within the first weeks of life. In rare instances, these tumors can lead to Kasabach–Merritt phenomenon (KMP), a life-threatening consumptive coagulopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and hypofibrinogenemia. In the present case, a 20-month-old patient is diagnosed with KMP. This case report highlights the challenges in diagnosis and management, reinforcing the importance of multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Vascular tumours (VT) with Kasabach-Merritt phenomenon (KMP) are rare and aggressive tumors. In absence of evidence based treatment guidelines, we studied varied presentation and response to therapy with vincristine and steroids in VT with KMP at our center. In this retrospective observational study, infants with a symptomatic/disfiguring rapidly growing VT with features of KMP were included. Demographic, treatment and outcome data was retrieved from patient file. Complete response (CR) was defined as complete clinical regression of VT with normalization of coagulopathy and thrombocytopenia. Partial response (PR) was defined as decrease in size of VT by more than 80%, absence of clinical bleed with normalization of coagulopathy and platelet count > 50,000/cumm. Five infants (2-male, 3-female) with age range (0–7 month) treated with daily prednisolone and weekly vincristine were included. The location of VT was: face (2), hemi-thorax (2) and urinary bladder (1). Four of five infants showed PR within two months; while two of these attained CR to treatment. There were no significant adverse effects over 9–32 (range) month follow-up. Two children (one in PR, one immediately after presentation) succumbed to intra-cranial hemorrhage. Combination therapy of steroids with vincristine is effective and safe in management of VT with KMP. [ABSTRACT FROM AUTHOR]

The conventional treatment of Kasabach-Merritt Phenomenon (KMP) consists of corticosteroids with vincristine/vinblastine or others. The aim of the study is to compare the first-year direct costs and effectiveness between sirolimus and conventional treatment. A retrospective case-control study of KMP patients was conducted at a mean age of 9 months (1 day to 12 years) between 2000 and 2022 from four tertiary centers in Thailand. The direct costs, hematologic and clinical complete response (HCR, CCR), hospitalization, length of stay, and complications were compared. Of 29 patients, 13 underwent sirolimus (four upfront and nine were refractory to the conventional). The first-year total cost had no statistically significant difference between sirolimus VS conventional treatment (8,852.63 VS 9,083.56 USD: p value: 0.94). The therapeutics achievement was the same in both HCR (244.75 VS 168.94 days; p value: 0.60) and CCR (419.77 VS 399.87 days; p value: 0.90). The subgroup analysis of the first-line sirolimus (n = 4) compared with the conventional (n = 25) showed a more reduced total cost (4,907.84 VS 9,664.05 USD; p value: 0.26) rendered net total cost of −4,756.21 USD per patient (cost saving). A more significant contrast of therapeutic achievement by reduction of both HCR (11.67 VS 224.20 days; p value: 0.36) and CCR (38.50 VS 470.88 days; p value: 0.04) was shown. The sirolimus had no difference in hospitalization, length of stay, and complications. Even though, it was unable to identify significant differences in cost-effectiveness. Sirolimus is suitable for all patients who have diagnosis of KMP either for rescue therapy or first-line treatment. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that often occurs in infants and young children. The goal of this study was to analyze the clinical characteristics of KHE patients with bone destruction and provide clinical guidance for diagnosis and treatment. We conducted a descriptive cohort study with follow-up from January 2007 to January 2023 to collect demographic information and tumor-related clinical information from KHE patients with bone destruction. A total of 269 KHE patients were included in the study, of whom 70 (26.0%) patients had tumors with bone destruction. The median age at diagnosis of patients with bone destruction was 19.0 months, which was much later than that of patients without bone destruction (P < 0.001). Patients with bone destruction were more likely to have a decreased range of motion (ROM) (P < 0.001). Metaphysis involvement was more likely to occur in the lower limb bones (P = 0.039), and the lower limb bones were more likely to be associated with decreased ROM (P = 0.001). Tumors involving extracompartmental bone were more likely to have decreased ROM (P = 0.003) and exhibit the Kasabach–Merritt phenomenon (P = 0.006). Based on the rarity and significant heterogeneity of KHE patients with bone destruction, we should give full play to the role of multidisciplinary teams in addressing disease to reduce the long-term complications of KHE with bone destruction and improve the quality of life of patients. Prognostic Study. Level II. [ABSTRACT FROM AUTHOR]

This retrospective study aimed to observe the efficacy of transcatheter arterial chemoembolization (TACE) combined with sirolimus in the treatment of haemangioma combined with the Kasabach–Merritt phenomenon (KMP). A total of 11 infants with KMP who were treated at our hospital from January 2016 to September 2021 were selected and treated with arteriosclerosis embolotherapy using a microsphere emulsion formed by bleomycin + ultra-fluid lipiodol + dexamethasone + contrast agent or bleomycin mixed microspheres as the embolising agent. The patients were administered sirolimus orally after TACE. The clinical efficacy and examination indicators before and after treatment were observed and compared. The 11 infants underwent TACE treatment by arteriosclerosis embolotherapy a total of 21 times; of these cases, 10 were cured, and 1 showed a moderate response. There were no cases of non-response or death. The platelet count rose from 10.0 (7.0, 18.0) x 109/L before TACE to 236.0 (188.0, 275.0) x 109/L six months after the first TACE, and the tumour size decreased from 49.0 (43.0, 111.7) cm3 before TACE to 7.0 (3.5, 17.0) cm3 six months after the first TACE. The differences were statistically significant (the Z values were −2.943 and −2.934, respectively, p < 0.05). The combination of TACE and sirolimus has significant efficacy on critical children with KMP. [ABSTRACT FROM AUTHOR]

AbstractObjective This retrospective study aimed to observe the efficacy of transcatheter arterial chemoembolization (TACE) combined with sirolimus in the treatment of haemangioma combined with the Kasabach–Merritt phenomenon (KMP).Methods A total of 11 infants with KMP who were treated at our hospital from January 2016 to September 2021 were selected and treated with arteriosclerosis embolotherapy using a microsphere emulsion formed by bleomycin + ultra-fluid lipiodol + dexamethasone + contrast agent or bleomycin mixed microspheres as the embolising agent. The patients were administered sirolimus orally after TACE. The clinical efficacy and examination indicators before and after treatment were observed and compared.Results The 11 infants underwent TACE treatment by arteriosclerosis embolotherapy a total of 21 times; of these cases, 10 were cured, and 1 showed a moderate response. There were no cases of non-response or death. The platelet count rose from 10.0 (7.0, 18.0) x 109/L before TACE to 236.0 (188.0, 275.0) x 109/L six months after the first TACE, and the tumour size decreased from 49.0 (43.0, 111.7) cm3 before TACE to 7.0 (3.5, 17.0) cm3 six months after the first TACE. The differences were statistically significant (the Z values were −2.943 and −2.934, respectively, p

Kaposiform haemangioendothelioma (KHE) is a rare vascular tumor, occurring predominantly in infancy, with an incidence of 0.091 per 100,000 children. Typically presenting as a solitary large cutaneous lesion with no distant metastases. Children with KHE are at high risk of developing the Kasabach-Merritt phenomenon (KMP), a consumptive coagulopathy. KMP is defined as profound thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. KMP has a mortality of 10 - 30%. Due to its rarity, there is little robust evidence for KHE management. Rapamycin (mTOR) inhibitors are a safe and effective treatment. This report is of a newborn full-term infant with no antenatal complications, presenting with abdominal cutaneous vascular malformation and, subsequently, coagulopathy. [ABSTRACT FROM AUTHOR]

Abstract Background Kaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops. Case presentation A 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon. Conclusion The diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management.

Background: Knowledge on the management of kaposiform hemanigioendothelioma (KHE) with kasabach-merritt phenomenon (KMP) rely on single case reports, case series and a few retrospective studies. Case report: A 3-month old girl was admitted for a violaceous, palpable mass in the right posterior neck. Laboratory studies showed thrombocytopenia and consumptive coagulopathy with hypofibrinogenemia and raised Dimer-D values. A diagnosis of KHE with KMP was confirmed histopathologically. Treatment regimen for KMP was commenced with oral prednisone and salicylic acid, followed by oral sirolimus at 1 mg/m2/day. It led to an involution of the vascular lesion at 12-month follow-up. Conclusion: The case contributes with another example of KHE with KMP that was effectively treated with the novel mTOR inhibitor. Prospective studies for sirolimus are urged and clinical guidelines are yet to be defined.

Treatment with sirolimus, an inhibitor of the mammalian target of rapamycin pathway, has improved the prognosis of patients with kaposiform hemangioendothelioma (KHE). However, the efficacy, durability and tolerability of long‐term sirolimus treatment in patients with KHE have not been well elucidated. We performed efficacy and safety assessments based on more than 4.5 years of follow‐up in patients receiving sirolimus therapy for KHE. One hundred sixty‐seven patients were analyzed, including 102 (61.1%) patients with the Kasabach‐Merritt phenomenon (KMP). Follow‐up was conducted after a median of 56.0 months. A total of 154 (92.2%) patients had a durable response to sirolimus treatment. No difference in durable response was found between patients without KMP and patients with KMP (95.4% vs 90.2%; difference, 5.2%; 95% confidence interval [CI], −4.0% to 13.1%). Rebound growth occurred in 17.3% of patients upon sirolimus discontinuation. Early treatment discontinuation (odds ratio [OR]: 3.103; 95% CI: 1.529‐6.299; P =.002) and mixed lesion type (OR: 2.271; 95% CI: 0.901‐5.727; P =.047) were associated with tumor rebound growth. No KHE‐related deaths occurred in this cohort. At the last follow‐up, approximately 17.4% of patients had active disease and/or changes in body structures to a variable extent. Serious adverse events occurred most commonly during the first year of sirolimus therapy. Follow‐up of almost 4.5 years demonstrated that the efficacy of sirolimus persisted over time and that long‐term treatment with sirolimus was not associated with unacceptable cumulative toxicities. However, nonresponse, tumor relapse and long‐term sequelae remained challenges despite intensified and prolonged sirolimus therapy. [ABSTRACT FROM AUTHOR]

The article focuses on a case of Kaposiform hemangioendothelioma (KHE) complicated by the Kasabach-Merritt phenomenon (KMP) in an infant. Topics include the diagnosis and treatment of KHE with sirolimus, the risks of biopsy-induced hemorrhage in hypervascular tumors, and the management of KMP exacerbation following intratumoral bleeding.

Background: Kaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops. Case presentation: A 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon. Conclusion: The diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management. [ABSTRACT FROM AUTHOR]

Objective: Kaposiform hemangioendothelioma (KHE) is a locally aggressive tumor of vascular origin. This study investigated the clinical and imaging features of KHE to provide a reference for its early diagnosis. Methods: The clinical and imaging findings of 27 clinically confirmed KHE cases (including 21 with focal lesions and 6 with diffuse lesions) between January 2016 and December 2021 were retrospectively analyzed. Results: The mean age of the 27 patients was 105 ± 80.27 days. Twenty-two (81.5%) of these patients had Kasabach–Merritt phenomenon. Most KHEs were located in the trunk and/or extremities (22/27). Ultrasonography showed heterogeneous echogenicity and/or striated hypoechoic bands with abundant or patchy blood flow within the tumor. On plain computed tomography (CT), they appeared as heterogeneous lesions isodense with the muscles, with a CT value of 29.58 ± 11.53 HU. In the arterial phase, the KHEs showed striated or lamellar heterogeneous enhancement, with a CT value of 153.91 ± 52.11 HU after enhancement. All KHEs showed uneven and high signal intensity on T2-weighted imaging, mixed high and low signal intensity on fat-saturated images, and no significant diffusion restriction on diffusion-weighted imaging. Conclusion: KHEs can occur in various locations and present as highly infiltrative and heterogeneous masses that can invade the skin, adjacent muscles, and bones. A vascularized mass with purpuric skin changes, with uneven and high T2WI signal is highly suggestive of the diagnosis of KHE.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multifocal venous malformations that can affect any organ or tissue. Kasabach-Merritt phenomenon (KMP) is a serious and extremely rare complication of BRBNS. This report describes a neonate with BRBNS with KMP who was successfully diagnosed and treated with low-dose sirolimus and glucocorticoids. A 13-day-old female infant was born with multiple tumors on her head, neck, shoulder, back, abdomen, limbs, perineum, etc. some of which were blue. Laboratory examinations showed thrombocytopenia, anemia and coagulopathy. BRBNS with KMP was diagnosed. Oral low-dose sirolimus combined with glucocorticoids was administered. After 6 months of regular follow-up, the lesions in the child were significantly decreased, and there were no signs of KMP recurrence. The presence of KMP should be considered in patients diagnosed with BRBNS who present with thrombocytopenia, anemia and coagulopathy. Sirolimus combined with glucocorticoid therapy can be administered to save the patient's life.

ObjectiveTo investigate the clinical features, diagnosis and treatment methods and prognosis of retroperitoneal Kaposiform hemangioendothelioma (R-KHE) in children.MethodsThe clinical data of an infant with R-KHE was retrospectively analyzed. Literature on R-KHE in pediatrics were retrieved in databases including Wanfang, CNKI and PubMed as of April 2022.ResultsA 1 month and 6 days female infant with R-KHE was reported. After the diagnosis was confirmed by biopsy and pathological examination, the patient was treated by interventional embolization, and a combined therapy with glucocorticoid, vincristine, sirolimus and propranolol. The patient has been followed up for 1 year and 2 months, and is still alive with tumor. Through literature search, a total of 15 children, together with the case in our report, were included. The main manifestations were diversity among those patients. 14 cases have combined Kasabach-Merritt phenomenon (KMP). 6 cases accepted surgery plus drug therapy. 4 cases accepted only surgery, and 4 cases only accepted drug therapy. While drug therapy plus radiotherapy were employed to 1 case. Improvement was observed in 11 cases, with significantly reduced tumor and survival with tumor. Tumor disappeared completely in 2 cases. While 2 cases suffered death.ConclusionR-KHE has diverse clinical presentations and non-specificity in symptoms and imaging examinations, and most cases accompanied with KMP. Methods for R-KHE treatment include surgical resection, interventional embolization and drug therapy. Close attention needs to be paid to the adverse reactions of the drug during the course of treatment.

Objective. To describe the case of a large cervical mass diagnosed in the late third trimester with development of Kasabach-Merritt phenomenon (KMP) in the immediate postnatal period, along with a literature review. Methods. Description of case-report and literature search through Medline/Pubmed, performed from inception to December 2022 for articles relating to the pre and postnatal diagnosis of KMP. Results. A 36-year-old multiparous woman was admitted to hospital for contractions at 40 weeks of gestation, in an otherwise uneventful pregnancy. Admission's ultrasound showed the presence of a voluminous mass of 14x15 cm of the posterior side of the neck, highly vascularized, and no signs of hemodynamic imbalance. Postnatally, blood tests showed the presence of severe anemia and thrombocytopenia requiring several transfusions of blood, plasma, platelets and clotting factors. Due to the association of congenital hemangioma and thrombocytopenia a diagnosis of KMP was made. After attempts of conservative treatment, surgical removal was needed to stop the hematological cascade with regression of symptoms. The review of the literature identified 14 articles including 9 cases of prenatally suspected KMP and 6 diagnosed in the immediate postnatal period and without signs of fetal hydrops. Adverse perinatal outcome, in terms of postnatal death/termination of pregnancy, was observed in 67% of cases (6/9) in the prenatally suspected group and 33% of cases in those with a postnatal diagnosis of KMP. Fetal hydrops was present in 83% of cases with adverse perinatal outcome. Conclusions. The Kasabach-Merrit syndrome is a rare condition, which can have a dangerous evolution when it develops in utero or in the immediate postnatal period carrying a risk of perinatal mortality of approximately 50%. Even if the fetus shows no signs of anemia or heart failure, the risk of developing it in the immediate postnatal period is high and should be mentioned to the couple. [ABSTRACT FROM AUTHOR]

Background: Vascular anomalies comprise a diverse group of rare diseases with altered blood flow and are often associated with coagulation disorders. The most common example is a localized intravascular coagulopathy in venous malformations leading to elevated D-dimers. In severe cases, this may progress to a disseminated intravascular coagulopathy with subsequent consumption of fibrinogen and thrombocytes predisposing to serious bleeding. A separate coagulopathy is the Kasabach–Merritt phenomenon in kaposiform hemangioendothelioma characterized by platelet trapping leading to thrombocytopenia and eventually consumptive coagulopathy. Our previous work showed impaired von Willebrand factor and platelet aggregometry due to abnormal blood flow, i.e., in ventricular assist devices or extracorporeal membrane oxygenation. With altered blood flow also present in vascular anomalies, we hypothesized that, in particular, the von Willebrand factor parameters and the platelet function may be similarly impacted. Methods: We prospectively recruited 73 patients with different vascular anomaly entities and analyzed their coagulation parameters. Results: Acquired von Willebrand syndrome was observed in both of our patients with Kasabach–Merritt phenomenon. In six out of nine patients with complex lymphatic anomalies, both the vWF antigen and activity were upregulated. Platelet aggregometry was impaired in both patients with Kasabach–Merritt phenomenon and in seven out of eight patients with an arteriovenous malformation. Conclusions: The analysis of coagulation parameters in our patients with vascular anomalies advanced our understanding of the underlying pathophysiologies of the observed coagulopathies. This may lead to new treatment options for the, in part, life-threatening bleeding risks in these patients in the future. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas (CHs) are rare vascular tumors and do not exhibit progressive postnatal growth. The incidence is less than 3% of all hemangiomas. Most CHs have a favorable prognosis; however, the Kasabach–Merritt phenomenon (KMP) is a rare but life-threatening complication in CHs that requires aggressive treatment. Medical treatments with corticosteroids and interferon have been suggested. Surgical resection can be considered for the treatment of complicated CHs in medically resistant lesions. Vascular embolization could be an alternative method if surgery is not considered feasible. Herein, we report a case of a 9-day-old newborn who underwent arterial embolization for a CH with KMP, combined with sirolimus treatment, and the outcome was favorable. The hemangioma completely regressed by 3 months and rapidly involuting congenital hemangioma (RICH) was diagnosed. Our successful experience with treating RICH associated with KMP revealed that RICH can have potentially serious complications although they usually resolve rapidly after birth without treatment. Surgical resection is considered to be the standard method for the treatment of medically resistant vascular tumors, but it is difficult to perform during the active phase of KMP due to acute bleeding and severe coagulopathy. Arterial embolization is feasible and can be used as an alternative to surgical resection, even in small babies.

Infantile fibrosarcoma (IF) is a well characterized pediatric malignancy marked by gene rearrangements involving members of the NTRK family. In this report, we present a case of IF that presented in the inguinal region-proximal thigh and was initially thought to be a kaposiform hemangioendothelioma (KHE) because it presented with a bleeding diathesis thought to be Kasabach-Merritt phenomenon (KMP). Subsequently, the placental examination showed a neoplasm in the perivascular-subendothelial space of stem villi, initially thought to be myofibromatosis. Ultimately, a biopsy of the thigh mass showed IF with an NTRK3-ETV6 fusion. Subsequent FISH analysis of the placenta showed an ETV6 rearrangement confirming that it was also IF. Review of the laboratory studies suggests that disseminated intravascular coagulation may have been more likely than KMP, highlighting the difficulty in making this distinction in some cases. We believe this to be the first report of an IF presenting in a soft tissue site and the placenta, and discuss the possible mechanisms that could have allowed the IF in the leg to spread to the placenta. [ABSTRACT FROM AUTHOR]

Kasabach-Merritt phenomenon (KMP) is a life-threatening condition caused by rare vascular tumors. To reduce drug resistance observed in monotherapy of KMP with prednisone, vincristine (VCR) or sirolimus, the present study evaluated the efficacy and safety of triad therapy in the treatment of KMP. A total of 10 KMP infants managed with prednisolone, VCR and sirolimus in The Second Affiliated Hospital of Xi'an Jiaotong University (Xi'an, China) between April 2017 and August 2021 were retrospectively reviewed. The three female and seven male infants with KMP underwent cocktail therapy with prednisone, VCR and sirolimus. At diagnosis, the infants, aged 49.1±41.0 days, showed laboratory test results with platelet counts 22±15.4x109/l, fibrinogen 81.7±26.9 mg/dl and D-dimer 38649±13443.6 ng/ml. The average maximal diameter of the tumors at diagnosis was 84.5±25.1 mm. KMP risk is increased by large tumors with deep lesions infiltrating the muscle. Platelet counts normalized after a median 10 days (range, 5-69 days) of treatment. With combination therapy maintained for 46.8±24.4 days, ultrasound showed that the thickness of the tumors decreased by 51% from 28.9±12.1 to 13.9±6.2 mm. Neutropenia and gastrointestinal disorders were the most common adverse effects. The present study found that the cocktail therapy with prednisolone, VCR and sirolimus has favorable tolerance and efficacy for life-threatening KMP. Once a stable condition has been achieved, cocktail therapy should be replaced by sirolimus monotherapy to reduce potential side effects. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KHE) is a rare borderline vascular tumor that usually presents as a mass of skin or deep soft tissue. We report a unique case of an 8-year-old KHE patient with bilateral symmetrical sites involving both femurs. The laboratory, radiographic, and pathological findings of the patient were minutely described. During the 6-month follow-up, the symptoms of pain and dysfunction of this patient were relieved. This study aimed to arouse clinicians’ concern about the symmetrical sites of KHE patients.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor usually seen in children. It is frequently associated with Kasabach-Merritt phenomenon. Here we report two cases of KHE: the first case being an 11-month-old boy who came with massive swelling on the face and violaceous discoloration. The second case was a 7-year-old boy who presented with respiratory distress and bleeding manifestations. CT scan chest showed a large mass involving the anterior mediastinum. Histologic examination of resected masses from both these cases showed features of KHE involving subcutaneous tissue and thymus, respectively. Although cutaneous and subcutaneous location is common, thymic involvement is unusual. It is important to distinguish KHE from infantile haemangioma, tufted angioma, spindle-cell haemangioma, verrucous malformation and Kaposi sarcoma. Histologic features, supportive immunohistochemistry and the clinical profile together are helpful to differentiate KHE from other vascular lesions.

Aims: Management of kaposiform haemangioendotheliomas (KHE) with Kasabach–Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal. As infants have a very low CYP3‐enzyme expression at birth, which rises during ageing, we hypothesize that a reduced metabolization of sirolimus might lead to high sirolimus serum levels and low dose may be sufficient without the side effects. Methods: A case series of 5 infants with kaposiform haemangioendothelioma with Kasabach–Merritt phenomenon was analysed retrospectively. All infants were treated with sirolimus 0.2 mg/m2 every 24 or 48 hours according to their age. Prednisone was added to the therapy for additional effect in 4 patients. Results: In all patients, low dose of sirolimus led to therapeutic sirolimus levels (4–6 ng/mL). All infants (aged 4 days–7 months) had a complete haematological response, without serious adverse events. In all patients, the Kasabach–Merritt phenomenon resolved, the coagulation profile normalized and tumour size reduction was seen. Conclusion: Low‐dose sirolimus treatment is safe for infants with kaposiform haemangioendothelioma and Kasabach–Merritt phenomenon. It is essential to realize that during the first months of life, metabolism is still developing and enzymes necessary to metabolise drugs like sirolimus still have to mature. To avoid toxic levels, the sirolimus dosage should be based on age and the associated pharmacological developments. [ABSTRACT FROM AUTHOR]

Abstract Background Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis. Methods The clinical features and imaging findings were retrospectively reviewed in 64 patients with pathological diagnosis of KHE. Results Of the 64 patients diagnosed with KHE, 36 patients were

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm associated with the Kasabach–Merritt phenomenon (KMP), which is a consumptive coagulopathy with associated potentially life-threatening thrombocytopenia. There are no standardized treatment protocols for the management of KHE with KMP. Moreover, there are limited reports regarding the treatment of cases of rebound. Herein, we describe a rare case of rebound of KHE/KMP, during systemic sirolimus treatment, successfully treated with embolization and vincristine infusion combined with microwave ablation.

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that mostly appears in infancy or early childhood. Most KHE occurred on the limbs and trunk with cutaneous lesions. Approximately 12% of KHE patients manifested as deep masses and spinal involvement is extremely rare. KHE may develop into life-threatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP), especially in patients with retroperitoneal involvement. The thrombocytopenia is usually severe, with a median platelet count of 21 × 109/L at the initial presentation of KMP. Here, firstly we described a case of a 13-month-old girl with KHE who presented the movement limitation of the lower extremity caused by spinal involvement with a normal platelet count. 18F-fluorodeoxyglucose-positron emission tomography/CT (18F-FDG PET/CT) showed mildly elevated metabolism in the lesion, suggesting a probably low-grade malignant tumor. Then the patient was diagnosed with KHE by biopsy. After 6-month sirolimus monotherapy, the size of the retroperitoneal lesion was reduced significantly and the patient showed improvement in clinical symptoms. This case demonstrated the advantage of 18F-FDG PET/CT in the evaluation of disease activity in KHE and the possibility of using 18F-FDG PET/CT to guide therapy and prognostication.

Abstract Objectives There are no cohort studies of chronic lymphedema in patients with kaposiform hemangioendothelioma (KHE). We sought to characterize the incidence, clinical features, risk factors and management of chronic lymphedema in patients with KHE. Methods We conducted a multicenter retrospective analysis of patients who had a minimum of 3 years of follow-up after the onset of KHE and/or Kasabach–Merritt phenomenon (KMP). Clinical features were reviewed to determine the possible cause of chronic lymphedema. The degree of lymphedema, risk factors and management strategies were analyzed. Results Among the 118 patients, chronic lymphedema was confirmed by lymphoscintigraphy 1 year after the onset of KHE and/or KMP in 13 patients. In 8 patients with lymphedema, extremity swelling was evident in the presence of KHE and/or KMP. In all patients with lymphedema, a unilateral extremity was affected, along with ipsilateral KHE. Most (84.6%) patients reported moderate lymphedema. Lymphedema was more common in patients with larger (≥ 10 cm) and mixed lesions involving the extremities (P 0.05). Overall, 76.9% of patients received sirolimus treatment after referral, including 53.8% who presented extremity swelling before referral. Seven (53.8%) patients received compression therapy. Five (38.5%) patients reported lymphedema-associated decreased range of motion at the last follow-up. Conclusions Chronic lymphedema is a common sequela of KHE and can occur independently of KMP and sirolimus treatment. Patients with large and mixed KHE involving extremities should be closely monitored for this disabling complication.

Abstract Background Systemic drug therapy is generally recommended for infant huge vascular anomalies associated with thrombocytopenia and coagulopathy, but some patients are not suitable due to drug unresponsiveness or life threatening conditions before the drug works, who will need to receive surgical treatment. This study retrospectively analyzed the clinical features, imaging features, and surgical outcomes of these patients. Methods The clinical data of 4 infants with huge vascular anomalies (2 vein malformations (VMs) and 2 kaposiform hemangioendothelioma (KHE)) associated with thrombocytopenia and coagulopathy treated from June 2016 to December 2017 were retrospectively analyzed. All patients received glucocorticoids, propranolol, vincristine or sirolimus treatment before admission, but the treatment was ineffective. Skin petechia, thrombocytopenia and coagulopathy were present at the time of admission. CT scanning was performed before operation. The patient’s general clinical data, hematological examination results, operation time, surgical bleeding volume, blood transfusion volume and surgical complications were collected for analysis. The patients were followed up for 10–26 months. Results CT scanning results of 2 patients showed special CT features without detectable enhancement within the lesion after CT enhanced scanning and multiple phleboliths formation. Four patients underwent surgical treatment successfully. Two patients underwent complete resection of the lesion, and 2 underwent cytoreductive surgery. Preoperative clinical symptoms such as skin petechia, thrombocytopenia and coagulopathy were normal at 1 week after surgery. Postoperative pathological results showed 2 cases of KHE and 2 cases of VMs. All patients were discharged from hospital without physical dysfunction, recurrence, or death. Conclusions Timely and appropriate surgical intervention can achieve satisfactory results for infants with huge VMs and KHE who were unresponsive to drug therapy or suffering from life-threatening occasion before the drug become effective.

Kaposiform hemagioendothelioma (KHE) is an uncommon locally aggressive vascular tumor that can involve soft tissues or bones. KHE that originates primarily from the temporal bone has been reported extremely rare in the literature. Here we present a case of KHE of the temporal bone in a 27 months-old girl and describe the findings of different radiological modalities along with histopathological characteristics of the tumor.

BackgroundDespite recent developments, the role of sirolimus in the heterogeneous spectrum of vascular anomalies is yet to be defined, in terms of indication, dosage, and therapy duration, recognizing both its potential and limitations.MethodsWe retrospectively analyzed 16 children with vascular anomalies treated with sirolimus in two pediatric centers between 2014 and 2020 [male: n = 7, the median age at diagnosis: 4.6 months (range, 0–281.4)]. In addition, repetitive volumetric analyses of the vascular anomalies were performed when possible (11 cases).ResultsTen patients were diagnosed with vascular malformations and 6 with vascular tumors. The mean therapy duration was 27.2 months (range, 3.5–65). The mean sirolimus level was 8.52 ng/ml (range, 5.38–12.88). All patients except one with central conducting lymphatic anomaly responded to sirolimus, with the most noticeable volume reduction in the first 4–6 months. Additional administration of vincristine was needed in five patients with kaposiform hemangioendothelioma and yielded a response, even in cases, refractory to sirolimus monotherapy. As a single agent, sirolimus led to impressive improvement in a patient with another vascular tumor—advanced epithelioid hemangioendothelioma. Complicated vascular malformations required long-term sirolimus therapy. Side effects of sirolimus included mucositis and laboratory abnormalities. No major infectious episodes were recorded. An infant with COVID-19, diagnosed while on sirolimus therapy, presented with a mild course.ConclusionIn the current series, we reported limitations of sirolimus as monotherapy, addressing the need to redefine its indications, and explore combination regimens and multimodal treatment strategies. Tools for objective evaluation of response trends over time could serve as a basis for the establishment of future therapeutic algorithms.

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. All children completed ultrasound examinations in our hospital. After pathological biopsy, 132 cases were diagnosed with KHE. Taking pathological biopsy as the gold standard, the diagnostic rate of ultrasound examination was determined. Our results showed that ultrasound examination diagnosed 124 cases with KHE. Compared with pathological biopsy, the diagnostic sensitivity, specificity, and accuracy of ultrasound were 81.82, 77.42, and 80.41%, respectively (Kappa = 0.725). Most of the children had a single lesion, often involving the skin. The tumor was hard in texture and red or purple in color and did not fade when pressed. The two-dimensional sonogram showed a solid heterogeneous echo mass in the soft tissue. The lesion was generally large, irregular in shape, and unclear in boundaries, extending to the adipose layer and forming a "tree root-like" change. Microscopically, crossed spindle-shaped cell bundles could be seen, showing diffuse multinodular infiltrative growth. Children with the Kasabach-Merritt phenomenon (KMP) generally had larger lesions. Subsequently, the children with KHE were assigned to a training set and a validation set in a ratio of 7:3. High-throughput data acquisition of the regions of interest (ROIs) on the ultrasound images was carried out to construct a KEH prediction model based on ultrasound radiomics, and validation analysis was conducted. We found the diagnostic accuracy rate of ultrasound radiomics was 91.41%, the sensitivity was 77.44%, and the specificity was 97.21%, which were better than those of the conventional ultrasound. In conclusion, ultrasound radiomics analysis is highly effective in the diagnosis of KHE, which can contribute to the early diagnosis rate of KHE., Competing Interests: None., (AJCR Copyright © 2024.)

The article presents a case study of an infant with Kasabach–Merritt phenomenon (KMP), a rare disease with vascular tumors, treated with everolimus (EVL). Topics discussed include the efficacy of EVL in KMP, the risk of pneumocystis pneumonia as a rare but potentially fatal complication of mTOR inhibitors, and the need for immunological evaluations and prophylactic measures during treatment.

Background and ObjectiveKaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE.MeasuresA total of 91 children with KHE participated in this cross-sectional study. The HRQOL was assessed by the age-specific Pediatric Quality of Life Inventory Version 4.0 (PedsQL™ 4.0) Infant Scales, Family Information Form (FIF), Family Impact Module (FIM) and Generic Core Scales (GCS). For comparison, demographically matched healthy children were recruited as a control group. The main outcome measure of HRQOL was analyzed in the two groups. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis.ResultsThe study found that the scores of each item in the family impact module (FIM) were lower than 75, which suggesting that KHE can make the parents of patients in a state of poor quality of life. The scores of physiological and psychosocial domains in all age groups of patients with KHE were lower than those of normal children (P < 0.01). Activity dysfunction is the factor influencing the physiological function score of all-age patients. KMP is the factor influencing the psychosocial function score of all-age patients.ConclusionsThe findings presented here suggest that patients with KHE have a poor HRQOL. KMP and activity dysfunction are risk factors for poor HRQOL in patients with KHE. However, lesion size, lesion location and education level of the mother and father were not related to the HRQOL.

Abstract Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm with high morbidity and mortality. The initiating mechanism during the pathogenesis of KHE has yet to be discovered. The main pathological features of KHE are abnormal angiogenesis and lymphangiogenesis. KHEs are clinically heterogeneous and may develop into a life-threatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). The heterogeneity and the highly frequent occurrence of disease-related comorbidities make the management of KHE challenging. Currently, there are no medications approved by the FDA for the treatment of KHE. Multiple treatment regimens have been used with varying success, and new clinical trials are in progress. In severe patients, multiple agents with variable adjuvant therapies are given in sequence or in combination. Recent studies have demonstrated a satisfactory efficacy of sirolimus, an inhibitor of mammalian target of rapamycin, in the treatment of KHE. Novel targeted treatments based on a better understanding of the pathogenesis of KHE are needed to maximize patient outcomes and quality of life. This review summarizes the epidemiology, etiology, pathophysiology, clinical features, diagnosis and treatments of KHE. Recent new concepts and future perspectives for KHE will also be discussed.

Kaposiform hemangioendothelioma (KHE) represents a rare, borderline vascular tumor with locally aggressive behavior. They are often associated with a potentially life-threatening coagulopathy known as Kasabach–Merritt phenomenon (KMP). Due to heterogeneous nature of the vascular lesion and lack of standardized treatment protocols, these patients pose a diagnostic dilemma and therapeutic challenge with morbidity and potential mortality. We report successful management of an infant with KHE and associated KMP. Difficulties encountered in diagnosis, initiation of therapy, and role of dual therapy with vincristine and steroids are discussed.

Kaposiform haemangioendothelioma (KHE) is a rare, locally invasive vascular tumour that is commonly associated with the Kasabach-Merritt phenomenon (KMP). A case of a five-month-old female infant admitted for dyspnoea, stridor, and skin haematoma is presented. Computerised tomography of the chest showed a tumour mass occupying mediastinum and most of the left hemithorax, while laboratory analysis revealed a thrombocytopaenia and a consumption coagulopathy. Histology of tumour biopsy was characteristic of KHE with a component of tufted angioma. Corticosteroid treatment initially induced a reduction in tumour size, but progression occurred four weeks later and led to a fatal outcome despite additional chemotherapy. After a literature search, we found only 18 cases of mediastinal KHE published so far, with 21 % fatality rate. In the present case several risk factors for adverse outcome were present: onset of disease in early infancy, a large volume of the tumour, mediastinal location, KMP, and partial response to available therapy.

Abstract Large abdominal purpuras may be caused by retroperitoneal kaposiform hemangioendothelioma with consumptive coagulopathy. Clinicians should perform serial ultrasonography studies to detect the sings of tumor until the final diagnosis is confirmed.

Introduction: Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor that mostly appears in infants and adolescents. KHE with spinal involvement is extremely rare. The aim of this study was to review the imaging features, clinical manifestations and treatment of KHE patients with spinal involvement.Materials and Methods: We reviewed patients with KHE who were admitted to Pediatric Surgery of West China Hospital of Sichuan University from April 2014 to August 2020, and the cases were evaluated.Results: Seven patients with spinal involvement were enrolled in the study, including four (57.1%) males and three (42.9%) females. The age at onset ranged from 1.0 day to 4.0 years, with an average of 1.6 years. Five (71.4%) had pain due to bone destruction, three patients (42.9%) had decreased range of motion (ROM), four (57.1%) patients had scoliosis, two (28.6%) patients developed claudication, and three patients (42.9%) presented with a soft tissue mass in the neck of the back. Five patients (71.4%) had the Kasabach-Merritt phenomenon (KMP), with a minimum platelet value of 8 × 109/L. All patients were treated with sirolimus, and showed regression of the lesion and/or normalization of the hematologic parameters.Conclusion: KHE with spinal involvement is difficult to diagnose due to its rarity and variable symptoms, which need to be recognized to start early treatment. The management of KHE with spinal involvement should be performed by a multidisciplinary team. Sirolimus can improve outcomes in patients with KHE with spinal involvement.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor in children, which can be accompanied by life‐threatening thrombocytopenia, referred to as Kasabach‐Merritt phenomenon (KMP). The mTOR inhibitor sirolimus is emerging as targeted therapy in KHE. As the sirolimus effect on KHE occurs only after several weeks, we aimed to evaluate whether additional transarterial embolization is of benefit for children with KHE and KMP. Seventeen patients with KHE and KMP acquired from 11 hospitals in Germany were retrospectively divided into two cohorts. Children being treated with adjunct transarterial embolization and systemic sirolimus, and those being treated with sirolimus without additional embolization. Bleeding grade as defined by WHO was determined for all patients. Response of the primary tumor at 6 and 12 months assessed by magnetic resonance imaging (MRI), time to response of KMP defined as thrombocyte increase >150 × 103/μL, as well as rebound rates of both after cessation of sirolimus were compared. N = 8 patients had undergone additive embolization to systemic sirolimus therapy, sirolimus in this group was started after a mean of 6.5 ± 3 days following embolization. N = 9 patients were identified who had received sirolimus without additional embolization. Adjunct embolization induced a more rapid resolution of KMP within a median of 7 days vs 3 months; however, tumor response as well as rebound rates were similar between both groups. Additive embolization may be of value for a more rapid rescue of consumptive coagulopathy in children with KHE and KMP compared to systemic sirolimus only. What's new? Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor in children. Up to 70% of these patients also develop a life‐threatening, consumptive coagulopathy known as Kasabach‐Merritt Phenomenon (KMP). Sirolimus is proving to be an effective therapy for both KHE and KMP. However, it can take several weeks for sirolimus to have an effect. In this study, the authors found that initial treatment with embolization provides a rapid rescue from the symptoms of KMP. Image‐guided, transarterial embolization may thus provide a valuable adjunct to sirolimus therapy of KHE/HMP. [ABSTRACT FROM AUTHOR]

Background: Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis.Methods: The clinical features and imaging findings were retrospectively reviewed in 64 patients with pathological diagnosis of KHE.Results: Of the 64 patients diagnosed with KHE, 36 patients were < 6 months and 28 patients were ≥ 6 months. The most common presenting features were Kasabach-Merritt phenomenon (KMP, 42.2 %), visible cutaneous lesions (90.6 %), oedema or swelling (43.8 %) and destructive changes or remodelling of adjacent bone (42.2 %). Compared with patients in the group ≥ 6 months, patients in the group < 6 months have higher odds of KMP (P = 0.000), infiltrative lesion with ill-defined borders (P = 0.044). The group ≥ 6 months have higher odds of destructive changes or remodelling of adjacent bone (P = 0.002). In all patients, the lesions in all of the 64 patients were hypointense or isointense compared with muscle on T1-weighted sequences, and hyperintense on T2-weighted or inversion-recovery sequences, nine patients (14.1 %) showed vascularity. There were 28 patients (43.8 %) with characteristic enhancing and infiltrative soft-tissue thickening.Conclusions: Presence of visible cutaneous lesions with ill-defined borders, destructive changes or remodelling of adjacent bone, severe thrombocytopenia and consumptive coagulopathy should favour the diagnosis of KHE. [ABSTRACT FROM AUTHOR]

We present a case of a neonate born with kaposiform hemangioendothelioma (KHE), complicated by Kasabach-Merritt phenomenon (KMP) and other serious conditions, who was successfully treated with sirolimus. In addition to complications from thrombocytopenia and fluid overload, during the course of therapy, our patient experienced supratherapeutic drug levels at the commonly accepted starting dose of sirolimus. Patients with KHE and KMP should be closely monitored for potential complications of both the initial disease and unexpected side effects of treatments.

Kasabach-Merritt Phenomenon (KMP) is a potentially life-threatening condition characterised by thrombocytopenic consumptive coagulopathy. KMP is almost exclusively associated with two uncommon vascular tumours, Kaposiform haemangioendotheliomas (KHE) and tufted angiomas (TA). It is clinically characterized by a rapid increase in the size of the pre-existing vascular plaque and deranged blood coagulation profiles. Early recognition and treatment is of crucial importance. Hemodynamic stability should be achieved as soon as possible with the use of fresh frozen plasma infusions. Surgical excision and embolization, when feasible, are the line of treatment. Oral prednisolone with or without vincristine are most commonly used and effective treatment. Oral sirolimus has shown its efficacy and safety in recent reports and-series. Propranolol and anti-platelet drugs are commonly used as a second-line therapy. Other drugs and combination therapies are used in non-responsive recalcitrant cases.

A 76-year-old woman was referred to our hospital due to massive gingival bleeding following teeth extraction. Laboratory findings suggested disseminated intravascular coagulopathy (DIC). Enhanced computed tomography and magnetic resonance imaging disclosed multiple hypervascular liver masses of 2-6 cm in diameter, the largest of which displaying an irregular enhancement pattern. We considered that her DIC was caused by the multiple liver masses and commenced repeated erythrocyte/fresh frozen plasma infusion and gabexate mesilate administration. However, the DIC proved uncontrollable and trans-arterial embolization could not be attempted. The patient eventually died 4 months after admission due to spontaneous hepatic tumor rupture and hepatic failure. Post-mortem hepatic tumor biopsy led to a final diagnosis of hepatic angiosarcoma with Kasabach-Merritt phenomenon (KMP). Among the 7 cases of hepatic angiosarcoma representing KMP found in the literature, mortality occurred within 4 months of the appearance of bleeding tendency primarily due to abdominal bleeding and hepatic failure. The possibility of hepatic angiosarcoma should be considered in patients with DIC and hypervascular liver tumors. Since treatment is uncertain and prognosis is poor, novel diagnostic and therapeutic advances are needed for angiosarcoma.