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Kaposiform hemangioendothelioma (KHE) is an aggressive disease with high morbidity and mortality. The aim of this study was to retrospectively evaluate the efficacy and safety of sirolimus for the treatment of progressive KHE. A multicenter, retrospective cohort study was conducted in patients with progressive KHE treated with sirolimus. A total of 52 patients were analyzed. Thirty-seven (71%) patients exhibited Kasabach-Merritt phenomenon (KMP) and were significantly younger than the patients without KMP (95% confidence interval [CI], 14.39-41.61; P 0.05). No patients permanently discontinued treatment due to toxicity-related events, and no drug-related deaths occurred. Sirolimus was effective and safe for the treatment of progressive KHE. Sirolimus may be considered as a first-line therapy or as part of a multidisciplinary approach for the treatment of KHE. This article is protected by copyright. All rights reserved.

Primary pancreatic tumours are a rare and unusual entity in children. In this article, we present the case of an 8-month-old girl who presented with obstructive jaundice. The differential diagnosis based on imaging studies was that of a pancreatic vascular neoplasm; however, with the laboratory evidence of Kasabach–Merritt phenomenon (KMP), this prompted the diagnosis of pancreatic kaposiform hemangioendothelioma. A core biopsy of the pancreatic mass was taken at laparotomy and confirmed this diagnosis. The pancreas is an exceedingly rare site of occurrence for this tumour, with only nine cases being published to date. The clinical, biochemical, imaging and pathological findings are discussed to highlight a rare and potentially life-threatening vascular tumour.

Kaposiform hemangioendothelioma (KHE) was first described by Zuckerberg et al in 1992.1 KHE is defined as a rare, locally aggressive infiltrative vascular neoplasm that typically occurs during infancy and childhood. KHE generally originates on the skin as a distinctive cutaneous lesion with ill-defined borders, later affecting deeper tissue by infiltrative growth.1, 2 This lesion occurs most commonly over the extremities and other sites such as the head, neck, trunk, and retroperitoneal or thoracic cavity.3, 4 According to a previous case series at a large referral center, the incidence of KHE is estimated at 0.07 per 100,000 children per year.2 KHE is commonly associated with Kasabach-Merritt phenomenon (KMP). KMP is triggered by intralesional platelet trapping within a vascular tumor leading to profound thrombocytopenia and consumptive coagulopathy.4, 5, 6 KHE is associated with a relatively high mortality rate (around 30%). However, deaths are almost always related to local invasion and compression of vital structures or are a result of KMP.1, 3 To date, it is particularly challenging to treat KHE complicated by KMP, as no controlled trials have been conducted to describe variable responses of the therapeutic options for this relatively rare neoplasm. According to the consensus-derived practice standards plan for complicated KHE published in 2013 there are several treatment modalities for KHE complicated by KMP, such as corticosteroids, vincristine, intravascular embolization, and surgery. These treatments are described with variable responses and many side effects.7 Recently, sirolimus, a mammalian target of rapamycin inhibitor, was reported to be effective and safe and seems to be a promising agent in treating patients with life-threatening refractory KHE.4, 6, 8 We report for the first time, to our knowledge in the Middle East, a successful outcome using oral sirolimus to treat refractory KHE complicated with KMP in a neonate who did not respond to multiple medical therapies.

Purpose To investigate the effectiveness and application of transcatheter arterial embolization (TAE) plus systemic vincristine for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants. Materials and Methods TAE was performed in 17 infants (average age, 4.3 mo ± 2.4; range, 1–10 mo) with corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon, followed by intravenous vincristine once weekly for systemic chemotherapy. The effects and complications were observed and evaluated after a cycle (1 cycle: TAE plus treatment with vincristine every 4 weeks). Cycles were repeated in infants with platelet counts 9 /L. Results In 17 patients, 36 treatment cycles were successfully performed. The platelet count for all patients increased to ≥ 100 × 10 9 /L for the first time at 6.0 days ± 3.5; the platelet level of 15 infants was maintained at levels > 150 × 10 9 /L at 57.5 days ± 16.5. Before treatment, two infants had a normal fibrinogen level (2.21 g/L and 2.34 g/L); the fibrinogen level in the other 15 infants was first found to be increased to ≥ 2.0 g/L at 7.0 days ± 3.4 and was stabilized at levels > 2.0 g/L at 55.9 days ± 13.8 after treatment. Complications were graded as major in four cases and as minor in 13 cases. Conclusions TAE plus vincristine can rapidly improve levels of platelets and fibrinogen, and it is an effective method for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants.

Vascular anomalies, comprised of vascular tumors and malformations, are frequently associated with coagulopathy. Recognition of and familiarity with these vascular anomaly-associated hematologic abnormalities prior to surgery or interventional procedures is essential for pre-operative pre-operative planning. Complicated coagulopathies present within the framework of either Kasabach-Merritt phenomenon (KMP) or localized intravascular coagulopathy (LIC), and their management benefits from the expertise of a hematologist for optimal intra- and peri‑operative care. Furthermore, with the recent broadening of understanding of vascular anomalies and the addition of new classification sub-groups, distinctions of these two classic coagulopathy phenotypes have been recognized. This review summarizes the main features of these coagulopathies, described according to their vascular anomaly type, highlighting clinical aspects relevant to surgical management.

We describe our experience with a 12 year-old girl with kaposiform hemangioendothelioma accompanied by Kasabach–Merritt phenomenon with exacerbation of the disease 10 years after the initial diagnosis. Kaposiform hemangioendothelioma infiltrated into the subcutaneous tissue of the facial skin with deterioration of coagulopathy despite conventional therapies including corticosteroid, vincristine, and propranolol. Sirolimus, a mammalian target of rapamycin inhibitor, produced rapid and dramatic improvement of the Kasabach–Merritt phenomenon and kaposiform hemangioendothelioma shrinkage. Eventually, multifocal lesions of kaposiform hemangioendothelioma disappeared on the images of magnetic resonance imaging and have remained in remission for 27 months after sirolimus cessation. We demonstrated that the AKT/mammalian target of rapamycin signaling pathway played a pivotal role in the kaposiform hemangioendothelioma growth. Sirolimus must be a strong candidate for molecular therapy targeting kaposiform hemangioendothelioma.

Kasabach-Merritt phenomenon (KMP) occurred uniquely in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We report the clinical characteristics of two patients with KHE involving the right upper arm. The patients demonstrated rapid enlargement of the lesion with severe KMP shortly after vaccination. Sirolimus was used to treat the KHE with KMP. The patients showed a quick normalization of the platelet level. The follow-up examination revealed that the size of the mass was significantly decreased. This report raises the intriguing possibility that extrinsic factors may contribute to the development of KMP in the context of an already existing KHE.

Kasabach-Merritt phenomenon (KMP) is a very rare life-threatening condition that combines a vascular tumor belonging to the kaposiform hemangioendothelioma (KHE) spectrum lesions, and thrombocytopenia.1,2 Thrombocytopenia results from platelet trapping within the tumor. Platelet activation leads to various degrees of decreased fibrinogen and elevated D-dimer levels. This coagulopathy is frequently protracted after thrombocytopenia resolution. Several treatments have been proposed with variable efficiency. This article is protected by copyright. All rights reserved.

Kaposiform hemangioendothelioma is a rare, potentially life-threatening vascular tumor often associated with a coagulopathy known as Kasabach–Merritt phenomenon (KMP). We report the case of an infant with satellite lesions around a large vascular tumor and associated KMP.

Background Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. We retrospectively reviewed 11 KMP cases treated over the past 13 years in our institute. Observations With the exception of 1 case, steroids were administered as the first-line therapy. Eight cases required second-line or third-line therapy. The effective salvage therapies include interferon (n=1), radiotherapy (n=1), and chemotherapy (n=5). One case continues to depend upon chemotherapy. Three refractory cases were therapy dependent over 1 year of age, whereas 8 were treated effectively by 6 months of age. Conclusions Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.

Kaposiform hemangioendothelioma (KHE) associated with Kasabach-Merritt phenomenon is a life-threatening vasculopathy. The current mainstay treatment for KHEs is corticosteroids and chemotherapy, but these medications do not work for all patients and carry significant side effects. We report a neonate with a large congenital KHE who responded extremely well to low-dose radiation therapy.

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.

Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This review highlights the clinical presentation, histopathology, management, and treatment of KMP associated with kaposiform hemangioendothelioma, and less frequently tufted angioma. A classic clinical case is described to illustrate the presentation and our management of a patient with KMP.

Kasabach–Merritt phenomenon (KMP) is a severe thrombocytopenic coagulopathy which usually occurs in the presence of enlarging vascular tumors such as kaposiform hemangioendothelioma (KHE) and tufted angioma. The treatment for this potentially fatal condition is challenging without a consensus on appropriate management. The authors report two cases of KHE with KMP, wherein improvement in size of tumor and coagulopathy occurred after treatment with prednisolone, vincristine, and aspirin.

Bei dem Patienten fiel am 4. Lebenstag ein tastbarer Strang links zervikal auf, mit Grosenprogredienz im Verlauf. Bei nicht verschieblicher, nicht druckdolenter, blaulich schimmernder, submandibularer Halsschwellung und intermittierendem biphasischem Stridor wurde anhand der Magnetresonanztomographie, der Probeexzision und der Blutwerte ein Kasabach-Merritt-Phanomen bei kaposiformem Hamangioendotheliom diagnostiziert. Mit Beseitigung des Tumors verschwindet auch die Verbrauchskoagulopathie.

The objective of this article is to provide a comprehensive overview of the Kasabach-Merritt Phenomenon. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology are discussed.

A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach-Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.

Kaposiform hemangioendothelioma is a very infrequent, locally aggressive vascular neoplasm, characterized by fascicular spindle cell proliferation. It occurs almost exclusively in infants and adolescents, and is often associated with Kasabach-Merritt phenomenon. The tumor is predominantly located subcutaneously or in the deep soft tissue of the extremities and trunk, peritoneum, or retroperitoneum. However, this tumor can sometimes be located on the head and neck. We report a case of kaposiform hemangioendothelioma of the maxillary sinus in a 4-month-old female infant presenting with cheek swelling, thrombocytopenia, anemia, and disseminated intravascular coagulation. Sinus computed tomography presented an enhancing, bone-destructing tumor. Magnetic resonance images showed an uncommon appearance as mostly low signal intensity on T2-weighted images.

Kasabach-Merritt phenomenon (KMP) is a rare thrombocytopenic consumption coagulopathy associated with an enlarging tufted angioma or kaposiform hemangioendothelioma. We report a case series of patients with KMP and discuss the treatment options and outcomes. Nine patients with a diagnosis of KMP were identified through retrospective chart review-6 had "definite KMP" and 3 had "less likely KMP." The hematologic features of KMP and those of chronic coagulopathy seen with other vascular malformations can be similar, which makes KMP difficult to distinguish.

Kaposiform haemangioendothelioma is a rare soft-tissue tumour of infants and children, and presents as a moderately aggressive malignancy. We present the MRI findings of a histologically proven case of Kaposiform haemangioendothelioma without Kasabach-Merritt phenomenon or typical skin changes. Our case also reveals that the multiple foci of the cutaneous tumour have different MRI morphologies. These findings have not been reported in the literature to date.

Vascular anomalies may be classified as either vascular malformations or vascular tumors. While the most common vascular tumors, infantile hemangiomas, are often benign and resolve without intervention, they can interfere with vital functions and be life threatening. Rare tumors, such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), can be more aggressive and be associated with Kasabach-Merritt phenomenon (coagulopathy and thrombocytopenia). Given the wide spectrum of disease for all vascular tumors and rare nature of KHE and TA, evidence-based recommendations for treatment do not exist, and management must be individualized, often necessitating consultation with an experienced multidisciplinary vascular anomaly center.

Background. The term "Kasabaha-Merit syndrome" is often used as a eponym for general description of the for coagulation disorders in different vascular lesions in both children and adults. However, subsequent studies show that for certain types of vascular tumors and vascular malformations different types of coagulopathy occur. The aim of the study is to determine which vascular anomalies are at risk for coagulopathy. Methods: 64 patients aged from 1 month to 15 years with vascular anomalies were enrolled in prospective investigation. Of the 26 patients with vascular tumors 20 have hemangiomas, including 6 liver hemangiomas, 2 have kaposhiforme hemangioendothelioma, 1 tufted angioma, and 3 PHACE syndrome. Vascular malformations have 38 patients, 15 of them have cystic lymphatic malformations, 8 have primary lymphedema, 11 have venous malformations, 2 have arteriovenous malformation, and two have CLOVES syndrome. Measurement of D-dimer levels, platelet count, and fibrinogen in blood, witсh was drawn from a peripheral vein not involved by the vascular anomalies. Results. None patients with hemangiomas had any coagulation disorders. Very low platelet count so called Kasabach-Merritt phenomenon had two patients younger than one year with rare local aggressive tumor kaposiform hemangioendothelioma. 14 year old patient with huge tufted angioma of extremities had normal platelet count. Among the 11 patients with venous malformations (VM) 6 (54.5%) had elevated D-dimer levels. Patients with multiple gastrointestinal venous malformations had very high D-dimer lever (≥4 µg/ml) associated with low fibrinogen level. Patients with large unifocal truncal VM and VM of perineum and pelvis had D-dimer levels above 1 µg/ml and normal fibrinogen level. In contrast, patients with small localized VM of limbs and children with lymphatic malformations had normal coagulations tests.Conclusions. Children with vascular anomalies have risk of coagulopathy. Very low platelet count is specific for kaposhiform hemangioendothelioma and tufted angioma and is age dependent. Elevated D-dimer level is highly specific for VMs and don’t depends of patients age only of VMs size., Вступление. Термин «синдром Касабаха-Меритт» неродко используется как общий эпоним для описания коагуляционных нарушений при разных сосудистых поражениях, как у детей, так и у взрослых. Однако, дальнейшие исследования показали, что для для определенных видов сосудистых опухолей и сосудистых мальформаций свойственны различные виды коагуляционных нарушений.Целью исследования было установить, какие сосудистые аномалии имеют риск коагулопатии.Материалы и методы. 64 пациента с сосудистыми аномалиями включены в проспективное исследование. Среди 26 пациентов з сосудистыми опухолями у 20 были гемангиомы, в том числе у 6 – гемангиомы печени, у 2 – капошиформная гемангиоэндотелиома, в 1 – tufted ангиома, и у 3 PHACE синдром. Сосудистые мальформации диагностированы у 38 пациентов, в 15 из них были кистозные лимфатические мальформации, в 8 - первичная лимфедема, в 11 - венозные мальформации, в 2 - артериовенозные мальформации, и у 2 CLOVES синдром. Определяли уровень D-димеров, тромбоцитов и фибриногена в периферической крови, полученной из вены, которая не поражена сосудистыми аномалиями.Результаты. Ни у одного пациента с гемангиомами не обнаружены коагуляционные нарушения. Очень низкое количество тромбоцитов, так называемый феномен Касабаха-Мерит, выявлено у двух детей первого года жизни с локально агресивной опухолью капошиформной гемангиоэндотелиомой. У 14-летнего пациента с гигантской tufted ангиомой конечностей количество тромбоцитов было в пределах нормы. Среди 11 пациентов с венозными мальформациями (ВМ) 6 (54,5%), имели повышенный уровень D-димеров. У пациентов с множественными ВМ желудочно-кишечного тракта уровень D-димеров был очень высоким (≥4 μg / мл), что сочеталось с низким уровнем фибриногена. У пациентов с большими локальными ВМ туловища, промежности и малого таза уровень D-димеров был более 1 μg / мл, уровень фибриногена в пределах нормы. В отличие е пациентов с обширными ВМ, у детей с малыми локализованными ВМ конечностей а также у пациентов с лимфатическими мальформациями коагуляционных нарушений не выявлено.Выводы. Дети с сосудистыми аномалиями имеют риск коагулопатии. Тяжелая тромбоцитопения характерна для капошиформной гемангиоэндотелиомы и tufted ангиомы и имеет возрастную зависимость. Повышение уровня D-димеров является высоко специфическим для ВМ, не зависит от возраста пациента, а определяется только размерами ВМ., Вступ. Термін «синдром Касабаха-Меріт» нерідко використовують як загальний епонім для описання коагуляційних порушень при різних судинних ураженнях як у дітей, так і у дорослих. Проте подальші дослідження доводять, що для певних видів судинних пухлин та судинних мальформацій властиві різні види коагуляцій них порушень.Метою дослідження було встановити, які судинні аномалії мають ризик коагулопатії.Матеріали і методи. 64 пацієнта з судинними аномаліями віком від 1 місяця до 15 років було включено у проспективне дослідження. Серед 26 пацієнтів з судинними пухлинами у 20 були гемангіоми, в тому числі у 6 – гемангіоми печінки, у 2 – капошиформна гемангіоендотеліома, в 1 – tufted ангіома, та у 3 PHACE синдром. Судинні мальформації діагностовано у 38 пацієнтів, у 15 з них були кістозні лімфатичні мальформації, у 8 – первинна лімфедема, в 11 – венозні мальформації, у 2 – артеріовенозні мальформації, та у 2 CLOVES синдром. Визначали рівень D-димерів, тромбоцитів та фібриногену у периферичній крові, отриманій із вени, яка не уражена судинними аномаліями.Результати. У жодного пацієнта з гемангіомами не виявлено коагуляційні порушення. Дуже низька кількість тромбоцитів, так званий феномен Касабаха-Меріт, виявлено у двох дітей першого року життя з локально агресивною пухлиною капошиформною гемангіоендотеліомою. У 14-річного пацієнта з гігантською tufted ангіомою кінцівок кількість тромбоцитів була в межах норми. Серед 11 пацієнтів з венозними мальформаціями (ВМ) 6 (54,5%), мали підвищений рівень D-димерів. У пацієнтів з множинними ВМ шлунково-кишкового тракту рівень D-димерів був дуже високим (≥4 µg/мл), що поєднувалося з низьким рівнем фібриногену. У пацієнтів з великими локальними ВМ тулуба та промежини і малого тазу рівень D-димерів був понад 1 µg/мл, рівень фібриногену в межах норми. На відміну ві пацієнтів з обширними ВМ, у дітей з малими локалізованими ВМ кінцівок а також у пацієнтів з лімфатичними мальформаціями коагуляційних порушень не виявлено.Висновки. Діти з судинними аномаліями мають ризик коагулопатії. Важка тромбоцитопенія є характерною для капошиформної гемангіоендотеліоми і tufted ангіоми і має вікову залежність. Підвищення рівня D-димерів є високо специфічним для ВМ, не залежить від віку пацієнта, а визначається лише розмірами ВМ.