Case report of neonatal kaposiform hemangioendothelioma in the spinal canal and mediastinum with Kasabach-Merritt phenomenon / 中华实用儿科临床杂志

Clinical data of one patient diagnosed with kaposiform hemangioendothelioma (KHE) in the spinal canal and mediastinum at the Children′s Hospital, Zhejiang University School of Medicine in October 2022 were retrospectively analyzed.Additionally, a literature review on mediastinal KHE was performed to summarize prior clinical characteristics and treatments.The patient, a 25-day-old boy, presented with refractory thrombocytopenia.Multiple chest radiographs suggested significant widening of the mediastinum and atypical atelectasis in the left upper lung, and enhanced chest CT and magnetic resonance imaging suggested KHE.After cocktail therapy with Prednisolone, Vincristine and Sirolimus, platelets increased to normal, and coagulation function improved.After 3 months of follow-up, hematologic parameters were stable, the mass became smaller, and no side effects occurred.

Progressive kaposiform hemangioendothelioma and sirolimus-related severe thrombocytopenia

Kaposiform hemangioendothelioma is a locally invasive tumor and we were unable to find any previous reports of multifocal progression. Sirolimus, a mammalian target of rapamycin inhibitor, has been widely used to treat kaposiform hemangioendothelioma. Herein, we report a case of multifocal progressive kaposiform hemangioendothelioma, wherein sirolimus treatment caused severe thrombocytopenia. A 12-year-old East Asian girl presented with indurated dark-purple masses on her back. The patient had received three surgical interventions following the first appearance of the masses in 2012 and subsequent reappearances in 2014 and 2016. Kaposiform hemangioendothelioma was diagnosed based on radiological and pathological findings. Two more masses appeared in the following year. The patient was treated with oral sirolimus (2.5 mg/ m2/day) and developed grade 3 thrombocytopenia 8 days later. The patient was uneventfully relieved 5 days later after the withdrawal of sirolimus and the administration of appropriate medications. This rare case indicated that kaposiform hemangioendothelioma could be progressive with local metastatic characteristics in children. Besides, the severe sirolimus-induced complication highlights the importance of serum drug level monitoring during treatment. Physicians should be extremely cautious while treating kaposiform hemangioendothelioma patients with sirolimus.

Kaposiform hemangioendothelioma in an unusual site: A report of two cases in children

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.

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura / Refractory kaposiforme hemangioendothelioma in the pediatric population: case report and literature review

Resumen Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente caracterizado por una invasión local agresiva y un síndrome de atrapamiento de plaquetas conocido como fenómeno de Kasabach-Merritt. Aunque muchos casos de HEK se tratan con éxito con control local o quimioterapia de baja intensidad, otros son resistentes y se cuenta con pocas opciones terapéuticas. El objetivo de este reporte es mostrar la experiencia del tratamiento con sirolimus por vía oral en un paciente pediátrico con HEK asociado a fenómeno de Kasabach-Merritt refractario al tratamiento de primera línea, quien mostró excelente respuesta al tratamiento. Caso clínico: Paciente de sexo masculino de 3 meses con un HEK refractario al manejo de primera línea (corticoides, propranolol, vincristina), sin posibilidad de hacer control local, por lo que se decide terapia combinada con sirolimus, presentando control local y resolución de la coagulopatía desde la primera semana de iniciado el manejo y con resolución de la malformación vascular después de 12 meses de seguimiento. Conclusiones: Aunque no existen pautas claras para el tratamiento del HEK refractario en la edad pediátrica, la evidencia actual demuestra que el sirolimus es un medicamento eficaz que puede ser considerado como opción terapéutica de primera línea en estos pacientes.

Abstract Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a platelet entrapment syndrome known as the Kasabach-Merritt phenomenon. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often resistant, with few therapeutic options available. Here, we report a pediatric patient with KHE associated with Kasabach-Merritt phenomenon refractory to first-line treatment, who demonstrated excellent response to treatment. Case report: We present the case of a 3-month-old male patient with a KHE refractory to first-line treatment (vincristine, corticosteroids, propranolol), without possibility of local control treatment. Therefore, combined therapy with sirolimus was decided, presenting local control and resolution of the coagulopathy from the first week after starting the management and with resolution of vascular malformation after 12 months of follow-up. Conclusions: Although there are no clear guidelines for the treatment of refractory KHE in the pediatric population, current evidence demonstrate that sirolimus is an effective option that could be considered as a first-line treatment in such patients.

Discussion on the treatment of 106 cases of Kaposiform hemagioendothelioma / 中华整形外科杂志

Objective@#To compare the curative effect of non-surgical treatment, early operation and advanced operation on the Kaposiform hemagioendothelioma (KHE). To explore the optimal treatment for KHE.@*Methods@#The 106 cases of KHE patients admitted by our hospital from January 2004 to December 2016 were selected as the study subjects. The age is the minimum 1d and the maximum 43 years, median age 94.5 d. According to different treatment methods, they were divided into non-surgical treatment and surgical treatment: according to the onset of the disease and the day of the operation, the early operation was performed less than three months since the onset of the disease, and the advanced operation more than 3 months. Efficacy evaluation criteria: The recovery meant that the tumor disappeared or mostly disappeared, the platelets returned to normal, and there was no recurrence more than half a year after treatment. " Effective" indicated that tumors were shrunken significantly and the platelet counting rose significantly. " Ineffective" indicated that the tumor was not shrunken or continued to increase and platelet counting did not rise or continued to decrease.The three groups of patients were followed up, and the efficacy of the three groups after treatment and the changes of platelet counting before and after treatment on day 1, 7 and 14 were compared. The two groups of early and late treatment were compared for operative and postoperative conditions.@*Results@#All patients were followed up for 6-23 months, 24 cases were effective, 12 cases were ineffective, and 5 cases died. After 7 days of treatment, the platelet counting of the three groups were 68 (31, 157)×109/L in the non-surgical treatment group, (294.75±150.59)×109/L in the early operation group, (221.48±148.71)×109/L in the late operation group, with statistical significance (P < 0.05). After 14 days of treatment, the platelet counting of the three groups were 78 (25.51, 54.5) in the non-surgical treatment group, 281 (234, 356)×109/L in the early operation group, and in the late operation group (219.67±140.95)×109/L, with statistical significance (P < 0.05). After at least half a year of follow-up, the total effective rate of clinical efficacy in the three groups was the non-surgical treatment group 62.07%, the early operation group 96.34% and the late operation group 81.82%; the difference was statistically significant between two groups (P < 0.05); There was significant difference between the early operation group and the late operation group in the amount of bleeding and operation time, the time of platelet counting and the total hospitalization time (P< 0.05).@*Conclusions@#Early surgical treatment of Kaposiform hemangioendothelioma is superior to late-operative and non-surgical treatment.Surgical treatment will be the preferred option for treatment of Kaposiform hemangioendothelioma, and examinations should be operated to potential patients for early diagnosis.

The application of multi-point puncture D2-40, CD31, CD34 staining for autologous skin grafting in Kaposiform hemangioendothelioma / 中华整形外科杂志

Objective@#To explore the feasibility of application of multi-point puncture immunohistochemical to determine the invasion extent of the epidermis in Kaposiform hemangioendothelioma before surgery and choose the surgical method.@*Methods@#From July 2013 to April 2016, 17 patients with Kaposiform hemangioendothelioma were admitted to the People′s Hospital of Zhengzhou University, including 7 males and 10 females. The first consultation age was 75.60±31.55 days. Preoperative multi-point pathological puncture D2-40, CD31, CD34 immunohistochemical staining was to test the invasion extent of the epidermis in Kaposiform hemangioendothelioma and to determine whether undergo autologous skin grafting. Skin graft survival areas were recorded postoperatively. Platelet values were examined after 1 week, 4 weeks, 12 weeks and 24 weeks.@*Results@#In these 17 cases, 15 cases with epidermis and dermis unaffected was performed in situ grafting. 2 cases with the tumor invasion, superficial skin grafting were repaired. In these 15 cases of in situ grafting, platelet values returned to normal at 1 week after the operations, and platelet values were higher than 100×109/L at 4 weeks, 12 weeks and 24 weeks. Among them, 12 cases of epidermal survival area were greater than 90%, 2 cases were 70%-90%, and 1 cases was about 50%.@*Conclusions@#During the complete resection of Kaposiform hemangioendothelioma, the multi-puncture D2-40, CD31 and CD34 staining are used to determine invasion extent of the tumor to the skin, which can maximize the retention of normal tissue surface, reduce postoperative complications. This is a new multi-disciplinary surgical approach.

The relationship between the expression of GRα, GRβ and hormone sensitivity in KHE children with KMP / 中华整形外科杂志

To investigate the relationship between glucocorticoid receptor (GRα, GRβ) expression and hormone sensitivity in kaposiform hemangioendothelioma (KHE) patients complicated by Kasabach-Merritt phenomenon (KMP).Methods@#In this study, 25 cases of KHE with KMP (mean age 2.4±1.5 months), including 9 males and 16 females at Henan Provincial People′s Hospital between May 2013 and May 2016 were included. All patients underwent surgical resection after being treated with hormone for one week, and were divided into sensitive group (10 cases) and resistance group (15 cases) according to the efficacy evaluation criteria. Normal tissues collected from 15 patients received surgical excision of lipoma was performed as a control group.Immunohistrochemical SP method was adopted in detecting GRα and GRβ expression in all groups. The expression intensity and positive rate were analyzed. Statistical significance was determined using the Wilcoxon rank sum test for the group samples and the Kruskal-Wallis test for multiple samples. Values of P<0.05 were considered statistically significant.@*Results@#①There was no significant difference among the control group, the resistance group and the sensitive group in expression intensity of GRα (control group 4.20±1.01, resistance group 4.10±0.99, sensitive group 3.53±0.52, P=0.632). The number of GRα positive cells in the sensitive group(46.20±2.57)was higher than that in the resistance group (40.93±5.18, P=0.032). ②The expression intensity of GRβ in the resistance group(5.40±0.51)was significantly higher than that in the sensitive group(2.60±0.52)and the control group(2.87±0.64, P=0.000, 0.002); there was significant difference among the sensitive group(29.70±2.50), the control group(36.07±3.47)and the resistance group (47.27±5.59)in the number of GRβ positive cells (P=0.000). ③The ratio of GRα/Grβ expression intensity was significantly lower in the resistance group than in the sensitive group and the control group; The ratio of GRα/GRβ positive cells was gradually decreased in the sensitive group, control group and resistance group, and the difference was statistically significant (P=0.000).@*Conclusions@#In the tumor tissue of KHE children with KMP, the increase of GRβ expression negatively regulats GRα, leading to imbalance of the expression ratio of the two, which may be an important cause for GC resistance.

Recent advances in the diagnosis and treatment of Kasabach-Merritt phenomenon / 国际儿科学杂志

Kasabach-Merritt phenomenon (KMP),also known as Kasabach-Merritt syndrome,is a rare syndrome associated with Kaposiform haemangioendothelioma or tufted angioma,and characterized by thrombocytopenia and consumptive coagulopathy.KMP onsets early and progresses quickly.If diagnosis and treatment delayed,it can be life-threatening.In this paper,the progress of clinical manifestations,pathogenesis,diagnosis and treatment of KMP are summarized.

Kaposiform hemangioendothelioma: a clinicopathological analysis of 29 cases / 临床与实验病理学杂志

Purpose To explore the clinicopathologic features, diagnosis and differential diagnosis of Kaposiform hemangioendothelioma. Methods To reported the histopathological and immunohistochemical features in 29 cases of Kaposiform hemangioendothelioma, combined with the clinical data and the literature review. Results Of the 29 patients, 15 were male and 14 were female, almost all patients were no more than 1 year old, except one patient aged 25 years.28 cases occurred in superficial skin, including 15 cases in limbs, 5 cases in head and neck, 4 cases in trunk, 1 case in armpit, 3 cases were multiple sites, and 1 case in pelvic cavity. The clinical manifestations were limited or diffuse skin painless plaque, and 16 cases with Kabasach-Merritt phenomenon. Microscopically, the tumor was composed of proliferating small blood vessels and spindle cells, with irregular nodular or lobulated distribution, and invasive growth. Spindle cells were arranged in bundles, with epithelioid cells and spindle cells arranged in nests, and glomerulus-like structures, and the latter with crescent shaped vascular fissures. Immunohistochemically, the tumor cells were strongly positive for VEGFR-3, CD31, CD34 and ERG. Partial tumor cells were positive for SMA and D2-40, but negative for CK, Glut-1 and HHV8. Conclusion Kaposiform hemangioendothelioma is a rare, intermediate, locally aggressive angiogenic tumor, mainly occurring in infants and young adults. The diagnosis relies on its unique morphology and immunophenotype, and it should be differentiated from tufted angioma, infantile hemangioma, congenital hemangioma, spindle cell hemangioma, verrucous venous malformation and Kaposi sarcoma. Extended resection is the best way to treat Kaposiform hemangioendothelioma.

Research progress of Kaposiform hemangioendothelioma / 中华实用儿科临床杂志

Kaposiform hemangioendothelioma(KHE) is a kind of organization form is similar to Kaposi's sarcoma(KS) and aggressive cancer,found in children and infants are rare and multiple in the limbs,torso,facial skin and deep soft tissue can also occur in the mediastinum,retroperitoneal etc.KHE is one of the main causes of Kasabach Meritt phenomenon(KMP) in infants and young children,the mortality rate is as high as 10％-37％,even 52％.At present,there is no uniform and effective treatment method at home and abroad.The clinical manifestations,diagnosis and treatment of KHE are reviewed.

Clinical analysis on 13 cases of Kaposiform hemangioendothelioma / 中华实用儿科临床杂志

Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma (KHE),deepen the understanding of KHE and Kasabach-Merritt phenomenon (KMP),and discuss the optimal treatment for KHE.Methods From January 2008 to August 2016,13 cases of KHE were confirmed by surgery or biopsy pathology and admitted to the First Affiliated Hospital of Sun Yat-Sen University,and the clinical diagnosis and treatment were analyzed retrospectively.Results There were 13 patients(7 males and 6 females) with a median age of 1.0 years(0.2-10.0 years),and 84.6%(11/13 cases) were infants and young children,76.9% (10/13 cases) involved with deep tissue,23.1% (3/13 cases) were associated with KMP and they were younger than 1 year old,and 15.4% (2/13 cases) coexisted with hemangioma or lymphangioma.The location,extent and infiltration depth of the lesion were observed by imaging examinations and histopathology showed nodule shaped spindle tumor cells.Radical resection was considered if possible.Dose of Vincristine (0.5 mg/m2 weekly) and Propranolol [1 mg/(kg·d)] were administered.The prognosis was different in thirteen cases undergoing different treatments.After 3 months to 9 years follow-up,41.7% (5/12 cases) survived after tumor treatment.Conclusions KHE happens mostly in infants and young children,with varying clinical manifestations and a high recurrence rate.The diagnosis of KHE is based on histological examination,computed tomography and magnetic resonance imaging while it still need explicit pathological diagnosis is needed.KHE may be accompanied by hemangioma or lymphangioma.Prognosis is affected by many factors and the comprehensive treatment is required.KMP should be remedied preferentially,individual treatment protocol and long term follow-up are necessary.

Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3‑year‑old female child, uncomplicated by Kasabach–Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.

Angioma en penacho / Tufted angioma

El Angioma en Penacho es un raro tumor vascular de la infancia, que comparte características con el hemangioendotelioma kaposiforme, teniendo ambos posibilidad de desarrollar Síndrome de Kasabach-Merrit. Presentamos una paciente portadora de un angioma en penacho desde el nacimiento, que hemos seguido por 13 años: clínicamente, con estudios de imágenes y biopsias. El caso presenta similitudes con la literatura, con algunas interesantes peculiaridades en su evolución. Consideramos que por el comportamiento clínico e histológico, el Angioma en Penacho es parte de la misma enfermedad que el Hemangioendotelioma Kaposiforme.

Tufted angioma is a rare vascular tumor of childhood that shares features with the Kaposiform hemangioendothelioma, having both tumors the possibility of developing Kasabach-Merrit phenomenon. We report the case of a patient with a tufted angioma since birth, which we have followed for 13 years: clinically, with imaging studies and biopsies. The case presents similarities with literature, with some interesting peculiarities in its evolution. We consider that because of the clinical and histological behavior, Tufted angioma is a part of the same disease that Kaposiform hemangioendothelioma.

A case report:Alimentary track hemorrhage caused by mesentery Kaposiform hemangioendothelioma and literature review / 实用肿瘤学杂志

Kaposiform hemangioendothelioma ( KHE ) is a vascular tumor characterized by intermediate malignancy.Retrospective analysis and literature review on the clinical pathologic,immunohistochemical and clini-cal data of a patient with mesentery KHE.The cardinal symptom of this patient is alimentary track hemorrhage, imageological diagnosed as a huge soft tissue mass of mesentery and invaded ileum.Clinical feature is without Ka-sabach-Merritt phenomenon,observing under the light microscope.The tumor is with cavernous vascular structure, and lymphocyte infiltration inside of stroma.The nodules are composed of disposed and lots of short spindle cell tumor;the tumor cells inside of the nodules are vertical and horizontal staggered and form tendon like or fissuring vessels.Immunohistochemistry:CD31(+),CD34(+),D2-40(+),C-Kit(-),SMA(-),Ki-67(1%).

Development and treatments of Kasabach-Merritt phenomenon / 中华实用儿科临床杂志

Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia with enlarging vascular tumour,KMP usually develops in infancy and is associated with significant morbidity and mortality,the mortality rate is reported as high as 30％.It commonly reported sites of tumor include extremities,trunk,retroperitoneum and neck.There is no consensus in treatment and various regimens have been used by different authors.This report is aim to learn the pathophysiology of the KMP and its diagnosis and treatment.

Multifocal kaposiform hemangioendothelioma of soft tissue with bilateral pulmonary involvement in an adolescent / 소아과

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.

Sirolimus (rapamicina) en pacientes con hemangioendotelioma kaposiforme: caso clínico / Sirolimus (rapamycin) in patients with kaposiform hemangioendothelioma: clinical case

Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente, que se asocia a coagulopatía grave, conocida como fenómeno de Kasabach-Merritt (FKM). Puede ser letal en 20 por ciento de los casos. Se han descrito múltiples tratamientos, pero a pesar de las complicaciones potencialmente severas que pueden presentar estos pacientes, no hay hasta ahora un protocolo único. Existen experiencias internacionales, en modelos experimentales, con el uso de Sirolimus (SRL), macrólido, inhibidor de mTOR, con actividad antiangiogénica. Sin embargo, la experiencia de su uso en niños en el tratamiento de anomalías vasculares como el HEK es limitada. Objetivo: Reportar la evolución de dos pacientes con HEK y FKM tratados con SRL en centros oncológicos, después de múltiples fallas con otras terapias. Casos clínicos: Reportamos dos recién nacidos con HEK profundo asociado a FKM, refractarios a terapias habituales y que evolucionaron con complicaciones amenazantes de la vida, en quienes se inicio tratamiento con SRL. El uso de SRL permitió que los dos pacientes mostraran mejoría en su estado clínico, con normalización del recuento de plaquetas y de las pruebas de coagulación, disminución del tamaño de la lesión, del dolor asociado, desaparición del riesgo vital y sin efectos adversos. Conclusión: SRL parece ser efectivo y seguro en el tratamiento de HEK, representando una nueva opción terapéutica, disponible en nuestro medio.

Introduction: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor, which is associated with the severe coagulopathy known as Kasabach-Merritt phenomenon (KMP). This condition has a mortality rate around 20 percent. Many treatments have been described, but despite the potentially severe complications that these patients may present, so far there is not a single protocol to follow. International experimental models have used Sirolimus (SRL), a macrolide mTOR inhibitor with antiangiogenic effect. However, its use in children against vascular anomalies, such as KHE, is limited. Objective: To report the development of two patients with KHE and KMP treated with SRL in cancer centers, after multiple failures with other therapies. Case reports: Two infants with severe KHE associated to FKM, resistant to standard therapies and who overcame life-threatening complications after treatment with SRL are presented. After SRL, the two patients showed normal platelet counts and coagulation tests, reduced lesion size and associated pain, no presence of life threatening conditions or side effects. Conclusion: SRL appears to be effective and safe in treating KHE, representing a new available therapeutic option.

Tratamiento con vincristina de tres tumores vasculares con fenómeno de Kasabach-Merritt / Vincristine treatment of three vascular tumors with Kasabach-Merritt phenomenon

El fenómeno de Kasabach-Merrit (FKM) es una coagulopatía por consumo que se presenta en las primeras semanas de la vida. Se caracteriza por presentar coagulopatía por consumo, con trombocitopenia y anemia hemolítica microangiopática, asociado a un tumor vascular. El objetivo de este trabajo es presentar tres pacientes con tumores vasculares que manifestaron FKM y fueron tratados con vincristina como droga de segunda línea, atendidos en el Servicio de Dermatología del Hospital de Pediatría J. P. Garrahan. Dos pacientes presentaron el tumor al nacimiento y otro a partir de los dos meses de vida. Dos fueron niñas y uno varón.Todos los pacientes tenían una trombocitopenia severa (3 000/mm_), bajos niveles de fibrinógeno y dimero D elevado. Los tumores estaban localizados en región proximal de miembro inferior, tronco y miembro superior, y región cervical. Ninguno de nuestros pacientes tuvo compromiso de órganos internos. El diagnóstico histológico en dos de ellos fue de hemangioendotelioma kaposiforme (HEK). Los corticoides fueron el tratamiento de primera linea: metilprednisona 3mg/kg/día por vía oral. En un paciente el tumor continuó creciendo a pesar de haber asociado interferón alfa 2 a la corticoterapia y realizarle secundariamente una embolización. Dada la falta de respuesta clínica y hematológica, se decidió iniciar tratamiento con vincristina 1mg/m2/dosis/IV semanal. Todos los pacientes normalizaron los parámetros hematológicos, con franca mejoría clínica, dos pacientes a la quinta y otro a la sexta dosis de vincristina, con involución gradual del tamaño del tumor. Ninguno de los pacientes presentó complicaciones secundarias al tratamiento ni recidivas de su enfermedad a la fecha. La duración promedio de tratamiento fue de 35±6 días. Podemos concluir que el uso de vincristina es considerado en la actualidad una droga de segunda línea en el tratamiento de tumores vasculares con FKM (AU)

Kasabach-Merritt phenomenon (KMP) is a consumptive coagulopathy that typicallly presents in the first few weeks of life. It is characterized by a triad of vascular tumor, thrombocytopenia and coagulopathy. We reviewed the clinical and hematologic data and response to therapeutic with vincristine in three patients who had a vascular tumor and KMP at the Dermatology Department of Hospital de Pediatría J. P. Garrahan.Tumors were present at birth in two patients and in one at two months old. Two were girls and one was a boy. All patients had severe thrombocytopenia (Lowest platelets count 3 000/mm_), consumption of fibrinogen and lower D-dimer levels.Tumors were localized on proximal lower limb, trunk and upper limb and cervical area. None of our patients had internal involvement. Histopathology finding in two of them was kaposiform hemangioendothelioma. First line of treatment was prednisolone 3-5mg/kg/day. In one patient the tumor size continued to increase in spite of simultaneous treatment with corticosteroid and interferon alfa-2a plus embolization. After corticosteroids treatment failure, correction of coagulopathy and tumor regression occurred in our three patients after 5 to 6 doses of vincristine 0.5-1mg/m_ IV weekly with almost complete tumor regression and correction of coagulopathy.The average duration of treatment was 35±6days. None of the patients developed complications due to this intervention nor experienced recurrence of the tumor.The use of vincristine is currently a second line treatment of vascular tumors with KMP (AU)

Cerebellopontine Angle Kaposiform Hemangioendothelioma: A Case Report / 대한이비인후과학회지

Hemangioendothelioma (HE) is an uncommon vascular neoplasm, and its intracranial occurrence is extremely rare, especially in the pediatric age group. The authors report a 7-month-old male patient with kaposiform HE who presented with left-sided facial paralysis and hearing loss. Initial magnetic resonance imaging (MRI) revealed a small lesion that filled the internal auditory meatus and exhibited the neuroimaging features of a vestibular schwannoma (VS). Six months later, follow-up MRI showed markedly enlarged mass. Biopsy disclosed kaposiform HE. After vascular embolization, partial resection was performed via transmastoid approach due to massive bleeding, followed by medical treatment with steroid and interferon-alpha. The tumor nearly disappeared 6 months after medical treatment. To our knowledge, it is a first reported case of kaposiform HE mimicking a VS in the cerebellopontine angle (CPA) area. A proper diagnosis is important because its treatment differs dramatically from that of VS. So, we report this case with a literature review.