Clinicopathological features of congenital hemangioma: a study of 40 cases / 中华病理学杂志

Objective: To investigate the clinical and pathologic features, diagnosis and differential diagnosis of congenital hemangioma (CH). Methods: Forty cases of CH were diagnosed from January 2017 to December 2020 in Henan Provincial People's Hospital. The clinical and pathological and immunohistochemical data were analyzed, with review of literature. Results: There were 24 male and 16 female patients. The lesions were located in the head, neck (11 cases), limbs (14 cases), and trunk (15 cases). The clinical manifestations were congenital painless plaques or masses, the larger ones protruded on the skin surface, mostly dusky purple or bright red, with surrounding white halos. Under low magnification, the tumor was lobular and well demarcated, composed of neo-microvascular lumen of different sizes. The vascular endothelial cells were cuboidal or hobnail in appearance, forming stellar drainage vessels within the lobules. Extra-medullary hematopoiesis was seen in one case of rapidly involuting CH; there were different number of tortuous and dilated vascular lumen between the lobular structures, and some non-involuting CH cases were vascular malformations, which were devoid of lobulated structures. Immunohistochemistry showed that endothelial cells were strongly positive for CD31, CD34 and ERG, while D2-40 and GLUT-1 were negative. Conclusions: CH is a benign congenital vascular tumor with characteristic lobulated growth and abnormal blood vessels in the stroma. Pathological diagnosis often needs to be differentiated from infantile hemangioma, pyogenic granuloma, kaposiform hemangioendothelioma and vascular malformation.

Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

Síndrome de Kasabach-Merritt esplénico en adulto. Presentación de un caso y revisión del tema / Splenic Kasabach-Merritt syndrome in an adult. Presentation of a case and review of the topic / Síndrome de Kasabach-Merritt esplênica em adulto. Apresentação de um caso e revisão do tema

RESUMEN Se presentó un paciente de 22 años de edad, masculino, de la raza negra con gran esplenomegalia debido a un hemangioma gigante del bazo, el cual presentó trombocitopenia y trastornos de la coagulación. Se estudió y se diagnosticó un síndrome de Kasabach-Merrit. Se le realizó una esplenectomía total anterior regresando a la normalidad el conteo de plaquetas y los trastornos de la coagulación. Su evolución fue favorable al cabo de los 18 meses de seguimiento. Se recomienda el tratamiento quirúrgico, la esplenectomía. Se revisó el tema por lo infrecuente de la enfermedad.

ABSTRACT A 22-year-old male black patient presented with big splenomegaly due to a giant hemangioma of the spleen, showing thrombocytopenia and bleeding disorders. A Kasabach-Merrit syndrome was diagnosed. An anterior total splenectomy was performed, returning the platelet count and coagulation disorders back to normal. His evolution was favorable after 18 months of follow-up. Splenectomy surgical treatment was recommended. The topic was reviewed due to the infrequency of the disease.

RESUMO Foi apresentado paciente do sexo masculino, 22 anos de idade, de raça negra, apresentou grande esplenomegalia devido a hemangioma gigante do baço, que apresentava plaquetopenia e distúrbios de coagulação. Uma síndrome de Kasabach-Merrit foi estudada e diagnosticada. Uma esplenectomia total anterior foi realizada, retornando a contagem de plaquetas e distúrbios de coagulação ao normal. Sua evolução foi favorável após 18 meses de seguimento. Tratamento cirúrgico é recomendado, esplenectomia. O tema foi revisado devido à raridade da doença.

Clinical features of Kasabach-Merritt syndrome: an analysis of 16 neonates / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease.@*METHODS@#A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020.@*RESULTS@#Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates.@*CONCLUSIONS@#KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.

Linfangiomatosis kaposiforme: reporte de un caso y revisión de la literatura / Kaposiform lymphangiomatosis: a case report and literature review

La linfangiomatosis es una patología benigna, de etiología desconocida, que afecta especialmente a población pediátrica, caracterizándose por desarrollo de masas subcutáneas que involucran uno o más órganos. Cuando se asocia con coagulopatía y derrames quilosos, recibe el nombre de linfangiomatosis kaposiforme (LK), cuya evidencia disponible es escasa. El objetivo de este manuscrito es reportar un caso de LK pediátrico atendido en el Instituto del Cáncer SOLCA, Cuenca, Ecuador. Paciente femenina de 1 año 7 meses, con hernia inguinal bilaterales y alteración de los tiempos de coagulación. En los estudios de imagen se evidenció una masa mediastínica asociada a presencia de líquido en espacio pleural y cavidad abdomino-pélvica. Además, se evidenció la existencia de múltiples imágenes hipogénicas en el bazo. Se practicó timectomía mínima, toma de biopsia de linfonodos mediastínicos para establecer el diagnóstico, y se instaló un dren en el tórax para tratar el derrame pleural antes señalado. Se descartaron patologías similares (linfomas, lupus, inmunológicas, etc.). Dado el continúo deterioro respiratorio, coagulopatía, lesiones hipogénicas esplénicas y drenaje abundante del quilotórax por el tubo torácico, se planteó el diagnóstico de LK. Se inició apoyo nutricional vía parenteral (sin lípidos); se indicó vincristina, y se realizó una pleurodesis con bleomicina sin buena respuesta. La paciente falleció a los 16 días de su ingreso. Se trata de un caso de mal pronóstico, con evolución rápidamente progresiva hacia un desenlace fatal.

Lymphangiomatosis is a benign pathology, of unknown etiology. Affects especially to pediatric population and is characterized by development of subcutaneous masses that involve one or more organs. When it presents with coagulopathy and chylous effusions, it is called kaposiform lymphangiomatosis (KL). The available evidence of KL is scarce. The objective of this manuscript was to report a case of pediatric KL treated at SOLCA Cancer Institute, Cuenca, Ecuador. Female patient of 1 year and 7 months age. She had a bilateral inguinal hernia and altered coagulation times. Imaging studies revealed a mediastinal mass associated with fluid in the pleural space and in the abdominalpelvic cavity. Furthermore, the existence of multiple hypogenic images in the spleen was evidenced. Minimal thymectomy, biopsy of mediastinal lymph nodes was performed to establish the diagnosis, and a drain was installed in the chest to treat the aforementioned pleural effusion. Other pathologies like lymphomas, lupus, etc. were ruled out. Given the continuous respiratory deterioration, coagulopathy, splenic hypogenic lesions and abundant drainage of the chylothorax through the chest tube, the diagnosis of KL was raised. Parenteral nutrition (lipid-free) was started; Vincristine was indicated, and a pleurodesis with bleomycin was performed without a good response. The patient died 16 days after her admission. It is a case of very bad prognosis, with a rapidly progressive evolution towards a fatal outcome.

Hemangioma esplênico no adulto associado à síndrome de Kasabach-Merritt / Kasabach-Merritt Syndrome-associated adult splenic hemangioma

O hemangioma esplênico, tumor benigno mais frequente do baço, porém, por vezes de difícil diagnóstico, pode integrar a síndrome de Kasabach-Merritt, afecção rara e potencialmente fatal, descrita como associação entre hemangioma, trombocitopenia e coagulopatia de consumo. Apresentamos um caso de paciente de 73 anos em acompanhamento com a hematologia por trombocitopenia crônica a princípio idiopática, que evoluiu com dor abdominal, anemia e derrame pleural. Foi realizada tomografia computadorizada de abdome, que evidenciou massa esplênica sólida heterogênea. A paciente foi submetida a esplenectomia total videolaparoscópica e evoluiu com melhora da dor abdominal e normalização da série plaquetária, sem recorrência do derrame pleural após o procedimento. A raridade e a complexidade do caso, somadas à dificuldade diagnóstica e à abordagem terapêutica, foram fatores que corroboraram para a apresentação desse caso. (AU)

Splenic hemangioma, the most common, but sometimes hard to diagnose, benign tumor of the spleen may integrate Kasabach- Merritt syndrome, a rare and potentially fatal condition described as an association ofhemangioma, thrombocytopenia and consumption coagulopathy. We present a case of a 73-year-old female patient being monitoring, with Hematology due to chronic idiopathic thrombocytopenia who progressed with abdominal pain, anemia and pleural effusion; a computed tomography scan of the abdomen was performed, showing a heterogeneous solid splenic mass. The patient underwent total videolaparoscopic splenectomy and progressed with improvement of abdominal pain and normalization of the platelet series, with no recurrence of pleural effusion after the procedure. The rarity and complexity of the case added to the diagnostic difficulty and therapeutic approach were factors that corroborated for the presentation of this case. (AU)

Síndrome de Kasabach- Merrit / Kasabach-Merritt syndrome

Introducción: El síndrome de Kasabach Merritt (SKM) es poco frecuente, tiene una alta mortalidad y se caracteriza por una lesión vascular de crecimiento rápido, asociado a coagulopatía de consumo y trombocitopenia. Materiales y métodos: revisión de literatura actual confrontando con artículos de revisiones de temas en búsqueda electrónica en bases de datos de RIMA, MEDLINE, PUBMED, MEDSCAPE, de 1940 a 2017. Resultados Se describe un caso clínico de un niño de 10 meses de edad con Síndrome de Kasabach Merrit sucedido en el Hospital Infantil Napoleón Franco Pareja (HINFP) Conclusiones El síndrome Kasabach-Merritt es una entidad poco frecuente, su diagnóstico inicia desde la sospecha clínica, que incluye al examen físico el hallazgo de un hemangioma acompañado de trombocitopenia y coagulopatía. El manejo no está pautado, se dispone de corticoide como terapia de inicio, interferón, antineoplásico, radioterapia o cirugía, y su pronóstico va a depender de la resolución pronta.

Introduction: The Kasabach-Merritt syndrome (SKM) is rare, has a high mortality and is characterized by a vascular lesion of rapid growth, associated with coagulopathy of consumption and thrombocytopenia. Materials and methods: review of current literature in comparison with articles of reviews of subjects in electronic search in databases of RIMA, MEDLINE, PUB-MED, MEDSCAPE, from 1940 to 2017. Results A clinical case of a 10-month-old child is described. of age with Kasabach Merrit syndrome occurred at the Napoleon Franco Franco Children's Hospital (HINFP) Conclusions The Kasabach-Merritt syndrome is a rare entity, its diagnosis starts from the clinical suspicion, which includes the physical examination the finding of a hemangioma accompanied by thrombocytopenia and coagulopathy. The management is not scheduled, corticoid is available as start therapy, interferon, antineoplastic, radiotherapy or surgery, and its prognosis will depend on the prompt resolution.

Hemangioma hepático congénito gigante: síndrome de Kasabach-Merritt / Giant Congenital Liver Hemangioma: Kasabach Merritt Syndrome

Resumen Los hemangiomas son las proliferaciones más frecuentes en la edad pediátrica, y el hemangioma hepático es de los más comunes entre los viscerales. El diagnóstico precoz es importante para orientar el tratamiento. Se presenta el caso de un neonato con compromiso ventilatorio secundario al efecto de una masa gigante, que ecográficamente correspondió a un hemangioma hepático. Se realizó resección quirúrgica y, ulteriormente, fue enviada a patología, que confirmó el diagnóstico.

Abstract Hemangiomas are the most frequent proliferations in children, and liver hemangiomas are the most common among those in the viscera. Early diagnosis to focus treatment is important. We present the case of a neonate with compromised breathing secondary to the effect of a giant mass that echographically appeared to be a hepatic hemangioma. Surgical resection and subsequent pathology confirmed the diagnosis.

Exome sequence analysis of Kaposiform hemangioendothelioma: identification of putative driver mutations

Abstract BACKGROUND: Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments. OBJECTIVE: In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. METHOD: The genomic DNA for exome sequencing was obtained from the tumor tissue and matched normal tissue from the same individual. Exome sequencing was performed on HiSeq2000 sequencer platform. RESULTS: Among oncogenes, germline missense single nucleotide variants were observed in the TP53 and APC genes in both the tumor and normal tissue. As tumor-specific somatic mutations, we identified 81 candidate genes, including 4 nonsense changes, 68 missense changes and 9 insertions/deletions. The mutations in ITGB2, IL-32 and DIDO1 were included in them. CONCLUSION: This is a pilot study, and future analysis with more patients is needed to clarify: the detailed pathogenesis of this tumor, the novel diagnostic methods by detecting specific mutations, and the new therapeutic strategies targeting the mutation.

Hemangioblastoma cerebeloso y trombocitopenia: reporte de caso / Cerebellar hemangioblastoma and thrombocytopenia: report of one case

The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.

Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient / 대한소화기학회지

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience

BACKGROUND: Kasabach-Merritt syndrome (KMS) is a rare but life-threatening illness. The purpose of this study is to report our single-center experience with KMS. METHODS: We reviewed the medical records of 13 patients who were diagnosed with KMS between 1997 and 2012 at Samsung Medical Center. Treatment response was defined as follows: 1) hematologic complete response (HCR) – platelet count >130×10⁹/L without transfusion; 2) clinical complete response (CCR) – complete tumor disappearance or small residual vascular tumor displaying lack of proliferation for at least 6 months after treatment discontinuation. RESULTS: Participants included 7 male and 6 female patients. The median initial hemoglobin levels and platelet counts were 9.7 g/dL (range, 6.6–11.6 g/dL) and 11×10⁹/L (range, 3–38×10⁹/L), respectively. Twelve patients received corticosteroid and interferon-alpha as initial treatment, and the remaining patient received propranolol instead of corticosteroid. Two patients with unsatisfactory response to the initial treatment received weekly vincristine. Successful discontinuation of medication was possible at a median of 301 days (range, 137–579) in all patients except one who was lost to follow-up. The median times to achieve HCR and CCR were 157 days and 332 days, respectively. The probabilities of achieving HCR and CCR were 77% and 54% at 1 year, and 88% and 86% at 2.5 years, respectively. CONCLUSION: The prognosis of KMS in our cohort was excellent. Our data suggest that individualized treatment adaptation according to response may be very important for the successful treatment of patients with KMS.

One case of nasal Kaposiform hemangioendothelioma / 临床耳鼻咽喉头颈外科杂志

Female patients, 41 years old, with the left nasal cavity bleeding intermittently and left nasal congestion 20 days as the chief complaint to the hospital. Physical examinationindicated dark red mass was at the front-end of left nasal cavity, which has not smooth surface with blood vessels and hemorrhagic secretions, and back up to the middle turbinate. Sinus enhancement 3D-CT showed soft tissue density can be found in the left nasal cavity,Scan CT value is 37-47 HU, and enhanced and delay is about 69-78 HU. Nasal septum,middle turbinate and inferior turbinate bonewas visible damage. The pathologic biopsy of left nasal cavity lesions results conform to the Kaposiform hemangioendothelioma.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

Comprehensive therapy for infant vascular tumor associated with Kasabach-Merritt phenomenon / 中华整形外科杂志

<p><b>OBJECTIVE</b>To summarize the management of infant vascular tumors with Kasabach-Merritt phenomenon (KMP) and to evaluate the effect of drug combined with sclerotherapy.</p><p><b>METHODS</b>From Feb. 2007 to Nov. 2014, 25 cases with KMP, who underwent drug therapy combined with sclerotherapy, were retrospectively studied. Oral corticosteroids (2 mg/kg per day) was used as the first-line therapy on all of the patients and intravenous vincristine (1.5 mg/m2 every week) was added when the platelet counts didn't recover obviously after 2-3 weeks. After the recovery of the platelet counts, the patients were admitted for sclerotherapy (average, 4.56 sessions per case) with 100% alcohol (1-3 ml per session), Lauromacrogol (1.25-5 ml per session) and betamethasone (0.25-1 ml per session). All the patients were followed up for 42 months ( range, 9 months to 6.5 years). Therapeutic outcomes were assessed by evaluating platelet counts, size of lesion, function of trunk and limb.</p><p><b>RESULTS</b>All the 25 cases got obvious recovery in the platelet counts [average, (94.3 ± 18.5) x 10(9)/L] after drug therapy, of which 16 were treated by single oral corticosteroids for 4-7 weeks and 9 were treated by corticosteroids plus intravenous vincristine for 2-5 weeks. Meantime, 11 cases received platelet transfusions, of which 3 were coupled with gamma globulin intramuscularly. During the first admission, each of the 25 cases received 1-4 sessions of sclerotherapy (average, 2.6 sessions each case). One week after the sclerotherapy, the platelet counts returned to (167-312) x 10(9)/L (average, (258.5 ± 34.4) x 10(9)/L). The hemoglobin and blood coagulation function returned to normal within 1-5 weeks. Meanwhile the mental condition, appetite, body weight, sleeping were greatly improved. The size of the lesions decreased gradually after the combined therapy including 13 cases within 3-12 months and 13 cases within 13-36 months. Long term follow-up indicated that only 1 case need treatment for recurrent decrease of platelet counts, and all of the 25 cases kept the normal weight, height, immunity as well as the growing development.</p><p><b>CONCLUSIONS</b>Oral corticosteroids plus intravenous vincristine combined with sclerotherapy is a reliable management with high cure rate, short course and minor side-effect.</p>

Kasabach-Merritt syndrome: clinical vs. surgical treatment / Síndrome de Kasabach-Merritt: tratamento clínico versus cirúrgico

Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient's clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.

A síndrome de Kassabach-Merritt é uma associação de hemangioma capilar e trombocitopenia, que promove sangramentos com petéquias, equimoses e hematomas espontâneos. A conduta é tratar com corticoide, interferon alfa ou quimioterápicos. Apresentamos um caso de criança (com 1 ano e 9 meses anos de idade) com um hemangioma gigante desde a raiz da coxa até altura do joelho, e plaquetopenia. O tratamento foi iniciado com corticoterapia sem melhora do quadro, surgindo de forma espontânea pontos de sangramento intratumoral e cutâneo. Sem condições clínicas de receber vincristina e o interferon, foi feita a ressecção tumoral de urgência por extrema plaquetopenia e sangramento. A criança começou então a desenvolver um quadro séptico com hipotensão e isquemia do tecido residual. Diante deste quadro, configurou-se um desafio terapêutico que será objeto deste artigo.

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.

Intractable Tufted Angioma Associated with Kasabach-Merritt Syndrome

No abstract available.

Intractable Tufted Angioma Associated with Kasabach-Merritt Syndrome

No abstract available.