Kasabach-Merritt Syndrome Research Articles

Successful Management of Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Vincristine and Ticlopidine

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually occurs in skin and retroperitoneum of infants and young children. We present a case of a newborn with a rapid tumor growth and a life-threatening Kasabach-Merritt syndrome with a progressive remission after treatment with vincristine and ticlopidine.

Superextended hepatectomy for resection of multiple giant hemangiomas: Report of a case

Most liver hemangiomas are small, asymptomatic, and require no treatment. Symptoms such as right upper quadrant abdominal pain and fullness are associated only with liver hemangiomas larger than 4 cm in diameter. Serious complications such as jaundice, Kasabach-Merritt syndrome, and rupture are rare. Surgical resection is the only effective treatment, but it is advocated only for patients with incapacitating symptoms or complications. We report a case of successful superextended hepatectomy with resection of segments III-VIII for multiple, bilobar hemangiomas. A 45-year-old woman, who had undergone transcatheter arterial embolization (TAE) for inoperable multiple giant liver hemangiomas 4 years earlier, was referred to our hospital for investigation of abdominal distension and consumption coagulopathy. Because of her severe and progressive symptoms despite treatment, the other hospital had considered her as a candidate for liver transplantation, which she had refused. After careful preoperative assessment of the future liver remnant volume and function, we considered that resection was possible. Based on our review of large surgical series in the literature from 1970, this is the first report of a superextended hepatectomy for a benign liver tumor.

Refractory Kaposiform Hemangioendothelioma That Expressed Vascular Endothelial Growth Factor Receptor (VEGFR)-2 and VEGFR-3

This report describes the case of a 10-month-old boy who was diagnosed to have kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt syndrome (KMS), which is a rare pediatric vascular tumor with a high mortality rate. Although both KHE with KMS were resistant to various therapies, such as oral prednisolone, sclerotherapy, and chemotherapy, repeated radiation therapy with methylprednisolone pulse therapy did reduce the volume of KHE and improved the symptoms of KMS. Unfortunately, a regrowth of KHE with KMS was observed 4 months after the cessation of treatment and the patient thereafter died from an intracranial hemorrhage and Pneumocystis carinii pneumonia, which is a complication related to repetitive radiation and steroid therapy. A histopathologic examination of autopsy specimens confirmed a diagnosis of KHE and immunohistologic staining was positive for vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3. These findings may provide the rationale to further investigate the role of VEGFRs in the pathogenesis of KHE and also to elucidate its prognostic value, along with the application of inhibitors for VEGFRs for the treatment of refractory KHE.

Use of radiotherapy to treat life‐threatening Kasabach–Merritt syndrome

Kasabach-Merritt syndrome involves thrombocytopenia, which is usually severe, in association with haemangiomata. We describe two newborn infants who developed life-threatening Kasabach-Merritt syndrome but who were successfully treated. Both had failed to respond adequately to corticosteroids and alpha-interferon. They subsequently received emergency radiotherapy. Both patients had an improvement in their platelet counts. The first infant's haemangioma gradually faded during the first 3 years of life, whereas the second infant's haemangioma had resolved 2 months after radiotherapy. No significant late effects of the radiotherapy have been noted at the 8 and 5 years follow up respectively. Radiotherapy remains an important treatment method in Kasabach-Merritt syndrome when patients fail to respond to other treatments.

Intracranial kaposiform hemangioendothelioma: proposal of a new malignant variant

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and early childhood that occurs mainly in soft tissue and the retroperitoneum. The pathological characteristics of a KHE are fascicles of spindleshaped endothelial cells and slitlike vascular channels with irregular tumor margins. In spite of benign features such as rare mitoses and a low MIB-1 labeling index, a KHE is categorized as an intermediate malignancy because of local invasiveness into adjacent lymph nodes or organs. Resection is the treatment of choice for this type of tumor and adjuvant medical therapy is prepared for the tumor remnants and the associated Kasabach-Merritt syndrome. In this paper the authors report a case of a KHE without Kasabach-Merritt syndrome arising from the right tentorium cerebelli in a 13-month-old male child. The biological behavior of the tumor, including its pathological traits and clinical course, was malignant in nature. This case may be not only the first intracranial extraaxial KHE, but also the first truly malignant variant. Kaposiform hemangioendothelioma should be considered in the differential diagnosis of intracranial and extraaxial tumors.

The use of recombinant activated factor VII during major surgery in a child with Kasabach-Merritt syndrome

The use of recombinant activated factor VII during major surgery in a child with Kasabach-Merritt syndrome

Atypical Hepatic Hemangioma

Hepatic hemangioma is a common incidental finding discovered with abdominal sonography. These lesions consist of collections of atypical blood vessels; structurally, the irregular vessels create blood-filled cystic spaces separated by fibrous connective tissue and endothelial cells. Although these benign lesions typically appear sonographically as small, hyperechoic, and well-circumscribed hepatic masses, atypical hemangiomas make up around one third of hemangiomas diagnosed with sonography. Giant hemangiomas, in particular, are of special significance to the sonographer because their atypical size may create symptoms that mimic other abdominal pathology, such as gallstones or pancreatitis; they may also lead to potentially deadly complications such as rupture, Budd-Chiari syndrome, or Kasabach-Merritt syndrome. If these large vascular lesions become symptomatic, surgical excision may be necessary. A case of hepatic hemangioma is presented that demonstrates the sonographic features of atypical hemangioma and emphasizes the potential of sonography as a high-quality first-line imaging modality.

SUCCESSFUL TREATMENT OF KASABACH-MERRITT SYNDROME WITH VINCRISTINE AND DIAGNOSIS OF THE HEMANGIOMA USING THREE-DIMENSIONAL IMAGING

Kasabach-Merritt syndrome is a life-threatening congenital disorder characterized by an enlarging hemangioma, thrombocytopenia, and consumption coagulopathy. We report the case of a one-month male infant who presented with a large cutaneous tumor in his right axilla with ecchymosis, thrombocytopenia, and chronic consumption coagulopathy. Three-dimensional computed tomography was useful for accurate diagnosis of the cutaneous tumor and for determining the precise vascular constitution of the hemangioma, suggesting the efficacy of this method for diagnosing Kasabach-Merritt syndrome. Although administration of a corticosteroid was not effective, additional administration of vincristine resulted in the reversal of thrombocytopenia and coagulopathy with reduction of the hemangioma.

An operated case of retroperitoneal venous hemangioma complicated with Kasabach-Merritt syndrome which was well controlled by danaparoid sodium

An operated case of retroperitoneal venous hemangioma complicated with Kasabach-Merritt syndrome which was well controlled by danaparoid sodium

Case report: Subutaneous hemangiomatosis causing Kasabach-Merritt syndrome - MRI features

Hemangiomatosis is an uncommon entity in which there is diffuse infiltration of soft tissue or bone by hemangioma. Kasabach-Merritt syndrome is an uncommon complication of large hemangiomas, in which there is thrombocytopenia and coagulopathy. Plain radiographs, in addition to showing a soft tissue mass, also show a variety of findings in the bones. MRI is the investigation of choice. A case of a 2-year-old child suffering from hemangiomatosis and a resultant Kasabach-Merritt syndrome is presented.

Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child

Most cavernous hemangiomas in the spleen are small lesions that are found incidentally and patients usually present with no symptoms. Imaging is able to detect the lesions that are considered as diagnostic evidence. But some patients with diffuse cavernous hemangioma may present with anemia, thrombocytopenia, coagulopathy and bleeding, which might be misdiagnosed as idiopathic thrombocytopenia with disseminated intravascular coagulation (DIC). Splenectomy is the most effective therapy for diffuse cavernous hemangiomas with symptoms. The history, imaging results, pathologic findings, diagnosis and treatment of a 34-month-old boy with severe petechiae were reviewed. The boy was diagnosed as having refractory idiopathic thrombocytopenia (ITP) because of low platelet count and bleeding at a local hospital. He had no response to a full-dose of corticosteroid and a high-dose of immunoglobulin (2 g/kg). Huge splenomegaly and DIC were found after 7 months. Diffuse cavernous hemangioma of the spleen was highly suspected, but it was not confirmed by B ultrasound, enhanced CT or MRI. DIC and bleeding were solved by low molecular weight heparin, supplement of fibrinogen and prothrombin complex. A diffuse cavernous hemangioma involving the whole spleen was confirmed pathologically following a successful splenectomy. The boy recovered completely without any complication after the operation. Diffuse cavernous hemangioma of the spleen should be differentiated from ITP associated with splenomegaly. Radiological and overall physical examination should be emphasized for refractory ITP cases.

Hemangioendotelioma kaposiforme e síndrome de Kasabach-Merritt

OBJETIVO: Descrever a apresentação e a evolução de um caso de hemangioendotelioma kaposiforme complicado por síndrome de Kasabach-Merritt. DESCRIÇÃO DO CASO: Lactente masculino de 45 dias, com febre, irritabilidade e choro, associados ao aparecimento de nodulações em tronco e membros. Evoluiu rapidamente com coagulopatia de consumo e queda do estado geral, necessitando de internação em Unidade de Terapia Intensiva Pediátrica (UTIP). Tomografia computadorizada revelou condensações parenquimatosas intra e extrapulmonares compatíveis com hemangiomas múltiplos e ausência de lesões em crânio e abdome. Houve complicação para síndrome Kasabach-Merritt em conseqüência de uma hemangiomatose kaposiforme. Optado por iniciar tratamento conservador, associando terapia de suporte com concentrado de glóbulos vermelhos, criopreciptado, plasma fresco, concentrado de plaquetas e uso das seguintes drogas: ácido tranexâmico e dexametasona. Após 20 dias em UTIP, já se encontrava em bom estado geral, em processo de remissão dos hemangiomas e normalização dos exames laboratoriais, sendo transferido para a enfermaria. Recebeu alta em tratamento com ácido tranexâmico e prednisona, com acompanhamento ambulatorial e laboratorial semanal. COMENTÁRIOS: A doença apresenta curso clínico rápido e grave, com necessidade de diagnóstico e tratamento precoces, para melhor prognóstico e sobrevida.

Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature

IntroductionHaemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels. They are the most common pediatric neoplasm. Kasabach-Merritt syndrome is a rare type of vascular lesion with peculiar characteristics. The diagnosis is based upon three basic findings; enlarging haemangioma, thrombocytopenia and consumption coagulopathy.Case presentationA 5 month old boy was admitted to the Pediatrics department for the management of an abdominal wall mass. He was the first child of consanguineous parents, born in a private hospital following uncomplicated pregnancy and delivery. At birth a bluish birth mark 5 cm × 5 cm was noted below the umbilicus. Over the next five months, this birth mark increased in size and evolved into a swelling. As a result, the patient was admitted to Maternal and Child Health (MCH) unit for the management of this swelling.The clinical findings and imagining studies followed by laboratory investigations strongly suggested the diagnosis of Kasabach-Merritt syndrome.Vincristine was initiated after a trial of corticosteroids when the platelet count was 6000/cmm. One week after the start of vincristine the size of the lesion started to decrease. At the end of 6th week the lesion size decreased to half and the platelet count increased to 49,000/cmm. Vincristine was continued for another 2 weeks, no further improvement in lesion size or platelet count was observed. Vincristine was discontinued and the patient was shifted to the paediatric surgery department. A fresh platelet transfusion was given and the haemangioma was excised completely.The histopathological examination of the excised mass revealed a caverno-capillary haemangioma with infiltration into skeletal muscles.ConclusionSix weeks treatment with vincristine in a dose of 0.5 mg/kg/week followed by surgical excision may be the best management in selected cases of Kasabach-Merritt syndrome.

Vascular Malformation and Hemangiomatosis Syndromes: Spectrum of Imaging Manifestations

The purpose of this review is to describe the role of imaging and associated findings in the diagnosis of blue rubber bleb nevus syndrome, Proteus syndrome, Klippel-Trénaunay syndrome, and Kasabach-Merritt syndrome. Blue rubber bleb nevus, Proteus, Klippel-Trénaunay, and Kasabach-Merritt syndromes are a diverse group of vascular malformation and hemangiomatosis syndromes. Both cutaneous and visceral vascular lesions are associated with these disorders. Accurate diagnosis of these syndromes is important because they can be associated with serious complications, including life-threatening hemorrhage.

Living donor liver transplantation in a patient with giant hepatic hemangioma complicated by Kasabach-Merritt syndrome: Report of a case

We herein present a case of unresectable giant hepatic hemangiomas with Kasabach-Merritt syndrome which was successfully treated by living donor liver transplantation using a left lobe graft. The patient was a 45-year-old woman who complained of abdominal distension. Two sessions of transarterial embolization were performed, but failed to reduce the size of the tumor. The hepatic tumors were thus judged untreatable and the only option for a cure was to offer living donor liver transplantation, because of the tumor size, its location, and the association with Kasabach-Merritt syndrome. A left lobe graft with the middle hepatic vein donated by her 47-year-old brother was transplanted under venovenous bypass. The postoperative course of the recipient was complicated by small-for-size graft syndrome, which developed after episodes of acute cellular rejection on postoperative day 8 and sepsis on day 31. The patient successfully recovered from the complications and was discharged on day 72, and she remains well at 10 months after transplantation. In conclusion, living donor liver transplantation was found to be an effective option for the treatment of a patient with unresectable giant hepatic hemangiomas complicated by Kasabach-Merritt syndrome.

Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life‐threatening consumption coagulopathy

Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.

Tufted Angioma–Associated Kasabach-Merritt Syndrome Treated with Embolization and Vincristine

Tufted Angioma–Associated Kasabach-Merritt Syndrome Treated with Embolization and Vincristine

Oropharyngeal Kaposiform Hemangioendothelioma

The kaposiform hemangioendothelioma is a very infrequent tumor proceeding from the endothelial-derived spindle cells, more often found on the limbs, although peritoneal, retroperitoneal and sacrum locations are also prevailing. Head and neck are exceptional locations. The kaposiform hemangioendothelioma is almost exclusively found in children and early adolescents, and it is highly associated with the Kasabach-Merritt syndrome and lymphangiomatosis. The main treatment is the tumor surgical removal, including wide margins, plus supporting therapy when Kasabach-Merritt syndrome is linked. We report an isolated oropharyngeal kaposiform hemangioendothelioma 9-10-year old male case.

Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult!

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor which usually occurs in infants. Clinically it appears as ill-defined red to purple indurated plaque. KHE is commonly associated with Kasabach-Merritt syndrome (KMS) and lymphangiomatosis. Microscopically, the tumor is composed of infiltrating lobulated nodules with slitlike or crescentic vessels which are poorly canalized and lined by spindle shaped endothelial cells. We report a 36-year old female who developed a reddish tumor on the chest. Histological examination revealed a KHE, which was clinically not associated with thrombocytopenia or bleeding complications, but lymphangiomatosis at the right submandibular region. The association of KHE in a female adult with lymphangioma rather than KMS in this case supports the hypothesis that such an association may represent a benign subform of this disease in an adult and excision seems to be curative.

Kasabach-Merritt Syndrome Associated with Angiosarcoma of the Scalp Successfully Treated with Chemoradiotherapy

Kasabach-Merritt Syndrome Associated with Angiosarcoma of the Scalp Successfully Treated with Chemoradiotherapy