Transcatheter Intervention for Infantile Hepatic Hemangioendothelioma with Amplatzer Duct Occluder II.

Introduction: Infantile Hepatic hemangioendothelioma is characterized by multifocal benign vascular dilatations involving the liver. The clinical course depends on tumor size, localization and complications. Case Report: 15 days old neonate with stormy postnatal period was referred for intractable congestive cardiac failure. Transthoracic 2D echocardiography showed multiple vegetations on all the four valves, noncompaction of left ventricle with multiple vascular channels in left lobe of liver. Computed tomography angiogram showed 58x29x50 mm markedly enhancing lesion in left lobe of liver suggestive of infantile hepatic hemangioendothelioma. The neonate was treated for bacterial endocarditis. Later 6x6 Amplatzer duct occluder II was parked in hepatic vein, then gel foam and polyvinyl chloride particles were injected into infantile hepatic hemangioendothelioma. Cardiac failure resolved with marked regression of lesion. Discussion: Infantile hepatic hemangioendothelioma is a rare anomaly causing heart failure in neonate causing death in up to 70% of untreated infants without adequate regression of lesion. Therefore aggressive treatment is warranted. For the first time in the world, we report a case of infantile hepatic hemangioendothelioma in a neonate, with pump failure due to noncompaction of left ventricle and vegetations on all four valves, successfully treated by combination of transcatheter deployment of Amplatzer duct occluder II to occlude the venous end and hand injection of gel foam and polyvinly chloride particles from the aortic end to close the feeder artery. Conclusion: The combination of closure of hepatic vein by Amplatzer duct occluder II and injection of gel foam and polyvinyl chloride particles is safe and effective in infantile hepatic hemangioendothelioma in neonate with heart failure.

Hemangioendotelioma epitelioide y fusiforme de ganglio linfático: Caso clínico / Spindle and epithelioid hemangio-endothelioma of the lymph node: Report of one case

Background: Primary vascular tumors of lymph nodes are extremely rare with the exception of AlDS-related Kaposi's sarcoma. The diagnosis of epithelioid hemangio-endothelioma (EH) is difficult to make without ancillary studies, since it is devoid of morphological features indicating its vascular nature and it may be overlooked when it appears as a primary tumor of lymph nodes. Spindle and epithelioid hemangio-endothelioma (SEH) is considered to be a variant of EH, which has been reported to occur exclusively in lymph nodes and the spleen. We report a 70-year-old male with chronic lymphocytic leukemia (CLL) and left cervical lymphadenopathy. An excisional biopsy was performed, and microscopically the lymph node showed effacement of nodal architecture by a tumor composed of spindle cells disposed in intersecting fascicles, and characterized by abundant eosinophilic cytoplasm, elongated nuclei and conspicuous nucleoli. A second population of cells had an epithelioid appearance with intracyto-plasmic vacuoles containing red blood cells. lmmunohistochemically, the tumor cells were positive for CD31 and CD34. The final diagnosis was SEH of the lymph node.

Coexistent hepatic and pulmonary epitheloid hemangioendothelioma.

Epitheloid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin known to arise in soft tissue, liver and lung. We describe a case of coexistent hepatic and pulmonary epitheloid hemangioendothelioma, proven on autopsy, and review the histological and radiological features of epitheloid hemangioendothelioma. The coexistence of hepatic with pulmonary EHE has been reported in only a few cases. Large confluent masses, peripheral location with capsular retraction, hypertrophy of uninvolved liver, invasion of portal and hepatic veins, enhancing margins and delayed enhancement and dense calcification are the typical features which provide a clue to diagnosis of hepatic EHE. In patients with both hepatic and pulmonary EHE it is difficult to say whether the tumor arose primarily in the lung or liver, or began simultaneously in both organs.

Hemangioendotelioma kaposiforme e síndrome de Kasabach-Merritt / Kaposiform haemangioendothelioma and Kasabach-Merritt syndrome

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.

To describe the clinical presentation of kaposiform hemangioendothelioma that was complicated by Kasabach-Merritt syndrome. CASE DESCRIPTION: A 45-day-old male infant presented fever, irritability and crying associated with the eruption of nodules in trunk and members. Soon after admission, the patient developed a consumptive coagulopathy, requiring admission in the Pediatric Intensive Care Unit (PICU). Computed tomography scans showed intra and extrapulmonary condensations suggestive of multiple thoracic hemangiomas, without the central nervous system and abdominal lesions. The diagnosis of Kasabach-Merritt syndrome in consequence of kaposiform hemagioendothelioma was established. Treatment option was conservative, including red blood cells, cryoprecipited, fresh frozen plasma and platelets transfusions associated to tranexamic acid and dexamethasone. After 20 days, patient recovered and he was discharged from PICU with significant remission of the hemangiomas. The child continued to receive tranexamic acid and prednisone at home, with weekly follow-up. COMMENTS: The disease presents quick and severe clinical course. Therefore, early diagnosis and treatment are needed to improve the outcome.

Childhood hepatic angiosarcoma--a case report.

Hepatic angiosarcoma (HAS) is an extremely rare liver tumor in children. We report a case of childhood HAS in a six year old girl who presented with acute abdominal pain and fever with a mass in epigastrium. Left hepatic lobectomy was performed with a clinical diagnosis of hepatoblastoma. Histopathological examination revealed features typical of hepatic angiosarcoma. The case is presented for its rarity and to discuss the interrelation between infantile hemangioendothelioma (IHE) and HAS.

Hipofosfatemia revertida al extirpar hemangioendotelioma compuesto del dedo mayor del pie / Reversion of hypophosphatemia after the excision of a composite hemangioendothelioma in the great toe

We report a 43 years old male admitted to the hospital for progressive lumbar pain, lasting 20 years, that caused severe disability. On admission the patient had a serum phosphate of 2 mg/dl, an urine phosphate excretion over 300 mg/dl and serum alkaline phosphatases over 750 U/L. Serum intact parathormone was normal and tubular maximum phosphorus/glomerular filtration was 0.7 mg/dl. Bone scintigraphy showed an increased radionuclide uptake in condro-costal joints. Bone densitometry showed femoral osteoporosis. A violet colored mass was detected in a great toe. It was removed and the pathological diagnosis was a composite hemangioendothelioma. After tumor excision, serum phosphate levels returned to normal values and symptoms disappeared within 15 days.

Use of tranexamic acid in pediatric Kaposiform hemangio-endothelioma complicated with Kasabach-merritt syndrome

Kasabach-Merritt syndrome is an infrequent combination of Kaposiform hemangio-endothelioma and severe thrombocytopenia, which may be life- threatening with an overall mortality rate of 20-30% and for which there are no definitive methods of treatment. A retrospective data collection of a single institute [KFSHRC]* reporting three cases of Kasabach-Merritt syndrome, showing a very good response to tranexamic acid with the reversal of the hematological disorder, decrease in steroid requirement and diminution of the size of the lesion in few months duration. The early use of tranexamic acid in the treatment of Kasabach-Merritt syndrome, especially the non-resectable vascular tumors, was recommended to be combined with interferon-alpha or steroid therapy for children

Malformación vascular como causa de proptosis a propósito de un caso: revisión de laliteratura / Vascularmalfotmations as cause of proptosis, apropos of a case: review of the literature

Se presenta un caso de malformaciones vascular como causa de proptosis. Se detalla el caso clínico con resumen de la historia, ultrasonido, seguimiento y resultado anatomopatológico. Se discuten los tipos de tumores orbitarios