Infantile Hepatic Hemangioendothelioma Research Articles

Tumor-Induced Osteomalacia- Like Syndrome with Rickets and Infantile Hepatic Hemangioendothelioma.

Tumor-induced osteomalacia (TIO), a rare paraneoplastic syndrome is seen in association with the overproduction of fibroblast growth factor-23 (FGF-23) by certain mesenchymal tumors in adults. In children, these phosphaturic mesenchymal tumors produce features of rickets similar to TIO. This condition is characterized by elevated blood levels of FGF-23, low phosphate, low or normal active vitamin D, and high alkaline phosphatase. Though the removal of the tumor is curative; in cases where surgical resection is not possible, medical treatment is successful with phosphate and active vitamin D in the improvement of symptoms. The case of a child with features of rickets is presented here to illustrate the importance of identifying this rare condition and instituting appropriate intervention.

Infantile hepatic hemangioendothelioma associated with pulmonary arterial hypertension and cardiac insufficiency successfully treated with oral propranolol—a rare case report

BackgroundInfantile hepatic hemangioendothelioma (IHHE) is a benign liver tumor associated with a varied spectrum of presentations ranging from asymptomatic hepatomegaly and cutaneous hemangiomas to some life-threatening complications like cardiac failure, consumptive hypothyroidism, pulmonary arterial hypertension, gastrointestinal bleeding, and coagulopathy. Hypothyroidism results from increased activity of type 3 iodothyronine deiodinase in the liver. Clinico-radiological correlation is essential for a definite diagnosis. Although children with asymptomatic lesions may experience spontaneous regression within a year, those with symptomatic lesions require aggressive management due to the risk of mortality once the symptoms commence. Treatment modalities can be either pharmacologic or interventional depending on the clinical presentation, with propranolol being considered the first-line agent.Case presentationHere, we describe a 2-month-old female infant with progressive abdominal distension, signs of congestive cardiac failure, and pulmonary arterial hypertension, confirmed as IHHE by abdominal ultrasonography and contrast-enhanced MRI. It was complicated by consumptive hypothyroidism and coagulopathy. The child was successfully treated with oral propranolol, levothyroxine, and supportive measures.ConclusionEarly aggressive treatment in a symptomatic patient is linked to successful outcomes. Hypothyroidism should be focused on and managed earlier to prevent intellectual and growth retardation.

Propranolol for infantile hepatic hemangioendothelioma: Clinical evaluation of drug efficacy and safety using a single-center patient cohort

Introduction and objectivesInfantile hepatic hemangioendothelioma (IHHE) is a benign liver tumor, associated with hypothyroidism and vascular malformations along the skin, brain, digestive tract and other organs. Here, we determined a single-center patient cohort by evaluating the effectiveness and safety of propranolol and sirolimus for the treatment of IHHE. Patients and methodsWe performed a monocentric and observational study, based on clinical data obtained from 20 cases of IHHE treated with oral propranolol and sirolimus at the Shanghai Children's Medical Center (SCMC), between December 2017 and April 2019. All cases were confirmed by abdominal enhanced CT examination (18/20, 90%) and sustained decrease of alpha fetoprotein (AFP) (2/20, 10%).Propranolol treatment was standardized as once a day at 1.0mg/kg for patients younger than 2 months, and twice a day at 1.0mg/kg (per dose) for patients older than 2 months. Sirolimus was used to treat refractory IHHE patients after 6 months of propranolol treatment, and initial dosing was at 0.8mg/m2 body surface per dose, administered every 12h. Upon treatment, abdominal ultrasound scanning was regularly performed to evaluate any therapeutic effects. All children were followed up for 6–22 months (mean value of 12.75 months). The clinical manifestations and therapeutic effects, including complications during drug management, were reviewed after periodic follow-up. ResultsThe effective rate of propranolol for the treatment of children with IHHE was 85% (17/20). In most cases, the AFP levels gradually decreased into the normal range. A complete response (CR) was achieved in 3 cases, partial response (PR) for 14 cases, progressive disease (PD) for 2 cases and stable disease (SD) was only detected once. Lesions decreased in two PD patients after administration of oral sirolimus. No serious adverse reactions were observed. ConclusionThis study indicates that both propranolol and sirolimus were effective drugs for the treatment of children with IHHE at SCMC.

Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma.

Infantile hepatic hemangioendothelioma (IHHE) is the most common hepatic vascular tumor in children. We report on the treatment outcome of our large single-center experience of patients with IHHE over a 9-year period. A retrospective analysis of all IHHE patients treated at the Children Cancer Hospital Egypt from April 2008 through April 2017. In total, 28 patients (18 females, 10 males) were diagnosed with IHHE with a median age at diagnosis of 3 months. The lesions were multifocal (n=12), focal (n=10), and diffuse (n=6). Six (21.4%) patients initially had low T3 and T4. Eleven patients did not receive any treatment, whereas 1 patient underwent resectional surgery. Sixteen patients received drug treatment, 9 of whom responded well to first-line propranolol/prednisolone, whereas 7 patients needed salvage treatment. Twenty-five patients are alive, whereas 3 patients have died. Overall, patients with IHHE do well, a significant percentage of whom do not require drug therapy, particularly for those with small focal lesions. In patients with multifocal/diffuse disease, there is a high incidence of low T3 and T4 and while some of these patients did well without additional therapy, those with rapidly progressive lesions during treatment may do poorly.

CEUS in paediatric hepatic lesions

Focal liver lesions (FLL) are commonly observed in adult patients and rarely reported in children. The reasons for this remain speculative and the exact data concerning the incidence of these lesions in childhood are lacking. Primary hepatic tumors are a diverse group of epithelial and mesenchymal tumors accounting for 0.5 and 2.0% of all pediatric neoplasms. Focal liver lesions in children can be benign, malignant, or indeterminate. About one-third of the pediatric primary liver masses are benign. In pediatric patients, most benign focal liver lesions are inborn and may grow like the rest of the body. Knowledge of pediatric liver diseases and their imaging appearances is essential in order to make an appropriate differential diagnosis. Selection of the appropriate imaging test is challenging, since it depends on a number of age-related factors. The presentation will focus on the most frequently encountered benign and malignant liver tumors in children (infantile hepatic hemangioendothelioma, mesenchymal hamartoma, focal nodular hyperplasia, nodular regenerative hyperplasia, and hepatocellular adenoma, hepatocellular carcinoma and metastases) .

Infantile hepatic hemangioendothelioma camplicated with persistent plmonary hypertension: three cases report

infantile hepatic hemangioendothelioma camplicated with persistent plmonary hypertension: three cases report

Transcatheter arterial sclerosis and embolization with bleomycin lipiodol emulsion and polyvinyl alcohol in the treatment of infantile hepatic hemangioendothelioma

Objective To evaluate the safety and effectivity of transcatheter arterial sclerosis and embolization (TASE) with bleomycin lipiodol emulsion and polyvinyl alcohol (PVA) in the treatment of infantile hepatic hemangioendothelioma (IHH). Methods Fortytwo children with IHH were diagnosed, including 20 males and 22 females, ranging from 26 days to 5.0 years with a median of 10 months. Celiac artery angiography under DSA was performed to conform IHH. Bleomycin lipiodol emulsion and PVA were injected to feeding arteries by super selective technique. The follow-up time was 3 to 36 months, with an average of (19±6) months. Results Total TASE was 80 times, 1 to 4 times/case;The tumor volume was reduced by about 63.4% after 3 months, and was completely disappeared in 39 cases one year later, 3 cases with diffuse lesions were reduced slightly and TASE was performed again after a year, the tumors were completely disappeared. Five cases showed slight elevation of serum aminotransferase, and recovered to be normal after symptomatic treatment. One case showed right femoral artery stenosis, and balloon dilation was performed one month later. One case had bilateral iliac artery thrombosis, and was back to be normal after anticoagulant therapy. One case had a transient pulmonary embolism, and the symptoms disappeared after 2 days with auxiliary respiratory support. Conclusion TASE with bolemicin lipiodol emulsion and PVA is a safe and effective method in the treatment of IHH. Key words: Infant; Hemangioma; Liver; Embolization, therapeutic; Bleomycin

The Value of Clinical and Ultrasound Features for the Diagnosis of Infantile Hepatic Hemangioendothelioma: Comparison with Contrast-Enhanced CT/MRI

The Value of Clinical and Ultrasound Features for the Diagnosis of Infantile Hepatic Hemangioendothelioma: Comparison with Contrast-Enhanced CT/MRI

Infantile Hepatic Hemangioendothelioma: An Uncommon Cause of Persistent Pulmonary Hypertension in a Newborn Infant

Multifocal and diffuse infantile hepatic hemangioendotheliomas commonly present with signs of high-output congestive heart failure. In addition, prolonged persistent pulmonary overcirculation eventually leads to the development of pulmonary hypertension at a later age. We report a 2-day old, full-term infant with multifocal, large infantile hepatic hemangioendothelioma, who presented with an early onset of pulmonary hypertension, managed successfully with supportive care and systemic therapy directed toward the involution of infantile hepatic hemangioendothelioma.

Infantile Hepatic Hemangioendothelioma (IHHE) – An Unusual Presentation

Hepatic tumours are relatively rare in children, accounting for 1% - 4% of all paediatric solid tumors, commonest amongst them are hepatoblastoma, neuroblastoma, and vascular tumors (hemangioendothelioma/haemangiomas). Infantile Hepatic hemangioendothelioma (IHHE) is classically characterised by hepatomegaly, jaundice, congestive cardiac failure with high cardiac output, skin haemangiomas, haemolytic anemia, thrombocytopenia(kasabach ‐ Merritt syndrome), normal Alfafeto protein (AFP) levels and hypothyroidism. We report a case of infantile hemangioendothelioma in a 5 day old female neonate with high output cardiac failure, unusually high alpha-fetoproteins, thrombocytosis and polycythaemia, which posed a diagnostic dilemma. Diffuse IHHE is a rapidly growing benign tumor which can lead to poor clinical outcome if not identified early. Serum AFP can’t be relied to exclude IHHE.

Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol

Steroid-resistant infantile hepatic hemangioendothelioma successfully treated with propranolol

Infantile hepatic hemangioendothelioma presenting as early heart failure: An autopsy case report

Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy, but is rarely reported because of the low incidence estimated to be about 1/20,000. We report an autopsy case of IHHE in a 20-day-old female who came with an initial manifestation of abdominal distension and congestive heart failure (CHF). Symptoms of cardiac decompensation gradually worsened in spite of treatment and child died within 2 days. On autopsy, grossly enlarged liver showed microscopy of large ecstatic vascular channels lined by single layer of plump endothelial cells and diagnosis was given as Type I IHHE. CHF is common complication due to arteriovenous shunts within the tumor and contributes to high morbidity and mortality in up to 70% of untreated infants with median age of presentation of 1-month. Therefore, if symptoms develop, aggressive treatments are warranted.

Benign liver tumors in pediatric patients - Review with emphasis on imaging features.

Benign hepatic tumors are commonly observed in adults, but rarely reported in children. The reasons for this remain speculative and the exact data concerning the incidence of these lesions are lacking. Benign hepatic tumors represent a diverse group of epithelial and mesenchymal tumors. In pediatric patients, most benign focal liver lesions are inborn and may grow like the rest of the body. Knowledge of pediatric liver diseases and their imaging appearances is essential in order to make an appropriate differential diagnosis. Selection of the appropriate imaging test is challenging, since it depends on a number of age-related factors. This paper will discuss the most frequently encountered benign liver tumors in children (infantile hepatic hemangioendothelioma, mesenchymal hamartoma, focal nodular hyperplasia, nodular regenerative hyperplasia, and hepatocellular adenoma), as well as a comparison to the current knowledge regarding such tumors in adult patients. The current emphasis is on imaging features, which are helpful not only for the initial diagnosis, but also for pre- and post-treatment evaluation and follow-up. In addition, future perspectives of contrast-enhanced ultrasound (CEUS) in pediatric patients are highlighted, with descriptions of enhancement patterns for each lesion being discussed. The role of advanced imaging tests such as CEUS and magnetic resonance imaging, which allow for non-invasive assessment of liver tumors, is of utmost importance in pediatric patients, especially when repeated imaging tests are needed and radiation exposure should be avoided.

Regression of multifocal infantile hepatic hemangioendothliomas after steroid therapy

Abstract Hepatic hemangioendotheliomas are rare childhood tumors. Infantile hepatic hemangioendothelioma (IHHE) is the most common vascular tumor of the liver in children accounting for 12% of all childhood hepatic tumors. We report an 8-month-old female infant presented at the age of 4 months with incidental hepatomegaly. On presentation her abdomen was soft and lax. The liver was at 8 cm below the right costal margin, not tender with rounded edge and nodular surface. The spleen was not felt and there were no other masses and no ascites. Her liver functions tests were normal. MRI of the liver showed numerous rounded lesions in all the hepatic segments with characteristic of low signal intensity at T1 and high intensity at T2. Post contrast, the lesions showed a peripheral thick rim of enhancement and in the delayed porto-venous phase showed central filling with contrast. The portal and hepatic veins were patent. Our findings were compatible with the diagnosis of multifocal infantile hemangioendothelioma of the liver. Oral prednisone was started for 2 weeks then was tapered over 4 months. A follow up MRI confirmed the decrease in the number and the size of hepatic lesions. A follow-up liver US 24 months after treatment showed residual solitary small lesion in left hepatic lobe measuring 1.2 × 1.0 cm. The authors report that steroid therapy was effective in the treatment of massive multifocal HHE without a need for other advanced therapies including liver transplantation.

Clinical experience with infantile hepatic hemangioendothelioma

Objective: Infantile hepatic hemangioendothelioma (IHHE), although rare, is the most common benign hepatic tumor in children. Aim of our study was to review our clinical experience with patients with IHHE.

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 Case Study Vijayalakshmi et al.; CA, 2(4): 317-324, 2014; Article no. CA.2014.4.017 318 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.

Infantile hepatic hemangioendothelioma in an 11-year-old child: A case report and review of literature

Infantile hepatic hemangioendothelioma (IHH) is a rare benign mesenchymal tumor. As the name implies, it is commonly seen in infancy and has variable clinical presentation and outcome. This tumor can spontaneously regress or can have expansile growth replacing whole of the liver. In very large tumors, there may be extensive arteriovenous shunting within the lesion, resulting congestive heart failure (CHF) in a significant number of patients with poor outcome. Since there is no tumor marker for this tumor, the diagnosis can only be suspected on the basis of clinical features and radiological investigations from among the various mass occupying lesions of the liver in this age group, which includes hepatoblastoma, mesenchymal hamartoma or hepatocellular carcinoma. As a result, the management strategy cannot be defined and in the literature also there are isolated reports with diverse treatment protocols. The medical management includes corticosteroid, interferon, cytotoxic agents and irradiation or even the radical interventions such as hepatic artery ligation, transcatheter hepatic artery embolization. The surgical options are biopsy, complete resection of the lesion or even liver transplantation. We are reporting a case of IHH who was managed with surgical resection and postoperative corticosteroid. However, the patient had recurrence of the tumor and developed CHF, which led to the demise of the patient. The idea of reporting this case is that this patient was 11-year-old child who very unusual for this tumor at this age, and the surgical resection of this lesion should be radical rather than just excision biopsy as was done in the present case.

Tratamiento exitoso con prednisona en un recién nacido crítico con hemangioendotelioma hepático infantil multifocal sintomático

Infantile hepatic hemangioendothelioma is the most common vascular tumor of the liver in infancy. The diagnosis can be enunciated without histological defnition, if the clinical, analytical and images fndings are very suggestive; particularly in children less than six months old, because the biopsy is not exempt of complications. We report a female patient, four days old and 3500 g, who developed a hypovolemic shock as a complication of the biopsy from a multifocal infantile hepatic hemangioendothelioma. She developed clinical manifestations of cardiac failure and thrombocytopenia. Because of the critical clinical status we choose the medical treatment with prednisone which was administered for fve month and complete remission of the tumor was obtained. We highlight the Doppler ultrasonography as a safe and no invasive diagnosis method. Management of this disease is commented as well.

Infantile Hepatic Hemangioendothelioma in Comparison with Hepatoblastoma in Children: Clinical and Ultrasound Features

BackgroundInfantile hepatic hemangioendothelioma (IHH) and hepatoblastoma (HBL) are respectively the most common benign and malignant liver tumors in children.ObjectivesTo study the clinical manifestations and the ultrasound features of the pediatric patients for distinguishing IHH from HBL.Patients and MethodsBetween 2002 and 2012, thirteen children with IHH and 38 children with HBL under the age of 10 years were included. We retrospectively reviewed the clinical and the ultrasound features of the two groups, especially including parameters as follows: age at diagnosis, gender, alpha-fetoprotein (AFP) elevation, venous involvement and Doppler ultrasound.ResultsCompared with HBL group, the age of IHH group was much younger (5.8 months vs. 35.1 months, P = 0.000), the AFP elevation was less likely to be detected in IHH group (23.1% vs. 89.5%, P = 0.000). Although the color flow were the same commonly observed (61.5% vs. 52.6%, P > 0.05), the spectral Doppler showed IHH was less likely to appear as arterial flow with resistance index (RI) > 0.7(12.5% vs. 75.0%, P < 0.05), characterized by arterial flow with RI < 0.7 and/or venous flow. Combined the clinical features including age (< 6 months) and normal AFP level yielded high capability in differential diagnosis, with sensitivity, specificity and Youden index of 77% (10/13), 95% (36/38), and 0.72, respectively. When combined clinical features (age and AFP) and spectral Doppler as the diagnostic criterion for distinguishing these cases with positive color flow signals, the sensitivity, specificity, accuracy and Youden Index were 88%, 95%, 89% and 0.83, respectively.ConclusionsThe clinical features are effective indicators for distinguishing IHH from HBL, and the spectral Doppler may be a useful adjunct parameter for differential diagnosis.

Spontaneous Regression of Infantile Hepatic Hemangioendothelioma

Many primary hepatic tumors such as the rare pediatric neoplasms can be evaluated by sonography. Infantile hepatic hemangioendothelioma (IHH), the most common hepatic benign vascular tumor in infancy, has an excellent prognosis and spontaneous involution within 6–8 months in most cases. A 25-day-old baby girl was admitted to the hospital due to nonbilious vomiting for 2 days. Sonography showed multiple hypoechoic masses in the liver, and abdominal computed tomography demonstrated multifocal hypervascular lesions that were characteristic of IHH. The patient was then treated with supportive care and discharged in a stable condition without medication. Serial follow-up sonographic examinations showed that the size of the masses decreased gradually. At the age of 11 months, a follow-up sonogram confirmed complete resolution of IHH. Sonography is a useful and convenient method for diagnosis and follow-up of IHH. Long-term follow-up is necessary for IHH, even if the lesions are in regression, to watch for rare but possible malignant transformation.