Infantile hepatic hemangioendothelioma with subsequent malignant degeneration

A 4 year old child was seen because of an enlarging epigastric mass and a rapidly falling hematocrit 31/2 years following steroid treatment for multiple hepatic hemangioendotheliomas. Technetium 99 m sulfur colloid liver scan and hepatic angiography confirmed the presence of a large, vascular mass involving the left lobe of the liver. A left hepatic lobectomy was performed and histopathology showed angiosarcoma. As children with hemangioendotheliomas of the liver begin to survive for longer periods of time, surveillance for the possible development of neoplastic disease is recommended.     

Infantile hepatic hemangioendothelioma treated by radiotherapy

Infantile hepatic hemangioendothelioma is a rare tumor of infancy, sometimes associated with cutaneous hemangiomatosis. It is clinically evident within the first six months after birth and can be life threatening because of heart failure, intraperitoneal hemorrhage or thrombocytopenia. In less severe forms spontaneous regression has been described. Current treatment may be surgical ligation of the hepatic artery, or pharmacological therapy with corticosteroids or radiotherapy. A 4-month infant is described, admitted with acute heart failure and huge hepatomegaly. Since a surgical approach was not possible and corticosteroid therapy failed to achieve the expected effect, radiotherapy was given with excellent results.     

婴儿型肝脏血管内皮细胞瘤并发心力衰竭2 例分析

目的探讨婴儿型肝脏血管内皮细胞瘤(IHHE)合并心力衰竭的临床特征、治疗及预后。方法回顾分析2例婴儿型IHHE合并心力衰竭患儿的临床资料,并复习相关文献。结果例1女性,产前超声示胎儿左腹部不均质回声团,生后第5天经磁共振成像、CT检查确诊,生后7天出现心力衰竭,于出生15天手术切除并经病理检查确诊IHHE,2月龄时复查无异常。例2男性,出生第15天时因新生儿黄疸入院,并很快出现心力衰竭,经磁共振、CT检查临床确诊IHHE。因病情进展迅速,于术前合并失代偿心力衰竭、呼吸衰竭死亡。结论婴儿型IHHE合并心力衰竭者病死率高,早识别、早干预、多学科干预可改善预后。     

Infantile hemangioendothelioma

Primary hepatic tumors are uncommon in children and account for only three per cent of the tumors in children. Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present before six months. An unusual presentation and progression of infantile hemangioendothelioma is reported in a 19-month-old female child. The diagnosis was arrived at by radiological and histopathological examination. The patient underwent excision surgery, following which made an uneventful recovery. On follow-up at six months, patient was asymptomatic with no evidence of recurrence.     

Infantile cardiac hemangioendothelioma

Summary A 4-month-old infant with cardiac hemangioendothelioma presented with thrombocytopenia, and pericardial effusion, as well as signs and symptoms of heart failure. This is the first reported case of infantile cardiac hemangioma successfully treated with steroids.     

Infantile hepatic hemangioendothelioma. A multifocal, bilobular and asymptomatic case with spontaneous regression

Infantile hepatic hemangioendothelioma is a severe disease with a high mortality rate. Nevertheless these vascular lesions may experience spontaneous regression within 12 to 18 months. The decision of trying a specific treatment and the choice among the several therapeutic options remains controversial, particularly in those asymptomatic cases of multifocal, bilobular involvement. We describe a case of multifocal, bilobular and asymptomatic infantile hepatic hemangioendothelioma, untreated, and with spontaneous regression before 2 years of age.     

Stromal-predominant mesenchymal hamartoma of the liver with elevated serum alpha-fetoprotein level

Mesenchymal hamartoma of the liver (MHL) is an uncommon, benign, tumor-like lesion and is usually diagnosed in the first 2 years of life. Its pathogenesis remains unclear. Treatment of choice is radical excision. The authors report a case of solid stromal predominant MHL in a 12-month-old male infant who also had an elevated serum alpha-fetoprotein level. He also had hypospadias, which might represent a spectrum of developmental anomalies. It usually presents as an asymptomatic mass, however, as in the reported case, it may cause several complications due to the compression of surrounding structures. He was successfully treated with total excision of the pedunculated large tumor without any complication.     

Infantile hemangioendothelioma of the pelvis associated with Kasabach-Merritt syndrome

A case of infantile hemangioendothelioma of the pelvis in a newborn male infant is described. Shortly after delivery, a large abdominal mass was found by external examination. CT scan revealed a hypervascular retroperitoneal pelvic mass invading the lumbar spinal column. Exploratory laparotomy was performed and biopsy revealed infantile hemangioendothelioma. The tumor was associated with profound thrombocytopenia, intratumoral hemorrhage, and right hydroureteronephrosis. After treatment with high dose steroids, Cytoxan, and external beam radiation for 2 months, the patient was discharged with persistent thrombocytopenia requiring platelet transfusions. The hydroureteronephrosis has also not improved. The literature on this subject is reviewed.     

Immature ovarian teratoma with peritoneal gliomatosis and elevated serum alpha-fetoprotein associated with a second mature teratoma

A 7-year-old girl had a huge immature grade 2 teratoma of the left ovary with peritoneal gliomatosis and elevated serum alpha-fetoprotein (AFP). The serum AFP level reached 3543 ng/ml and returned to normal after left salpingo-oophorectomy and chemotherapy. Twenty-two months later, a second mature teratoma was removed from the left subdiaphragmatic region. The AFP level had been within the normal range and the patient had been symptom-free for 3 years following the previous operation. AFP was positive in the tubular epithelium of the immature tissues on immunohistochemical study. Offprint requests to: A. Hokama     

Infantile hepatic hemangioendotheliomas associated with hemihypertrophy

An infant presented with congenital hemihypertrophy, hepatomegaly, and a low thoracic paraspinal mass. Liver scan showed multiple defects in the uptake of radioisotope. Surgical exploration revealed multiple infantile hemangioendotheliomas of the liver and a paraspinal hemangioendothelioma. In review of 69 reported cases of infantile hemangioendothelioma of the liver and viscera, there has been no previous note of hemihypertrophy associated with this abnormality.     

Thyroid abnormalities in infantile hepatic hemangioendothelioma

We report an infant with hepatic hemangioendothelioma (HAE) associated with compensated hypothyroidism. The hepatic lesions regressed with steroid therapy and his thyroid function normalized with high doses of thyroxine supplement.     

Symptomatic hepatic hemangioendothelioma in a newborn

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.     

Massive hepatic hemangioendothelioma and consumptive hypothyroidism

Hypothyroidism was diagnosed in an infant with massive hepatic hemangioendothelioma. Markedly elevated serum reverse-triiodothyronine levels confirmed consumptive hypothyroidism. Large doses of thyroxine were initially required to normalise TSH. Thyroxine was tapered as the tumor regressed and was discontinued at 16 months old. Thyroid function remains normal without treatment.     

Maternal serum alpha-fetoprotein screening

Maternal serum alpha-fetoprotein screening should be offered to all patients as a routine component of prenatal care. The benefits of MSAFP screening have far exceeded early expectations. This technology not only provides an efficient and cost-effective method of screening for neural tube defects that is applicable to all pregnancies but also provides the physician with important information relevant to a number of other birth defects and complications of pregnancy. Our knowledge of variables affecting MSAFP levels measured during pregnancy is rapidly evolving, and further refinements in the application of MSAFP screening will almost certainly occur. Such refinements can serve only to extend the benefits of this new and very valuable technology in improving the health and well being of mothers and infants.     

Hepatic hemangioendothelioma of infancy associated with elevated alpha fetoprotein and catecholamine by-products

Five cases of hepatic hemangioendothelioma (HH) were seen in infants ranging from 2 days to 5 months of age. The cases were studied by clinical chemistry, immunohistochemical staining, and electron microscopic techniques. Serum alpha-fetoprotein (AFP) levels were elevated in each of the four patients in whom levels were obtained. The highest elevation was noted in a 2-day-old infant with a diffuse, unresectable lesion involving the right lobe. Two patients who underwent complete resections of their solitary HH had normalization of their AFP levels. This study suggests that AFP levels in patients with HH are closely related to the patients' age, with the youngest patient having the highest levels of AFP. Catecholamine by-products (VMA and HVA) were elevated in one of four patients in whom levels were obtained, this infant was 3 months old at the time of presentation. She also had an elevated AFP level, a diffuse unresectable lesion involving the entire liver, multiple pulmonary nodules, and cutaneous hemangiomas. Immunohistochemical study failed to demonstrate the source of AFP or the catecholamine by-products in the tumor of this patient, suggesting that the source of catecholamine by-products could be stress-induced catecholamine secretion.     

Infantile hepatic haemangioendothelioma: an association with hemihypertrophy

A 3-month-old infant with obvious hypertrophy affecting the right arm, leg, and face presented with a haemangioendothelioma arising from the right lobe of the liver that was not associated with cardiac failure. At diagnostic laparotomy the nature of the mass was confirmed and the right hepatic artery was ligated. Follow-up examination at 10 months revealed complete resolution of the intrahepatic mass. This appears to be the first report of an association between hemihypertrophy and hepatic haemangioendothelioma. Correspondence to: M. Davenport