Infantile hemangioendothelioma case reports international. It is the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life. Benign infantile hemangioendothelioma ihh is a rare neonatal benign vascular tumor usually located in the liver. The presenting complaint was abdominal distention which started soon after. Clinical features of infantile hepatic hemangioendothelioma. Infantile hepatic hemangioendothelioma ihh is the most common vascular tumor of the liver in infancy. Dedicated to the mission of bringing free or lowcost educational materials and information to the global ultrasound community.
Histologically, ihh can be present in different, more or less immature types of hemangioma. Infantile hepatic hemangioendothelioma is a rare tumor of infancy, sometimes associated with cutaneous hemangiomatosis. Infantile hepatic hemangioendothelioma libre pathology. Diffuse infantile hepatic hemangioendothelioma with early central. Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by dail and liebow as an aggressive bronchoalveolar cell carcinoma. Hemangioma of infancy is a benign proliferation of small blood vessels capillaries that is present either at birth congenital onset, or occurs right after. The prognosis is better for eh than for other parenchymal tumors, such as sarcoma, although extrahepatic metastases do occur. Infantile hepatic hemangioendothelioma associated with. Niedt gw, greco ma, wieczorek r, blanc wa, knowles dm 2nd.
Imaging plays a vital role in making an accurate diagnosis, thus helping to avoid. The type ii variant is also referred to as infantile angiosarcoma. A case report of hemangioendothelioma of the hard palate. Infantile hemangioendothelioma of liver case reports. Hemangioendothelioma of bone portnotes orthopaedicsone. Current management of hemangiomas and vascular malformations jennifer j. Histopathology images of epithelioid hemangioendothelioma. Mesenchymal tumours in the liver mesenchymal hamartoma. My wife was recently diaognised with epithelioid hemangioendothelioma ehe after she underwent a surgery for excision of an aggressive sebaceous cyst at the back of her skull on the scalp. The topic benign infantile hemangioendothelioma you are seeking is a synonym, or alternative name, or is closely related to the medical condition hemangioma of infancy.
But they are slowgrowing and do not usually spread. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum. Kaposiform hemangioendothelioma also known as infantile kaposiform hemangioendothelioma is an uncommon vascular tumor, first described by niedt, greco, et al. Epithelioid hemangioendothelioma is a rare type of vascular tumor that affects the epithelial cells, which line the inside of blood vessels. The term hemangioendothelioma has been used to designate various groups of vascular tumours. Mulliken, mdb adivision of plastic surgery, department of surgery, cincinnati childrens medical center, ml2020, 3333 burnet avenue, cincinnati, oh 45229, usa bdivision of plastic surgery, department of surgery, childrens hospital and harvard medical school, hunnewell 178. Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Glut1 positive lesions classified as hepatic infantile haemangioma. Of 16 infants with infantile hepatic hemangioendothelioma, 14 88% presented before age 3 months. It typically presents between two and four months of age as a rapidly growing mass. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. It is the most common symptomatic vascular tumor of infancy, with 85% presenting in the first 6 months of life. Infantile hepatic hemangioendothelioma benign infantile.
Almost 85% of patients with ihhe are diagnosed during the first 6 months of life, and ihhe is the most common symptomatic tumor occurring during this time period. Hemangioendothelioma, haemangioendothelioma, cellular hemangioma, histiocytoid hemangioma, and angioendothelioma are are phrases that may be used interchangeably. Please remove adblock adverts are the main source of revenue for dovemed. The lesion is composed predominately of endothelial cells and has characteristic phases of rapid growth caused by cellular proliferation and spontaneous involution which can be accelerated by the use of angiogenesis inhibitors. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. The biopsy of the cyst which was large in size which had grown in a samall benign form over last ten to twelve years reveleade it to be ehe. Hemangiomas are the most common type of benign hepatic tumor. Benign infantile hemangioendothelioma cellular hemangioma of infancy. Infantile hepatic hemangioendothelioma, abbreviated ihh, is a type of hemangioendothelioma. Ihh is the most common benign hepatic tumor found in infants, but malignant transformations have been reported 1, 11, 12.
By contrast, infantile hemangioma ih, also referred to as infantile hemangioendothelioma, is a vascular liver tumor arising in. Draper et al cured 3 patients of diffuse lesions by combining. The case 2yearold male child palpable mobile abdominal lumps recurrent episodes of subacute intestinal obstruction weakness and weight loss on radiological evaluation intestinal. Full text infantile hemangioendothelioma case reports. Type 2 lesions tend to be more aggressive and more often associated with congestive cardiac failure 5. Infantile hemangioendothelioma of the liversonographic diagnosis and followup. Pathology of hemangioendothelioma dr sampurna roy md. It is the most common vascular tumour of the liver in infants. Infantile hemangioendothelioma of the parotid gland. Well demarcated or infiltrative 35% pure type 1 change. Most of the lesions are diagnosed in the 1 st few months of life.
Hemangioendothelioma an overview sciencedirect topics. Infantile hepatic hemangioma is a proliferative endothelial cell neoplasm that involves the liver. Infantile hepatic hemangioendothelioma ihhe is the most common vascular tumor of the liver in children, accounting for 12% of all childhood hepatic tumors. Multiple cutaneous infantile hemangiomas associated with hepatic.
Other vascular tumours in the liver haemangioma, epithelioid. Hepatic tumors in children are relatively uncommon and account for 23% of all pediatric tumors, however infantile hemagioendothelioma is the third most common hepatic tumor accounting for 12% of all childhood tumors. In adults, the major histological type is a cavernous hemangioma. However, a distinct, diseasedefining genetic alteration recently described for ehe indicates that it is an entirely separate entity from both angiosarcoma and hemangioma. Apparently, there is no good correlation between tumor size and the degree of congestive heart failure 4. In less severe forms spontaneous regression has been described. Learn indepth information on infantile hemangioendothelioma of salivary gland, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. Epithelioid hemangioendothelioma is a rare malignant neoplasm which can occur only in bone or as a multiorgan disease230. Imaging diagnosis and followup of infantile hepatic haemangioendothelioma. Epithelioid hemangioendothelioma tumors most commonly affect the soft tissues, liver, lungs and bones. Hemangioendothelioma may be classified into 4 groups including epitheloid hemangioendothelioma most common, kaposiform hemangioendothelioma, retiform hemangioendothelioma, and infantile hemangioendothelioma. Infantile hepatic hemangioendothelioma ihh is a vascular tumor originating from the mesenchymal liver tissue. Infantile hemangioendothelioma of the parotid gland year. End of skin nonmelanocytic tumors vascular tumors hemangioendothelioma and subtypes.
Mulliken, md,a and odile enjolras, mdb boston, massachusetts, and paris, france rapid postnatal growth and slow involution in childhood characterize the common infantile hemangioma. The term hemangioendothelioma describes several types of vascular neosplasms and includes both noncancerous benign and cancerous malignant growths. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide. It is a benign condition that undergoes spontaneous regression and does not warrant intervention. Infantile hepatic hemangioendothelioma ihh is rare which can regress. Infantile hemangioendothelioma is a rare benign vascular tumor of the liver. Infantile hemangioendothelioma an interesting case dr ratnadeep ganguly md,dnb,miac,mnams dept of pathology 2. Some of these entities are benign non neoplastic reactive lesions. Infantile hemangioendothelioma of the liver revisited. Vascular tumors of intermediate malignancy frequently present in the skin and often cause diagnostic diffi culty.
Nopper, md, the section on dermatology, section on otolaryngologyhead and neck surgery, and section on plastic surgery abstract infantile hemangiomas ihs are the most common tumors of childhood. It is clinically evident within the first six months after birth and can be life threatening because of heart failure, intraperitoneal hemorrhage or thrombocytopenia. The terms arteriovenous malformation, infantile hemangioma capillary or cavernous, and hemangioendothelioma are used synonymously to describe vascular hepatic tumors described before 6 months of age 1. For seven cases 44%, the diagnosis was suspected from antenatal ultrasonographic findings. Skin tumor nonmelanocytic hemangioendothelioma and. Pseudomyogenic hemangioendothelioma was recognized as a distinctive entity under the name epithelioid sarcomalike hemangioendothelioma in 2003. Usually the lesion is present in the first six months of life 710. Infantile hemangioendothelioma is the most com mon symptomatic. An unusual presentation and progression of infantile hemangioendothelioma is reported in a 19monthold female child. Introduction infantile hemangioendothelioma is the third most common hepatic tumor in children, seen in 12% of all childhood hepatic tumors. The term has also been applied to those that show borderline behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas.
Epithelioid hemangioendothelioma eh is a rare tumor of adults that should be distinguished from infantile hemangioendothelioma, which occurs in infants and children. We report here a case of infantile hemangioendothelioma type ii, which is unusual for its presentation and histomorphology. Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence. Korecc a bs t rac t infantile hemangioendothelioma which is the most common primary. The cells generally had a rounded, epithelioid character, regular nuclei, and relatively few mitotic figures. Name l define l risk l detect l treatment l stagegrade l followup l metsrecur l link. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. The trip database provides clinical publications about. Hemangioendothelioma definition of hemangioendothelioma.
Request pdf on researchgate hepatic hemangioendothelioma. We report a case of hepatic hemangioendothelioma in an 8 monthold female infant who presented with hepatomegaly and respiratory distress, which was successfully treated with oral prednisolone for six months. Diagnosis and management of infantile hemangioma david h. However, infantile hemangioendothelioma is the third most common hepatic tumor in children 12% of all childhood hepatic tumors, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the.
It is usually considered as a tumor with a benign course. Hemangioendothelioma article about hemangioendothelioma. Hemangioendotheliomas are caused by abnormal growth of blood. A clinicopathologic study of lnfantile hemangioendothelioma of the liver chang won ha, chong jai kim and je g.
They may undergo spontaneous regression but can sometimes be lifethreatening due to congestive cardiac failure ccf andor consumptive thrombocytopenia and coagulopathy. Infantile hemangiomas, the most common tumours of infancy, are vascular tumours characterized by rapid proliferation of endothelial cells during the first few months of postnatal life followed by slow spontaneous involution. We report the case of 12 week and 11 day old female patient who was retroviral disease rvd positive. Utility of immunohistochemistry for endothelial markers in distinguishing epithelioid hemangioendothelioma from carciinoma metastatic to bone. Epithelioid hemangioendothelioma ehae is a rare tumor, first characterized by sharon weiss and franz enzinger that both clinically and histologically is intermediate between angiosarcoma and hemangioma. This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patients clinical data using reasonable medical judgment.