Multiple Cutaneous Hemangiomas Research Articles

Prospective Study of the Frequency of Hepatic Hemangiomas in Infants with Multiple Cutaneous Infantile Hemangiomas

Multiple cutaneous infantile hemangiomas have been associated with hepatic hemangiomas. Screening of infants with five or more cutaneous infantile hemangiomas with abdominal ultrasound is often recommended. The aim of this study was to determine the frequency with which hepatic hemangiomas occur in infants with five or more cutaneous infantile hemangiomas compared to those with one to four cutaneous infantile hemangiomas and to characterize the clinical features of these hepatic hemangiomas. A multicenter prospective study of children with cutaneous infantile hemangiomas was conducted at pediatric dermatology clinics at Hemangioma Investigator Groups sites in the United States, Canada, and Spain between October 2005 and December 2008. Data were collected, and abdominal ultrasonography was performed on infants younger than 6 months old with five or more cutaneous infantile hemangiomas and those with one to four cutaneous infantile hemangiomas. Twenty-four (16%) of the 151 infants with five or more cutaneous infantile hemangiomas had hepatic hemangiomas identified on abdominal ultrasound, versus none of the infants with fewer than five (p = 0.003). Two of the 24 infants with hepatic hemangiomas received treatment specifically for their hepatic hemangiomas. Infants with five or more cutaneous infantile hemangiomas have a statistically significantly greater frequency of hepatic hemangiomas than those with fewer than 5. These findings support the recommendation of five or more cutaneous infantile hemangiomas as a threshold for screening infants younger than 6 months old for hepatic hemangiomas but also demonstrate that the large majority of these infants with hepatic hemangiomas do not require treatment.

An unusual case of congenital cardiac cyst of the interventricular septum

We present a very rare case of a neonatal multiple haemangiomatosis with a cystic interventricular cardiac haemangioma. An anechoic cyst of 10 mm of diameter, located in the superior third of the interventricular septum was found by foetal echocardiography at 30 weeks of pregnancy. The baby was born at 39 weeks of pregnancy and the skin was covered with multiple purple cutaneous haemangiomas. As neonatal haemangiomatosis may be associated with visceral haemangiomas, the diagnosis of cardiac haemangioma was considered and transthoracic echocardiography revealed a translucent cyst consistent with this diagnosis. At 6 months of age, the cardiac mass was barely visible and most cutaneous lesions had regressed without treatment. Cardiac haemangiomas must be considered in the diagnosis of cystic masses, and the present case emphasizes the tendency towards spontaneous regression of these lesions.

Low-Dose Propranolol for Multiple Hepatic and Cutaneous Hemangiomas With Deranged Liver Function

We report here the case of an infant with multiple hepatic and cutaneous infantile hemangiomas (IHs) associated with deranged liver function who was treated successfully with low-dose propranolol. We also discuss our recent data that show that IH is a developmental anomaly of hemogenic endothelium derived from primitive mesoderm with a neural crest-cell phenotype. We previously presented evidence that this hemogenic endothelium is governed by the renin-angiotensin system, which we propose can account for both the action of propranolol and the process of spontaneous involution of IH. We further speculate on the possibility of using inhibitors of angiotensin-converting enzyme and that of angiotensin II receptor 2 as potential alternative therapies.

Multiple Hemangiomas and Hemangiomatosis - Risk Factors and Outcome over an Eight Year Period

Hemangiomas are the most common tumors of infancy. Multiple cutaneous hemangiomas may be associated with the presence of hemangiomas in inner organs. However, there is little data on the risk factors for organ involvement and the outcome of a large sample of patients. patients with 3 or more cutaneous hemangiomas were evaluated with regard to patient characteristics, distribution of hemangiomas, results of radiological abdominal/cerebral imaging, clinical course, and therapeutic approach. We analyzed the risk factors for organ involvement and complications/outcome. The average gestational week at birth was 32.8; radiological imaging showed liver hemangiomas in 13.5% and mesenteric lesions in 1 (1.9%) but no cerebral lesions. Preterm infants (p=0.02) and patients with high numbers of cutaneous hemangiomas (p=0.02) were at higher risk of organ involvement. A life-threatening event occurred in 1 patient (1.9%). None of the patients died. Organ manifestation is relatively common in patients with multiple hemangiomas, complications are rare, but potentially life-threatening. We recommend abdominal imaging for patients with 3 or more hemangiomas, especially in preterm infants.

Vincristine, an efficacious alternative for diffuse neonatal haemangiomatosis

Diffuse neonatal haemangiomatosis (DNH) is an uncommon condition characterized by multiple cutaneous and visceral haemangiomas frequently causing severe complications. Corticosteroids constitute the first therapeutic line; however, when they fail, other alternatives are available, provided possible side effects are closely monitored during and after treatment. We present a case of life-threatening DNH, non-responsive to corticosteroids, successfully treated with Vincristine with minor side effects. We conclude that Vincristine is a valid alternative in corticosteroid-resistant DNH.

Intracranial hemangiomas in a patient with POEMS syndrome

POEMS syndrome is a rare multi-system disease with typical features of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder and skin changes. We describe a 44-year-old woman with polyneuropathy, hepatomegaly, IgA lambda-plasmacytoma, thrombocytosis, papilledema with elevated protein levels in cerebrospinal fluid and multiple cutaneous hemangiomas who was diagnosed with three intracranial lesions. Histology revealed capillary hemangiomas, one of them displaying partially glomeruloid features.

Spectrum of hepatic hemangiomas: management and outcome

Purpose Infants with multiple cutaneous hemangiomas often present with hepatic hemangiomas. They can follow a benign clinical course or require complex management. We reviewed our experience in the management of hepatic hemangiomas. Methods We performed a retrospective review of patients (1996-2007) with hepatic hemangiomas treated in our institution. Results Twenty-six patients were diagnosed with hepatic hemangiomas as follows: 8 focal, 12 multiple, and 6 diffuse lesions. Nineteen (73%) patients had associated cutaneous hemangiomas. Sixteen patients had multiple and 3 patients had single cutaneous hemangiomas. All patients with multiple or diffuse liver lesions were screened for heart failure and hypothyroidism. Congestive heart failure developed in 4 patients, 3/4 of these patients had diffuse lesions. Two patients required thyroid replacement because of elevated thyroid-stimulating hormone. Because of progression of disease, 9 patients required steroid treatment. Two patients were treated with vincristine and 3 patients received α-interferon because of poor response to steroid treatment. Two patients went on to surgical resection for failed response to medical management and worsening heart failure (left lobectomy, liver transplant). Both patients had uncomplicated postoperative courses. Five patients had a previously undescribed constellation of rapidly involuting cutaneous hemangiomas (gone by 3 months, glut-1–negative) with associated liver lesions also resolving at a faster pace (mean resolution of cutaneous hemangiomas, 1.9 vs 7.9 months; P = .001; liver, 5.8 vs 25.3 months; P = .004). All patients in our series survived. Conclusion Patients with multiple cutaneous hemangiomas should be screened for hepatic lesions. Patients with diffuse or multifocal liver hemangiomas should be screened for congestive heart failure and hypothyroidism. A subgroup of rapidly involuting cutaneous hemangiomas have a significantly shorter time for involution of hepatic lesions. The status of cutaneous lesions can be used as indicators for the liver hemangiomas.

Retrospective evaluation of hepatic hemangiomas in patients with multiple cutaneous hemangiomas

Retrospective evaluation of hepatic hemangiomas in patients with multiple cutaneous hemangiomas

Simultaneous occurrence of infantile hemangioma and congenital melanocytic nevus: Coincidence or real association?

Both infantile hemangiomas (IH) and congenital melanocytic nevi (CMN) are common birthmarks that affect approximately 4% to 5% of infants and 1% of newborns, respectively. No previous association has been established between these birthmarks. We report a series of 6 patients who were remarkable for either the proximity or extent of anatomic involvement of their IH and CMN. Patient 1 had a large CMN in the area of her left eye and upper face in addition to a parotid IH. Likewise, patient 5 had a giant CMN on her back and a beard distribution IH. Patients 2, 3, and 4 were found to have small CMNs and localized IH in close anatomic proximity. Patient 6 was seen for a large left parietal scalp CMN with satellite nevi and multiple cutaneous hemangiomas. Although IH and CMN are common birthmarks, the clinical characteristics in these cases suggest a real, not coincidental association. Possible pathogenetic mechanisms to explain this association are discussed.

PHACE Syndrome with Intracerebral Hemangiomas, Heterotopia, and Endocrine Dysfunction

PHACE is an acronym to describe the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, and eye abnormalities. More than 200 cases have been reported. The present report presents the cases of two female infants with PHACE syndrome, both of whom had additional congenital defects of subependymal gray matter heterotopia, craniofacial arterial anomalies, and pituitary dysfunction. One had an extensive segmental facial hemangioma with ipsilateral intracranial hemangiomas. The other had multiple cutaneous hemangiomas, but no segmental facial hemangioma. These two cases suggest a further expansion of the spectrum of PHACE to include other forms of disordered cerebral development and endocrine dysfunction.

Umbilical cord hemangioma associated with multiple cutaneous hemangiomas in a newborn

Umbilical cord hemangiomas are rare with fewer than 50 cases reported in the English literature. Associated fetal cutaneous hemangiomas are exceptional. A 39 year old G3P1021 underwent ultrasound at 12 weeks. No abnormalities were detected. She underwent another ultrasound at 19+ weeks. A 1.8×1.9 cm homogeneous echogenic mass was noted at the base of the cord where it inserted into the placenta, interpreted as a hemangioma. Adjacent to the mass were two cystic structures, possibly pseudocysts, measuring 8.9×6.6 cm and 8.7×5.6 cm. Amniocentesis showed normal male karyotype. Serial ultrasounds revealed polyhydramnios and persistence of the mass. She presented at 37 weeks, in labor and a low transverse cesarean section was performed. A live 3455 gram male was delivered with Apgar scores of 9.9. The newborn had numerous port-wine stains involving the face bilaterally including the eyelids, neck, posterior occiput, superior chest, left shoulder, buttocks, and bilateral extremities. At 2 days old, the baby experienced mild tremors which resolved. Brain imaging revealed no evidence of brain parenchymal, leptomeningeal, or vascular abnormality, although the extradural space in the area of the confluence of sinuses showed a heterogeneous prominence, possibly a hemangioma. The child was discharged 5 days after delivery for follow-up. The placenta weighed 420 g. The trivascular umbilical cord measured 15 cm in length and showed fusiform dilation up to 7.3 cm in maximum diameter, over a length of 8 cm, beginning 1.2 cm from the site of placental insertion. A separate multicystic myxoid area was present adjacent to the maximally dilated cord on the fetal side (Fig. 1). Histologically, the lesion was consistent with hemangioma. Cord hemangioma, although rare, is the most common umbilical cord tumor. There is a significant morbidity and mortality association, including prematurity and fetal death [1]. Associated fetal anomalies include cutaneous and systemic hemangiomas [2,3]. Cutaneous hemangiomas are the most common benign tumor of infancy. Although most remain asymptomatic, some can cause significant morbidity. Our case shares some features with Sturge–Weber and Klippel–Trenaunay–Weber Syndromes but does not quite fit either. Interestingly, this case showed polyhydramnios which is infrequently seen in association with umbilical cord hemangioma [2]. Patients should be counseled of the risk for neonatal mortality and morbidity, including cutaneous or visceral hemangioma when umbilical cord hemangioma is diagnosed prenatally. Antepartum fetal surveillance may be of benefit.

Multiple Cutaneous Infantile Hemangiomas Associated With Hepatic Angiosarcoma: Case Report and Review of the Literature

Multiple cutaneous hemangiomas can be associated with internal hemangiomas, with the liver being the most common site. Here we report a case of a premature female neonate who presented with cardiac failure at birth and had typical-appearing infantile hemangiomas on the skin in association with vascular lesions in the liver. Her clinical presentation was felt to be consistent with cutaneous and hepatic infantile hemangiomas. After failure to respond to systemic steroids and chemotherapy, she underwent liver transplantation. Histopathologic evaluation of the liver revealed a diagnosis of type 2 infantile hepatic hemangioendothelioma (regarded as synonymous with angiosarcoma) rather than benign infantile hemangioma of the liver. Subsequent skin biopsies confirmed that her multiple cutaneous lesions were infantile hemangiomas and not metastatic angiosarcoma. We report this case and a review of the literature on pediatric angiosarcoma of the liver associated with cutaneous infantile hemangiomas.

Hepatic Infantile Hemangioendothelioma with Unusual Manifestations

Hepatic Infantile Hemangioendothelioma with Unusual Manifestations

Two infants with life‐threatening diffuse neonatal hemangiomatosis treated with cyclophosphamide

Diffuse neonatal hemangiomatosis (DNH) is a rare, frequently fatal disorder characterized by multiple cutaneous and visceral hemangiomas. Complications include high-output cardiac failure, hemorrhage, hepatic failure, and consumption coagulopathy. We present two cases of neonates with DNH. We prescribed cyclophosphamide after failure of corticosteroids. Cyclophosphamide induced a fast regression of the lesions with no side effects in both infants. We were able to stop treatment after 3-4 courses of cyclophosphamide in both infants because of the excellent response. Cyclophosphamide seems to be a safe and effective treatment in patients suffering from life-threatening DNH unresponsive to corticosteroids.

Diffuse Neonatal Hemangiomatosis

Multiple cutaneous hemangiomas of the skin have commonly been recognized as a marker to potentially life-threatening visceral hemangiomas. When cutaneous hemanigomas coexist with visceral hemangiomas, a rare condition known as diffuse neonatal hemangiomatosis is formed. Hemangiomas appear to form sporadically. Sonography is the initial imaging modality of choice. The advantages of sonography are that it is noninvasive, portable, and less expensive. An infant girl presented at 1 week of age with three cutaneous hemangiomas located on her left cheek, shoulder, and chest. A routine sonogram of the patient’s liver demonstrated several well-marginated, hypoechoic lesions within the liver. The pediatrician was notified, and a series of biweekly sonograms and steroid therapy were started. In this case, steroid therapy was successful, and the patient currently has no problems with hemangiomas.

Cutaneous haemangioma: prevalence and sonographic characteristics of associated hepatic haemangioma

AIMS: To determine the prevalence of hepatic haemangiomas (HH) occurring with multiple or large cutaneous haemangiomas (CH), and compare the ultrasound (US) characteristics of HH with morphology of concurrent CH. MATERIALS AND METHODS: Retrospective and prospective sonographic and clinical analysis was undertaken on 69 patients referred for US to exclude silent HH. This comprised 62 patients referred over 6 years with six or more CH or one large CH (⩾5 cm) and seven patients referred over 1 year with three to five small CH. RESULTS: HH occurred in 45% miliary CH and 40% mixed small and large CH, but also in 12–14% of patients with one large or three or more small CH. None had HH progression. There was association between HH sonographic characteristics and CH number and morphology. CONCLUSION: Clinically silent HH occurred in nearly one in four of infants presenting overall with six or more small CH or large CH. Previous studies have documented HH mortality between 18 and 80%, although this was not shown in our study. Therefore, if clinical suspicion exists, US is indicated to enable early steroid treatment if significant HH are present.

Tumores vasculares como marcadores sindrómicos

Haemangiomas can be indicators or clue signs for serious syndromes. Although less well known than those related to vascular malformations, there are some syndromes of important diagnostic value that are associated with haemangomias. Early recognition of problematic haemangiomas, together with a prompt intervention, may help to minimise their future morbidity. Neonatal haemangiomas warrant a special follow-up, since their growth pattern might be unpredictable at such an early age. Several clinical presentations are relevant because of their risk of syndromic association: cervicofacial haemangiomas, especially the extensive ones, may be markers for severe dysmorphic conditions like the PHACE(S) syndrome. Those distributed in the beard area are occasionally associated with haemangiomas of the airway. Lumbosacral haemangiomas usually hide an underlying spinal dysraphism or anorectal and urogenital anomalies. Multiple cutaneous haemangiomas may be a sign of visceral haemangiomatosis, most often hepatic, which becomes complicated by cardiac insufficiency or thyroid disease. Finally, there are two vascular neoplasms of rapid and invasive growth – kaposiform haemangioendothelioma and angioblastoma or tufted angioma – which, unlike infantile haemangioma, are markers for the Kasabach-Merritt syndrome.

Concurrent Infantile Hemangioendothelioma and Mesenchymal Hamartoma in a Developmentally Arrested Liver of an Infant Requiring Hepatic Transplantation

A newborn female underwent a surgical resection for a hepatic mass discovered prenatally by ultrasonography, and diagnosed pathologically as a mesenchymal hamartoma. Within 4 months after surgery, multiple cutaneous hemangiomas developed and a multinodular mass was detected in the liver. A liver biopsy showed an infantile hemangioendothelioma with type I features. An orthotopic liver transplant was performed due to the extensive nature of the hepatic involvement and progressive respiratory compromise. Virtually the entire liver was involved by a large infantile hemangioendothelioma. A multicystic mesenchymal hamartoma was also found on the left side. In addition, the uninvolved hepatic parenchyma had features recapitulating the fetal liver. This simultaneous involvement of the liver by a mesenchymal hamartoma and infantile hemangioendothelioma is unique. A review of the literature revealed only one incompletely characterized case with similar findings.

Diffuse Neonatal Hemangiomatosis Associated with Simpson-Golabi-Behmel Syndrome: A Case Report

Diffuse neonatal hemangiomatosis (DNH) is a rare disorder characterized by multiple cutaneous and visceral hemangiomas that usually presents in newborns and has a high mortality rate. While previously reported cases of DNH describe multiple cutaneous hemangiomas, we present a patient with a single hemangioma of the ear, who also had DNH of the central nervous system and visceral organs. Furthermore, in this report we present a new constellation of findings, namely, a Simpson-Golabi-Behmel syndrome (SGBS). The practical implication on the basis of the experience with our patient is the need to ensure adequate diagnostics for patients with large hemangiomas as well as for patients with multiple cutaneous hemangiomas, because DNH also can occur in single hemangiomas in rare cases.

Maffucci's Syndrome Associated with Chondrosarcoma and Aneurysm: Case Report

Maffucci syndrome is a rare congenital non-inherited condition characterized by multiple enchondromas and cutaneous hemangiomas. It is associated with increased risk of malignancy, including chondrosarcomas, and because of generalized mesodermal dysplasia, aneurysms can develop. We present a case of Maffucci syndrome associated with intracranial chondrosarcoma and aneurysm.