Visceral Hemangiomas Research Articles

Generalized Haemangiomas with Visceral Involvement: Case Report

Haemangiomas are the most common benign tumours of infancy. Most cases of haemangiomas present as solitary lesions, but about 20% have more than one lesion. The presence of 3 lesions is uncommon and the presence of 4 or more lesions is rare. The presence of multiple cutaneous haemangiomas greatly enhance the chances that the child could have visceral haemangioma. We report an 11- month old infant with multiple cutaneous haemangiomas with visceral involvement complicated by congestive cardiac failure. Initially,he responded to treatment with prednisolone and antifailure drugs, but died later as a result of exacerbation. Some of the problems associated with managing such cases in a resource-poor country are highlighted.

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.

Overlap of Sturge-Weber syndrome and Klippel-Trenaunay syndrome

Sturge–Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel–Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge–Weber syndrome in combination with Klippel–Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes.

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.

La chorioangiomatose : une cause rare d’anasarque fœtoplacentaire non immune. Implications pratiques obstétrico-pédiatriques

We report a case of chorioangiomatosis with hydrops fetalis as a complication. Hydrops fetalis associated with fetal distress led to preterm birth at 33 GW. Resuscitation was needed at birth. A systematic histologic exam showed diffuse chorioangiomatosis. There was no congenital hemangioma. We describe the features of chorioangiomatosis, a rare cause of nonimmune hydrops fetalis. Prenatal diagnosis of chorioangiomatosis before early recognition of severe maternal and fetal complications during pregnancy is infrequent, thus precluding early management of the pregnancy. Reports of associated chorioangioma and infantile hemangioma are frequent, illustrated with a recent pathophysiological hypothesis involving embolism of a placental angioblast as for the cause of congenital hemangioma. Investigations for associated cutaneous or visceral infantile hemangioma should be undertaken when chorioangioma is diagnosed.

An Immunohistochemical Analysis of Canine Haemangioma and Haemangiosarcoma

The aim of the present study was to investigate immunohistochemically aspects of the biology of canine endothelial neoplasia. Forty samples of canine cutaneous and visceral haemangiosarcoma (HSA), 29 samples of cutaneous and visceral haemangioma (HA) and 10 control samples of granulation tissue (GT) were labelled with antisera specific for vimentin, smooth muscle actin, von Willebrand factor (vWF), CD117 (KIT), vascular endothelial growth factor receptor-3 (VEGFR-3), vascular endothelial growth factor-C (VEGFC) and CD44. Further antisera were employed to determine the level of cellular proliferation (MIB-1 index) and toluidine blue staining was used to detect populations of tumour-infiltrating mast cells (MCs). There was greater expression of CD117, VEGFR-3 and CD44 in HSA than in HA, suggesting that these proteins might be suitable targets for the future development of novel therapeutic approaches to canine HSA. Marked infiltration of MC was detected in HA, suggesting a possible role for these cells in the pathogenesis of benign vascular neoplasia in the dog.

Diffuse Hemangiomatosis With Predominant Central Nervous System Involvement

Diffuse neonatal hemangiomatosis presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. Described here is a case of diffuse neonatal hemangiomatosis with predominant central nervous system involvement. The early appearance of central nervous system lesions on imaging can overlap with that of cavernous malformations, confounding diagnosis; however, rapid growth, response to steroids, cystic appearance with sedimentation levels of the mature lesions, and involvement of other visceral organs can help confirm the diagnosis.

Blue Rubber Bleb Nevus Syndrome

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by the presence of cutaneous and gastrointestinal (GI) hemangiomas. These BRBNS-associated hemangiomas, possibly numbering into the hundreds, may also be identified in multiple visceral locations. Although the presence of benign cutaneous lesions is of small concern, GI lesions are cause for bleeding. Heavy visceral organ involvement can lead to intravascular coagulation, consumptive coagulathopy, and organ failure. Therefore, GI-related complaints, anemia, the visual identification of a cutaneous blue-colored lesion located on the upper extremity or trunk, and multiple visceral hemangiomas involving a large portion of the organ may indicate the presence of BRBNS. It is important that this unusual combination of patient signs, symptoms, and sonographic findings be presented to the interpreting physician to facilitate inclusion of this rare condition as a possible differential diagnosis to be considered.

P26.14: Aortic aneurysm and sternal cleft: A variant of the PHACES syndrome?

Case: We report a case and associated images of an infant prenatally diagnosed with an aortic aneurysm and chest wall deformity. Postnatally, echocardiogram and MRI revealed a large aneurysm of the proximal descending aorta measuring 1.1 × 1.8 cm. The aneurysm began just distal to the origin of the left carotid artery, and a moderate pre-aneurysmal coarctation was noted. The origin of the left subclavian artery was distal to the aneurysm. In addition, partial sternal cleft with agenesis of the manubrium was noted. The intracranial anatomy was normal, and U/S and MRI revealed no hemangiomas of the head and neck vessels. Dermatologic evaluation revealed no evidence of cutaneous hemangiomas. Bronchoscopy revealed no hemangiomas of the respiratory tract. Ophthalmologic exam revealed normal ocular anatomy. Conclusion: Hemangiomas are the hallmark of both the sternal malformation/vascular dysplasia association and PHACES syndrome. We have, however, identified a case of sternal cleft with aortic aneurysm in the absence of cutaneous or visceral hemangiomas. Although these malformations may be unrelated, this case may also represent a variant of the PHACES syndrome.

Neonatal lupus erythematosus complicated by mucocutaneous and visceral hemangiomas

Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune syndrome resulting from transplacental passage of maternal anti-Ro/SSA and/or anti-La/SSB antibodies to the fetus. Characteristic manifestations of NLE include transient dermatitis, hepatic and hematologic abnormalities and congenital heart block. Skin lesions in NLE resemble subacute cutaneous lupus erythematosus and typically consist of annular, erythematous, scaly plaques. Telangiectasias, vascular abnormalities resulting from dilation of superficial dermal vessels, may also affect the skin in a minority of patients. The etiology of telangiectasias in NLE is unknown, but disordered angiogenesis likely plays a role. Hemangiomas are a common disorder of angiogenesis frequently encountered in infancy. There have been no reported cases of neonatal lupus associated with the development of hemangiomas. We present a case of an infant diagnosed with NLE after manifesting classic dermatitis, hepatic and hematologic abnormalities who later developed mucocutaneous and visceral hemangiomas. We further postulate that disordered angiogenesis, possibly dysregulated production of vascular endothelial growth factor, may play a primary role in the development of these cutaneous vascular lesions in NLE.

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.

A Rare Case of Diffuse Neonatal Hemangimatosis

Diffuse neonatal hemangiomatosis (DNH) is a rare neonatal condition in which cutaneous and visceral hemangiomas coexist. If left untreated, DNH is usually fatal at an early age. We report a case of a 6-month-old male infant who was brought to our institution with hepatosplenomegaly and a history of anemia and thrombocytopenia since 1 month of age. Cytogenetic analysis and liver biopsy were normal and bone marrow aspirate was nondiagnostic. Congenital red blood cell abnormality was ruled out. Ultrasound confirmed an increase in size of the spleen from 5 to 15 cm, and magnetic resonance imaging demonstrated intense splenic enhancement consistent with a hemangioma or vascular malformation. Despite severe thrombocytopenia, an exploratory laparotomy was done and the patient underwent a splenectomy and omentectomy. The final pathology confirmed hemangiomatosis of the spleen and omentum. In the neonate with unexplained anemia and thrombocytopenia, DNH should be considered as part of the differential diagnosis. In our case, the patient not only exhibited no obvious cutaneous involvement, but also had rare splenic involvement. Although there are risks involved when operating on a thrombocytopenic patient, the benefits of operating on a patient with DNH far outweigh the risks, and operative intervention should proceed without delay.

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.

Klippel-Trenaunay syndrome: clinical features, complications and management in children.

Clinical features and management of 47 children with Klippel-Trenaunay syndrome treated since 1970 were reviewed. Haemangiomas and soft tissue and/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.

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.

The blue rubber bleb nevus syndrome: a case with prominent head and neck findings

Abstract The blue rubber nevus syndrome describes a clinical entity that presents with cutaneous and visceral hemangiomas, particularly those found in the gastrointestinal tract. These lesions may appear at birth or in early childhood and can increase in size and frequency with age. Oral cavity hemangiomas occur in 59% to 64% of cases. Successful diagnosis requires a high index of suspicion, and treatment is symptomatic. This report discusses a unique presentation and a review of the literature.

A Retrospective Study of Visceral and Nonvisceral Hemangiosarcoma and Hemangiomas in Domestic Animals

Cases of hemangiosarcoma submitted to the Colorado State University Veterinary Diagnostic Laboratory during a 6-year period were reviewed. Visceral hemangiosarcomas represent less than 2% of canine specimens submitted for histologic examination and nonvisceral hemangiosarcoma less than 1%. Most nonvisceral hemangiosarcomas of dogs occur in skin. Hemangiosarcomas are less common in cats and usually occur in skin. They are also rare in other animal species. Animals with nonvisceral hemangiosarcomas are usually mature; dogs and cats average 10 years of age. The tumors develop in many different locations, and there is no sex predilection. A wide variety of dog breeds are affected, but Italian greyhounds, greyhounds, and whippets are overrepresented. Clinical outcomes of 76 cases of nonvisceral hemangiosarcomas in dogs and cats were obtained from submitting veterinarians. Completeness of excision of a tumor is the most important factor that can be used in predicting clinical outcome for an affected dog or cat. In all cases in which the animals were clinically normal for at least 1 year after surgical removal of a nonvisceral hemangiosarcoma, the margins were reported to be free of neoplastic cells. Degree of differentiation, mitotic rate, size of tumor, and presence or absence of epidermal ulceration, mast cells, or solar elastosis did not correlate with clinical outcome.

Kasabach–Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association

Kasabach-Merrit syndrome (KMS) is very rarely observed in adults associated with visceral hemangiomas. Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare clinical entity with an intermediate malignant potential and a mortality rate of 20-30%. We described a case of KMS associated with HEHE in a 70 year old male patient who presented coagulation abnormalities, low platelet count and high fibrin degradation products that were typical features of KMS. Histopathology of the hepatic tissue and a positive immunostaining for endothelial markers confirmed the diagnosis of an HEHE. The first description of KMS was made in children in 1940. It was described as a "consumptive coagulopathy with capillary hemangiomas". However, recent reports have shown that this condition could be associated with kaposiform hemangioendotheliomas, an aggressive form of giant hemangioma. A giant hemangioma in adults as well as an hemangioendothelioma in children could be associated KMS, but an HEHE has been rarely reported in association with this syndrome in adult patients.

Complicaciones en la evolución de los hemangiomas y malformaciones vasculares

The differentiation of haemangiomas and vascular malformations is histological, clinical and prognostic. Although the majority of haemangiomas evolve towards spontaneous resolution, as many as 10% of cases can develop complications with ulceration, pain and haemorrhaging. Besides, the localisation of haemangiomas in the head and neck, next to vital structures, can compromise their functions. Hence, compression of the airway might be a vital emergency. Periorbital haemangiomas can give rise to amblyopia due to sensory deprivation or due to a restrictive strabismus. Lumbosacral haemangiomas must be studied with Nuclear Magnetic Resonance because of their frequent association with alterations in the midline at the level of the spine, anus, genitals or kidneys. Amongst visceral haemangiomas, hepatic haemangiomas are the most serious due to their association with congestive cardiac insufficiency. The association of extensive facial haemangiomas with anomalies of the central nervous system, vascular, cardiac, ocular and sternal anomalies, is denominated PHACE syndrome and is frequently complicated by mental deficiency, convulsions or ictus. Vascular malformations of trigeminal localisation are associated in up to 15% of cases with glaucoma or choroidal or leptomeningeal haemangiomas (Sturge-Weber syndrome). Combined vascular malformations localised in the extremities can become complicated with thrombophlebitis, regional osteolysis and even distant thromboembolisms (Klippel-Treneaunay Syndrome). On the other hand, there is a coagulopathy due to consumption (Kassabach-Merrit Syndrome) that can complicate some vascular tumours such as the Kaposiform haemangioendothelioma and the tufted angioma. Finally, the complications of the treatments employed are reviewed.

Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome

PurposeTo report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas. DesignInterventional case report. MethodsReview of clinical findings, radiologic studies, and treatment of the patient. ResultsA 36-year-old female with multifocal, bilateral orbital lesions was seen with severe proptosis and decreased visual acuity of the left eye. Surgical debulking of multiple cavernous hemangiomas of the orbit resulted in decreased proptosis and an improvement in visual acuity. Subsequent histopathologic analysis and the results of a systemic workup revealing multiple, distinctive, cutaneous and visceral lesions were found to be consistent with the blue rubber bleb nevus syndrome. ConclusionsAlthough hemangiomas of the orbit are relatively common occurrences, multifocal, bilateral lesions may exist in the setting of underlying systemic hemangiomatosis. Bilateral or multifocal hemangiomas associated with cutaneous or visceral lesions should alert the clinician to the possibility of a number of syndromes that may have life-threatening consequences.