Cutaneous Hemangiomas Research Articles

Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules

To investigate and define a morphologically distinctive group of cutaneous papillary haemangiomas. Eleven patients (seven male, four female, age range 1-77 years, median 57) were identified with a solitary bluish cutaneous papule (median size 11 mm) arising in the head and neck region. Most lesions had been present for several years. None of the patients had associated systemic disease or polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome. Only one lesion recurred locally. The lesions showed predominantly intravascular papillary growth within multiple ectatic dermal vessels. The papillae had cellular cores containing pericytes and stromal cells, arranged around normal small capillaries. The surfaces of the papillae were covered by focally swollen endothelial cells containing numerous hyaline globules, ultrastructurally representing giant lysosomes containing organelle debris and fat vacuoles (so-called thanatosomes). These endothelial cells were immunopositive for CD31 and CD34 but negative for D2-40 (podoplanin). Papillary haemangioma is a distinctive benign cutaneous lesion containing eosinophilic hyaline globules consistent with dysfunction of the autophagocytic-lysosomal pathway.

P30.13: MRI and 3D ultrasound: a combined evaluation for placenta percreta? Case report

Congenital hemangiomas are rare solitary lesions, mostly located in the head and the limbs. Giant hemangiomas are frequently associated with life-threatening complications such as high-output heart failure, anemia, thrombocytopenia and disseminated intravascular coagulopathy (Kasabach-Merritt syndrome). Prenatal diagnosis and management is possible in the second and third trimesters of pregnancy with twoand three-dimensional sonography, color Doppler ultrasound and magnetic resonnace imaging. We present two cases of cutaneous limb hemangioma. In the first fetus, anemia and heart failure were the presenting features associated to the diagnosis of a giant hemangioma of the leg at 26 weeks of pregnancy. After full information on diagnosis and prognosis, future parents decided to terminate the pregnancy. In the second fetus, an upper limb hemangioma was diagnosed at 33 weeks of pregnancy. Delivery was anticipated because anemia was suspected. After birth, a low platelet count was descovered, pointing to a Kasabach-Merritt sequence. The female newborn was started on high dose prednisone with a rapid reduction in size of the tumor. These examples illustrate the variable outcome of seemingly similar hemangiomas and underlines the value of proper imaging studies in making accurate decisions with respect to handling.

P30.12: Prenatal diagnosis of velamentous cord insertion and vasa previa by MRI: a case report

Congenital hemangiomas are rare solitary lesions, mostly located in the head and the limbs. Giant hemangiomas are frequently associated with life-threatening complications such as high-output heart failure, anemia, thrombocytopenia and disseminated intravascular coagulopathy (Kasabach-Merritt syndrome). Prenatal diagnosis and management is possible in the second and third trimesters of pregnancy with twoand three-dimensional sonography, color Doppler ultrasound and magnetic resonnace imaging. We present two cases of cutaneous limb hemangioma. In the first fetus, anemia and heart failure were the presenting features associated to the diagnosis of a giant hemangioma of the leg at 26 weeks of pregnancy. After full information on diagnosis and prognosis, future parents decided to terminate the pregnancy. In the second fetus, an upper limb hemangioma was diagnosed at 33 weeks of pregnancy. Delivery was anticipated because anemia was suspected. After birth, a low platelet count was descovered, pointing to a Kasabach-Merritt sequence. The female newborn was started on high dose prednisone with a rapid reduction in size of the tumor. These examples illustrate the variable outcome of seemingly similar hemangiomas and underlines the value of proper imaging studies in making accurate decisions with respect to handling.

Management of urethral hemangiomas associated with Klippel–Trenaunay–Weber syndrome by endoscopic sclerotherapy

Klippel-Trenaunay-Weber syndrome (KTS) is an unusual congenital anomaly characterized by cutaneous hemangiomas, varicosities and bony hypertrophy of the extremities. Herein the case is reported of a 24-year-old man with urethral bleeding from hemangiomas associated with KTS that were successfully managed by endoscopic sclerotherapy. A 23-G puncture needle was inserted into the bleeding vein to inject a 5% solution of monoethanolamine oleate (Oldamine), which is typically used for sclerotherapy of esophageal vasix. At a 4-month follow-up, the patient only had slightly bloodstained urethral discharge, and is doing well. This is the first case reporting endoscopic sclerotherapy for a KTS-associated urethral hemangioma.

Circular excision and purse-string suture technique in the management of facial hemangiomas

Hemangiomas are the most common soft tissue tumors in infancy. Treatment options for hemangiomas are numerous. The scar after lenticular excision and linear closure is often unacceptable. Localized cutaneous infantile hemangioma acts like a tissue expander. Therefore, circular excision and purse-string suture technique are applicable for hemangioma at any stage. For 2-year period we have operated 14 patients with hemangioma using circular excision and purse-string suture technique. In eight patients hemangioma was localized in the head and neck region. Two infants with large hemangioma of the face are presented in this article. One patient had hemangioma with ulceration resistant to conservative treatment. Second patient had giant hemangioma, which caused partial disturbation of visual axis. We applied circular excision and purse-string suture technique in both patients. The results were good, without any postoperative complications.

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.

CAVERNOUS HEMANGIOMA OF THE CAVERNOUS SINUS, SKIN, AND RETINA

There are several reports concerning cavernous hemangiomas of the skin and central nervous system. Additional retinal involvement has also been reported. The authors report a 69-year-old woman with a giant extra-axial cavernous hemangioma of the right cavernous sinus involving the supra- and parasellar region, retina, and skin. Shrinkage of its cutaneous part lead to subsequent increase of the volume of the intracranial part. Owing to compression of the optic and the oculomotor nerves, oculomotor disturbances, ptosis, and visual impairment to 0.2 occurred. Via a pterional approach microsurgical removal of the tumor except for a remnant of the intracavernous part was performed. Hemodynamic connection between cutaneous, retinal, and intracranial hemangiomas should be considered.

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.

Cutaneous Hemangioma Associated With Persistence of the Trigeminal and Both Proatlantal Arteries

The authors describe a girl who was evaluated at 7 years old because of facial segmental hemangioma associated with unilateral persistent trigeminal artery, bilateral proatlantal arteries, hypoplasia of 1 posterior cerebral artery, kinking of 1 internal carotid artery at 2 different levels, and transdural collateral vascularization supplying the posterior areas of the cerebral hemispheres. This is the first patient known to have a cutaneous hemangioma associated with bilateral proatlantal arteries despite having a unilateral facial hemangioma.

Corticosteroids therapy in the management of infantile cutaneous hemangiomas.

To determine the effectiveness of systemic and intra-lesional corticosteroids, in the management of infantile cutaneous hemangiomas and to compare the results with untreated lesions. Randomized controlled trial. Surgical Unit B of National Institute of Child Health, Karachi, from January 2001 to January 2003. A total of 75 cases with cutaneous hemangiomas were included in the study. Three groups of 25 patients each were made by allocating first patient to control group, every second patient to oral steroid group and every third patient to intra-lesional steroid group. In control group, patients were managed conservatively with repeated hospital visits in order to note down changes occurring in the lesion. In oral steroid group, tablet prednisolone was given at a dose of 2mg/kg/day on alternate days. The dose was gradually tapered over a period of 8 weeks. In intra-lesional steroid group, injection triamcinolone was given at a dose of 1 to 5 mg/kg, with a maximum dose of 30mg. Six injections of triamcinolone in monthly intervals were given. Outcome measures noted were the change in size before and after treatment, duration of proliferative phase, change in morphology and complications. In the control group,76% lesions showed little or no change, 5.8% had less than 50% reduction and 20% lesions showed actual increase in size. In oral steroid group, 32% lesions showed greater than 50% reduction in size, 44% lesions had less than 50% reduction and 24% lesions had little or no change. In intra-lesional steroid group, 44% lesions showed greater than 50% reduction in size, 32% lesions showed less than 50% reduction and 24% lesions had little or no change. Morphology change in terms of color change, surface bosselation and soft consistency was found in 88% lesions of oral steroid group, 92% in intra-lesional steroid group and 16% in observation group. Complication rate was 16% in observation group, 20% in oral steroid group and 24% in intra-lesional steroid group. Hemangiomas when left untreated behave in an unpredictable manner. Low dose steroids whether given orally or intra-lesionally, showed remarkable reduction in size with minimal side effects.

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.

Absent/reduced glucose transporter-1 protein expression in infantile subglottic haemangiomas

Positive immunohistochemical staining for glucose transporter-1 protein (GLUT1) is a characteristic of cutaneous infantile haemangiomas. To examine GLUT1 expression in subglottic haemangiomas. Review of clinical notes and biopsy tissue with immunostaining for GLUT1 in 14 patients with subglottic haemangiomas. GLUT1 immunostaining was negative in 11 cases, and focally positive in three. No subglottic haemangiomas demonstrated the intense diffuse positive GLUT1 staining seen in cutaneous infantile haemangiomas. Five patients had cutaneous as well as subglottic haemangiomas, one of whom had a GLUT1-negative subglottic haemangioma and a GLUT1-positive cutaneous haemangioma of the lip. Subglottic haemangiomas appear immunohistochemically different from cutaneous infantile haemangiomas, which may reflect differences in endothelial cell differentiation or underlying aetiology.

P157: Treatment of Cutaneous Hemangiomas with Topical Imiquimod 5%

P157: Treatment of Cutaneous Hemangiomas with Topical Imiquimod 5%

Tumores neonatales: características clínicas y terapéuticas. Análisis de 72 casos del hospital infantil La Fe de Valencia

Neonatal tumors, which represent only 1.5-2% of all pediatric tumors, have distinctive features. Their incidence is imprecise, as selection criteria vary and there are few published studies. To analyze and disseminate findings on the histological, clinical, therapeutic and follow-up characteristics of neonatal tumors. We performed a retrospective study of the clinical records of patients diagnosed with neonatal tumors in La Fe University Children's Hospital in Valencia (Spain) between January 1990 and December 1999. Hamartomas, flat and cavernous cutaneous hemangiomas, nevi, lipomas, subcutaneous fibroma, lymphangiomas, and epidermal cysts were excluded. A systematic review of the literature published in the previous 25 years was performed using Medline, Cancerlit, Index Citation Science, and Embase. The search profile combined neonatal or congenital and tumor or cancer or neoplasm. The most interesting studies, as well as the most relevant references contained in these studies and published before the search period, were selected. The clinical records of 72 patients with neonatal tumors (40 boys and 32 girls), representing 2.8% of all pediatric tumors, were reviewed. The most frequent tumors were hemangiomas (20.8%, 15 patients), neuroblastomas (16.7%, 12 patients), teratomas (12.5 %, 9 patients), and soft tissue tumors (9.7 %, 7 patients). Eighty-six percent of the patients were symptomatic during the first week of life. Although diagnosis was prenatal in 22.2 % of the patients, the most frequent findings on physical examination were identification of a mass or cutaneous lesion in 24 patients (33.3%) and an abdominal mass or hepatomegaly in 13 patients (18%). An associated disease, malformation or syndrome was found in 15 patients (20.8 %). Treatment included surgery (50% of patients) and drugs as monotherapy or coadjuvant therapy (13.9%). Thirteen patients received irradiation after the neonatal period. Spontaneous complete remission took place in six patients. Twenty patients died (27.8%). Mortality was highest in patients with central nervous system tumors or leukemias (83.3% and 75 % respectively). By contrast, none of the patients with hemangiomas or teratomas died. Mortality was low in patients with neuroblastoma (8.3%). At the time of the study, survival was 73 %, with a median follow-up of 8 years. Due to their biological features, neonatal tumors represent a distinctive subgroup in pediatric oncohematology. The concept of neonatal tumor should be unified to allow the results of different research groups to be analyzed and compared. Despite the methodological limitations found, the clinical, diagnostic, therapeutic, and follow-up characteristics of our patients are similar to those of other published series. The differences found could be explained by the diverse selection criteria employed.

Les hémangiomes cutanés : aspects cliniques

Infantile cutaneous hemangioma is a benign vascular tumour present at 10% of the infants. It forms part of the group of the vascular tumours in the classification of International Society for Vascular Anomalies (ISSVA). Clinical diagnosis is easy in its triphasic typical form with a phase of sometimes brutal postnatal growth, a phase of stabilization and a phase of slow secondary regression. Classically, it is presented in the form of a mass or stains cutaneous red, of a subcutaneous mass or, generally, of a mixed form associating the two aspects.

Deviated nasal septum in case of Klippel-Trenaunay-Weber Syndrome

Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare entity. It includes cutaneous hemangioma (Port-wine stains) of the face and extremities with associated varicosities and hypertrophy of underlying soft tissue and bone. Craniofacial involvement is rare in this syndrome. We report a case of Klippel-Trenaunay-Weber Syndrome who presented with craniofacial deformity leading to deviated nasal septum, nasal obstruction and intermittent nasal bleed. These patients can have involvement of oral cavity and nasal mucosa with angiomatous malformation, which can give rise to epistaxis and excessive bleeding during oral and nasal surgeries. We discuss the review and management of such cases from Otolaryngologists point of view.

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

PurposeTo explain the considerations governing the anesthetic management of pregnant patients with Klippel-Trenaunay syndrome (KTS). Klippel-Trenaunay syndrome is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy; and is associated with both hemorrhagic and thrombotic complications. The importance of this diagnosis, including the presence of neuraxial vascular anomalies, is often under-appreciated by both obstetric and anesthesia providers. While regional anesthetic management of patients with KTS has been discussed by others, we present a case in which regional anesthesia presented an unwarranted risk to the patient.Clinical featuresAn obese, 18-yr-old parturient with a fetus in the breech position underwent Cesarean delivery at 35 weeks gestation secondary to evolving preeclampsia. Unfortunately, no neurovascular imaging of this patient’s spine was available. The patient underwent an attempted external cephalic version, a failed obstetric induction, and, ultimately, a Cesarean delivery under general anesthesia. The resulting infant was without any stigmata of KTS. Both mother and infant did well during the course of their hospitalization, and were discharged home without incident.ConclusionsThe posterior cutaneous hemangiomas of KTS may be associated with underlying epidural and subdural vascular malformations. Disruption of these vascular anomalies during regional anesthesia may lead to neuraxial hematoma formation, which may be further compounded by a consumptive coagulopathy observed in some cases of KTS. If neuraxial vascular anomalies cannot be ruled out radiographically, regional anesthesia should be avoided. Additionally, regardless of the anesthetic technique chosen, the coagulation profile of these patients should be verified for signs of coagulopathy.

Hemangiomas of the Pelvis

Hemangiomas of the colon and rectum are rare but often misdiagnosed causes of hematochezia. They are characterized by the clinical triad of recurrent episodes of painless rectal bleeding, multiple ectopic phleboliths on plain radiographs, and cutaneous hemangiomas. The majority of these tumors involve the rectosigmoid region. Diffuse cavernous lesions are the most common histological type. Endoscopy is important to define the extent and number of lesions. Imaging by computed tomography or magnetic resonance imaging is used to define invasion into pelvic structures. Surgical resection with preservation of the sphincters is the recommended treatment.

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.

Role of Intralesional Bleomycin in the Treatment of Complicated Hemangiomas: Prospective Clinical Study

Hemangioma is the most common tumor of infancy. Although it has a basically benign nature and usually spontaneously regresses, a small percentage (5%) have complications that need treatment. Many different therapeutic modalities can be used in this tumor. To investigate the effect of a new method of treatment (intralesional bleomycin injection) in complicated hemangiomas. In the Department of Radiation Oncology at Nemazee Hospital in Shiraz, Iran, from April 1992 to October 1998, 32 patients with complicated hemangioma were treated with four to six courses of direct injection of bleomycin into the lesion. After a minimum follow-up of 6 years, there was 70 to 100% regression in 18 patients, 50 to 70% in 7 cases, and less than 50% reduction in 7 patients. Intralesional injection of bleomycin is an easy, safe, and effective therapeutic modality in complicated cutaneous hemangiomas.