Early surgical treatment of cutaneous hemangiomas

Infantile hemangioma appears after the birth as a vascular tumor, which is known for its characteristic evolution in 3 phases: rapid augmentation then stabilization and involution on several years with in the best cases, classical "restitutio ad integrum". Usual surgical attitude is abstention and surgery is proposed only in order to treat sequel. But some particular situations require early surgery for life-threatening lesions or in case of functional impairment. Surgery consists in these cases in a simple volumetric diminution of the tumor with no aim for esthetic improvement. Other kinds of hemangiomas require an early surgical treatment before their complete involution. In some particular locations, deformation or growth delay can occur due to the lesion's development. In some cases, hemangiomas present a delayed involution with minor regression capacity; these are mainly located on the median part of the face and have principally a subcutaneous development. Early surgery can be proposed in order to avoid definitive deformation or growth impairment of adjacent structures. It should be performed before school age and before occurrence of psychological difficulties. Surgery is indicated in a perspective of esthetic improvement. Surgical procedure consists first in modeling excision followed by simple repair technique as linear suture or purse string closure; complex surgical procedures inducing their own sequels are usually inappropriate.     

Nerve distribution in hemangiomas depends on the proliferative state of the microvasculature

Hemangiomas appear at birth and undergo gradual regression within several years. Recent published studies have documented increased nerve numbers in port-wine stains and intramuscular vascular tumors. The aim of this study was to establish a relationship between angiogenesis and nerve growth in lesions that undergo neovascular proliferation followed by vessel involution. Twenty-two hemangiomas and arteriovenous malformations were studied using indirect immunocytochemistry with antibodies against the nerve markers protein gene product 9.5 (PGP 9.5) and calcitonin gene-related peptide (CGRP). Nerves and vessels were counted and compared. Our results indicate that PGP 9.5(+) and CGRP(+) nerves were most numerous in growing hemangiomas and numbers were reduced in involuting hemangiomas and vascular malformations. The percentage of CGRP(+) sensory nerves was markedly increased in growing hemangiomas (45.3%) compared with involuting hemangiomas (21.2). These data indicate that hemangiomas with increasing neovascularization have increased sensory nerve growth. Sensory nerve-derived neuropeptides are known to act as endothelial cell mitogens and may contribute to the angiogenesis in these vascular tumors. Conversely, angiogenic endothelial cells may secrete mediators that promote nerve fiber growth. These results suggest that endothelial cell proliferation and sensory nerve fiber growth may be closely related.     

Biomolecular markers and involution of hemangiomas

Background/purpose

Vascular anomalies are a diverse set of lesions with distinct clinical behaviors, whose biomolecular characteristics are largely undefined. Common hemangiomas proliferate during the first year of life, then involute at a variable pace over several years. Other vascular tumors may involute much more quickly (rapidly involuting congenital hemangiomas [RICH]), not at all (lymphatic malformation), or display malignant behavior (angiosarcoma). Key cytokines driving angiogenesis include vascular endothelial growth factor (VEGF) family members/receptors (placental growth factor [PIGF], VEGF-A, and VEGF-C) and angiopoietins. The authors hypothesized that involuting hemangiomas would display biologic markers distinctly different from noninvoluting vascular lesions.

Methods

Six patient samples were analyzed: (1) RICH, (2) proliferating hemangioma, (3) involuting hemangioma, (4) tufted angioma, (5) hepatic angiosarcoma, and (6) lymphatic malformation. Detailed examination of endothelial/vascular mural cell status was performed by fluorescent double-label immunostaining using specific markers (PECAM-1, αSMA) in combination with markers of proliferation (anti-phospho-histone H3) or apoptosis (TUNEL). Expression of PIGF, VEGF-A, VEGF-C, and Ang-1 was localized by in situ hybridization.

Results

Involuting/proliferating common hemangiomas demonstrated vasculature with abundant vascular mural cells (αSMA+); in contrast, αSMA(+) cells were rare in RICH vessels. Endothelial apoptosis was increased dramatically, but proliferation was unchanged during involution. VEGF-A was expressed in all lesions except lymphatic malformation, which displayed VEGF-C and Ang-1 upregulation. Strikingly, PIGF expression was increased markedly in the lesions predicted to involute/actively involuting but was virtually absent from noninvoluting tumors.

Conclusions

Vessel architecture and endothelial/vascular mural cell status differed between lesions, differentiating even common versus rapidly involuting hemangioma and corresponded to clinical involution. VEGF-A expression characterized endothelial-derived lesions, whereas VEGF-C marked lymphatic-derived cells. PIGF expression occurred only in vascular anomalies predicted to involute or actively involuting, a pattern potentially linked to PIGF function as a conditional antagonist of VEGF-A. Thus, distinct patterns of morphology and angiogenic factor expression characterize vascular anomalies with different clinical behaviors.     

Preoperative treatment of a parotid hemangioma with 100% ethyl alcohol

Hemangiomas are one of the most common childhood neoplasms, occurring in approximately 12% of infants younger than one year of age. The lesions typically appear shortly after birth, increase in size over the first year and characteristically regress over the next decade. Because hemangiomas can be visible during an important stage of a child’s social development, numerous authors have pursued alternative treatment strategies to avoid or reduce this lengthy involution process. Unfortunately, no effective medical treatment has been reported for children with large, deforming hemangiomas of the parotid gland and overlying cheek. In the present case, a patient with a large parotid hemangioma was treated preoperatively with an intralesional injection of 100% ethyl alcohol solution to reduce the size of the mass. The mass was removed 28 days later with no major postoperative complications.     

Current knowledge of the pathogenesis of infantile hemangiomas

Infantile hemangiomas are the most common benign tumor of infancy, occurring shortly after birth in 5% to 10% of white infants. Hemangiomas occur in infants of all races but are most common in those who are white. These lesions are preponderant in females compared with males at rates of 3:1 to 5:1. Many hemangiomas are discrete, well-circumscribed masses present in the head and neck. Some hemangiomas are segmental and diffuse, often involving large areas of the extremities or the head and neck. Chorionic villus sampling at 9 to 12 weeks of gestation has been associated with a 21% increased incidence of hemangiomas in infants. Most hemangiomas occur sporadically without a hereditary component. However, in a few families, hemangiomas segregate as a highly penetrant, autosomal dominant trait. Gene linkage studies of familial infantile hemangiomas show evidence of linkage to chromosome 5q31-33.     

Endovascular treatment of arteriovenous malformation

Vascular anomalies are common congenital or neonatal abnormalities. According to the approved classification of vascular lesions by Glowacki and Mulliken, hemangiomas and vascular malformations are distinguishable. Hemangiomas usually appear during the first days or weeks after birth and grow faster than the whole body of the infant. They are proliferating benign tumors that often involute. The opposite of hemangiomas, vascular malformations are present at birth, grow commensurately with the patient, demonstrate normal endothelial turnover, and never involute. The case of a young woman with an arteriovenous malformation (AVM) located on the left side of her face beneath the lower lip is described. The patient did not have any specific complaints except the cosmetic effect, which was a reddish and bluish discoloration of the skin over the lesion. The AVM was embolized with polyvinyl alcohol, and no subsequent surgery was performed. Follow-up ultrasound examination after a 12-month period showed no flow within the lesion area.     

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.     

Vascular tumors of the breast. II. Perilobular hemangiomas and hemangiomas

Nearly 100 vascular tumors of the breast have been studied. Sixty-two were angiosarcomas lesions. Twenty-four patients with mammary hemangiomas are the subject of this report. Five of 11 microscopic perilobular hemangiomas, not clinically apparent (2 mm or less), were atypical and had nuclear hyperchromasia or focal anastomoses among vascular channels. Whether treated by excision or mastectomy, all 11 patients remain well with follow-up of up to 10 years. Fourteen clinically or grossly apparent macroscopic hemangiomas (0.3-2.5 cm) included eight characterized as cytologically atypical. With one exception, lesions designated hemangiomas were well circumscribed and tended to be divided into lobules. In more than half, origin from large, non-neoplastic "feeding" vessels that were seen branching into the lesion was demonstrated. Whether treated by excision or mastectomy, no hemangioma has recurred after follow-up, up to 5 years in some cases. The diagnosis of vascular tumors of the breast requires thorough microscopic study of the entire lesion. The majority are angiosarcomas. However, about one-third constitute a spectrum of apparently benign and atypical lesions, in which the single largest group are perilobular hemangiomas and hemangiomas. Size appears to be an important characteristic for distinguishing hemangiomas from angiosarcomas, as few lesions larger than 2 cm qualify as hemangiomas, whereas angiosarcomas are rarely smaller than 2 cm. It is possible that atypical perilobular hemangiomas or atypical hemangiomas are precursors to angiosarcoma, but this relationship remains to be demonstrated.     

Understanding vascular anomalies: a common language for doctors

Vascular anomalies are benign vascular lesions of childhood and complex lesions should be managed within a specialized multidisciplinary team. They have been poorly understood owing to a lack of a common nomenclature among the various medical specialities as the subject remains neglected in undergraduate and postgraduate courses. The seminal work of Mulliken and Glowacki in 1982 established the foundations of the understanding of vascular anomalies by classifying them according to their histopathological and clinical features. Based on these findings, in 1996 the International Society for the Study of Vascular Anomalies classified vascular anomalies into vascular tumours (haemangiomas and others) and vascular malformations (capillary, lymphatic, venous, arteriovenous or a combination). Their characteristics and management differ greatly. Haemangiomas are the most frequent tumours of infancy. They are benign, transitory and self-limiting vascular lesions that exhibit cellular proliferation. Usually not present at birth, haemangiomas undergo transformation through a cycle of rapid proliferation over 6 months, to a variable period of involution and spontaneous regression over years. Their management is usually conservative, with active treatment reserved for the presence of functional or cosmetic complications (ulceration, obstruction and distortion of vital structures). Vascular malformations are structural anomalies of vascular morphogenesis without cellular proliferation. They present at birth, do not regress spontaneously and are subclassified as low-flow (capillary, lymphatic and venous) and high-flow (arteriovenous) lesions. Their effects may be those of a space-occupying lesion: infection, bleeding, pain or coagulopathy. Treatment options include dye laser (capillary), percutaneous sclerotherapy and surgery (venous and lymphatic) and embolization and surgery (arteriovenous).     

Follow‐up outcomes of benign vascular lesions of breast diagnosed on core needle biopsy: A study of 117 cases

Benign vascular lesions of breast are uncommon findings on core biopsy and surgical excision has been recommended to rule out a more serious lesion. However, a recent study suggested that excision may be spared for vascular lesion without atypia. The aim of this study was to assess the follow‐up outcomes in lesions yielding benign vascular lesions on core biopsy. We retrospectively reviewed 117 patients with diagnosis of hemangioma (106 patients) and atypical hemangioma (11 patients) on core biopsy at our institution over an 18‐year period. Majority of benign vascular lesions were followed‐up clinically and/or radiologically. Surgical excision was performed on 18 patients (16.9%) with benign hemangiomas and all 11 patients (100%) with atypical hemangiomas. Upon excision, the majority of patients (82.8%, 24/29) retained benign final pathology and five patients (17.2%) were atypical hemangioma. There was no upgrade on excision. All patients had a benign course regardless whether the lesions were excised or not. Our findings support the recent study that benign vascular lesion of breast may not require surgical excision.     

Management of vascular and lymphovenous malformations

Perhaps the most important event in relation to the vascular and lymphovenous group of malformations has been the development of a classification which is useful to physicians, patients, and parents. The broad classification consists of hemangiomas and vascular malformations; the latter group is further subdivided into venous, arteriovenous, and lymphovenous malformations [5, 11]. Hemangiomas are present at or just after birth in 40% of infants. They grow rapidly and involute slowly; there is increased endothelial cell activity. Venous malformations are present at birth in 70% of cases; they grow with the child and have a normal rate of endothelial cell turnover. Lymphovenous malformations occur at an early age and may or may not resolve. Arteriovenous malformations are usually progressive. The treatment is a combination of embolization and surgery.      

Outcome of dorsolumbar vertebral hemangiomas presenting with neuraxial compression

Background:

Hemangiomas are benign vascular tumors associated with proliferation of blood vessels in bone or soft tissue and they are usually incidental findings in vertebrae. When symptomatic, they present with features of radiculopathy, myelopathy, or vertebral fractures. Treatment options are varied, include sole embolization, embolization combined with surgical excision, surgical excision alone, percutaneous ablation, and radiotherapy. We hereby describe a series of seven cases of symptomatic vertebral hemangiomas operated from 2006 to 2009.

Materials and Methods:

Their clinical and radiological profile and outcome have been described. All patients were subjected to surgical excision followed by instrumentation. Outcome was assessed at a followup of 2 years following surgery with Frankel grading system.

Results:

Seven patients (five females and two males) were included in the study. The mean age was 33.85 years with the mean duration of symptoms of 12 months. All seven cases were symptomatic vertebral hemangiomas with cord compression and underwent surgical excision. Preoperatively, patients with poor Frankel grade such as A and B improved postoperatively to C, D, or E.

Conclusion:

Surgical excision of these lesions is difficult due to the tremendous amount of intraoperative bleeding. During surgery, brisk bleeding is usually encountered, but can be brought under control with adequate preoperative preparation and expertize. Preoperative embolization may help to reduce the bleeding, but at times it may be difficult to do if vertebrae are replaced by a solid hard mass. In spite of the risks associated with surgery, it still is the treatment of choice as a single intervention, especially in aggressive vertebral hemangiomas.     

Intraosseous hemangioma of the clivus: a case report and review of the literature

Intraosseous hemangiomas are benign vascular tumors that are encountered most commonly in vertebrae and rarely in the skull. When presenting in the skull, they are commonly found in the calvarium in frontal and parietal bones and seldom in the skull base. We encountered a patient with an incidental finding on magnetic resonance imaging (MRI) of an enhancing lesion in the clivus. Here we report an unusual location of a clival intraosseous hemangioma. A 62 year old man worked up for carpal tunnel syndrome had imaging of his cervical spine that revealed an enhancing clival lesion, which extended into the left occipital condyle. Endoscopic endonasal biopsy was performed on the abnormality revealing a capillary hemangioma. Patient tolerated the biopsy well and no further surgical intervention is indicated at this time. Patient will be followed at six month intervals. Primary intraosseus hemangiomas of the skull are extremely rare and usually occur in the calvarium. This is one of the few reported case of an intraosseus hemangioma in the clivus. We present this case in part because it is unusual, but more importantly, with the wider use of MRI, it is likely that these lesions will be discovered more frequently, and conceivably confused for more dangerous lesions.     

Classification, diagnosis, and interventional radiologic management of vascular malformations

Vascular anomalies are comprised of either hemangiomas or vascular malformations.Low-flow vascular malformations can be divided into capillary, venous, and lymphatic types and are usually present at birth, undergo pari passu growth, and produce symptoms related to mass effect or stasis. High-flow malformations are comprised pre-dominantly of arteriovenous malformations that follow a more aggressive clinical course of hyperemia, adjacent mass effect, steal phenomenon, tissue destruction, and ultimately high output failure. Ultrasound, CT, nuclear medicine, angiography, and particularly MRI have greatly enhanced diagnostic accuracy and provide detailed information for percutaneous and surgical treatment planning and an objective means of following therapeutic efficacy. Interventional radiologic percutaneous sclerotherapy for low-flow lesions and embolosclerotherapy for high-flow lesions with or without adjunctive surgical intervention have become the mainstay of therapy.     

Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations

BackgroundThere is no way to predict the size that proliferative infantile hemangiomas (IHs) can reach and to expect the occurrence of complications. Moreover, there are no well-known characteristics that can affect the rate of involution of IHs and to predict its completion. Accordingly, intervention is frequently indicated. Different modalities have been reported for treatment of IHs. The possible mechanisms of action of propranolol on IHs are complex.MethodsFifty infants presented with 80 IHs treated by oral propranolol at a dose of 2 mg/kg body weight per day. Treatment outcomes were clinically and radiologically evaluated.ResultsThe first noticeable effects on propranolol treatment were the changes in color and softening of IHs, followed by regression of their sizes. The clinically elicited color changes of superficial IHs and superficial components of compound IHs have been objectively proven by statistically significant color clearance (P ≤ .001) and resisting index (P ≤ .01) (～50% increase) as a good indicator of lower vascular activity within IHs. Moreover, the softening of lesions followed by the clinically elicited regression of sizes of deep IHs and deep components of compound IHs has been objectively proven by statistically significant changes at lesions' thickness (P ≤ .01) (～50% regression) and resisting index (P ≤ .01) (～50% increase).ConclusionsCollectively, high efficacy and tolerance of propranolol treatment have been elicited. However, propranolol treatment of IHs is still an issue suitable for more studies to confirm the safety and efficacy of the drug and to investigate whether there are some hemangiomas that are, perhaps, nonresponsive to propranolol treatment.     

Intracranial capillary hemangioma: case report and review of the literature

BACKGROUND: Capillary hemangiomas are benign vascular lesions that commonly present at birth or in early infancy on the face, scalp, back, or chest. The authors present an exceedingly rare case of an intracranial capillary hemangioma arising in an adult. Only 4 biopsy-proven cases have been reported in the pediatric population previous to this case report. CASE DESCRIPTION: A 31-year-old pregnant woman presented at 38 weeks of gestation with severe headaches, nausea, and vomiting. Imaging revealed an extra-axial mass lesion arising from the tentorium with both supra- and infratentorial components. The patient underwent a resection of her tumor, which was diagnosed as a capillary hemangioma by histopathologic examination. The patient required 2 further resections after the lesion exhibited a rapid regrowth from residual tumor in the left transverse sinus. The patient has remained free of disease 41 months out from her third surgery. CONCLUSIONS: Intracranial capillary hemangiomas are exceedingly rare entities, with a capability for rapid growth. When gross total resection cannot be achieved, these patients should be observed closely, and the use of adjuvant radiotherapy should be considered.     

Argon laser treatment of head and neck vascular lesions

Port wine stains (PWSs), hereditary hemorrhagic telangiectasias (HHTs), hemangiomas, arterial venous malformations (AVMs), vascular granulomas and polyps, glomus tumors, and nasopharyngeal angiofibromas are vascular lesions of the head and neck potentially responsive to treatment with the argon laser. One hundred consecutive patients with PWSs, 25 patients with HHTs, three with subglottic hemangiomas, three with oral and/or lingual hemangiomas, two with labial AVMs, three with vascular laryngeal polyps, and one patient with inoperable glomus tumor were treated with the argon laser. Results were good to excellent in 94% of the facial PWS patients. All treated patients in the HHT group demonstrated improvement. Results with the other lesions were variable. The argon laser is a valuable treatment modality in vascular lesions of the head and neck. Posttreatment scarring and failure to achieve desired results are complications encountered. Pretreatment counseling will assist the patient in understanding the expected results.     

Cavernous hemangiomas of the internal auditory canal

Cavernous hemangiomas are vascular malformations that are seldom found in the central nervous system. Four cavernous hemangiomas of the internal auditory canal and one of the cerebellopontine angle have been reported previously. We present a series of seven (six of which have never been reported) cavernous hemangiomas limited primarily to the internal auditory canal. Whereas all cases but one had marked unilateral sensorineural hearing loss, only two had preoperative facial nerve dysfunction. There are no specific symptoms or physical or audiometric findings to differentiate cavernous hemangiomas from acoustic neuromas. However, a hemangioma should be suspected when facial paralysis is present in conjunction with a small intracanalicular tumor as demonstrated by computed tomography or magnetic resonance imaging. The former reveals calcium stippling in the lesions, whereas magnetic resonance imaging reveals a high signal intensity on both T1- and T2-weighted images.     

Living donor liver transplantation for multiple intrahepatic portosystemic shunts after involution of infantile hepatic hemangiomas

We describe a 6-year-old girl presenting with multiple intrahepatic portosystemic shunts after the involution of infantile hepatic hemangiomas (IHHs), who successfully underwent living donor liver transplantation. The chronological changes of radiologic findings indicated that remnant portovenous shunts at the time of IHHs involution developed gradually on the background of atrophic intrahepatic portal veins. This suggests that patients should be carefully followed up for the late onset of intrahepatic portosystemic shunts after the involution of IHHs.