Juvenile nasopharyngeal angiofibroma. Awareness in anesthesia of the juvenile nasopharyngeal angiofibroma.
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Adolescent↗
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[Expression of members of the cadherin-/catenin-protein family in juvenile angiofibromas].
BACKGROUND: Angiofibromas are benign, but locally aggressive tumors occurring nearly exclusively in adolescent males. Pathogenesis of this fibrovascular neoplasm is still unknown. Detection of beta-catenin gene mutations and immunohistochemical localization of beta-catenin only in the nuclei of stromal cells has been proposed as evidence for the stromal cells to be the neoplastic cells in angiofibromas (Abraham et al., 2001). METHODS: Paraffin embedded tissue of 13 angiofibromas was analysed immunohistochemically for expression of E-cadherin, N-cadherin, alpha-catenin, beta-catenin and gamma-catenin. RESULTS: In all angiofibromas stromal cells showed in their cytoplasm and their nuclei strong immunoreaction for beta-catenin. In 10 out of 13 angiofibromas strong immunoreaction was also observed in endothelial cells covering the irregular lined vascular spaces. While gamma-catenin was detected in 7 out of 13 angiofibromas in the stromal cells and endothelial cells, N-cadherin was found only in the stromal cells in 8 out of 13 angiofibromas. No significant immunoreaction in angiofibromas was seen for E-cadherin and alpha-catenin. CONCLUSIONS: Proof of strong beta-catenin-expression in stromal cells and endothelial cells stress possible importance of the APC/beta-catenin-pathway in angiofibromas, but gives no certain evidence for the assumption that stromal cells may be the neoplastic cells. As beta-catenin can increase sensitivity of the androgen receptor, it may be assumed that the increased beta-catenin-expression in angiofibromas is involved in the typical growth stimulus of this tumor in adolescent males.
Adolescent↗
Loss of expression of tuberin and hamartin in tuberous sclerosis complex-associated but not in sporadic angiofibromas.
BACKGROUND: Angiofibromas occur sporadically, and they develop in most patients with tuberous sclerosis complex (TSC), which is associated with alterations of the tumor suppressor genes TSC1 or TSC2. Loss of tuberin, the protein product of TSC2, has been shown in the interstitial fibroblast compartment of TSC-associated angiofibromas. It is unclear whether there is also a loss of hamartin, the product of TSC1 in TSC-associated and sporadic angiofibromas. METHODS: The expression of hamartin and tuberin was analyzed by immunohistochemistry in 59 TSC-associated and 12 sporadic angiofibromas using affinity-purified antibodies. RESULTS: Loss of expression of both tuberin and hamartin was detected in 14 angiofibromas, loss of only tuberin in three, and loss of only hamartin in four TSC-associated angiofibromas; but there was no loss in the sporadic angiofibromas. Only the interstitial cells, but not the vascular cells, showed a loss of expression of tuberin or hamartin. CONCLUSIONS: Loss of tuberin or hamartin occurred in a minority of the TSC-linked angiofibromas, but not in the sporadic angiofibromas. The absence of both tuberin and hamartin in some of the tumors suggests that the stability of tuberin and hamartin, which are believed to form an active complex in vivo, is negatively affected by the absence of either of the partners.
Angiofibroma↗
Growth factors and receptors in juvenile nasopharyngeal angiofibroma and nasal polyps: an immunohistochemical study.
BACKGROUND: Juvenile nasopharyngeal angiofibroma is a rare nasopharyngeal tumor that occurs exclusively in adolescent boys. It is a histologically benign but locally persistent growth of stromal and vascular tissue. Although male hormones and some growth factors, such as transforming growth factor beta1 (TGF-beta1), insulin-like growth factor II (IGF-II), and, lately, the proto-oncogene beta-catenin, have been implicated in the histogenesis of the tumor, the biologic signaling pathways that drive this peculiar fibrovascular proliferation are still nuclear. OBJECTIVE: To evaluate immunoexpressions of beta-catenin, c-Kit, p130Cas, TGF-beta3, bone morphogenic protein 4, nerve growth factor (NGF), and the IGF receptor (IGF-1R) in a series of juvenile nasopharyngeal angiofibromas and to compare to that of a group of nasal polyps. DESIGN: A standard immunohistochemical technique was used on paraffin sections of 12 sporadic juvenile nasopharyngeal angiofibromas and 15 nasal polyps with microwave or steam antigen retrieval. Immunoreactivity was analyzed semiquantitatively in stromal cells and endothelial cells of each case. RESULTS: The expressions of beta-catenin (nuclear), c-Kit (cytoplasmic), and NGF (cytoplasmic) were higher and more frequent in stromal cells of juvenile nasopharyngeal angiofibromas than those of nasal polyps. Both juvenile nasopharyngeal angiofibromas and nasal polyps showed similarly frequent and strong immunoreactivity for p130Cas and TGF-beta3 and weak immunoreactivity for bone morphogenic protein 4 in both stromal cells and endothelial cells. No IGF-1R immunoreactivity was detected in any case of either group. CONCLUSIONS: Our results support the role of beta-catenin in juvenile nasopharyngeal angiofibromas and suggest a potential involvement of c-Kit and NGF signaling pathways in the juvenile nasopharyngeal angiofibromas. Although the biologic significance of c-Kit in juvenile nasopharyngeal angiofibromas has yet to be defined, the finding of frequent and high c-Kit expression might have therapeutic importance for patients with juvenile nasopharyngeal angiofibromas.
Adolescent↗
[Aetiology of Angiofibromas: Reflection on their Specific Vascular Component].
BACKGROUND: Even though numerous theories have speculated either on the vascular or fibrous tumour component as tissue of origin, aetiology of angiofibroma still remain unclear. Histological investigations recently led Beham and coworkers to the assumption that angiofibromas have to be considered as vascular malformations. METHODS: After giving a literature review of the various theories on tumour origin the proposal to consider angiofibromas as vascular malformations is discussed on an embryological base. Taking typical clinical features of the tumour and knowledge of vasculogenesis into account a new explanation for origin of the vascular tumour component is presented. RESULTS: The vascular component of angiofibromas can be explained embryologically due to incomplete regression of the first branchial arch artery (vascular atavism). This vessel arises regularly between embryological day 22 and 24 and recedes during regular development completely until delivery via temporary formation of a vascular plexus. In the late stages of embryological development remnants of the plexus are found at the area of the sphenopalatine foramen, the typical site of angiofibroma origin. Incomplete regression of the vascular plexus of the former first branchial arch artery may form the vascular component of an angiofibroma arising due to growth stimulation at the time of adolescents. CONCLUSIONS: Incomplete regression of the first branchial arch artery presenting an atavism is suited to explain the vascular tumour component of angiofibromas considering main tumour characteristics (origin in the posterior nasal cavity close to the sphenopalatine foramen, main blood supply from the maxillary artery with possible feeders arising from the internal carotid artery). Our embryological contributions support to define angiofibromas as vascular malformations.
Adolescent↗
[Early childhood angiofibroma in an unusual site].
Angiofibromas are usually characterized by their occurrences in adolescent males with a typical localization and involvement of the posterior nasal cavity and nasopharynx. Even the suspicion of an extranasopharyngeal angiofibroma or an angiofibroma in young children must be viewed in general with skepticism, although reports of angiofibromas with atypical localizations and manifestations in young children have appeared in the literature. Three cases of this fibro-vascular neoplasm with manifestations in the first decade of life and atypical localizations are presented: (1) an angiofibroma medial to the left lacrimal sac in a 15-month old boy, (2) a right paranasal localization in a 9-year-old boy and (3) an angiofibroma limited to the right sphenoid sinus in a 6-year old boy. In all three cases the neoplasm was resected via an endonasal, micro-endoscopic approach that avoided an external incision. According to the literature and based on our own experiences with typical histological findings in all three cases, the clinician has to be aware that the rare angiofibroma can occur in preadolescent children with atypical localizations. The endonasal surgical approach is without any doubt the least traumatic one and in selected cases allows resection of a circumscribed tumor considering functional and aesthetic aspects.
Angiofibroma↗
First evidence of genetic imbalances in angiofibromas.
OBJECTIVE/HYPOTHESIS: Angiofibromas are clinically well characterized by their origin at the posterior lateral nasal wall close to the sphenopalatine foramen, their occurrence in male adolescent patients, and the histological findings of a benign fibrovascular neoplasm with irregular, endothelium-lined vascular spaces in a fibrous stroma. However, their etiology and genetic causes remain unknown. The present study addresses genetic imbalances in angiofibromas. STUDY DESIGN: The present pilot study compared genomic hybridization in three angiofibromas to search for chromosomal abnormalities in this rare tumor. METHODS: Fluorescence-marked normal DNA and angiofibroma DNA were compared using genomic hybridization screening to detect chromosomal abnormalities. Their binding ratio to metaphase chromosomes were analyzed by special digital image analysis. RESULTS: Chromosomal gains and losses showing a high level of agreement were detected in all three angiofibromas. Specifically, DNA gains were observed on chromosomes 3q, 4q, 5q, 6q, 7q, 8q, 12p, 12q, 13q, 14q, 18q, 21q, and X, and DNA losses were screened on chromosomes 17, 19p, 22q, and Y. Finding chromosomal abnormalities at the sex chromosomes X and Y of this rare tumor is remarkable. Concurrent chromosomal gain on 8q12q22 was noted in all three tumor specimens. CONCLUSIONS: Comparative genomic hybridization is suitable for screening angiofibromas on a genetic level. The results on these screens indicate that further genetic investigations of this rare benign tumor may provide more details about the tumor's genetic abnormalities and perhaps clarify the etiology of angiofibromas.
Angiofibroma↗
Genetic heterogeneity of the MYC oncogene in advanced juvenile angiofibromas.
Despite their benign histological appearance, juvenile angiofibromas sometimes exhibit an aggressive growth behavior. Molecular and genetic analyses have detected beta-catenin mutations and androgen receptor gene gains in this tumor. Because intensive cross-talk among beta-catenin, androgen receptor, and C-MYC has been detected recently, we analyzed expression of the C-MYC protooncogene (MYC) on the genetic, transcriptional and translational level in seven sporadic juvenile angiofibromas. Two-color in situ hybridization analyses for chromosome 8 and MYC found in all seven juvenile angiofibromas significant MYC losses. In the three advanced juvenile angiofibromas of this series (Fisch stages III and IV) additional significant MYC gains were observed demonstrating a genetic heterogeneity for the MYC protooncogene. In cases of genetic MYC heterogeneity, reverse transcriptase-polymerase chain reaction (RT-PCR) analysis, Western blot investigations, and immunohistology showed increased C-MYC mRNA and protein levels. Semiquantitative RT-PCR analyses from laser microdissected endothelial cells and fibroblasts found no differences of C-MYC mRNA levels, leaving open the question of the neoplastic cell in juvenile angiofibromas. The finding of genetic MYC heterogeneity associated with C-MYC overexpression on the mRNA and protein level in advanced juvenile angiofibromas indicates involvement of the MYC oncogene in aggressive growth behavior.
Angiofibroma↗
Atypical angiofibromas: a report of four cases.
In this study, our four patients with angiofibroma with various atypical features are presented. Related literature is reviewed and criteria for atypicality are proposed. 14 patients, diagnosed and treated for angiofibroma in the Otorhinolaryngology Department of Faculty of Medicine in Uludag University between January 1992 and December 2003, have been evaluated. The files of the patients were examined and four patients with atypical characteristics have been included in the study. Angiofibromas presenting with at least one of the following criteria such as origin or location other than nasopharynx, presenting complaints other than nasal obstruction or epistaxis, aged younger than seven or older than 25, female sex, atypical histopathology and multifocalitiy were considered as "atypical". Four of 14 angiofibromas (28.5%), which were diagnosed and treated in our clinic, had atypical features. The reasons for atypicality were unusual localization in three patients and uncommon initial symptom in one case. In the atypical localization group, neoplasm was located in tonsil in one case, and in inferior turbinate in two patients. The only patient with atypical initial symptom presented with a bulging in the cheek. The patients, who have different characteristics other than classical angiofibromas, may be called "Atypical Angiofibroma (AAF)", under the scope of the related literature and our experience. These patients should be included in atypical group according to the characteristics such as, localization, symptom, age, sex, histopathology and multifocality.
Adolescent↗
Extranasopharyngeal angiofibroma.
Juvenile nasopharyngeal angiofibromas are vascular neoplasms, which originate characteristically in the posterior lateral wall of the nasopharynx. Although angiofibromas extend beyond the nasopharynx commonly, they rarely originate outside the nasopharynx. Reports of primary extranasopharyngeal angiofibromas have appeared sporadically in the literature. We present an unusual case of an angiofibroma arising from the middle turbinate. The clinical characteristics of extranasopharyngeal angiofibromas do not conform to that of nasopharyngeal angiofibromas. Therefore, they can present diagnostic challenges. A methodic evaluation and a high index of suspicion are essential in establishing the proper diagnosis and treatment.
Adolescent↗
Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?
Juvenile nasopharyngeal angiofibroma is a benign neoplasm affecting the nasopharynx of male adolescents. Two patients treated at Temple University Hospital for this condition were also diagnosed with familial adenomatous polyposis. Familial adenomatous polyposis results from the inheritance of a mutated adenomatous polyposis coli gene in an autosomal dominant pattern. The development of colorectal carcinoma in middle age is seen almost invariably in familial adenomatous polyposis, if a prophylactic colectomy is not performed. To identify a possible association between juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis, chart reviews and patient interviews were carried out for all patients treated for juvenile nasopharyngeal angiofibroma at Temple University Hospital between 1985 and 1993. Single-strand conformational polymorphism was performed to detect the presence of certain adenomatous polyposis coli gene mutations within the germline DNA of those juvenile nasopharyngeal angiofibroma patients not previously found to have familial adenomatous polyposis. Although no more patients with both juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis were found by these methods, the two patients with both disorders previously identified constitute 22% of our juvenile nasopharyngeal angiofibroma series. The implications of these findings are discussed.
Adenomatous Polyposis Coli↗
Extranasopharyngeal angiofibroma of the head and neck in women.
Extranasopharengeal angiofibroma of the head and neck in women is very rare, and clinical characteristics do not confirm to that of nasopharyngeal angiofibroma. We present clinical characteristics, etiology, preoperative evaluation, and management of 3 unusual cases of angiofibroma, 1 case originating from larynx and the other 2 cases from nasal septum in women. Endolaryngeal endoscopic excision for laryngeal and local excision for septal angiofibromas were performed. The patients are free of disease. In the etiology of extranasopharyngeal angiofibroma, we suspect that ectopic tissue may become located further away than usual place and may have been the cause of the extranasopharyngeal location. Computed tomography scan, magnetic resonance imaging, and carotid angiogram are valuable in the diagnosis of extranasopharyngeal angiofibroma. Excision of the mass is the treatment of choice, and recurrence is rare.
Adolescent↗
Treatment of angiofibromas with a scanning carbon dioxide laser: a clinicopathologic study with long-term follow-up.
BACKGROUND: Facial angiofibromas in tuberous sclerosis have been managed with various treatment modalities, including carbon dioxide (CO(2)) laser resurfacing. OBJECTIVE: Our purpose was to perform a long-term clinicopathologic assessment of CO(2) laser treatment of angiofibromas. METHODS: This was a retrospective case review of 10 patients treated with a scanning CO(2) laser to flatten lesions. Baseline clinical photographs and those taken at 6, 12, and 24 months after the operation were assessed by a blinded observer. Patients also evaluated outcomes. Biopsy specimens taken immediately and at 4 months after the operation were reviewed. RESULTS: Three groups of patients were identified: the first comprised 2 patients with sustained excellent and good outcomes. A second group (3 patients) had excellent outcomes in the early and medium term but then demonstrated partial deterioration. The last group (5 patients) had a range of early results with invariably poor outcomes at 24 months. In contrast, patients' self-assessment at 24 months was good or excellent in 8 of 10 cases. All biopsy specimens taken immediately after the procedure demonstrated ablation extending into the papillary dermis. Residual angiofibromas were present in 6 biopsy specimens. At 4 months, all biopsy specimens showed a band of superficial dermal fibrosis, but distinguishing between this and adjacent angiofibromas was often difficult. Long-term side effects included 2 cases of subtle hypopigmentation. CONCLUSION: The long-term results of CO(2) laser treatment of angiofibromas are unpredictable. The marked improvement obtained at 6 months is sustained in only a minority of cases at 24 months. Despite this, patient satisfaction appears relatively high. Initial clinical improvement may be the result of a combination of destruction of angiofibromas and their sequestration under postoperative fibrosis. The benefits of therapy should be weighed against both early morbidity and the risks of long-term complications such as scarring and hypopigmentation.
Adult↗
Recent advances in the treatment of juvenile angiofibroma.
PURPOSE OF REVIEW: Juvenile nasopharyngeal angiofibroma is a rare vascular tumor almost exclusive to the nasopharynx of adolescent males. Traditionally, juvenile nasopharyngeal angiofibroma has been treated surgically using open surgical approaches and has been associated with frustratingly high recurrence rates. This article reviews recent contributions to the study and treatment of this disease. In particular, advances in minimally invasive endoscopic resection of juvenile nasopharyngeal angiofibroma are evaluated. RECENT FINDINGS: The growth patterns of juvenile nasopharyngeal angiofibroma are evaluated. Young age does not appear to correlate with more aggressive disease. The major recent advance in the treatment of juvenile nasopharyngeal angiofibroma has been the application of endoscopic endonasal surgery to the treatment of select tumors. This article reviews the indications and inclusion criteria recently put forth to help select patients for this minimally invasive approach. In properly selected patients with Radkowski stage I and II lesions, recurrence rates range between 0 and 7%. Advanced lesions continue, in most cases, to require open approaches, some of which are also presented. SUMMARY: With proper patient selection, endoscopic resection of juvenile nasopharyngeal angiofibroma is feasible and may be preferable to traditional open approaches. Results suggest that after endonasal resection, disease recurrence is low. Most larger lesions, especially those with intracranial spread, continue to require open approaches for complete resection.
Angiofibroma↗
Nasopharyngeal angiofibroma: an APC-gene-associated tumor?
Nasopharyngeal angiofibromas are extremely rare, locally invasive tumors of unknown cause exclusively occurring in male adolescents. Recently, 6 cases have been reported in patients with familial adenomatous polyposis coli (Gardners syndrome). Mutations or allelic loss of the adenomatous polyposis coli (APC) gene have therefore been implied in the pathogenesis of nasopharyngeal angiofibroma. The authors analyzed 11 cases of nasopharyngeal angiofibromas from 9 male patients for mutations in the mutation cluster region and allelic loss of the APC gene. Six were primary tumors; 2 first recurrences; and 1, primary tumor with 2 recurrences. Direct sequence analysis was performed on several overlapping polymerase chain reaction (PCR) products. Detection of allelic loss was performed by restriction length polymorphism analysis at a polymorphic locus. No mutation was detected in 11 tumors of 9 different patients. None of the 9 informative (heterozygous) cases carried an allelic loss. We conclude that alterations of the APC gene do not play a major role in the development of nasopharyngeal angiofibroma. The coincidence of nasopharyngeal angiofibromas and adenomatous polyposis coli in some families implies the possibility that another gene in this region might be responsible for the development of nasopharyngeal angiofibromas. HUM PATHOL 31:1411:1413.
Adolescent↗
Unilateral facial angiofibromas--a segmental form of tuberous sclerosis.
Multiple facial angiofibromas are thought to be a pathognomonic and common, feature of tuberous sclerosis. In contrast, it is rare to see multiple angiofibromas limited to one side of the face. We have studied a patient with multiple angiofibromas of one cheek, in order to search for other manifestations of tuberous sclerosis and to determine the histochemical identity of the angiofibromas. No evidence of other pathology known to be associated with the tuberous sclerosis complex was uncovered. Histopathology of the unilateral lesions revealed focal fibroplasia and positive cellular staining for factor XIIIa and vimentin, similar to that described for bilateral angiofibromas. We suggested that the segmental expression of tuberous sclerosis, in the form of unilateral facial angiofibromas, may result from a postzygotic mutation.
Adult↗
Resurfacing of facial angiofibromas in tuberous sclerosis patients using CO2 laser with flashscanner.
BACKGROUND: Angiofibromas are a common presentation of tuberous sclerosis. They cause considerable cosmetic and hygienic morbidity for patients. Treatments of angiofibromas have included curettage, cryosurgery, chemical peel, dermabrasion, shave excision, and 13-cis retinoic acid. Results from these modalities in many cases were not satisfactory from a cosmetic standpoint. Copper vapor, argon, pulsed dye, and CO2 lasers have been used with success in isolated cases. OBJECTIVE: The purpose of this study was to evaluate the efficacy of CO2 laser resurfacing with flashscanner in the treatment of facial angiofibromas. METHODS: Two patients with angiofibromas on the face were treated with a CO2 laser with flashscanner. The cheek and nose were treated in one patient, and entire face was done in the other. RESULTS: Both patients showed remarkable cosmetic improvements without scarring. Mild hyperpigmentation was found in both patients, which disappeared in 1 month with the application of topical agents. Facial erythema persisted about 2 months in both patients. CONCLUSION: A CO2 laser equipped with flashscanner causes less residual thermal damage than conventional CO2 lasers and enables controlled depth vaporization for more precise and regular removal of angiofibromas. In whole-face resurfacing, more cosmetically acceptable results are possible because localized treatment leaves marginal prominences. Laser resurfacing is an effective alternative in the treatment of multiple protuberant angiofibromas even though we cannot permanently clear these lesions due to their nature.
Adult↗
Angiofibroma of the larynx: report of a case with clinical and pathologic literature review.
BACKGROUND: Angiofibromas are uncommon vascular tumors with a strong predilection for the nasopharynx of adolescent males. Although they are slow growing and histologically benign, they have the potential to cause significant morbidity with laryngeal involvement. METHODS: We describe the clinical characteristics, histopathologic findings, differential diagnosis, preoperative evaluation, and management of a case of laryngeal angiofibroma. RESULTS: The patient was initially seen with a 2 1/2-year history of progressive dyspnea and dysphagia. Preoperative evaluation suggested a vascular mass involving the left supraglottic larynx. A partial laryngopharyngectomy was performed without complication. The patient is alive and disease free 3 years postoperatively. Final histopathologic diagnosis is consistent with angiofibroma. CONCLUSIONS: Laryngeal angiofibroma is an extremely rare entity. Adequate preoperative imaging is necessary to confirm the vascularity of this lesion, because ill-planned biopsy may lead to significant blood loss. The role of preoperative embolization of other laryngeal vascular lesions has been well documented and may be useful in the management of laryngeal angiofibroma.
Angiofibroma↗