Treatment of a nasal angiofibroma in a cat using stereotactic body radiation therapy.

A 14-year-old cat presented with right-sided epistaxis, right facial swelling, hyporexia, and sneezing. A right nasal mass was diagnosed based on dental radiography and computed tomography (CT), and nasal angiofibroma was diagnosed based on histopathology. Treatment consisted of stereotactic body radiation therapy in three consecutive daily doses. Self-limiting grade 3 oral mucositis developed which resolved within 6 weeks. Recheck CT 169 days after treatment confirmed a partial response by RECIST(1) based on digital CT measurements . Disease progression was confirmed on CT 642 days after treatment, per RECIST criteria, with the longest tumor diameter measuring 3.4 cm.

Nasal angiofibroma treatment outcome using intensity-modulated radiation therapy in a dog.

A 6-year-old dog presented with a modified Adams stage 3 angiofibroma of the right nasal cavity, causing fluid accumulation along the right frontal sinus. Treatment consisted of step-and-shoot intensity-modulated radiation therapy in 12 daily treatments of 3.5 Gy, for a total dose of 42 Gy to 95% of the planning target volume. The dog developed self-limiting grade 2 oral mucositis which resolved within 2 weeks of course completion. A recheck exam 668 days after treatment confirmed a stable disease response by RECIST and a tumor volume decrease of 55.4%.

Advantage of 68Ga-PSMA PET/CT on Postradiotherapy Response Assessment of Juvenile Nasal Angiofibroma.

ABSTRACT: Prostate-specific membrane antigen (PSMA) is universally expressed in the endothelial cells of tumor-associated neovasculature of juvenile nasopharyngeal angiofibroma. Functional 68Ga-PSMA PET/CT also enables easier differentiation of the residual tumor from postoperative changes. An 18-year-old man with the residual intracranial disease received radiotherapy after surgery. In PSMA PET/CT imaging, uptake was noted initially only in the residual disease, and the same was absent after successful response to radiotherapy, unlike contrast MRI, which showed persistent enhancement. Functional 68Ga-PSMA PET/CT may be a very useful tool clinically for identifying early responses to radiotherapy compared with conventional structural imaging.

Juvenile nasopharyngeal angiofibroma with intracranial extension - diagnosis and treatment.

INTRODUCTION: This retrospective study analyzes radiological findings, therapeutic management and outcomes of patients with intracranial extension of JNA. The routes of intracranial spread, incidence of intracranial disease and influence on therapeutic approach are discussed. MATERIAL AND METHODS: An evaluation on the records of 62 patients with JNA was performed and 10 patients with intracranial tumors were included in the study. All patients were males aged 10 to 19 years. R esults: According to Andrews' classification 8 patients presented with stage IIIb, 1 patient stage IVa and another patient stage IVb tumor. Intracranial invasion was extradural in 8 cases and intradural in 2 patient. Surgery was performed in 9 cases and the most common was combined approach: infratemporal fossa and sublabial transantral. One patient was referred for radiotherapy. Follow-up ranged from 8 to 26 years. There was extracranial recurrence in 2 (22%) of 9 operated patients. C onclusions: The superior orbital fissure is the most frequent route of intracranial spread in patients with extensive involvement of the infratemporal fossa. Due to high risk of recurrence and potential serious complications advanced cases of JNA should be managed by experienced multidisciplinary team, preferably in tertiary referral centers, with an access to modern diagnostic and therapeutic modalities.

Angiofibroma de fosa temporal: reporte de un caso / Temporal fossa angiofibroma: a case report

RESUMEN El angiofibroma nasofaríngeo es el tumor benigno más frecuente de la nasofaringe, representando el 0,05% del total de las neoplasias de cabeza y cuello. Los angiofibromas en localizaciones distintas a la nasofaringe son entidades raras. Ellos son descritos esporádicamente en la literatura, ubicándose principalmente en el seno maxilar. En este artículo presentamos un caso de fibroangioma extranasofaríngeo localizado en fosa temporal derecha seguido de una revisión de literatura.

ABSTRACT Nasopharyngeal angiofibroma is the most common benign tumor of the nasopharynx, representing 0.05% of total neoplasms of the head and neck. Extranasopharyngeal angiofibromas are rare entities described sporadically in the literature, being located mainly in the maxillary sinus. We present a case of an extra-nasopharyngeal fibroangioma located in the right temporal fossa followed by a literature review.

Long-term treatment outcomes of juvenile nasopharyngeal angiofibroma treated with radiotherapy.

Juvenile nasopharyngeal angiofibroma (JNA) is a disease of adolescent males characterised by high vascularity with local aggressiveness. This analysis was intended to see the effectiveness of radiation in locally advanced JNA. We included patients treated from 1990-2012. A total of 31 patients met study criteria. Median age was 16 years (range: 12-33 years). Radiation was used for refractory, residual or unresectable locally advanced disease. The median radiation dose was 30 Gy (range: 30-45 Gy). Median follow-up was 36 months (Range: 1-271 months). The median progression-free survival [PFS] was not reached. PFS at 3, 5 and 10 years was 91.7, 70.7 and 70.7% respectively. Three patients progressed at 38, 43 and 58 months after completion of treatment and opted for alternative therapy. One patient developed squamous cell carcinoma of the nasal ale 15 years after radiation.

Conformal radiotherapy for locally advanced juvenile nasopharyngeal angio-fibroma.

PURPOSE: To assess the efficacy of radiation in the treatment of juvenile nasopharyngeal angiofibroma (JNA). MATERIALS AND METHODS: Data were retrieved for JNA treated with radiotherapy from 1987-2012. The demographics, treatment and outcome data were recorded in predesigned proforma. RESULTS: Data of 32 patients were retrieved. Median age was 17 years (range: 12-33 years). All patients received radiation because of refractory, residual or unresectable locally advanced disease. All patients were planned with a three-dimensional conformal technique (3DCRT). The median radiation dose was 30 Gray (range: 30-45 Gray). Median follow-up was 129 months (range: 1-276 months). At the last follow-up, 13 patients were found to have a radiological complete response. Two patients progressed 38 and 43 months after completion of treatment and opted for alternative treatment. One patient developed squamous cell carcinoma of the nasal ale 15 years after radiation. CONCLUSION: Conformal radiotherapy shows promise as an alternative treatment approach for locally advanced JNA and confers long-term disease control with minimal toxicity.

[Juvenile nasopharyngeal angiofibroma with orbital extension]. / Angiofibroma nasofaríngeo juvenil con extensión orbitaria.

CLINICAL CASE: The case is presented of a 21 year-old male with a history of left proptosis and diplopia of two weeks of onset. The MRI showed an ethmoid-orbital vascular lesion with anterior skull base invasion and orbital extension. Biopsy of the ethmoid confirmed fibrovascular tissue, which supported the diagnosis of angiofibroma. DISCUSSION: It is a benign neoplasm with local characteristics of malignancy due to its ability to invade adjacent areas. In this case, the debut presented with manifestations of orbital extension. A broad and multidisciplinary approach is needed in order to improve prognosis.

Nasal hemophilic pseudotumor: favorable response to radiotherapy.

Hemophilic pseudotumors are rare but dangerous complications of Hemophilia. We hereby report a 3-year-old boy with Hemophilia B, presenting with nasal pseudotumor, showing favorable response to radiotherapy after unsuccessful treatment with factor IX replacement therapy. The diagnosis and treatment of this rare condition is also reviewed.

Angiofibroma juvenil nasofaríngeo / Juvenile nasopharyngeal angiofibroma

Se presenta el caso de un angiofibroma juvenil nasofaríngeo, en un paciente del consultorio médico No. 9, que pertenece al Policlínico Integral Docente Carlos Manuel Portuondo de Marianao. Se reconoce la importancia de realizar una historia clínica detallada, así como un minucioso examen físico, por el médico de familia y el otorrinolaringólogo del área de salud, que incluyó la rinoscopia posterior, para poder llegar al diagnóstico de esa patología, y realizar la extirpación precoz del angiofibroma, mediante el proceder quirúrgico. Se concluyó que el diagnóstico clínico se correspondió con el histopatológico, y que la conducta quirúrgica temprana es resolutiva en la afección

It is presented a case of juvenile nasopharyngeal angiofibroma in a patient with this pathology, from the clicial practice No. 9, in Comprehensive Teaching Polyclinic Carlos Manuel Portuondo in Marianao. It recognizes the importance of a thorough clinical history and careful physical examination by the family physician and the otolaryngologist in this health area, including a posterior rhinoscopy, to diagnose this disease and to achieve early removal of the angiofibroma, by a surgical procedure. It was concluded that the clinical diagnosis corresponded to the histopathological diagnosis, and that early surgical treatment is resolute in this condition

Angiofibroma juvenil nasofaríngeo / Juvenile nasopharyngeal angiofibroma

Se presenta el caso de un angiofibroma juvenil nasofaríngeo, en un paciente del consultorio médico No. 9, que pertenece al Policlínico Integral Docente Carlos Manuel Portuondo de Marianao. Se reconoce la importancia de realizar una historia clínica detallada, así como un minucioso examen físico, por el médico de familia y el otorrinolaringólogo del área de salud, que incluyó la rinoscopia posterior, para poder llegar al diagnóstico de esa patología, y realizar la extirpación precoz del angiofibroma, mediante el proceder quirúrgico. Se concluyó que el diagnóstico clínico se correspondió con el histopatológico, y que la conducta quirúrgica temprana es resolutiva en la afección(AU)

It is presented a case of juvenile nasopharyngeal angiofibroma in a patient with this pathology, from the clicial practice No. 9, in Comprehensive Teaching Polyclinic Carlos Manuel Portuondo in Marianao. It recognizes the importance of a thorough clinical history and careful physical examination by the family physician and the otolaryngologist in this health area, including a posterior rhinoscopy, to diagnose this disease and to achieve early removal of the angiofibroma, by a surgical procedure. It was concluded that the clinical diagnosis corresponded to the histopathological diagnosis, and that early surgical treatment is resolute in this condition(AU)

[Surgical treatment of children presenting with juvenile angiofibroma of the skull base].

A total of 475 children have been operated at the Russian Children's Clinical Hospital during the last 20 years for the removal of benign and tumour-like neoplasms localized mostly in the nasal cavity, paranasal sinuses, and nasopharynx. As many as 361 (76%) of these patents were found to have juvenile angiofibroma of the base of the skull. In most of them, the tumour was diagnosed at the late stages of development. Accordingly, the scarcity and non-specific character of early clinical manifestations of the disease taken together with complicated topographic anatomy of angiofibromas and the difficulty of detailed visualization of the affected zone in young children account for their late hospitalization. The angiomatous lesions prevailed in the spheno-ethmoidal and basiliar regions. The children were operated through the nasomaxillary approach as described by Denker (in V.S. Pogosov's modification) without ligation of external carotid arteries. Since 1996, surgery has been performed after endovascular occlusion of the vessels feeding the tumour, with the maximally possible sparing of bone structures and taking into account the continuing active growth and formation of the facial skeleton. Relapses were documented in 43 (12%) patients. Postoperative radiotherapy had to be applied in the children showing recurrent growth of the tumour or its intracranial extension and in the cases when radical removal of the tumour proved impracticable because of its localization in the vitally important anatomical regions.

Conformal radiotherapy in the treatment of advanced juvenile nasopharyngeal angiofibroma with intracranial extension: an institutional experience.

PURPOSE: To describe the results of conformal radiotherapy in advanced juvenile nasopharyngeal angiofibroma in a tertiary care institution. METHODS AND MATERIALS: Retrospective chart review was conducted for 8 patients treated with conformal radiotherapy between 2006 and 2009. The median follow-up was 17 months. All patients had Stage IIIB disease with intracranial extension. Radiotherapy was considered as treatment because patients were deemed inoperable owing to extensive intracranial/intraorbital extension or proximity to optic nerve. All but 1 patient were treated with intensity-modulated radiotherapy using seven coplanar fields. Median (range) dose prescribed was 39.6 (30-46) Gy. Actuarial analysis of local control and descriptive analysis of toxicity profile was conducted. RESULTS: Despite the large and complex target volume (median planning target volume, 292 cm(3)), intensity-modulated radiotherapy achieved conformal dose distributions (median van't Reit index, 0.66). Significant sparing of the surrounding organs at risk was obtained. No significant Grade 3/4 toxicities were experienced during or after treatment. Actual local control at 2 years was 87.5%. One patient died 1 month after radiotherapy secondary to massive epistaxis. The remaining 7 patients had progressive resolution of disease and were symptom-free at last follow-up. Persistent rhinitis was the only significant toxicity, seen in 1 patient. CONCLUSIONS: Conformal radiotherapy results in good local control with minimal acute and late side effects in juvenile nasopharyngeal angiofibromas, even in the presence of advanced disease.

Manifestações neoplásicas em rinofaringe / Neoplasic manifestation in rhinopharynx

As neoplasias primárias de nasofaringe são raras na infância e adolescência. Sintomas como obstrução nasal persistente, cefaleia, epistaxes recorrentes e abaulamento paranasal ou de palato são comuns à maioria delas e podem ser confundidos com outras doenças infecciosas ou alérgicas.As neoplasias mais frequentes na faixa etária pediátrica são os rabdomiossarcomas, os linfomas não Hodgkin, o estesioneuroblastoma, o carcinoma de nasofaringe e o angiofibroma juvenil. A biópsia ou ressecção da lesão quando possível, seguida de tratamento quimio e radioterápico, quando indicado, compõem o tratamento destas neoplasias.

Angiofibroma juvenil de nasofaringe: actualización de los resultados de la radioterapia / Nasopharyngeal juvenile angiofribroma: updating of radiotherapy results

INTRODUCCIÓN. El angiofibroma juvenil de nasofaringe es un tumor benigno infrecuente, compuesto de tejido conectivo fibroso y abundancia de espacios vasculares revestidos de endotelio. Es casi exclusivo del sexo masculino y de la adolescencia. El tratamiento de elección es la exéresis, sin descartar otras posibilidades como la radioterapia. El objetivo del presente estudio fue presentar los resultados de esta última como opción terapéutica. MÉTODOS. Se estudió una serie de 11 pacientes, todos del sexo masculino, con edades entre 9 y 16 años, que fueron tratados en el Instituto Nacional de Oncología y Radiobiología de La Habana entre 1990 y 2005. Los pacientes fueron seguidos entre 48 y 306 meses. La radioterapia aplicada consistió en la irradiación de todo el volumen tumoral, con un margen de seguridad, en dosis de 40 a 60 Gy, con 1,8 Gy por sesión. Asociado a la radioterapia se utilizó interferón, durante y después de la irradiación, y poliquimioterapia en 1 paciente. RESULTADOS. Se obtuvo la remisión completa mantenida, sin recidivas, en 10 pacientes. No hubo pacientes con un segundo tumor. Presentaron complicaciones tempranas todos los pacientes, y tardías, solo algunos. Entre las complicaciones tempranas se halló radiomucositis y conjuntivis radiógena, y las más graves de las tardías fueron la pérdida permanente de las pestañas del párpado inferior en un caso y cataratas radiógenas en 4 pacientes. CONCLUSIONES. La radioterapia es un tratamiento que conserva su utilidad e indicaciones específicas(AU)

INTRODUCTION: The nasopharyngeal juvenile angiofribroma is a uncommon benign tumor composed of fibrous connective tissue and many vascular spaces covered by endothelium. It is almost exclusive of male sex and of adolescents. Choice treatment is the exeresis without obviate other possibilities as the radiotherapy. The aim of present study was to show the results of this latter as therapeutical option. METHODS: Authors studied a series of 11 male patients, aged between 9 and 16, seen in National Institute of Oncology and Radiobiology of La Habana between1990 and 2005. Patients were followed during 48 and 306 months. Radiotherapy applied was the irradiation of all the tumor volume with a accuracy margin in dose of 40 and 60 Gy, with 1,8 Gy by session. Together with radiotherapy we used Interferon, during and after irradiation and polychemotherapy in one patient. RESULTS: There was a maintained complete remission without relapses in 10 patients. There weren't patients with a second tumor. All patients had early complications and late in some of them. Among the early complications we found radiomucositis and radiogenic conjunctivitis and the more severe of the late complications were the permanent loss of eyelash of lower eyelid in a case and radiogenic cataract in 4 patients. CONCLUSIONS: Radiotherapy is a treatment maintaining its usefulness and specific indications(AU)

[Treatment modalities of nasopharyngeal angiofibroma]. / Nazofarenks anjiyofibromlarinda tedavi yaklasimlari.

Nasopharyngeal angiofibroma is a rare, benign vascular tumor originating from the sphenopalatine foramen. It primarily affects adolescent males. Due to its propensity to locally destructive growth, the tumor may lead to fatal epistaxis, intracranial extension, and life-threatening complications such as intraoperative hemorrhage. Many treatment modalities have been used for the management of nasopharyngeal angiofibroma, but surgery and external beam radiation therapy have proved to be the only effective treatment modalities with acceptable morbidity. While endoscopic surgery provides successful results for early stage tumors, recent technological advances in radiotherapy offer significant advantages in advanced and recurrent tumors.

[Efficacy of remote gamma-therapy in patients with juvenile angiofibroma of the base of the skull].

The results of radiation therapy of 48 patients with juvenile nasopharyngeal angiofibroma were analysed. It was found that radiation therapy can produce 80% stable remissions in postoperative recurrence or unresectable tumor. The response did not depend on primary size of the tumor and was better in a total dose over 35 Gy and in children over 14 years of age. Long-term side effects were minimal and did not deteriorate quality of life.

[Nasopharyngeal angiofibroma with intracranial extension: situating the chemotherapy-radiotherapy association]. / L'angiofibrome nasopharyngien avec extension intracrânienne : place de l'association chimiothérapie-radiothérapie.

Nasopharyngeal angiofibroma is a locally aggressive, although histologically benign, vascular neoplasm. This neoplasm accounts for 0.05% of head and neck tumours and affects almost exclusively male adolescents. Surgery is considered as the primary treatment of nasopharyngeal angiofibroma. Other treatment modalities such as radiotherapy and chemotherapy are still recommended for intracranial extension involving the cavernous sinus or the internal carotid artery. We report a rare case of nasopharyngeal angiofibroma, further complicated with a Kennedy syndrome in a 34 year-old women. The treatment consisted in a chemotherapy (adriamycine, decarbazine) followed by radiotherapy. We discuss the relevance and outcome of the association chemotherapy-radiotherapy in the treatment of nasopharyngeal angiofibromas with a consistent intracranial extension (stage III B of Arch Otolaryngol Head Neck Surg 122 (2003) 122-129).

Nasopharyngeal angiofibroma treated with radiotherapy.

Nasopharyngeal angiofibroma is a rare, highly vascular, benign, locally aggressive tumor, affecting boys of adolescent age. The aggressiveness and high vascularity makes surgery and even a biopsy difficult in majority of cases. Although surgery is the treatment of choice in early cases, considerable debate exists regarding the treatment of advanced disease with intracranial extension. Radiotherapy provides a good response and also avoids surgery-associated morbidity. We are herewith reporting a case of nasopharyngeal angiofibroma who showed complete hemostasis and improvement in vision to radiotherapy.