Paratesticular cellular angiofibroma: a case report.

INTRODUCTION: Paratesticular cellular angiofibroma is a rare benign mesenchymal tumor. The optimal management is surgical resection due to the difficulty of preoperative accurate diagnosis. CASE PRESENTATION: A 51-year-old Japanese male visited our hospital complaining of asymptomatic left scrotal swelling. Physical examination revealed a nontender elastic paratesticular mass (5.5 cm in diameter). Although testicular germ cell tumor was ruled out clinically, the possibility of malignant potential remained for the tumor. Since the patient consented to complete resection, a transinguinal radical orchiectomy was performed. The pathological diagnosis revealed cellular angiofibroma. The patient recovered without perioperative complications, and no apparent recurrence was observed at 5 years after surgery. CONCLUSION: The pathological findings were compatible for cellular angiofibroma. The tumor was successfully resected, and no apparent recurrence was observed at 5 years after surgery.

Giant cell angiofibroma of gingiva in tuberous sclerosis complex: a case report and literature review.

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare, complex genetic disorder characterized by hamartomas and neoplastic lesions in various organ systems. With the development of radiology and gene testing, the diagnostic criteria for TSC were updated in 2012 at the International Consensus Conference. Intraoral fibromas have long been associated with TSC. However, the incidence of giant cell angiofibroma (GCA) in TSC patients is extremely rare. Here, we report the first case of GCA in the gingival tissue of a patient with TSC. CASE PRESENTATION: A 41-year-old woman first visited the Department of Oral and Maxillofacial Surgery, Chonnam National University Dental Hospital, complaining of gingival enlargement. Clinical examination revealed several manifestations associated with TSC, including intraoral fibromas, facial angiofibromas, dental enamel pits, ungual fibromas, "confetti" skin lesions, hypomelanotic macules, and a shagreen patch. Intraoral examination revealed a 6.0 × 5.0 cm gingival overgrowth on the left mandible. Surgical excision was performed, and subsequent histopathological examination confirmed the diagnosis of GCA. There was no evidence of recurrence within the 24- months of surgery. CONCLUSIONS: We report the first case of GCA in the gingival tissue of a patient with TSC. This report would contribute to an improved understanding of this rare disease. However, further case reports are necessary to clarify the relationship between GCA and TSC.

Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review.

BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns. OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented. METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages. RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively). CONCLUSION: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.

[Association between vascularization and its impact for recurrence of juvenile nasoangiofibroma]. / Asociación entre vascularización y su impacto en la recurrencia de nasoangiofibroma juvenil.

Background: Juvenile nasoangiofibroma (JNA) is a rare, highly vascular, locally aggressive benign tumor which affects male adolescents. It accounts for 0.05-0.5% of head and neck tumors with recurrence rates of 6-50%. The internal maxillary artery is the main source of JNA. Objective: To evaluate the relationship between vascular supply as a factor associated with JNA recurrence. Material and methods: An cohort study was performed in patients diagnosed with NAJ. We collected demographic data, vascular contribution by angiography and tomography results to classify them according to their stage (Radkowski classification), and if they received adjuvant radiotherapy. Post-surgical CT scans were requested to evaluate recurrence and if any of the variables were related to this. Results: A sample of 14 male patients who met the inclusion criteria was collected. The mean age was 14.71 ± 4.08 years. According to Radkowski classification, stage IA, IIA and IIC were reported in 14.3%, IIB and IIB in 7.1% and IIIA in 42.9%. 42.9% had recurrence and out of these, 66.7% had irrigation of the right carotid system and the same percentage of patients received radiotherapy as adjuvant treatment. Conclusions: There is a tendency in tumor recurrence associated with vascular contribution from the right carotid system, as well as with patients who received radiotherapy.

Introducción: el nasoangiofibroma juvenil (NAJ) es un tumor benigno, raro, altamente vascular y localmente agresivo que afecta a adolescentes del sexo masculino. Representa de 0.05 a 0.5% de los tumores de cabeza y cuello con tasas de recurencia del 6-50%. La arteria maxilar interna se considera el principal aporte de los NAJ. Objetivo: evaluar la relación entre el aporte vascular como factor asociado con la recurrencia de NAJ. Material y métodos: se realizó un estudio de cohorte en pacientes con diagnóstico de NAJ. Se recabaron datos demográficos, el aporte vascular por resultados de angiografía y de tomografía para clasificarlos según su estadio (clasificación de Radkowski), y si recibieron radioterapia adyuvante. Se solicitaron tomografías postquirúrgicas para evaluar la recurrencia y si alguna de las variables tiene relación con esta. Resultados: se recolectó una muestra de 14 pacientes del sexo masculino que cumplieron con los criterios de inclusión. La edad promedio fue de 14.71 ± 4.08 años. Según la clasificación de Radkowski, se reportó un estadio IA, IIA y IIC en 14.3%, IIB y IIB en un 7.1% y IIIA en 42.9%. El 42.9% tuvo recurrencia y de estos, el 66.7% tenía irrigación del sistema carotídeo derecho y recibieron radioterapia como tratamiento adyuvante el mismo porcentaje de pacientes. Conclusiones: existe una tendencia en la recurrencia del tumor asociada al aporte vascular proveniente del sistema carotídeo derecho y también a los pacientes que recibieron radioterapia.

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.

Extended Endonasal Endoscopic (EEE) Surgery with Almost No Use of Adjuvant Radiotherapy for Juvenile Nasopharyngeal Angiofibroma (JNA).

Background and Objectives: Juvenile nasopharyngeal angiofibroma (JNA) is an angiomatous hamartoma of the nasal cavity. It is a benign but locally aggressive vascular tumor of the nasopharynx affecting adolescent males. Many surgical procedures are in practice, but the extended endonasal endoscopic (EEE) approach for JNAs is a suitable and effective technique. Materials and Methods: Fifteen adolescent patients having JNA who underwent extended endonasal endoscopic (EEE) surgery from January 2010 to January 2022 were studied retrospectively. Patients having residual and recurrent JNAs and those who underwent surgery other than EEE were excluded. Results: The average age of the patients was 18.3 years of age. A total of six patients (40%) each had stage V and IV while three patients (20%) had stage III JNAs. Gross total removal was achieved in eight (53.3%) patients and seven (43.7%) had partial removal. There was no per or postoperative mortality. All the patients had at least 3 years of postoperative follow-up and during follow-ups, seven patients were found to have residual tumors, and two had recurrences. Discussion: During the last decades, the endoscopic approach for the resection of JNAs has gained increasing popularity due to its obvious advantages over transfacial approaches. The magnified and angled field of view "behind the corner" helping in a more complete inspection for the resection and shorter hospitalization time makes it a better choice than the other approaches. Conclusions: Endoscopy is an excellent approach for primary JNA. It allows well visualization and precise removal of the angiofibroma. An endoscopic multiangle, multicorridor skull base approach including Denker's anteromedial maxillotomy is suitable and preferable for the resection of extensive JNAs.

[Surgical approach analysis of endoscopic resection of juvenile nasopharyngeal angiofibroma].

Objective:To investigate the surgical approach for the resection of juvenile nasopharyngeal angiofibroma（JNA） under nasal endoscopy. Methods:The clinical data of 87 patients undergoing endoscopic resection of nasopharyngeal fibroangioma were retrospectively analyzed. We classified JNA according to tumor site, size, invasion scope and anatomic position relationship between tumor and midline of pupil. Three endoscopic surgical approaches were selected according to the classification, and the postoperative symptoms, complications and recurrence were investigated and analyzed. Results:The tumor resection rate of 87 cases by nasal endoscopic surgery was 100%. Thirty-five cases were approached through the middle nasal passage（small tumors located in the nasal sinuses and pterygopalatine fossa）, forty-five cases were approached through the lateral wall of the nasal cavity（tumor invaded the pterygopalatine fossa but did not exceed the midline of the pupil） , and seven cases were approached via the lateral wall of nasal cavity + ipsilateral anterior wall of maxillary sinus（tumor invaded the infratemporal fossa beyond the midline of pupil or invaded the cavernous sinus and the middle cranial fossa epidural）, Postoperative patients with nasal congestion, nasal bleeding, headache, dizziness, vision loss and other symptoms showed varying degrees of improvement. No surgical death or intracranial infection occurred. The postoperative follow-up was 6-78 months, and the recurrence rate was 3.44%. Conclusion:Endoscopic resection of nasopharyngeal fibroangioma is the main treatment method for JNA. Selecting suitable endoscopic approach to resect JNA, To maximize the advantage of nasal endoscopic equipment according to the inherent anatomical space of the human nasal cavity, In order to achieve the purpose of JNA resection, reduce intraoperative and postoperative complications, reduce the recurrence rate and improve the prognosis.

Angiomiofibroblastoma vulvoperineal, presentación de un caso / Vulvoperineal angiomyofibroblatoma, a case report

An angiomyofibroblastoma is a benign tumor that may present itself in the genital tract of a reproductive age woman. Despite it being a benign neoplasia, unable to be invasive, it can reach large sizes, producing discomfort, pain, and anatomical deformation. A late diagnosis may result in increased surgical difficulty for resection. The following is the clinical case of a woman with a vulvo-perineal tumor of 6 years of evolution, with a final diagnosis of angiomyofibroblastoma, in which complete resection of the lesion was achieved with good cosmetic results. The highlight of this tumor is its slow, silent, but progressive growth, leading to diagnoses that are often confused and late, with a consequent delay in treatment. Among the common differential diagnoses are the Bartholin gland cyst and the aggressive angiomyxoma.

El angiomiofibroblastoma es un tumor benigno que puede presentarse en el tracto genital de la mujer en edad reproductiva. A pesar de ser una neoplasia benigna, sin capacidad invasora, puede alcanzar grandes tamaños, produciendo molestia, dolor y alteración importante de la anatomía. Si el diagnóstico es tardío, aumenta la dificultad quirúrgica en su resección. A continuación, se presenta el caso clínico de una mujer con un tumor vulvoperineal de 6 años de evolución, con diagnóstico final de angiomiofibroblastoma, en el que se logró la resección completa de la lesión con un buen resultado estético. La importancia de este tumor está dada por su crecimiento lento, silencioso, pero progresivo, llevando a que su diagnóstico sea en muchas ocasiones confuso y tardío, con el consecuente retraso en el tratamiento. Dentro de los diagnósticos diferenciales comunes se encuentran el quiste de la glándula de Bartholino y el angiomixoma agresivo.

Preoperative Embolization of Primary Juvenile Nasopharyngeal Angiofibroma: Is Embolization of Internal Carotid Artery Branches Necessary?

PURPOSE: To determine the effects of blood supply from internal carotid artery (ICA) on the surgical outcomes of primary juvenile nasopharyngeal angiofibroma (JNA) after transarterial embolization (TAE). METHODS: A retrospective analysis was performed on primary JNA patients who underwent TAE and endoscopic resection in our hospital between December 2020 and June 2022. The angiography images of these patients were reviewed, and then they were divided into ICA + external carotid artery (ECA) feeding group and ECA feeding group according to whether the ICA branches were part of the feeding arteries. Tumors in ICA + ECA feeding group were fed by both ICA and ECA branches, while tumors in ECA feeding group were fed by ECA branches alone. All patients underwent tumor resection immediately after ECA feeding branches embolization. None of the patients underwent ICA feeding branches embolization. Data on demographics, tumor characteristics, blood loss, adverse events, residual and recurrence were collected, and case-control analysis was performed for the two groups. Differences in characteristics between the groups were tested using Fisher's exact and Wilcoxon tests. RESULTS: Eighteen patients were included in this study: nine in ICA + ECA feeding group and nine in ECA feeding group. The median blood loss was 700 mL (IQR 550-1000 mL) in ICA + ECA feeding group versus 300 mL (IQR 200-1000 mL) in ECA feeding group, with no significant statistical difference (P = 0.306). Residual tumor was found in one patient (11.1%) in both groups. Recurrence was not observed in any patient. There were no adverse events from embolization and resection in either group. CONCLUSION: The results of this small series suggest that the presence of blood supply from ICA branches in primary JNA has no significant effect on intraoperative blood loss, adverse event, residual and postoperative recurrence. Therefore, we do not recommend routine preoperative embolization of ICA branches. LEVEL OF EVIDENCE: Level 4, Case-control.

Combined endoscopic endonasal and sublabial transmaxillary approaches for resection of intracranially extended juvenile nasopharyngeal angiofibroma.

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are rare, benign, and locally invasive nasopharyngeal tumors. Endoscopic endonasal resection is effective, non-invasive, with low complication rates. Until recently, endoscopic resection was not suitable for intracranially invasive tumors. PURPOSE/METHOD: We describe resection steps of an intracranially extending JNA with a combined endoscopic endonasal and endoscopic-assisted sublabial transmaxillary approaches. Indications, advantages, and approach-specific complications are also discussed. The main surgical steps are shown in an operative video. CONCLUSION: Surgical excision of JNAs by a combined endoscopic endonasal and sublabial transmaxillary approaches represents a safe and effective treatment for selected intracranially invasive JNA.

'Juvenile' nasal angiofibroma presenting in adulthood.

Juvenile nasopharyngeal angiofibromas (JNAs) are rare hyper vascular, benign tumours typically demonstrating a locally aggressive growth pattern. The cardinal presenting symptoms are unilateral nasal obstruction and recurrent, spontaneous epistaxis. Cases outside the adolescent male population are exceedingly rare and present a diagnostic challenge. We present the case of a man in his 30s referred to our tertiary skull base centre, presenting with left nasal obstruction. Examination showed left nasopharyngeal fullness without a discrete mass. Cross-sectional imaging detailed a 2.5×2.1×1.3 cm mass localised to his left sphenoid sinus with bony erosion. Due to the suspicion of malignancy, multidisciplinary consensus was to perform a diagnostic excisional biopsy and this revealed a JNA. He remains clinically well and asymptomatic following surgery. This case highlights the potential for subtle symptomatology in the presentation of these tumours and the challenge in diagnosing a JNA outside the adolescent male population.

Anatomic Considerations Guiding Single Versus Multiportal Endoscopic Approaches for Resection of Juvenile Nasopharyngeal Angiofibroma: Cases Series With Graded Multicorridor Resections.

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are characterized by expansive and destructive growth, often invading the midline/paranasal sinuses, pterygopalatine fossa, and infratemporal fossa and can extend into the orbit, cavernous sinus, or intracranially. OBJECTIVE: To evaluete the major benefits of the extended endoscopic endonasal approach (EEA) for JNA resection as compared with more traditional and invasive transpalatal and transfacial approaches. When JNAs extend into lateral anatomic compartments, the optimal operative trajectory often requires additional approach strategies or surgical staging. METHODS: We retrospectively reviewed 8 cases of large JNAs arising in symptomatic adolescent boys (University of Pittsburgh Medical Center Stages II, III, and V) and discuss anatomic and tumor considerations guiding the decision of a pure EEA vs combined EEA and sublabial transmaxillary approach (Caldwell-Luc). RESULTS: A pure extended EEA was used in 6 JNA cases (UPMC Stages II-III); a multiportal EEA + Caldwell-Luc maxillotomy was used in 2 cases. One of the 2 patients (UPMC Stage V) previously treated with multiportal EEA + Caldwell-Luc maxillotomy underwent staged left temporal/transzygomatic craniotomy, obtaining gross total resection. Seven patients ultimately underwent complete removal without recurrence. One patient with a small residual JNA (UPMC II) underwent stereotactic radiosurgery without progression to date. CONCLUSION: JNAs with lateral extension into the infratemporal fossa often benefited from additional lateral exposure using a Caldwell-Luc maxillotomy. Cases with significant skull base and/or dural involvement may undergo staged surgical treatment; temporalis + transzygomatic craniotomy is often useful for second-stage approaches for residual tumor in these lateral infratemporal or intracranial regions. SRS should be considered for residual tumor if additional surgery is not warranted.

Bulky Recurrent Juvenile Nasopharyngeal Angiofibroma.

Juvenile nasopharyngeal angiofibroma is a rare and highly vascularized tumor that accounts for 0.05 to 0.5% of all head and neck neoplasms. The aim of this work was to present a case of a large recurrent juvenile nasopharyngeal angiofibroma coexisting with a facial lipoma in a 16-year-old boy. The patient was referred to our institution because of frequent unilateral epistaxis. Computed tomography revealed a hypervascular tumor with ethmoidal cell destruction and spread to the nasopharynx. Operative treatment of nasal cavity tumors was carried out using a transpalatal approach. After 6 months, the recurrence of the angiofibroma was verified radiologically. Primary as well as secondary surgical procedures were assisted with an endoscopic procedure. Accurate preoperative assessment and staging are essential for choosing a surgical procedure. The primary treatment is surgical excision. Early diagnosis, accurate staging, adequate treatment, and regular postoperative follow-up are essential in the treatment of these lesions.

Giant Cell Angiofibroma in the Buccal Mucosa - A Rising Entity?

BACKGROUND: Tumors of the oral cavity must be differentiated into benign and malignant. Rare tumors must also be considered throughout the differential diagnosis when dealing with pathologic changes in the oral mucosa. Examples of rare benign tumors within the oral cavity are solitary fibrous tumors (SFTs). In recent years, individual case reports of SFTs in the oral cavity have been published showing a rising incidence of this rare entity. CASE REPORT: The present case report describes the occurrence of a subtype of SFT in the right buccal mucosa, the so-called giant cell angiofibroma (GCA). Histopathologically, GCA are distinguishable from SFT (NOS) by pseudovascular spaces lined by multinucleated giant cells. GCA generally shows a benign tumor behavior. The treatment of choice was surgical excision through an intraoral approach. CONCLUSION: To the best of our knowledge, this is one of a few reports of GCA arising in the buccal mucosa.

Juvenile Nasopharyngeal Angiofibroma: Outcomes Analysis Based Upon Hospital Volume.

OBJECTIVE: To evaluate the management of juvenile nasopharyngeal angiofibroma (JNA) from a national perspective with outcomes comparison based on hospital volume. STUDY DESIGN: Ten-year Pediatric Health Information Systems (PHIS) data analysis. METHODS: The PHIS database was queried for the diagnosis of JNA. Data regarding demographics, surgical approach, embolization, length of stay, charges, readmission, and revision surgery was collected and analyzed. Hospitals were classified as low volume if fewer than 10 cases and high volume if greater than or equal to 10 cases during the study period. A random effects model compared outcomes based on hospital volume. RESULTS: A total of 287 JNA patients were identified with a mean age of 13.8 (± 2.7) years. Nine hospitals were classified as high volume, accounting for a total of 121 patients. The mean length of hospitalization, blood transfusion rate, and 30-day readmissions did not differ significantly by hospital volume. Patients cared for at high-volume institutions were less likely to require postoperative mechanical ventilation (8.3% vs. 25.0%; adjusted RR = 0.32; 95% CI 0.14-0.73; p < 0.01) or return to the operating room for residual disease than patients admitted to low-volume hospitals (7.4% vs. 20.5%; adjusted RR = 0.38; 95% CI 0.18-0.79; p = 0.01). CONCLUSIONS: The management of JNA is complex from both an operative and perioperative management standpoint. Over the past decade, nearly half (42.2%) of JNA patients have been managed at nine institutions in the United States. These centers have significantly lower rates of postoperative mechanical ventilation and the need for revision surgery. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3216-3220, 2023.