Cellular angiofibroma of the female pelvic cavity: a case report.

BACKGROUND: Cellular angiofibroma is a rare benign mesenchymal tumor that mostly occurs in the genital area. Its occurrence outside this region, particularly in the pelvis, is extremely rare. To our knowledge, this study reports the first case of cellular angiofibroma occurring in the pelvic cavity, except for one case reported in the retroperitoneum. CASE PRESENTATION: A 25-year-old female patient with chronic, intermittent, dull pain in the lower abdomen that lasted for several months was referred to our clinic. Imaging studies revealed a tumor in the pelvic cavity anterior to the bladder. The radiographic characteristics of this tumor indicated a hypervascular nature, suggesting the possibility of a pheochromocytoma or a neuroendocrine tumor. The patient underwent surgical excision of the lesion. To date, no recurrence has been observed four months after excision. CONCLUSIONS: Cellular angiofibroma, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Immunohistochemical staining can help confirm the diagnosis of this condition. Treatment is generally straightforward, involving local excision of the tumor followed by postoperative monitoring.

A Systematic Review of Laser Treatment for Angiofibromas in Tuberous Sclerosis.

BACKGROUND: While mammalian target of rapamycin inhibitors have revolutionized the management of angiofibroma in tuberous sclerosis complex (TS), physical modalities such as laser are still indicated for recalcitrant lesions. OBJECTIVE: The authors performed a systematic review of the efficacy and safety of laser treatment for TS-related facial angiofibroma. METHODS: The electronic databases such as MEDLINE, Embase, PubMed, Cochrane Central Register of Controlled Trials, and Web of Science were searched from inception to October 10, 2023, for eligible records. RESULTS: Forty-seven articles met the inclusion criteria, representing a total of 217 patients with TS-related facial angiofibroma who received laser treatment. Several lasers have been trialed in patients including carbon dioxide ( n = 95, 43.7%), pulsed dye ( n = 21, 9.7%), argon ( n = 16, 7.4%), neodymium-doped: yttrium aluminum garnet ( n = 12, 5.5%), copper vapor ( n = 9, 4.1%), potassium titanyl phosphate ( n = 7, 3.2%), erbium: yttrium aluminum garnet ( n = 2, 0.9%), lasers and various combination therapies ( n = 55, 25.3%). CONCLUSION: Potassium titanyl phosphate, pulsed dye, and neodymium-dopsed:yttrium aluminum garnet lasers are better suited to manage the vascular components of angiofibroma while ablative lasers such as erbium: yttrium aluminum garnet and carbon dioxide lasers may present better options for lesions with a prominent fibrous component. While several lasers have been trialed with broadly favorable results, the low level of evidence precludes definitive conclusions, and no single laser appears superior.

BiZact-Assisted Endoscopic Resection of a Sinonasal Tumor.

The BiZact device, a bipolar electrosurgical scissor designed for tonsillectomy, minimizes thermal tissue damage and seals blood vessels <3 mm in diameter while dividing the soft tissue. This study describes the authors' experience with sinonasal tumor surgery using a BiZact and discusses its clinical utility and advantages. The authors analyzed BiZact-assisted endoscopic sinonasal tumor surgery cases between January 2021 and May 2023. Data were collected on patients' demographics, histopathology, extent of tumor involvement, surgical records, and postoperative medical records. Clinical utility was assessed using the success rate of complete tumor excision, estimated blood loss during surgery, device-related complications, and operation time. A survey of the surgeons' BiZact experience was also conducted. The diagnoses of the 20 patients in this study included squamous cell carcinoma (n = 2), malignant melanoma (n = 1), sarcoma (n = 1), natural killer cell lymphoma (n = 1), inverted papilloma (n = 12), angiofibroma (n = 2), and schwannoma (n = 1). This pilot study demonstrated a shortened operative time, with a median of 0.8 hours and <100 mL of intraoperative blood loss. In addition, no BiZact-related complications were observed. The BiZact device allows efficient sinonasal surgery because it has the unique advantage of one-step sealing and cutting. BiZact-assisted endoscopic sinonasal tumor surgery is a beneficial and safe procedure that reduces blood loss during surgery, shortens the operative time, and minimizes postoperative complications.

Radiotherapy for recurrent juvenile nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare paediatric tumour known for its local destructiveness and high recurrence rate. Surgery is the primary treatment modality for JNA, though other options, such as hormonal therapy, embolisation and radiotherapy, exist for inoperable cases. The location of the tumour makes surgical intervention challenging. A 14-year-old male presented with epistaxis and headaches as the chief complaints and was diagnosed with nasopharynx angiofibroma by computed tomography (CT) scan in 2018. Pre-operative embolisation was performed and followed by surgical removal of a 4 cm tumour in January 2019. Pathological examination revealed CD34 positivity, S100 negativity and Ki-67 positivity (5 to 10%), confirming angiofibroma. In October 2019, a 3.6 cm recurrent tumour was treated with embolisation and a second surgery. Pathological findings again confirmed JNA. The patient underwent four surgeries in total, but epistaxis persisted. In 2021, local radiotherapy was administered using intensity-modulated radiation therapy (IMRT) at a dose of 60 Gy in 25 fractions. Serial magnetic resonance imaging (MRI) post-radiotherapy showed a decreasing tumour size, with no further epistaxis and no observed radiation side effects 2 years post-treatment. Radiation therapy remains a strong alternative for managing recurrent JNA.

Cellular angiofibroma in the retroperitoneal space during pregnancy: A case report.

BACKGROUND: Cellular angiofibroma (CA) is a rare, benign mesenchymal tumor first described by Nucci et al. (Am J Surg Pathol 21:636-644, 1997. 10.1097/00000478-199706000-00002). It affects both men and women, although it is more common in middle-aged women. CA is well circumscribed and usually observed on the body surface, primarily in distal genital regions. Aggressive angiomyxoma and angiomyofibroblastoma are clinically and histologically similar; therefore, it may be necessary to distinguish between CA and these similar tumors. We present a rare case of CA, with atypical features, in the retroperitoneal space during pregnancy. CASE PRESENTATION: The presence of a 130 mm tumor was detected in a 19-year-old woman. The tumor, located in the retroperitoneal space, was found during first pregnancy examination. At 16 weeks of gestation, the woman developed nausea and fever, and it was diagnosed with acute pyelonephritis. After a few days, the amniotic membranes prematurely ruptured, leading to a miscarriage. The woman underwent a tumor resection, after miscarriage. This case presented with atypical features of CA. This included the young age of the patient, and presence of a tumor in the retroperitoneal space. CONCLUSION: In this case, the diagnosis of CA was difficult due to the rarity of the disease and its atypical clinical features. From this experience, we recommend that the discussion on the efficacy of surgical treatment and pregnancy outcomes should be done based on individual case, and not generalized.

Perioperative management of pediatric patients undergoing juvenile angiofibroma resection. A case series and educational review highlighting patient blood management.

BACKGROUND: Juvenile nasopharyngeal angiofibromas are one of the most enigmatic, bloody tumors encountered by otorhinolarygnologists, head and neck surgeons, neurosurgeons, and anesthesiologists. Juvenile nasopharyngeal angiofibromas are rare, benign, highly vascular tumors with a propensity towards aggressive local invasion. Surgery, open or endoscopic, to remove the growth is the primary treatment of choice for Juvenile nasopharyngeal angiofibromas. Historically, surgical resection was associated with massive, rapid blood loss, traditionally managed by blood product transfusion and deliberate hypotension. Preventative management employing multimodal blood conservation strategies should be an essential standard of perioperative care for patients with Juvenile nasopharyngeal angiofibromas. METHODS: We describe a contemporary and comprehensive approach in the management of patients with high grade Juvenile nasopharyngeal angiofibromas. This includes surgical strategies such as preemptive external carotid artery embolization, endoscopic surgical approach, and staged operations, as well as anesthetic strategies including antifibrinolytic therapy and acute normovolemic hemodilution. These surgeries, once synonymous with massive transfusion, may potentially be performed without allogeneic blood transfusion, or deliberate hypotension. AIMS: Using a case series, the authors introduce a contemporary approach to multimodal, multidisciplinary blood conservation strategies for Juvenile nasopharyngeal angiofibromas surgery. RESULTS: Here in the authors report on an updated contemporary perioperative clinical approach to patients with Juvenile nasopharyngeal angiofibromas. From an anesthetic perspective, we describe the successful use of normal hemodynamic goals, restrictive transfusion strategy, antifibrinolytic therapy, autologous normovolemic hemodilution, and early extubation in the care of three adolescent males with highly invasive tumors. We demonstrate that new surgical and anesthetic strategies have yielded a significant decrease in intraoperative blood loss and eliminated the need for transfusion of autologous red blood cells, which enable improved outcomes. CONCLUSIONS: The perioperative approach to elective surgery for Juvenile nasopharyngeal angiofibromas management is presented from a multidisciplinary patient blood management perspective.

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.

Endoscopic, endoscopic-assisted and open approaches in the treatment of juvenile angiofibroma: what has been new in the past decade (and 1586 cases)?

PURPOSE: Juvenile angiofibroma (JA) is a benign, but locally invasive tumor of the nasopharynx. Surgical resection of JA is performed through endoscopic (EA), endoscopic-assisted (EAA), or open approaches (OA). The management of these tumors is constantly evolving. We aimed to compare the surgical efficiency and morbidity of EA, EAA, and OA in JA treatment by conducting a systematic review of the literature published over the last 10 years. METHODS: A systematic review of the English literature on surgical cases of JA published between 2012 and 2022 was performed. Eligible articles were analyzed for individual patient data (IPD) and aggregate patient data (APD). The primary predictor variable was the surgical approach. The primary outcome variable was recurrence rate. RESULTS: The search retrieved 75 articles reporting 1586 JA surgical cases; 129 in IPD, and 1457 in APD data sets. Within the IPD data set, recurrence rates were significantly lower in cases completed by EA than that by OA (p < 0.05). There was no significant difference in recurrence rates between the EA and EAA groups (p > 0.05). EAA had a lower recurrence rate than that of OA (p < 0.05). For the APD data set, the recurrence rate following EA was significantly lower than that following OA (p < 0.05). There was no significant difference in recurrence between the EA and EAA groups (p > 0.05), and between the EAA and OA groups (p > 0.05). CONCLUSIONS: EA represents the method of choice for mild and moderately advanced JA. EAA and OA still play important roles in the treatment of advanced-stage JA.

Harmonic Scalpel-Assisted Endonasal Endoscopic Resection of the Vascular Tumors of the Anterior Skull Base.

Vascular tumors represent a challenging pathologic subset for surgical treatment as they show a propensity for profuse bleedings. This is especially applicable to the skull base region, where surgical access is difficult due to its complex anatomy. To overcome this problem, the authors introduced the use of a harmonic scalpel in endoscopic skull base surgery for vascular tumors. Here, the authors report the outcomes of endoscopic harmonic scalpel-assisted surgery in 6 juvenile angiofibromas and 2 hemangiomas. All surgeries were performed using Ethicon Endo-Surgery HARMONIC ACE 5 mm Diameter Shears. The median intraoperative blood loss was 400 mL (range: 200-1500 mL). The median length of hospital stay was 7 days (range: 5-10 days). Recurrence was recorded in 1 patient with juvenile angiofibroma, which was successfully resolved with revision surgery. In this institutional experience, ultrasonic technology showed precise cutting with minimal bleeding, resulting in reduced surgical morbidity compared with conventional endoscopic instruments.

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.

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.

A case report of an extremely rare type of cardiac tumor: Primary cardiac angiofibroma.

Cardiac angiofibroma is a very rare diagnosis when a patient develops an intracardiac mass. It is a primary benign cardiac tumor with a scarcity of information in the literature. This case report illustrates a 26-year-old man with a complaint of chronic chest tightness who was first diagnosed with right ventricle tumor by echocardiography then underwent cardiac magnetic resonance imaging (CMR) which confirmed the presence of a highly-vascular tumor with radiologically benign behavior. Then his tumor was excised, his postoperative course was uncomplicated and he was well within almost 2 months after discharge. Ultimately the histopathologic findings demonstrated vascular and stromal tissue in favor of angiofibroma and excluded the other diagnoses with immunohistochemical and trichrome staining. Angiofibroma is a benign, highly vascular tumor, mostly discovered in the nasopharynx. When it is found in the heart, CMR and pathology are pivotal to rule in its diagnosis. It is isointense in T1 weighted and hyperintense in T2 weighted sequences with intense enhancement following contrast injection. Its pathology contains an admixture of vasculatures with CD31 positive immunoreactivity for endothelial cells and fibrotic tissue with bluish coloration in trichrome staining. Eventually, its treatment includes merely surgical excision given its benign nature.

Nasopharyngeal rhinosporidiosis with intracranial extension masquerading as juvenile angiofibroma: an unusual entity.

Rhinosporidiosis is a chronic fungal inflammatory disease prevalent in India and Sri Lanka. Its manifestations are mostly nasal and extranasal lesions are relatively rare. Occasional atypical presentations of this disease lead to diagnostic dilemma. Herein we report on a case of nasopharyngeal rhinosporidiosis having extensive involvement of paranasal sinuses along with intracranial extension which mimicked radiologically as juvenile nasopharyngeal angiofibroma. To our knowledge, this is the first reported case of rhinosporidiosis having intracranial extension. We discuss the pathology, treatment and briefly review the literature of this rare disease.

Extranasopharyngeal Angiofibroma of the Natural Ostium of Maxillary Sinus.

ABSTRACT: Extranasopharyngeal angiofibroma which occurs in all head and neck regions is extremely rare. Unlike most angiofibromas which show nasal congestion and recurrent epistaxis as their symptoms, extranasopharyngeal angiofibromas (ENAF) may lead to various symptoms depending on their location. Nasal septum is the most frequent site of origin of ENAF. No study of ENAF originating in natural ostium of maxillary sinus has been reported. We present a case of 27-year-old male who has extranasophar- yngeal angiofibroma arising from the natural ostium of maxillary sinus in an adult patient whose symptom was right sided nasal obstruction. With this study, although uncommon, angiofibroma should be considered as a differential diagnosis in patient with mass lesion in the middle nasal meatus.

Cellular angiofibroma of orbit- a rare tumor at the rarest location.

Cellular angiofibroma (CAF) is a rare, site-specific benign tumor of mesenchymal origin, which is exclusive of the genital region except very few reported cases of extragenital CAF. Very recently the first case report of orbital CAF has been published and we are presenting the second documented case of CAF in this unusual location, in a 37-year-old man. Magnetic resonance imaging with T2 weighted image revealed a hyper-intense globular mass in the superomedial extraconal region of the left orbit. Histopathology of the excised mass showed numerous bland spindle cells embedded in wispy collagen, suggesting spindle cell tumor. On immunohistochemistry, the specimen was strongly positive to CD34 but negative to desmin, SMA, S-100, C-kit, ERG, EMG, CD31, and CD63, that further confirmed the diagnosis of CAF. CAF is a rare mesenchymal tumor and to the best of the authors' knowledge, this would be the second documented case of orbital CAF.

[The maxillary swing approach in surgycal treatment of the extensive juvenile nasopharyngeal angiofibroma]. / Ispol'zovanie metoda transpozitsii verkhnei chelyusti v khirurgicheskom lechenii mestnorasprostranennoi yuvenil'noi angiofibromy osnovaniya cherepa.

The objective of this article is to demonstrate a clinical case with a good outcome by using a maxillary swing approach during the surgical treatment of extensive locally advanced form of the juvenile nasopharyngeal angiofibroma (JNA) stage Fisch-Andrews IIIb (Radkowski IIIb). Thus the maxillary swing approach has given an adequate exposure of the skull base and is an effective approach in the surgical treatment of extensive JNA with a good overview of anatomical structures and has a minimal risk of complications and to evaluate an efficacy of the results.

Endoscopic Endonasal and Transmaxillary Approach for Resection of Juvenile Nasopharyngeal Angiofibroma With Preoperative Embolization in a Child.

ABSTRACT: A 13-year-old Japanese boy with a 6-month history of bilateral nasal obstruction and a 3-week history of recurrent epistaxis from the right nose was admitted to our department. Nasal endoscopy revealed a reddish, smooth-walled tumor occupying the right nasal cavity. Computed tomography scan revealed a 3.5 × 4.5 × 7.0-cm heterogeneously enhancing mass involving the right nasal cavity and extending posteriorly to the nasopharynx, and laterally to the pterygopalatine fossa and the medial part of the infratemporal fossa. We diagnosed as juvenile nasopharyngeal angiofibroma with Radkowski classification stage IIC. The internal maxillary and ascending pharyngeal arteries were embolized with polyvinyl alcohol followed by Embosphere using a conventional Seldinger technique. En bloc resection was performed with an endoscopic ipsilateral endonasal and sublabial Caldwell-Luc transmaxillary approach under general anesthesia. As of 3 years postoperatively, no recurrence has been found. We report a child case of juvenile nasopharyngeal angiofibroma successfully treated with less invasive surgery with preoperative embolization.

Midfacial degloving for juvenile angiofibroma: A case-series of 21 adult males: An alternative to the endoscopic approach and when it should be considered.

OBJECTIVES: To describe the midfacial degloving approach and compare the varying surgical approaches to juvenile angiofibromas. To demonstrate the efficacy of midfacial degloving in treating large juvenile angiofibromas in a unique patient cohort. DESIGN: A retrospective case-series between 2006 and 2019. SETTING: All patient care was undertaken at a regional skull base referral centre. PARTICIPANTS: Twenty-one male patients with a median age of 18 (range 16-45 years). MAIN OUTCOME MEASURES: Presenting symptoms, imaging, stage, age at operation, residual disease, estimated blood loss and operative time were all recorded. Postoperative outcomes included complications, length of stay and recurrence. RESULTS: The median surgical time was 105 minutes (range 55-219 minutes), median estimated blood loss 600 mls (range 150-900 mls) and median length of stay was 4 days (range 2-13 days.). Complications included two episodes of epistaxis, one requiring packing and one return to theatre. 14% (3/21) of patients had residual disease, none requiring further treatment and one patient had recurrence. CONCLUSIONS: MFD for JNA in our series resulted in low recurrence rate and no progression of residual disease. The approach has been successful in our cohort of patients and is an option in males over the age of 16 years, with JA extending beyond the nasopharynx and sinuses, involving the infratemporal fossa, cavernous sinus or orbital region.

Cellular angiofibroma of the orbit.

Cellular angiofibroma is a benign mesenchymal tumor most commonly located in the distal genital tract of both men and women. Although extragenital locations have been reported rarely, this is the first report of cellular angiofibroma of the orbit. A 58-year-old man presented with a mass in the left superomedial orbit since 2 years. Magnetic resonance imaging showed a well-demarcated lesion with a homogeneous intermediate signal intensity on both T1- and T2-weighted images, homogeneous contrast enhancement and high signal intensity on diffusion-weighted images. Complete excision was performed through a medial upper eyelid crease incision. Histopathology showed a vascular CD34-positive and STAT6-negative spindle cell tumor with monoallelic loss of FOXO1, indicating cellular angiofibroma.