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1.
BMJ Case Rep ; 16(1)2023 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-36634988

RESUMO

A juvenile nasopharyngeal angiofibroma (JNA) is a benign vascular tumour that arises from the pterygopalatine fossa. It is seen near exclusively in young males though female cases have been reported. Symptoms are due to their high vascularity and mass effect. Commonly reported symptoms include: nasal obstruction, epistaxis and nasal discharge. The mainstay of treatment is surgical resection either via an endonasal endoscopic approach or open surgical resection. Preoperative embolisation has been shown to decrease intraoperative bleeding. Embolisation may be undertaken via a transarterial (TA) approach or, more recently, via direct tumorous puncture (DTP). Options for recurrent or residual disease may include revision surgery, radiotherapy or close clinical surveillance. The following case presentation describes the management of a recurrent JNA in an adult male using preoperative embolisation via a combination of TA and DTP embolisation and an open surgical resection via a subtemporal-preauricular infratemporal fossa approach.


Assuntos
Angiofibroma , Fossa Infratemporal , Neoplasias Nasofaríngeas , Adulto , Humanos , Masculino , Feminino , Angiofibroma/cirurgia , Angiofibroma/diagnóstico , Fossa Infratemporal/patologia , Endoscopia , Neoplasias Nasofaríngeas/cirurgia , Neoplasias Nasofaríngeas/diagnóstico , Punções
2.
Front Endocrinol (Lausanne) ; 13: 1029041, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36325452

RESUMO

Multiple endocrine neoplasia type 1 (MEN1), an autosomal-dominantly inherited tumor syndrome, is classically defined by tumors arising from the "3 Ps": Parathyroids, Pituitary, and the endocrine Pancreas. From its earliest descriptions, MEN1 has been associated with other endocrine and non-endocrine neoplastic manifestations. High quality evidence supports a direct association between pathogenic MEN1 variants and neoplasms of the skin (angiofibromas and collagenomas), adipose tissue (lipomas and hibernomas), and smooth muscle (leiomyomas). Although CNS tumors, melanoma, and, most recently, breast cancer have been reported as MEN1 clinical manifestations, the published evidence to date is not yet sufficient to establish causality. Well-designed, multicenter prospective studies will help us to understand better the relationship of these tumors to MEN1, in addition to verifying the true prevalence and penetrance of the well-documented neoplastic associations. Nevertheless, patients affected by MEN1 should be aware of these non-endocrine manifestations, and providers should be encouraged always to think beyond the "3 Ps" when treating an MEN1 patient.


Assuntos
Angiofibroma , Ilhotas Pancreáticas , Neoplasia Endócrina Múltipla Tipo 1 , Humanos , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/patologia , Estudos Prospectivos , Angiofibroma/patologia , Ilhotas Pancreáticas/patologia , Estudos Multicêntricos como Assunto
3.
Medicine (Baltimore) ; 101(35): e30293, 2022 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-36107545

RESUMO

BACKGROUND: Cellular angiofibroma (CAF), a rare benign mesenchymal tumor, is histologically characterized by abundant thick-walled vessels with a spindle cell component. As one of the female reproductive system tumors, its clinical and pathological features are not well characterized. METHODS: A 47-year-old woman presented for the removal of intrauterine device on October 28, 2021, as she had achieved menopause one year back. The patient had no discomfort or awareness of any mass in her vagina. She has history of breast cancer and papillary thyroid cancer. Till date, no progression of thyroid cancer or breast cancer has been observed. Her menstrual cycle was regular, and she had one child delivered vaginally. RESULTS: Pelvic examination revealed a mass sized 2.5 × 2.0 cm located near the fornix in the upper segment of the left vaginal wall. Thin prep cytologic test (TCT) revealed negative intraepithelial lesion or malignancy (NILM). HPV test was negative and leucorrhea routine inspection cleanliness II degree. No cervical mass was detected by ultrasound examination. The patients underwent the operation for intrauterine device removal plus vaginal tumor resection on November 1, 2021. Postoperative antibiotics (intravenous cefuroxime sodium 0.75 g bid for 1 day) were administered to prevent infection. The patient showed no signs of recurrence at one-month follow-up. CONCLUSION: In summary, CAF is a rare benign soft tissue tumor. Surgery is the only treatment method, and the definitive diagnosis of CAF is based on histopathological examination of surgical specimen. Long-term follow-up is needed for surveillance of recurrence.


Assuntos
Angiofibroma , Neoplasias da Mama , Angiofibroma/diagnóstico , Angiofibroma/patologia , Angiofibroma/cirurgia , Cefuroxima , Feminino , Humanos , Pessoa de Meia-Idade , Sódio , Vagina/patologia , Vagina/cirurgia
4.
Rom J Morphol Embryol ; 63(1): 105-111, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36074673

RESUMO

Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor that affects predominantly males and is known by its highly vascular character. We have performed a 3-year retrospective study of patients with JNA surgically treated within the third ENT Department of Prof. Dr. Dorin Hociota Institute of Phonoaudiology and Functional ENT Surgery, Bucharest, Romania. In all the cases, the patients were investigated both clinically and through medical imaging before surgery and all tumors were embolized. Our study comprised of eight cases, of which seven were solved by endoscopic endonasal approach and one case was treated through a combined endonasal-external approach. JNA should always be managed through a multidisciplinary team (MDT) approach in centers with adequate experience, to gain favorable results.


Assuntos
Angiofibroma , Neoplasias Nasofaríngeas , Angiofibroma/diagnóstico , Angiofibroma/patologia , Angiofibroma/cirurgia , Endoscopia/métodos , Feminino , Humanos , Masculino , Neoplasias Nasofaríngeas/diagnóstico por imagem , Neoplasias Nasofaríngeas/patologia , Nariz/patologia , Estudos Retrospectivos , Romênia
5.
Medicine (Baltimore) ; 101(36): e30125, 2022 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-36086676

RESUMO

RATIONALE: Cellular angiofibroma (CA) is a rare tumor of the soft tissue classified as a benign fibroblastic/myofibroblastic tumor. Considering this, the literature regarding CA mainly, but not exclusively, comprises single case reports and case series. Here, we report a case of giant CA of the vulva with comprehensive literature review. PATIENT CONCERNS: We present a case of a massive vulvar CA arising in 53-year-old woman with no notable medical or surgical history. The mass has grown considerably over time, causing pain and difficult urination, defecation, and movement. The patient had normal regular menstrual cycle with no previous contraception use. Vaginal examination exposed a right-sided large tender vulvar mass with normal-looking vagina. DIAGNOSES: Pelvic magnetic resonance imaging with contrast revealed a large right vulvar heterogeneously enhancing soft tissue mass measuring 13.1 × 10.9 × 10.7 cm expending the left vulva, with internal and peripheral voids resembling feeding vessels. The mass was surgically removed, and subsequent histopathology showed skin-covered dermal-based lesion composed of fibroblast-like bland and spindle cell proliferation with thin-walled blood vessels of various sizes. Immunohistostaining of CD34 and smooth muscle antigen were both positive, while desmin was found to be negative. A diagnosis of vulvar angiofibroma was made based on the clinical scenario, imaging, and histopathology. INTERVENTIONS: Mass vulvectomy was performed starting with a circumferential incision at the base of the mass and structural dissection to separate the mass from the vulvar wall. The incision was successfully closed, and subcuticular stitches were applied to the skin. OUTCOMES: The patient's complaints were significantly relieved with no postoperative complications and the patient is being followed regularly in an outpatient setting. LESSONS: Due to its extremely benign nature of CA, and the implausible ability of its recurrence, it was decided to surgically excise it. Despite its rarity, it can be readily identified at its earlier stages preventing the vexing and exasperating symptoms accompanied with increased size as mentioned.


Assuntos
Angiofibroma , Neoplasias de Cabeça e Pescoço , Neoplasias Vulvares , Angiofibroma/patologia , Angiofibroma/cirurgia , Diagnóstico Diferencial , Feminino , Neoplasias de Cabeça e Pescoço/diagnóstico , Humanos , Pessoa de Meia-Idade , Vulva/patologia , Vulva/cirurgia , Neoplasias Vulvares/patologia
7.
Am J Otolaryngol ; 43(5): 103532, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35714497

RESUMO

INTRODUCTION: Juvenile nasopharyngeal angiofibroma(JNA) are highly vascular benign tumours originating in the sphenopalatine fossa and may extend to the pterygopalatine fossa, paranasal sinuses, and nasal cavity. The management of JNA has evolved greatly with development of endoscopy. Treatment modality has changed from open approach to endoscopic approach due to various advantages offered by the endoscopic approach. Bleeding during the surgery can compromise the exposure and surgical excision of JNA endoscopically. There have been many techniques to decrease intraoperative bleeding including external carotid ligation, hypotensive anaesthesia and more recently embolization of the feeding vessels. Embolization of the tumour has made the endoscopic excision of JNA easier because of less bleeding. This study was taken to find out the outcomes of surgical excision of JNA after embolization. METHODOLOGY: We retrospectively reviewed 22 histopathological proven JNA cases that underwent preoperative embolization followed by resection during the period of June 2015 to December 2020 in our tertiary care hospital. From the records demographic, clinical, radiological imaging (CECT nose and PNS), angiographic, pre-operative embolization and operative details were evaluated. RESULT: In the present study a total of 22 cases of angiofibroma were taken up for surgical excision after embolization. A complete endonasal endoscopic resection could be done in 21/22 cases with average operative time of 141 minutes (80 minutes-190 minutes range). The mean blood loss during surgery was 1163 ml (Range 500 ml- 1900 ml). In 7/22 (31.81%) subjects required intraoperative blood transfusion. No perioperative surgical complications occurred. CONCLUSION: Endoscopic excision of JNA has now become the primary operative technique and pre operative embolization in such cases can reduce the intraoperative time, blood loss and tumour relapse rate. It is a relatively safe procedure in a hand of an expert and must be done where ever available.


Assuntos
Angiofibroma , Embolização Terapêutica , Neoplasias Nasofaríngeas , Angiofibroma/patologia , Embolização Terapêutica/métodos , Endoscopia/métodos , Humanos , Neoplasias Nasofaríngeas/patologia , Recidiva Local de Neoplasia/patologia , Estudos Retrospectivos
8.
Vestn Otorinolaringol ; 87(1): 91-93, 2022.
Artigo em Russo | MEDLINE | ID: mdl-35274899

RESUMO

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.


Assuntos
Angiofibroma , Neoplasias Nasofaríngeas , Angiofibroma/diagnóstico , Angiofibroma/cirurgia , Humanos , Maxila , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/cirurgia
9.
J Coll Physicians Surg Pak ; 32(4): 528-530, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35330531

RESUMO

Solitary fibrous tumour (SFT) is a ubiquitous benign mesenchymal tumour of fibroblastic origin, which occurs most often in middle-aged adults. It usually presents as lung mass originating from pleura, but extrapleural occurrence is also common. Tumour is characterised by hypo- and hyper-cellular areas of spindle-shaped cells, arranged in haphazard manner with dispersed staghorn-shaped vessels. Surgical excision is the curative treatment. SFTs of the primary testicular or paratesticular region are extremely rare, but they exhibit histologic findings similar to SFTs originating at other body sites. Here, we report the case of a paratesticular SFT in a 37-year male, who presented with a non-tender, firm, mobile, left-sided retrotesticular swelling with unique histological features closely mimicking cellular angiofibroma, a common tumour of paratesticular location. Key Words: Solitary fibrous tumour, Paratesticular, benign, angiofibroma.


Assuntos
Angiofibroma , Tumores Fibrosos Solitários , Adulto , Angiofibroma/diagnóstico , Angiofibroma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Fibrosos Solitários/diagnóstico , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Testículo/patologia
13.
Aust Dent J ; 67(3): 281-285, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35152431

RESUMO

This case series presents two asymptomatic cases of juvenile angiofibroma which were initially incidentally identified in pre-orthodontic radiographs. Juvenile angiofibroma is an uncommon, locally aggressive benign, vascular neoplasm with invasive growth patterns. Due to the hypervascularity of these tumours, there are biopsy associated risks and multi-slice computed tomography, magnetic resonance imaging and angiography are usually employed for diagnosis. Early pre-symptomatic identification of this lesion facilitates early management and limiting potential life-threatening complications. This highlights the importance of thorough interpretation of dental radiographs, including the evaluation of structures which are not in the primary region of interest. © 2022 Australian Dental Association.


Assuntos
Angiofibroma , Neoplasias de Cabeça e Pescoço , Neoplasias Nasofaríngeas , Angiofibroma/diagnóstico por imagem , Angiofibroma/patologia , Austrália , Humanos , Achados Incidentais , Imageamento por Ressonância Magnética , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/patologia , Tomografia Computadorizada por Raios X
14.
Int J Mol Sci ; 23(4)2022 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-35216046

RESUMO

The etiology of juvenile angiofibroma (JA) has been a controversial topic for more than 160 years. Numerous theories have been proposed to explain this rare benign neoplasm arising predominately in adolescent males, focusing mainly on either the vascular or fibrous component. To assess our hypothesis of JA's being a malformation arising from neural crest cells/remnants of the first branchial arch plexus, we performed immunohistochemical analyses of neural crest stem cells (NCSC) and epithelial-mesenchymal transition (EMT) candidates. Immunoexpression of the NCSC marker CD271p75 was observed in all investigated JA's (n = 22), mainly around the pathological vessels. Close to CD271p75-positive cells, high MMP3-staining was also observed. Additionally, from one JA with sufficient material, RT-qPCR identified differences in the expression pattern of PDGFRß, MMP2 and MMP3 in MACS®-separated CD271p75positive vs. CD271p75 negative cell fractions. Our results, together with the consideration of the literature, provide evidence that JA's represent a malformation within the first branchial arch artery/plexus remnants deriving from NCSC. This theory would explain the typical site of tumor origin as well as the characteristic tumor blood supply, whereas the process of EMT provides an explanation for the vascular and fibrous tumor component.


Assuntos
Angiofibroma/patologia , Crista Neural/patologia , Células-Tronco Neurais/patologia , Adolescente , Adulto , Angiofibroma/metabolismo , Criança , Humanos , Masculino , Pessoa de Meia-Idade , Proteínas do Tecido Nervoso/metabolismo , Crista Neural/metabolismo , Células-Tronco Neurais/metabolismo , Adulto Jovem
15.
BMJ Case Rep ; 15(2)2022 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-35131785

RESUMO

Bleeding nasal mass in adolescent boys has customarily been attributed to Juvenile nasopharyngeal angiofibroma. However, little is known regarding the extranasopharyngeal origin of angiofibroma, as highlighted in this case report of a 15-year-old boy who presented with recurrent epistaxis and nasal obstruction. On constructing a working diagnosis of nasal haemangioma, the patient was taken up for endoscopic excision under general anaesthesia. Intraoperative endoscopic findings and histopathological examination revealed the diagnosis of middle turbinate angiofibroma, which is an extremely rare extranasopharyngeal angiofibroma. Consequently, this represents the third described case of a juvenile angiofibroma arising from the middle turbinate.


Assuntos
Angiofibroma , Obstrução Nasal , Neoplasias Nasofaríngeas , Neoplasias Nasais , Adolescente , Angiofibroma/diagnóstico por imagem , Angiofibroma/cirurgia , Humanos , Masculino , Obstrução Nasal/etiologia , Obstrução Nasal/cirurgia , Neoplasias Nasofaríngeas/diagnóstico por imagem , Neoplasias Nasofaríngeas/cirurgia , Neoplasias Nasais/diagnóstico por imagem , Neoplasias Nasais/cirurgia , Conchas Nasais/diagnóstico por imagem , Conchas Nasais/cirurgia
16.
Histol Histopathol ; 37(8): 717-722, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35211945

RESUMO

Angiofibroma of soft tissue (AFST) is a new soft tissue tumor entity described in the 2020 World Health Organization Classification of Soft Tissue and Bone Tumors. It most often arises in the lower extremities of middle-aged adults and pursues a benign clinical course with a low rate of non-destructive local recurrence. Histologically, the lesion consists of uniform bland spindle cells in a fibromyxoid stroma with a prominent vascular network. The vascular component forms a complex arrangement of small, thin-walled branching blood vessels. By immunohistochemistry, AFST is variably positive for epithelial membrane antigen, desmin, smooth muscle actin, CD34, CD68, CD163 and estrogen receptor. The exact etiology of AFST remains unknown, but it appears genetically distinct, with a balanced t(5;8)(p15;q13) translocation resulting in a fusion of aryl hydrocarbon receptor repressor (AHRR) and nuclear receptor coactivator 2 (NCOA2). Knowledge of this recently described entity is important because it can mimic a variety of intermediate and malignant soft tissue tumors, including solitary fibrous tumor, low-grade fibromyxoid sarcoma, myxoid liposarcoma and low-grade myxofibrosarcoma. We review AFST, with an emphasis on the diagnostic spectrum, recent molecular genetic features and the differential diagnosis.


Assuntos
Angiofibroma , Neoplasias de Cabeça e Pescoço , Neoplasias de Tecidos Moles , Actinas , Adulto , Angiofibroma/diagnóstico , Angiofibroma/genética , Angiofibroma/patologia , Desmina , Diagnóstico Diferencial , Humanos , Pessoa de Meia-Idade , Mucina-1 , Coativador 2 de Receptor Nuclear/genética , Coativador 2 de Receptor Nuclear/metabolismo , Receptores de Hidrocarboneto Arílico , Receptores de Estrogênio , Neoplasias de Tecidos Moles/patologia
17.
Photodiagnosis Photodyn Ther ; 37: 102725, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35041984

RESUMO

Facial angiofibromas are one of the dermatological hallmarks of tuberous sclerosis complex. Facial angiofibromas often lead to disfigurement and cosmetic concerns, which has a serious negative effect on the quality of life of the patients. There are no guidelines or consensus on the management of facial angiofibromas up to now. We report a patient with extensive facial angiofibromas treated with the combination of photodynamic therapy and ultrapulse carbon dioxide laser, achieving satisfying results. We suggest this might be a promising therapeutic option for facial angiofibromas in tuberous sclerosis complex.


Assuntos
Angiofibroma , Neoplasias Faciais , Terapia a Laser , Lasers de Gás , Fotoquimioterapia , Esclerose Tuberosa , Angiofibroma/complicações , Angiofibroma/tratamento farmacológico , Angiofibroma/cirurgia , Neoplasias Faciais/complicações , Neoplasias Faciais/tratamento farmacológico , Humanos , Terapia a Laser/métodos , Lasers de Gás/uso terapêutico , Fotoquimioterapia/métodos , Qualidade de Vida , Esclerose Tuberosa/complicações , Esclerose Tuberosa/terapia
18.
Clin Nucl Med ; 47(3): e315-e317, 2022 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-35025780

RESUMO

ABSTRACT: Angiofibroma of soft tissue is a rare benign mesenchymal neoplasm. We reported a case of angiofibroma of soft tissue in a 61-year-old woman. MRI examination indicated a soft tissue mass in the right lower thigh, which showed isosignal intensity on T1-weighted imaging and heterogeneous high signal intensity on T2-weighted imaging. This lesion had intense tracer uptake, with an SUVmax of 7.24 on FDG PET/CT. Our case revealed that when soft tissue masses with intense FDG uptake on PET/CT are encountered, angiofibroma of soft tissue should be considered in the differential diagnosis.


Assuntos
Angiofibroma , Fluordesoxiglucose F18 , Angiofibroma/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Tomografia por Emissão de Pósitrons
19.
Clin Nucl Med ; 47(3): e268-e270, 2022 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-35020647

RESUMO

ABSTRACT: We present the case of a left-sided recurrent juvenile nasopharyngeal angiofibroma (JNA) in a 16-year-old boy who underwent endoscopic excision 2 years back. The preoperative contrast-enhanced MRI and histopathology were in favor of JNA. The follow-up 68Ga-prostate-specific membrane antigen (PSMA) PET/CT scan at 3-month interval revealed complete clearance of disease. Currently, the patient presented with recurrent bleeding and nasal endoscopic examination, and contrast-enhanced MRI revealed a presence of a recurrent tumor. The tumor did not show any uptake on 68Ga-PSMA PET/CT, and postoperative histopathology evaluation confirmed the presence of JNA. Although regarded as a criterion-standard imaging modality for assessment of recurrent or residual JNA, 68Ga-PSMA PET/CT is never 100% specific.


Assuntos
Angiofibroma , Neoplasias Nasofaríngeas , Adolescente , Angiofibroma/diagnóstico por imagem , Isótopos de Gálio , Radioisótopos de Gálio , Humanos , Masculino , Neoplasias Nasofaríngeas/diagnóstico por imagem , Recidiva Local de Neoplasia/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Próstata
20.
Prog Brain Res ; 268(1): 279-302, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35074086

RESUMO

This chapter examines the results of GKNS on a variety of extraparenchymal skull base tumors some benign and some malignant. For the benign tumors there is good evidence on the effectiveness of the method for pretty much all diagnoses. For malignant extraparenchymal tumors the results are more limited and GKNS only has a supportive role in these lesions.


Assuntos
Angiofibroma , Cordoma , Angiofibroma/diagnóstico , Angiofibroma/patologia , Cordoma/diagnóstico , Cordoma/patologia , Humanos
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