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Ear, Nose, & Throat Journal Oct 2023Juvenile nasopharyngeal angiofibroma is a benign vascular tumor seen predominantly in adolescent males in the second decade of life. Extranasopharyngeal angiofibroma...
Juvenile nasopharyngeal angiofibroma is a benign vascular tumor seen predominantly in adolescent males in the second decade of life. Extranasopharyngeal angiofibroma includes vascular fibrous masses that occur outside the nasopharynx. The diagnosis of an angiofibroma is based on the clinical presentation and imaging, with biopsies being avoided to avoid excessive bleeding. Computed tomography scan is considered sufficient for the diagnosis of extranasopharyngeal angiofibroma as it clearly delineates and identifies the tumor.
Topics: Male; Adolescent; Humans; Nose Neoplasms; Angiofibroma; Nasopharyngeal Neoplasms; Nasal Septum; Nasopharynx; Respiratory Tract Neoplasms
PubMed: 34134540
DOI: 10.1177/01455613211026517 -
Indian Journal of Otolaryngology and... Mar 2017The extranasopharyngeal angiofibroma is a separate clinical entity but those involving infratemporal fossa and cheek resemble juvenile nasopharyngeal angiofibroma (JNA)...
The extranasopharyngeal angiofibroma is a separate clinical entity but those involving infratemporal fossa and cheek resemble juvenile nasopharyngeal angiofibroma (JNA) and hence have been labelled as juvenile perinasal angiofibroma (JPA) in this paper. This paper presents a 7th case of JPA and attempts to review the world literature on JPA, along with a proposal of staging the disease. A 16 year male presented with a painless compressible facial swelling since 7 months without any epistaxis or nasal obstruction. Initially a vascular lesion was suspected but JNA without nasal extension was strongly suspected on imaging. A deep trucut biopsy confirmed the histopathology. The vascular enhancement was significant and the tumour was excised through open approach (Weber Fergusson). JPA that can be regarded as a variant of JNA that fails to extend medially. Imaging demonstrates classical JNA findings with a clear nose/nasopharynx. A deep trucut biopsy under control in inpatient settings may sometimes help. JPA presents most commonly in Stage II where an open facial approach preferably following selective preoperative embolization is indicated. Hence with painless compressible (or non-compressible) cheek swelling suspected to be of a vascular etiology, a high degree of clinical suspicion for JPA needs to maintained in order to prevent a misdiagnosis.
PubMed: 28239582
DOI: 10.1007/s12070-016-1050-9 -
BMJ Case Reports Jul 2020Cellular angiofibroma is a rare type of benign mesenchymal tumour that arises mostly in middle-aged women. It needs to be distinguished from other neoplasms and has a...
Cellular angiofibroma is a rare type of benign mesenchymal tumour that arises mostly in middle-aged women. It needs to be distinguished from other neoplasms and has a predilection for the vulvovaginal region. To our knowledge, this is the first case of a cervical cellular angiofibroma. A 34-year-old nulligravid woman was referred with a large mass bulging in the fornix posterior. Ultrasound scanning and MRI showed a large solid mass projecting in the pouch of Douglas. Laparoscopic surgical excision was performed. Histopathological examination showed a well-demarcated, unencapsulated tumour, consisting of short fascicles of spindle cells in-between thick-walled medium-sized vessels. On immunohistochemistry, there was strong reactivity with antibodies against CD34 and oestrogen receptor. Angiofibromas are benign mesenchymal tumours mostly occurring in middle-aged women. They can cause abnormal swelling and uterine bleeding and need to be distinguished from other (malignant) neoplasms.
Topics: Adult; Angiofibroma; Cervix Uteri; Female; Humans; Uterine Cervical Neoplasms; Vagina
PubMed: 32675122
DOI: 10.1136/bcr-2020-235241 -
Journal of Neurological Surgery. Part... Feb 2018Juvenile angiofibroma (JA) is a benign, highly vascular tumor which is diagnosed on the basis of clinical and imaging features. It has a characteristic pattern of spread... (Review)
Review
Juvenile angiofibroma (JA) is a benign, highly vascular tumor which is diagnosed on the basis of clinical and imaging features. It has a characteristic pattern of spread commonly involving the pterygopalatine fossa and pterygoid base. The mainstay of treatment is surgery, while radiotherapy is rarely used for the treatment of recurrent lesion. Endoscopic endonasal surgery is currently the treatment of choice for small to intermediate size JAs, and is feasible even for advanced lesions; however, this should only be practiced in well-experienced centers.
PubMed: 29404237
DOI: 10.1055/s-0037-1615810 -
BMJ Case Reports Mar 2022
Topics: Angiofibroma; Endoscopy; Humans; Nasopharyngeal Neoplasms
PubMed: 35260405
DOI: 10.1136/bcr-2021-248023 -
Frontiers in Endocrinology 2022Multiple endocrine neoplasia type 1 (MEN1), an autosomal-dominantly inherited tumor syndrome, is classically defined by tumors arising from the "3 Ps": Parathyroids,... (Review)
Review
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 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.
Topics: Humans; Multiple Endocrine Neoplasia Type 1; Prospective Studies; Angiofibroma; Islets of Langerhans; Multicenter Studies as Topic
PubMed: 36325452
DOI: 10.3389/fendo.2022.1029041 -
Ear, Nose, & Throat Journal Jun 2015Staging of tumors is very important in treatment and surgical decision making, as well as in predicting disease recurrence and prognosis. This review focuses on the... (Review)
Review
Staging of tumors is very important in treatment and surgical decision making, as well as in predicting disease recurrence and prognosis. This review focuses on the different available classifications of juvenile nasopharyngeal angiofibroma (JNA) and their impact on the evaluation, management, and prognosis of JNA. The literature was reviewed, and publications on JNA staging were examined. Our MEDLINE search of the entire English-language literature found no review article on the current available staging systems for JNA. In this article, we review the common JNA classification systems that have been published, and we discuss some of their advantages and disadvantages. The most commonly used staging systems for JNA are the Radkowski and the Andrews-Fisch staging systems. However, some newer staging systems that are based on advances in technology and surgical approaches-the Onerci, INCan, and UPMC systems-have shown promising utility, and they will probably gain popularity in the future.
Topics: Angiofibroma; Child; Humans; Nasopharyngeal Neoplasms; Neoplasm Staging
PubMed: 26053985
DOI: 10.1177/014556131509400615 -
Cancer Imaging : the Official... May 2012More than 70 benign and malignant sinonasal tumours and tumour-like conditions have been described. However, sinonasal tumours are rare, and sinonasal cancers comprise... (Review)
Review
More than 70 benign and malignant sinonasal tumours and tumour-like conditions have been described. However, sinonasal tumours are rare, and sinonasal cancers comprise only 3% of all head and neck cancers and 1% of all malignancies, with a peak incidence in the 5th to 7th decades and with a male preponderance. The early symptoms and imaging findings of sinonasal tumours are similar to rhinosinusitis with runny and stuffy nose, lacrimation and epistaxis and therefore neglected both by the patients and doctors. When late symptoms such as anosmia, visual disturbances, cranial neuropathy (Cn II, IV, V, VI) or facial swelling appear, the patient is referred to sinonasal endoscopy or imaging. At the time of correct diagnosis more than half of the tumours have reached an advanced stage with a poor prognostic outcome. Even if imaging is performed in the early stages, a radiologist inexperienced with sinonasal anatomy and tumour features may easily interpret early signs of a malignant tumour as rhinosinusitis or a lesion that does not require follow-up. This article presents the imaging findings in some of the most common benign and malignant sinonasal tumours, and the TNM classification and staging of sinonasal carcinomas.
Topics: Angiofibroma; Carcinoma; Delayed Diagnosis; Diagnosis, Differential; Endoscopy; Humans; Lymphoma, Non-Hodgkin; Magnetic Resonance Imaging; Melanoma; Multimodal Imaging; Neoplasm Staging; Neuroendocrine Tumors; Nose Neoplasms; Osteoma; Papilloma, Inverted; Paranasal Sinus Neoplasms; Positron-Emission Tomography; Rhinitis; Sarcoma; Sinusitis; Tomography, X-Ray Computed
PubMed: 22571851
DOI: 10.1102/1470-7330.2012.0015