Nasal septal angiofibroma, a subclass of extranasopharyngeal angiofibroma




Abstract


Objective


Extranasopharyngeal angiofibromas (ENA) arising from the nasal septum or nasal septal angiofibromas are extremely rare; only 13 such cases have been reported in the international literature. Our objective is to describe the presentation, workup, and surgical management of these lesions.


Study design


Case reports were done.


Setting


The setting was a tertiary care referral center and the Veterans Affairs Medical Center.


Patients, interventions, and results


We present 2 cases of extranasopharyngeal angiofibroma occurring on the nasal septum.


Conclusions


In this report, we discuss the occurrence, the histopathologic findings, and the treatment of nasal septal angiofibroma.



Introduction


Extranasopharyngeal angiofibromas (ENA) arising exclusively from the nasal septum, nasal septal angiofibromas (NSA), are extremely rare with only 13 cases to date . Although the clinical presentation of these tumors differs vastly from the classically described juvenile nasopharyngeal angiofibroma (JNA), the histopathology is similar. A dense fibrous stroma with spindled or stellate cells and variably sized, thin-walled vascular spaces characterize the entity histopathologically. Angiofibroma must be considered in the differential diagnosis of vascular lesions in the sinonasal cavity. In this series, we report 2 cases of angiofibroma arising from the nasal septum, which would represent the 14th and 15th reported in the literature. As a case report, this project was exempt from institutional review board approval.





Case 1


A 60-year-old man was referred to the Division of Otolaryngology at the Veterans Affairs Medical Center for a 2-month history of unilateral, right-sided epistaxis. He had a right septal deviation with rightward septal spur, limiting visualization. He underwent silver nitrate cauterization of an exposed blood vessel on the right anterior septum. This was repeated twice at an outside emergency department for recurring epistaxis.


Upon follow-up, physical examination revealed a right-sided 1-cm lobulated mass on the anterior nasal septum at the site of previous cauterization. It was unclear if this represented granulation tissue, an arteriovenous malformation, or a tumor. A computed tomographic (CT) scan of the nasal sinuses demonstrated a 1-cm anterior mucosal mass that partially penetrated the septum. He was taken to the operating room for examination under anesthesia and surgical management. Under general anesthesia, nasal endoscopy was performed, demonstrating a 1-cm lobulated mass involving the right anterior nasal septum. A wide local excision was performed with electrosurgical cauterization of the stalk at the base of the lesion. Histopathologic examination revealed an angiofibroma. Hematoxylin and eosin stains display the histoarchitecture of the tumor ( Fig. 1 ). Immunohistochemical stains for an endothelial cell marker (CD34) ( Fig. 2 ), smooth muscle actin, and β -catenin ( Fig. 3 ) showed staining, consistent with an angiofibroma. An immunostain for c-kit (CD117) showed staining only of mast cells. The patient had no complications during the surgery. Blood loss was minimal. At 2-year follow-up, the patient was without epistaxis, and the operative site was without evidence of recurrence.




Fig. 1


The angiofibroma (right) abuts normal sinonasal respiratory mucosa (left), supporting an extrapharyngeal location. The tumor is composed of variably sized thin vascular channels (arrows) with intervening fibrous connective tissue and spindled-stellate stromal cells (asterisk) (hematoxylin-eosin stain; original magnification ×40).



Fig. 2


Endothelial cells (arrow) are highlighted by CD34 immunoreactivity within the tumor (CD34 immunoperoxidase stain; original magnification ×40).



Fig. 3


Nuclear and cytoplasmic immunoreactivity for β -catenin within stromal myofibroblasts (arrows) ( β -catenin immunoperoxidase stain; original magnification ×200).





Case 1


A 60-year-old man was referred to the Division of Otolaryngology at the Veterans Affairs Medical Center for a 2-month history of unilateral, right-sided epistaxis. He had a right septal deviation with rightward septal spur, limiting visualization. He underwent silver nitrate cauterization of an exposed blood vessel on the right anterior septum. This was repeated twice at an outside emergency department for recurring epistaxis.


Upon follow-up, physical examination revealed a right-sided 1-cm lobulated mass on the anterior nasal septum at the site of previous cauterization. It was unclear if this represented granulation tissue, an arteriovenous malformation, or a tumor. A computed tomographic (CT) scan of the nasal sinuses demonstrated a 1-cm anterior mucosal mass that partially penetrated the septum. He was taken to the operating room for examination under anesthesia and surgical management. Under general anesthesia, nasal endoscopy was performed, demonstrating a 1-cm lobulated mass involving the right anterior nasal septum. A wide local excision was performed with electrosurgical cauterization of the stalk at the base of the lesion. Histopathologic examination revealed an angiofibroma. Hematoxylin and eosin stains display the histoarchitecture of the tumor ( Fig. 1 ). Immunohistochemical stains for an endothelial cell marker (CD34) ( Fig. 2 ), smooth muscle actin, and β -catenin ( Fig. 3 ) showed staining, consistent with an angiofibroma. An immunostain for c-kit (CD117) showed staining only of mast cells. The patient had no complications during the surgery. Blood loss was minimal. At 2-year follow-up, the patient was without epistaxis, and the operative site was without evidence of recurrence.




Fig. 1


The angiofibroma (right) abuts normal sinonasal respiratory mucosa (left), supporting an extrapharyngeal location. The tumor is composed of variably sized thin vascular channels (arrows) with intervening fibrous connective tissue and spindled-stellate stromal cells (asterisk) (hematoxylin-eosin stain; original magnification ×40).

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Aug 25, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Nasal septal angiofibroma, a subclass of extranasopharyngeal angiofibroma

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