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Juvenile Angiofibroma


Introduction

Juvenile angiofibroma (JNA) is a rare, benign tumor that is found almost exclusively in adolescent males. The pathogenesis of JNA remains undefined, although hormonal and genetic factors have been implicated. JNA arises in the lateral nasopharynx near the sphenopalatine foramen and can spread in multiple directions to the nasal cavity, oropharynx, paranasal sinuses, orbit (via inferior orbital fissure), and infratemporal fossa (via pterygomaxillary fissure). Intracranial extension occurs through the skull base foramina and by the erosion of bone. Early symptoms include unilateral nasal obstruction and recurrent unilateral epistaxis. Large tumors can displace normal structures, resulting in facial swelling, proptosis, diplopia, visual loss, and facial hypesthesia. The primary treatment is surgical excision, with endoscopic surgery replacing open approaches for most tumors. The greatest challenge in surgery is bleeding caused by the hypervascular nature of JNA. Treatment options for recurrent tumor include surgery or radiation therapy (RT).

Key Operative Learning Points

  • Unilateral nasal obstruction and recurrent epistaxis in an adolescent male is suggestive of angiofibroma.

  • Diagnosis can be established radiographically without biopsy in most cases.

  • Staging and surgical planning should consider residual vascularity following embolization.

  • Preoperative embolization of the tumor’s blood supply reduces intraoperative bleeding.

  • The base of the pterygoid plate is a frequent site of recurrence and should be drilled to remove all remnants of the tumor.

  • An anterior endoscopic approach can be augmented with a transmaxillary approach.

  • A lateral infratemporal approach can be combined with an anterior approach to provide access to the middle cranial fossa.

  • Large tumors should be divided into vascular segments and may require multiple staged surgeries.

  • Residual tumor on follow-up scans should be observed for growth; not all residual tumors require additional treatment.

Preoperative Period

History

  • Nasal obstruction (unilateral or bilateral)

  • Recurrent epistaxis

  • Facial swelling

  • Diplopia

  • Numbness of the face (V2 distribution)

  • Pain in the face/sinus pressure

  • Hearing loss.

Physical Examination

  • Nasal endoscopy: reddish, smooth lobulated mass occupying the posterior nasal cavity

  • Swelling of the cheek or temporal area

  • Proptosis or displacement of the globe

  • Middle ear effusion with conductive hearing loss

  • Hypesthesia of the maxillary division of the trigeminal nerve.

Imaging

  • Computed tomography (CT) with contrast ( Fig. 122.1 )

    Fig. 122.1, Computed tomography (CT) scan of juvenile angiofibroma (JNA) (UPMC stage V) demonstrates bony erosion with extension of tumor to the orbits and cranial cavity. CT is superior for the visualization of bone and for estimating the vascularity of the tumor.

    CT is the preferred initial imaging modality since it demonstrates the vascular nature of the tumor and the bony architecture of the sinuses, orbit, and skull base. A characteristic feature of JNA is widening of the pterygopalatine space with remodeling of the posterior wall of the maxillary sinus. For surgical planning, a CT angiogram is obtained (skull base protocol with navigation protocol) for demonstration of the carotid artery and bony landmarks.

  • Magnetic resonance imaging (MRI) ( Fig. 122.2 )

    Fig. 122.2, Magnetic resonance imaging (MRI) of juvenile angiofibroma (JNA) (UPMC stage V) is superior for the interface of the tumor with soft tissue planes. The internal carotid arteries (arrows) and tumor vasculature appear as flow voids.

    MRI shows the interface of tumor with soft tissues (orbit, masticator space, dura) when there is bony erosion or extension of the tumor through the foramina. It can also differentiate the tumor from obstruction of a sinus with retained secretions. Without MRI, CT can be misleading, suggesting an aggressive neoplasm, such as a sarcoma.

  • Angiography

    Angiography is not necessary in most cases but helps to confirm the diagnosis (characteristic hypervascularity of JNA) and demonstrates the blood supply of the tumor. The blood supply is usually derived from branches of the external carotid artery (internal maxillary and ascending pharyngeal arteries) and is often bilateral in larger tumors. Advanced tumors also receive blood supply from the internal carotid artery (ICA) via the vidian artery and cavernous branches ( Fig. 122.3 ). For advanced tumors with skull base involvement and proximity to the ICA, angiography is important to assess collateral intracranial circulation (competency of the circle of Willis).

    Fig. 122.3, Angiography demonstrates a large amount of residual vascularity from the internal carotid artery following embolization of the internal maxillary artery (see coils in proximal trunk).

Indications

  • Surgery is the treatment of choice for all JNA, even advanced tumors with intracranial extension.

Contraindications

  • Residual or recurrent tumor that is not growing can be observed.

  • A prohibition against blood products (Jehovah’s Witness) is a relative contraindication for an advanced tumor with residual vascularity following embolization.

  • Medical condition that precludes surgery.

Preoperative Preparation

  • Biopsy

    Histologic confirmation is not necessary unless there are atypical radiological features suggestive of malignancy (sarcoma). If a biopsy is necessary, it should be performed under general anesthesia with control of the airway.

  • Embolization of the tumor

    Bilateral angiography is performed the day before surgery to identify and embolize the blood supply to the tumor. We prefer to use Onyx due to its superior penetration of tissues. Coils are placed in the proximal internal maxillary arteries. For small tumors, an alternative to embolization is surgical ligation of the internal maxillary artery lateral to the tumor margin at the beginning of the operation.

  • Staging

    A variety of staging systems have been proposed over the years for JNA. Only one staging system considers the vascularity of the tumor (University of Pittsburgh Medical Center (UPMC) staging system). There are five stages based on tumor extent and residual vascularity following the embolization of blood supply from the external carotid artery ( Table 122.1 ). Bleeding from the tumor is the greatest risk factor for complications during surgery, and the UPMC staging system provides superior prediction of intraoperative blood loss and risk of residual/recurrent tumor.

    TABLE 122.1
    UPMC Staging System for Juvenile Nasopharyngeal Angiofibroma
    Stage UPMC Staging System
    I Nasal cavity, medial pterygopalatine fossa
    II Paranasal sinuses, lateral pterygopalatine fossa
    No residual vascularity
    III Skull base erosion, orbit, infratemporal fossa
    No residual vascularity
    IV Skull base erosion, orbit, infratemporal fossa
    Residual vascularity
    V Intracranial extension, residual vascularity
    M: medial extension
    L: lateral extension

  • Balloon test occlusion of ICA

    If there is tumor encasement of the ICA, assessment of the adequacy of collateral circulation can be done with balloon test occlusion with neurological monitoring. This knowledge can guide the management of the patient in case there is injury to the ICA during surgery. Fortunately, most young patients can tolerate sacrifice of the ICA if necessary.

  • Perioperative blood loss

    Despite adequate embolization, packed red blood cells should be available for transfusion. If desired, directed donors can be used for homologous transfusions. In older patients, surgery can be delayed for several weeks to allow banking of autologous blood for transfusion. When a large amount of blood loss is anticipated, a cell-saver device may be used to recycle the patient’s own blood collected during surgery.

Operative Period

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