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Juvenile Nasopharyngeal Angiofibroma

last modified on: Mon, 04/05/2021 - 14:59

Juvenile Nasopharyngeal Angiofibroma

(Evaluation and Work-Up)

Return to: Paranasal Sinus Surgery Protocols ;

See also: Juvenile Nasopharyngeal Angiofibroma (JNA) Anatomy: the Pterygopalatine Fossa

See also: Juvenile Angiofibroma - Rads

See also (with virtual slide): Angiofibroma (pathology)

  1. GENERAL
    1. Overview

      1. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior nasopharynx.
      2. It occurs almost exclusively in adolescent males, with an average age at diagnosis of 15 years old.
      3. Highly vascular tumor
      4. Locally destructive, spreading submucosally, but rarely invasive
    2. Origin
      1. The internal maxillary artery is the most common vascular source, other vessels include the ascending pharyngeal artery and external, internal, and common carotid arteries.
      2. Origination is usually in the posterolateral nasal wall at the sphenopalatine foramen, other sources cite origination at the vidian canal.
    3. Anatomy, see Juvenile Nasopharyngeal Angiofibroma (JNA) Anatomy: the Pterygopalatine Fossa
    4. Extension and Spread
      1. Medial: into the nasopharynx and nasal cavity
      2. Lateral: the sphenopalatine fossa opens into the pterygopalatine fossa laterally, advancing into the infratemporal fossa
      3. Superior: expansion into the sphenoid sinus.
      4. Intracranial extension in 20-36%. Superior spread through the pterygoid process may extend into the middle cranial fossa. As the lateral and posterior walls of the sphenoid sinus are eroded, the cavernous sinus (which contains CN III, IV, V1, V2, VI, and the internal carotid artery) or pituitary and sella turcica may be involved.
    5. Histology
      1. Myofibroblast cells surrounded by a fibrous pseudocapsule
      2. The angiofibroma consists of a collagenous tissue network containing many vascular channels of abundant endothelial cells.
      3. These vessels lack a true muscular layer. This hallmark characteristic predisposes the tumor to hemorrhage because of its ability to vasoconstrict
      4. See also virtual slide at Angiofibroma

        JNA - low power

         
         
  2. CLINICAL EVALUATION
    1. Clinical Observations
      1. Nasal obstruction unilateral or bilateral
      2. Rhinorrhea
      3. Epistaxis, usually only mild and episodic
      4. Facial asymmetry
      5. Proptosis
      6. Blindness
      7. Epiphora
      8. Mental status changes
    2. Endo-Nasal Examination
      1. Rhinorrhea
      2. Pale or vascular-appearing mass in posterior nasal cavity
      3. Submucosal mass may also be visible in nasopharynx or produce asymmetry in the nose or lateral buccal area.
    3. Radiological Studies, see Juvenile Angiofibroma - Rads
      1. CT scan axial and coronal, with and without contrast, bone and soft tissue windows; identifies the extent of bone destruction.
      2. MRI multi-planer, T2 and T1 with and without gadolinium; identifies tumor relationship to adjacent soft tissue structures, including the orbits, optic nerves, pterygomaxillary space, buccal and masticator space, carotid arteries, and anterior cranial fossa.
        1. Holman-Miller sign: anterior bowing of the maxillary sinus wall
      3. Angiography with embolization
        1. Should be planned for 24-72 hrs prior to resection
        2. Aid in surgical planning
        3. Decrease intraoperative blood loss
        4. Possibly decrease tumor size
        5. Complications:
          1. Potential for CVA if carotid is affected
          2. Blindness of ophthalmic artery is embolized
          3. Necrosis of skin and soft tissue 

Biopsy: 

  • Should be avoided unless there is clinical evidence that would question the diagnosis of JNA. If a biopsy is done, it should be delayed until the completion of the CT scan and MRI and should only be done in the operating room where potential extensive bleeding can be controlled.

Classification:

  • There have been many modifications to angiofibroma staging since the first in 1981 and none are universally accepted, however, the Radkowski JNA Classification System is the most recent and focuses on the tendency of the tumor to extend posteriorly and on the degree of skull base extendion (Radkowski 1996).
     

     STAGE
    FEATURES 
     IA Limited to nose or nasopharynx
     IB Extension into at least one paranasal sinus
     IIA

    Minimal extension into sphenopalatine foramen,

    Includes minimal part of medial pterygomaxillary fossa

     IIB

    Full occupation of pterygomaxillary fossa with Holman-Miller sign,

    Lateral or anterior displacement of maxillary artery branches,

    May have superior extension with orbital bone erosion

     IIC Extension through pterygomaxillary fossa into cheek, temporal fossa, or posterior to pterygoids
     IIIA Skull base erosion with minimal intracranial extension
     IIIB Skull base erosion with extensive intracranial extension +/- cavernous sinus
     
  1. TREATMENT
    1. Surgical, with preoperative embolization 
      1. Gold standard
      2. Approaches
        1. Endoscopic transnasal
          1. Middle turbinectomy may be performed for improved exposure
          2. Middle meatus antrostomy
          3. Resection of posterior maxillary wall
          4. Sphenopalatine artery ligation
          5. Tumor resection from pterygopalatine fossa
        2. Transpalatal
          1. Soft palate is split and retracted
          2. Hard palate resection for increased exposure
          3. Palatine bone and interior aspect of pterygoid plate resected
        3. Denker approach
          1. Wide anterior antrostomy
          2. Removal of ascending process of maxilla
          3. Removal of inferior half of lateral nasal wall
        4. Facial translocation
          1. Midface Degloving with Masillary Osteotomies
            1. Gingivobuccal incision
            2. Nasal intercartilaginous incisions with transfixion incision
            3. Soft tissue elevation
            4. Le Fort I Osteotomy
        5. Medial maxillectomy
        6. Infratemporal fossa with or without craniotomy
        7. Alternative approaches
          1. Lateral rhinotomy
          2. Weber-Ferguson incision
          3. Weber-Ferguson with Lynch extension
          4. Weber-Ferguson with lateral subciliary extension
          5. Weber-Ferguson with subciliary extension and supraciliary extension
      3. Surgical Planning (Hossenini 2005)
        1.  Smaller tumors (Class IA, IB, IIA, IIB, IIC)
          1. Trans-nasal
          2. Transpalatal
          3. Transantral, for lesions extending laterally up to pterygopalatine fossa
        2. Larger tumors (IIIA, IIIB)
          1. Lateral rhinotomy
          2. Midface degloving
        3. More extensive resections have higher associated morbidity, however, limited resections have a higher rate of recurrence 
    2. Radiation Therapy
      1. Radiation therapy has been shown to decrease or eliminate tumor growth.
      2. May be useful in treating large, aggressive, or multiple recurrent tumors in the postoperative period.
    3. Chemotherapy
      1. If recurrent after resection and/or radiation
    4. Hormone Therapy
      1. Estrogen, progesterone, and androgen receptors have been found in some JNAs
      2. Estrogens and antiandrogens used to decrease tumor size/vascularity
      3. No advantage to treatment with Flutamide, a non-steroidal antiandrogen (Labra 2004)
      4. Poorly tolerated in adolescence 
  2. FOLLOW-UP
    Follow-up CT scan or MRI in 3 months and 6 months, for 3 years, then yearly if no signs of recurrence
    1. Recurrence rate of 27% in retrospective chart review of 37 patients with surgical intervention (Hosseini 2005)
  3. SUGGESTED READING
    Chandler JR, Goulding R, Moskowitz L, Quencer RM. Nasopharyngeal angiofibromas: staging and management. Ann Otol Rhinol Laryngol. 1984;93:322-329.
    Andrade NA, Pinto JA, Nóbrega Mde O, et al. Exclusively endoscopic surgery for juvenile nasopharyngeal angiofibroma. Otolaryngol Head Neck Surg 2007; 137(3):492-496.
    Herman P, Lot G, Chapot R, et al. Long-term followup of juvenile nasopharyngeal angiofibromas: analysis of recurrences. Laryngoscope 1999; 109(1):140–147.
    Liu L, Wang R, Huang D, et al. Analysis of intra-operative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Clin Otolaryngol Allied Sci 2002; 27(6):536-40.
    Wiatrak BJ, Koopmann CF, Turrisi AT. Radiation therapy as an alternative to surgery in the management of intracranial juvenile nasopharyngeal angiofibroma. Int J Pediatr Otorhinolaryngol 1993; 28(1):51-61.
    Radkowski D, McGill T, Healy GB, et al. Angiofibroma. Arch Otolaryngol Head Neck Surg 1996; 122(2):122-129.
    Hosseini SM, Borghei P, Borghei SH, et al. Angiofibroma: an outcome review of conventional surgical approaches. Eur Arch Otorhinolaryngol 2005; 262(10):807-812.
    Wormald PJ, Van Hasselt A. Endoscopic removal of juvenile angiofibromas. Otolaryngol Head Neck Surg 2003; 129(6):684-691.
    Yiotakis I, Eleftheriadou A, Davilis D, et al. Juvenile nasopharyngeal angiofibroma stages I and II: a comparative study
    Labra A, Chavolla-Magaña R, Lopez-Ugalde A, et al. Flutamide as a preoperative treatment in juvenile angiofibroma (JA) with intracranial invasion: report of 7 cases. Otolaryngol Head Neck Surg 2004; 130(4):466-469.