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Juvenile nasopharyngeal angiofibroma

Last updated: February 28, 2019

Summary

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. JNAs originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. As the tumor is largely space-occupying and highly vascular, patients typically present with a progressive, unilateral nasal obstruction, and recurrent, severe epistaxis. Other symptoms include rhinorrhea, anosmia, and facial swelling (e.g., exophthalmos). The diagnosis is based on the clinical findings and confirmed through cranial CT scans. Surgical excision is the treatment of choice, since JNAs have a high recurrence rate. Radiation (stereotactic gamma knife) is reserved for recurrent cases or JNAs with intracranial extension.

Definition

  • Juvenile nasopharyngeal angiofibroma is a rare, benign, but locally aggressive tumor of the nasopharynx. It is composed of vascular and fibrous tissue.

Epidemiology

  • Incidence: rare, accounts for 0.05% of all head and neck tumors
  • Sex: occurs exclusively in males
  • Age: 10–20 years

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiology

  • JNAs originate from a hamartomatous nidus in the roof of the nasopharynx
  • JNAs grow rapidly, extending into and distorting adjacent structures (e.g., nasopharynx and nasal cavity, paranasal sinuses, orbit, cranial cavity )
  • The tumor expresses androgen receptors (testosterone dependent); hence, JNAs are seen exclusively in males, especially around puberty, when testosterone levels begin to peak.
  • JNAs have vascular and fibrous components
  • The vessels are devoid of a muscular layer → severe epistaxis

References:[1]

Clinical features

References:[1]

Diagnostics

References:[1][2]

Treatment

  • Surgical excision of the tumor
    • Surgical approach depends on size and extent of the tumor (e.g., transmaxillary, transnasal)
    • Preoperative embolisation of feeding vessels necessary to reduce intra-operative blood loss
  • Stereotactic radiatiotherapy (e.g., gamma knife)
    • Reduces tumor vascularization and size
    • Indicated in recurrent cases or evidence of intracranial extension
    • Risk of damage to adjacent structures (e.g., eye, brain, spinal cord) is minimised compared to external beam radiotherapy.
  • Hormone therapy with flutamide

References:[1]

Prognosis

  • JNA is known to recur after surgery.
  • Most recurrences occur within four years after surgery and hence, annual/biannual follow-up is required for at least five years.

References:[3]

References

  1. Tewfik TL. Juvenile Nasopharyngeal Angiofibroma. Juvenile Nasopharyngeal Angiofibroma. New York, NY: WebMD. http://emedicine.medscape.com/article/872580-overview#showall. Updated: January 12, 2016. Accessed: February 16, 2017.
  2. Juvenile nasopharyngeal angiofibroma. https://radiopaedia.org/articles/juvenile-nasopharyngeal-angiofibroma. Updated: February 16, 2017. Accessed: February 16, 2017.
  3. Park CK, Kim DG, Paek SH, Chung HT, Jung HW. Recurrent juvenile nasopharyngeal angiofibroma treated with gamma knife surgery. J Korean Med Sci. 2006; 21 (4): p.773-777. doi: 10.3346/jkms.2006.21.4.773 . | Open in Read by QxMD