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Nasopharyngeal Angiofibroma

Synonyms: Juvenile Angiofibroma

Definition

Benign mesenchymal tumor mainly of young men, composed of an intricate mixture of blood vessels and fibrous stroma, arising from the posterolateral wall of the roof of the nose.

Clinical Features

  • Almost exclusively males aged 10–25 years1
  • Sometimes associated with familiar colonic polyposis2
  • Can grow to occlude involved nares completely
  • May protrude below free edge of soft palate, extend into antrum, and grow:
    • to external orifice of nares
    • posteriorly into nasopharynx
    • into orbit and cranial cavity1,3

Gross Pathology

Histopathology

  • Intricate mixture of:
    • blood vessels
    • fibrous stroma (Fig. 2
      Nasopharyngeal angiofibroma. The dense fibrous quality of the stroma and numerous thin-walled vessels are characteristic.

      Fig. 2: Nasopharyngeal angiofibroma. The dense fibrous quality of the stroma and numerous thin-walled vessels are characteristic.

      ):
      • varies from loose and edematous, with stellate fibroblasts and numerous mast cells, to
      • dense, acellular, and highly collagenized:
        • particularly striking and diagnostic
  • Vessels:
    • range from capillary size to venous size
    • larger vessels at base:
      • may have irregular or incomplete smooth muscle coat, but lack elastic fibers
    • smaller capillary-like vessels with plump endothelial cells common at ‘growing edge'4,5
  • Ultrastructurally:

Special Stains and Immunohistochemistry

  • Androgen receptors:
    • stromal and endothelial nuclear immunostaining in 75% of cases
  • Very few positive for progesterone receptors
  • None positive for estrogen receptors7
  • Contains protein basic fibroblast growth factor (bFGF)8
  • Strong nuclear expression of β-catenin9

Other Investigations

  • CT scan and MRI:
    • to determine confines
    • largely replaced use of selective carotid arteriograms

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Nasopharyngeal Angiofibroma

(View full diagnosis)


  • Capillary hemangiomas:
    • in general, in hemangiomas:
      • less fibrous tissue
      • often some lobulation
      • vessels do not have ‘erectile tissue’ appearance so characteristic of nasopharyngeal angiofibroma
    • location important:
      • question diagnosis of angiofibroma if:
        • not in area described
        • limited to nasal cavity

References

1 Hicks JL, Nelson JF. Juvenile nasopharyngeal angiofibroma. Oral Surg Oral Med Oral Pathol. 1973;35:807–817.

2 Guertl B, Beham A, Zechner R, Stammberger H, Hoefler G. Nasopharyngeal angiofibroma: an APC-gene-associated tumor?. Hum Pathol. 2000;31:1411–1413.

3 Harma RA. Nasopharyngeal angiofibroma. Acta Otolaryngol (Stockholm). 1958;146(Suppl):1–74.

4 Beham A, Beham-Schmid C, Regauer S, Aubock L, Stammberger H. Nasopharyngeal angiofibroma: true neoplasm or vascular malformation?. Adv Anat Pathol. 2000;7:36–46.

5 Sternberg SS. Pathology of juvenile nasopharyngeal angiofibroma. A lesion of adolescent males. Cancer. 1954;7:15–28.

6 Topilko A, Zakrewski A, Pichard E, Viron A. Ultrastructural cytochemistry of intranuclear dense granules in nasopharyngeal angiofibroma. Ultrastruct Pathol. 1984;6:221–228.

7 Hwang HC, Mills SE, Patterson K, Gown AM. Expression of androgen receptors in nasopharyngeal angiofibroma: an immunohistochemical study of 24 cases. Mod Pathol. 1998;11:1122–1126.

8 Schiff M, Gonzalez AM, Ong M, Baird A. Juvenile nasopharyngeal angiofibroma contains an angiogenic growth factor. Basic FGF. Laryngoscope. 1992;102:940–945.

9 Zhang PJ, Weber R, Liang H-H, Pasha TL, LiVolsi VA. Growth factors and receptors in juvenile nasopharyngeal angiofibroma and nasal polyps. An immunohistochemcial study. Arch Pathol Lab Med. 2003;127:1480–1484.

10 Deschler DG, Kaplan MJ, Boles R. Treatment of large juvenile nasopharyngeal angiofibroma. Otolaryngol Head Neck Surg. 1992;106:278–284.

11 Kasper ME, Parsons JT, Mancuso AA, Mendenhall WM, Stringer SP, Cassisi NJ, et al. Radiation therapy for juvenile angiofibroma. Evaluation by CT and MRI, analysis of tumor regression, and selection of patients. Int J Radiat Oncol Biol Phys. 1993;25:689–694.

12 Neel HB, Whicker JH, Devine KD, Weiland LH. Juvenile angiofibroma. Review of 120 cases. Am J Surg. 1973;126:547–556.

13 Waldman SR, Levine HL, Astor F, Wood BG, Weinstein M, Tucker HM. Surgical experience with nasopharyngeal angiofibroma. Arch Otolaryngol. 1981;107:677–682.

14 Howard DJ, Lloyd G, Lund V. Recurrence and its avoidance in juvenile angiofibroma. Laryngoscope. 2001;111(9):1509–1511.

15 Gullane PJ, Davidson J, O'Dwyer T, Forte V. Juvenile angiofibroma. A review of the literature and a case series report. Laryngoscope. 1992;102:928–933.

16 Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM. Diagnosis and treatment of juvenile nasopharyngeal angiofibroma. Eur Arch Otorhinolaryngol. 2001;258(3):120–124.

17 Fields JN, Halverson KJ, Devineni VR, Simpson JR, Perez CA. Juvenile nasopharyngeal angiofibroma. Efficacy of radiation therapy. Radiology. 1990;176:263–265.

18 Goepfert H, Cangir A, Lee Y-Y. Chemotherapy for aggressive juvenile nasopharyngeal angiofibroma. Arch Otolaryngol. 1985;111:285–289.

19 Chen KTK, Bauer FW. Sarcomatous transformation of nasopharyngeal angiofibroma. Cancer. 1982;49:369–371.

20 Spagnolo DV, Papadimitriou JM, Archer M. Postirradiation malignant fibrous histiocytoma arising in juvenile nasopharyngeal angiofibroma and producing alpha-1 antitrypsin. Histopathology. 1984;8:339–352.

21 Dohar JE, Duvall AJ. Spontaneous regression of juvenile nasopharyngeal angiofibroma. Ann Otol Rhinol Laryngol. 1992;101:469–471.

22 McCombe A, Lund VJ, Howard DJ. Recurrence in juvenile angiofibroma. Rhinology. 1990;28:97–102.

23 McGavran MH, Sessions DG, Dorfman RD, Davis DO, Ogura JH. Nasopharyngeal angiofibroma. Arch Otolaryngol. 1969;90:94–104.

Last updated: 5 Mar 2006

Nasopharyngeal Angiofibroma

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