Skip to main content
SpringerLink
Log in

Paraganglioma of the nasal cavity: a case report

  • Rhinology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

We describe the case of a 72-year-old woman presenting with a 1-year history of recurrent epistaxis and unilateral progressive nasal obstruction with associated rhinolalia resulting from the presence of a tumor mass occupying two-thirds of the right nasal cavity. Histopathologically, neoplastic cells or "chief cells" were arranged in well-defined nests, which had the classic alveolar or so-called "zellballen" pattern. Immunohistochemical studies highlighted the presence of S-100 protein-positive sustentacular cells located at the periphery of the clusters of chief cells. The chief cells showed a diffuse and intense positivity for neuron-specific enolase and synaptophysin. A diagnosis of paraganglioma was made. The lesion was excised completely and the patient did not develop recurrences or distant metastases after 8 months of follow-up. Paragangliomas arising in the nasal cavity and paranasal sinuses are extremely rare tumors. We report on the clinical, histopathological and immunohistochemical findings of our case and review the cases previously described in the literature.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1.
Fig. 2.
Fig. 3.
Fig. 4.
Fig. 5.

Similar content being viewed by others

References

  1. Fries JG, Chamberlin JA (1968) Extra-adrenal pheochromocytoma: literature review and report of a cervical pheochromocytoma. Surgery 63: 268–279

    Google Scholar 

  2. Glenner GG, Grimley PM (1974) Tumors of the extra-adrenal paraganglion system (including chemoreceptors). In: Armed Forces Institute of Pathology, Atlas of tumor pathology, 2nd series, fascicle 9. Washington, DC

  3. Parisier SC, Sinclair GM (1968) Glomus tumor of the nasal cavity. Laryngoscope 78: 2013–2024

    CAS  PubMed  Google Scholar 

  4. Talbot AR (1990) Paraganglioma of the maxillary sinus. J Laryngol Otol 104: 248–251

    CAS  PubMed  Google Scholar 

  5. House JM, Goodman MI, Gacek RR, Green GL (1972) Chemodectomas of the nasopharynx. Arch Otolaryngol 96: 138–141

    CAS  PubMed  Google Scholar 

  6. Mulligan RM (1950) Chemodectoma in the dog. Am J Pathol 26: 680–681

    Google Scholar 

  7. Lattes R (1950) Nonchromaffin paraganglioma of ganglion nodosum, carotid body and aortic arch bodies. Cancer 3: 667–694

    Google Scholar 

  8. Mulligan RM (1951) Syllabus of human neoplasm. Lea and Febiger, Philadelphia, p 98

  9. Melone CR, Tucci J, Canary JJ (1966) Cushing's syndrome due to bilateral adrenocortical hyperplasia caused by a benign adrenal medullary tumor. J Clin Endocrinol Metabol 26: 1192–1200

    Google Scholar 

  10. Levit SA, Shep SO, Espinosa RE, Remine WH, Harrison EG (1969) Catecholamine secreting paraganglioma of glomus-jugulare region resembling pheochromocytoma. N Engl J Med 281: 805–811

    CAS  PubMed  Google Scholar 

  11. De Lellis RA, Roth JA (1971) Norepinephrine in a glomus jugulare tumor. Arch Pathol 92: 73–75

    PubMed  Google Scholar 

  12. Schteingart DE, Conn JW, Orth DN, Harrison TS, Fox JE, Bookstein JJ (1972) Secretion of ACTH and β-MSH by an adrenal medullary paraganglioma. J Clin Endocrinol Metabol 34: 676–683

    CAS  Google Scholar 

  13. Zak FG, Lawson W (1982) The paraganglionic chemoreceptor system. Springer, New York, pp 276–285

  14. Apple D, Kreines K (1982) Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma. Am J Med Sci 283: 32–35

    CAS  PubMed  Google Scholar 

  15. Harkins WB (1957) Nonchromaffin paraganglioma of the nasal sinuses. Laryngoscope 67: 246–259

    Google Scholar 

  16. Rosenwasser H (1958) Metastasis from glomus jugulare tumors. Discussion of nomenclature and therapy. Arch Otolaryngol 67: 197–203

    CAS  Google Scholar 

  17. Moran TE (1962) Nonchromaffin paraganglioma of the nasal cavity. Laryngoscope 72: 201–206

    CAS  Google Scholar 

  18. Bolkov YN, Schechkin VN (1976) Chemodectoma of the nose and of the accessory sinuses. Vestn Otorhinolaringol 4: 56–59

    Google Scholar 

  19. Lack EE, Cubilla AL, Woodruff JM, Farr HW (1977) Paragangliomas of the head and neck region. A clinical study of 69 patients. Cancer 39: 397–409

    CAS  PubMed  Google Scholar 

  20. Gosavi DK, Mohidekar AT (1978) Chemodectoma of the nose and sphenoid sinus. J Laryngol Otol 92: 813–816

    CAS  PubMed  Google Scholar 

  21. Himelfarb MZ, Ostrzega NL, Samuel J, Shanon E (1983) Paraganglioma of the nasal cavity. Laryngoscope 93: 350–352

    CAS  PubMed  Google Scholar 

  22. Straehler-Pohl HJ (1985) Paraganglioma of the nasal cavity. Laryngol Rhinol Otol 64: 399–402

    CAS  Google Scholar 

  23. Ueda N, Yoshida A, Fukunishi R, Fujita H, Yanagihara N (1985) Nonchromaffin paraganglioma in the nose and paranasal sinuses. Acta Pathol Jpn 35: 489–495

    CAS  PubMed  Google Scholar 

  24. Betkowski A, Pogorzelski A, Krymski W, Sanakiewicz Z, Garska W (1987) Chemodectoma jamy nosowej. Otolaryngol Pol 41: 169–172

    CAS  PubMed  Google Scholar 

  25. Urquiza R, Vicioso L, Cantera T, Gallardo A, Gonzalez M (1988) Quemodectoma de la fosa nasal y el seno etmoidal. Acta Otorrinolaringol Esp 39: 101–105

    CAS  PubMed  Google Scholar 

  26. Watson DJ (1988). Nasal paraganglioma. J Laryngol Otol 102: 526–529

    CAS  PubMed  Google Scholar 

  27. Branham GH, Gnepp DR, O'McMenomey S, Friedman WH (1989) Malignant paraganglioma. A case report and literature review. Otolaryngol Head Neck Surg 101: 99–103

    CAS  PubMed  Google Scholar 

  28. Kuhn JA, Aronoff BL (1989) Nasal and nasopharyngeal paragaglioma. J Surg Oncol 40: 38–45

    CAS  PubMed  Google Scholar 

  29. Shimoda M, Shibuya N, Sato O (1989) Nasal cavity paraganglioma with intracranial extension. Neurol Med Chir 29: 132–136

    CAS  Google Scholar 

  30. Alarcos Llorach A, Matesanz Sanz A, Alarcos Tamayo E, Ovelar Arribas Y (1992) A glomus tumor of the nasal fossa and ethmoid sinus. Acta Otorinolaringol Esp 43: 291–295

    Google Scholar 

  31. Nguyen QA, Gibbs PM, Rice DH (1995) Malignant nasal paraganglioma: a case report and review of the literature. Otolaryngol Head Neck Surg 113: 157–161

    CAS  PubMed  Google Scholar 

  32. Welkoborsky HJ, Gosepath J, Jacob R, Mann WJ, Amedee RG (2000) Biologic characteristics of the nasal cavity and paranasal sinuses. Am J Rhinol 14: 419–426

    CAS  PubMed  Google Scholar 

  33. Kliewer KE, Wen DR, Cancilla PA, Cochran AJ (1989) Paragangliomas: assessment of prognosis by histologic, immunohistochemical, and ultrastructural techniques. Hum Pathol 20: 29–39

    Google Scholar 

  34. Johnson TL, Zarbo RJ, Lloyd RV, Crissman JD (1988) Paragangliomas of the head and neck: Immunohistochemical neuroendocrine and intermediate filament typing. Mod Pathol 1: 216–223

    CAS  PubMed  Google Scholar 

  35. Biscoe TJ, Stehbens WE (1966) Ultrastructure of the carotid body. J Cell Biol 30: 563–578

    CAS  PubMed  Google Scholar 

  36. Lack EE, Cubilla AL, Woodruff JM (1979) Paragangliomas of the head and neck region. Hum Pathol 10: 191–217

    CAS  PubMed  Google Scholar 

  37. Martinez-Madrigal F, Bosq J, Micheau Ch, Nivet P, Luboinski B (1991) Paragangliomas of the head and neck: immunohistochemical analysis of 16 cases in comparison with neuro-endocrine carcinomas. Pathol Res Pract 187: 814–823

    CAS  PubMed  Google Scholar 

  38. Achilles E, Padberg BC, Holl K, Kloppel G, Schroder S (1991) Immunocytochemistry of paragangliomas-value of staining for S-100 protein and glial fibrillary acid protein in diagnosis and prognosis. Histopathology 18: 453–458

    CAS  PubMed  Google Scholar 

  39. Nadji M, Albores-Saavedra J, Ziegels-Weissman J, Morales AR (1986) Antigenic profile of benign, locally invasive and metastatic paragangliomas. Lab Invest 54: 46A

    Google Scholar 

  40. Gould VE (1988). Neuroendocrine tumors. Pathol Res Pract 183: 117–118

    Google Scholar 

  41. Kliewer KE, Cochran AJ (1989) A review of the histology, ultrastructure, immunohistology, and molecular biology of extra-adrenal paragangliomas. Arch Pathol Lab Med 113: 1209–1218

    CAS  PubMed  Google Scholar 

  42. Linnoila Rl, Keiser HR, Steinberg SM, Lack EE (1990). Histopathology of benign versus malignant sympathoadrenal paragangliomas. Hum Pathol 21: 1168–1180

    CAS  PubMed  Google Scholar 

  43. Rosai J (1996) Adrenal gland and other paragangliomas. In: Ackerman's surgical pathology, 8th edn. Mosby, St Louis, pp 1015–1057

Download references

Author information

Authors and Affiliations

  1. Dipartimento di Patologia Umana ed Oncologia, Università degli Studi di Firenze, Viale G.B. Morgagni 85, 50134 , Firenze, Italia

    Sheyda Ketabchi, Daniela Massi & Alessandro Franchi

  2. Dipartimento di Scienze Chirurgiche Neuro-oto-oftalmologiche, Università degli Studi di Firenze, Firenze, Italia

    Roberto Santoro

Authors
  1. Sheyda Ketabchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniela Massi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Roberto Santoro
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alessandro Franchi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alessandro Franchi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ketabchi, S., Massi, D., Santoro, R. et al. Paraganglioma of the nasal cavity: a case report. Eur Arch Otorhinolaryngol 260, 336–340 (2003). https://doi.org/10.1007/s00405-002-0569-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00405-002-0569-4

Keywords

Search

Navigation