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Olfactory Neuroblastoma

Synonyms: Esthesioneuroblastoma, Esthesioneurocytoma, Esthesioneuroepithelioma, Olfactory Placode Tumor

Definition

Malignant neuroectodermal tumor thought to arise from neuroepithelial elements of the olfactory placode.

Clinical Features

Pathogenesis

Histopathology

  • Several patterns, which often merge11,12 (Fig. 1 and 2
    Olfactory neuroblastoma. The pattern of growth is nesting.

    Fig. 1: Olfactory neuroblastoma. The pattern of growth is nesting.

    Olfactory neuroblastoma. The pattern of growth is diffuse.

    Fig. 2: Olfactory neuroblastoma. The pattern of growth is diffuse.

    )
  • Most easily recognizable and common appearance:
    • cellular tumor composed of uniform small cells with:
      • round nuclei
      • scanty cytoplasm
      • indistinct nuclear membrane
      • prominent fibrillary or reticular background, similar to that seen in other neurogenic tumors (such as ganglioneuroblastoma) (Fig. 3
        Olfactory neuroblastoma. The tumor shows typical neurofibrillary background.

        Fig. 3: Olfactory neuroblastoma. The tumor shows typical neurofibrillary background.

        )
      • may be:
        • rosettes of Homer Wright-type:
          • but differentiation into mature ganglion cells rare13
        • abundant fibrovascular stroma separating tumor cells in clusters
        • melanin in cytoplasm of tumor cells14
      • vascular proliferation can be so florid as to obscure true nature of tumor15 (Fig. 4
        Olfactory neuroblastoma associated with a striking lobular proliferation of blood vessels. This proliferation is a feature often seen in neuroepithelial and neuroendocrine neoplasms.

        Fig. 4: Olfactory neuroblastoma associated with a striking lobular proliferation of blood vessels. This proliferation is a feature often seen in neuroepithelial and neuroendocrine neoplasms.

        )
  • In other examples tumor cells exhibit an epithelial appearance and are:
    • larger
    • have more abundant cytoplasm
    • grow in solid nests
  • Ultrastructurally:
    • consistent presence of:
      • neurofilaments
      • neurotubules
    • dense-core neurosecretory cytoplasmic granules16–18

Special Stains and Immunohistochemistry

Diagnosis

Differential Diagnosis

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  • Small cell type may be confused with:
    • malignant lymphoma
    • plasmacytoma
    • embryonal/alveolar rhabdomyosarcoma
    • Ewing sarcoma/PNET tumor family
  • Larger cell type often confused with undifferentiated carcinoma29

Management

  • Combination of surgery and radiation therapy:

References

1 Mills SE, Frierson HF. Olfactory neuroblastoma. A clinicopathologic study of 21 cases. Am J Surg Pathol. 1985;9:317–327.

2 Harrison D. Surgical pathology of olfactory neuroblastoma. Head Neck Surg. 1984;7:60–64.

3 Meneses MS, Thurel C, Mikol J, Ramina R, Maniglia JJ, Arruda WO, et al. Esthesioneuroblastoma with intracranial extension. Neurosurgery. 1990;27:813–819.

4 Hutter RVP, Lewis JS, Foote FW, Tollefsen HR. Esthesioneuroblastoma. Am J Surg. 1963;106:748–753.

5 Devaney K, Wenig BM, Abbondanzo SL. Olfactory neuroblastoma and other round cell lesions of the sinonasal region. Mod Pathol. 1996;9:658–663.

6 Oberman HA, Rice DH. Olfactory neuroblastomas. A clinicopathologic study. Cancer. 1976;38:2494–2502.

7 Sorensen PH, Wu JK, Berean KW, Lim JF, Donn W, Frierson HF, et al. Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma. Proc Natl Acad Sci USA. 1996;93:1038–1043.

8 Argani P, Perez-Ordonez B, Xiao H, Caruana SM, Huvos AG, Ladanyi M. Olfactory neuroblastoma is not related to the Ewing family of tumours: absence of EWS/FLI1 gene fusion and MIC2 expression. Am J Surg Pathol. 1998;22:391–398.

9 Kumar S, Periman E, Pack S, Davis M, Zhang H, Meltzer P, et al. Absence of EWS/FLI1 fusion in olfactory neuroblastomas indicates thses tumors do not belong to the Ewing's sarcoma family. Hum Pathol. 1999;30:1356–1360.

10 Mashberg A, Thoma KH, Wasilewski EJ. Olfactory neuroblastoma (esthesioneuroepithelioma) of the maxillary sinus. Oral Surg Oral Med Oral Pathol. 1960;13:908–912.

11 Gerard-Marchant R, Micheau C. Microscopical diagnosis of olfactory esthesioneuromas. General review and report of five cases. J Natl Cancer Inst. 1965;35:75–82.

12 Ordoñéz NG, Mackay B. Neuroendocrine tumors of the nasal cavity. Pathol Annu. 1993;28–2:77–111.

13 Chan JK, Lau WH, Yuen RWS. Ganglioneuroblastic transformation of olfactory neuroblastoma. Histopathology. 1989;14:425–428.

14 Curtis JL, Rubinstein LJ. Pigmented olfactory neuroblastoma. A new example of melanotic neuroepithelial neoplasm. Cancer. 1982;49:2136–2143.

15 Gaudin PB, Rosai J. Florid vascular proliferation associated with neural and neuroendocrine neoplasms. A diagnostic clue and potential pitfall. Am J Surg Pathol. 1995;19:642–652.

16 Lloreta-Trull J, Mackay B, Troncoso P, Ribalta-Farres T, Smith T, Khorana S. Neuroendocrine tumors of the nasal cavity. An ultrastructural and morphometric study of 24 cases. Ultrastruct Pathol. 1992;16:165–175.

17 McCluggage WG, Napier SS, Primrose WJ, Adair RA, Toner PG. Sinonasal neuroendocrine carcinoma exhibiting amphicrine differentiation. Histopathology. 1995;27:79–82.

18 Taxy JB, Hidvegi DF. Olfactory neuroblastoma. An ultrastructural study. Cancer. 1977;39:131–138.

19 Hirano T, Aida T, Moriyama M, Asano G, Suzuki I, Yuge K. Primary neuroblastoma of the nasal cavity and review of literature. Acta Pathol Jpn. 1985;35:183–191.

20 Micheau C. A new histochemical and biochemical approach to olfactory esthesioneuroma. A nasal tumor of neural crest origin. Cancer. 1977;40:314–318.

21 Amesen MA, Scheithauer BW, Freeman S. Cushing's syndrome secondary to olfactory neuroblastoma. Ultrastruct Pathol. 1994;18:61–68.

22 Axe S, Kuhajda FP. Esthesioneuroblastoma. Intermediate filaments, neuroendocrine, and tissue-specific antigens. Am J Clin Pathol. 1987;88:139–145.

23 Choi H-SH, Anderson PJ. Immunohistochemical diagnosis of olfactory neuroblastoma. J Neuropathol Exp Neurol. 1985;44:18–31.

24 Taxy JB, Bharani NK, Mills SE, Frierson HF, Gould VE. The spectrum of olfactory neural tumors. A light-microscopic immunohistochemical and ultrastructural analysis. Am J Surg Pathol. 1986;10:687–695.

25 Trojanowski JQ, Lee V, Pillsbury N, Lee S. Neuronal origin of human esthesioneuroblastoma demonstrated with anti-neurofilament monoclonal antibodies. N Engl J Med. 1982;307:159–161.

26 Silva EG, Butler JJ, Mackay B, Goepfert H. Neuroblastoma and neuroendocrine carcinomas of the nasal cavity. A proposed new classification. Cancer. 1982;50:2388–2405.

27 Mackay B, Luna MA, Butler JJ. Adult neuroblastoma. Electron microscopic observations in nine cases. Cancer. 1976;37:1334–1351.

28 Miller DC, Goodman ML, Pilch BZ, Shi SR, Dickersin GR, Halpern H, et al. Mixed olfactory neuroblastoma and carcinoma. A report of two cases. Cancer. 1984;54:2019–2028.

29 Kahn LB. Esthesioneuroblastoma. A light and electron microscopic study. Hum Pathol. 1974;5:364–371.

30 Chao KS, Kaplan C, Simpson JR, Haughey B, Spector GJ, Sessions DG, et al. Esthesioneuroblastoma: the impact of treatment modality. Head Neck. 2001;23:749–757.

31 Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer. 1976;37:1571–1576.

32 O'Connor TA, McLean P, Juillard GJF, Parker RG. Olfactory neuroblastoma. Cancer. 1989;63:2426–2428.

33 Appelblatt NH, McClatchey KD. Olfactory neuroblastoma. A retrospective clinicopathologic study. Head Neck Surg. 1982;5:108–113.

34 Olsen KD, DeSanto LW. Olfactory neuroblastoma. Biologic and clinical behavior. Arch Otolaryngol. 1983;109:797–802.

Last updated: 5 Mar 2006

Olfactory Neuroblastoma

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