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OJHAS Vol. 9, Issue 4:
Oct-Dec, 2010) |
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Olfactory Neuroblastoma: Diagnostic Difficulty |
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Vidya MN, Registrar,
Department of Pathology, Apollo
Hospitals, Bannerghatta Road, Bangalore 76
Swarna
Shivakumar, Consultant
Pathologist, Apollo
Hospitals, Bannerghatta Road, Bangalore 76
Siddhartha Biswas, Consultant
Pathologist, Apollo
Hospitals, Bannerghatta Road, Bangalore 76
Vijay Shankar
S, Associate
professor, Department of Pathology, Adichunchangiri Institute
of Medical Sciences, BG Nagara, Nagamangala taluk, Mandya district,
Karnataka
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Address For Correspondence |
No 45, Ananda
shylam,
3rd cross, 1st
main ,
KN extension, Yeswanthpur,
Bangalore - 560022,
Karnataka, India.
E-mail:
vijayshankarpatho@gmail.com |
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Vidya MN, Shivakumar S, Biswas S, Vijay Shankar S.
Olfactory Neuroblastoma: Diagnostic Difficulty. Online J Health Allied Scs.
2010;9(4):18 |
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Submitted: Dec 6,
2010; Accepted: Dec 28, 2010; Published: Jan 20, 2011 |
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| Abstract: |
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Olfactory neuroblastoma is an uncommon malignant tumor of sinonasal
tract arising from the olfactory neuro epithelium. The olfactory neuroblastomas
presenting with divergent histomorphologies like, epithelial appearance
of cells, lacking a neuro fibrillary background and absence of rosettes
are difficult to diagnose. Such cases require immunohistochemistry
to establish the diagnosis. We describe the clinical features, pathological
and immunohistochemical findings of grade IV Olfactory neuroblastoma
in a 57 year old man
Key Words: Olfactory neuroblastoma; Nasal cavity; Immunohistochemistry
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Olfactory
neuroblastoma is an uncommon malignant neoplasm, representing 2-3%
of sinonasal tract malignancies. It is a malignant neuro ectodermal
neoplasm arising from the olfactory neuro epithelium found in the upper
1/3 to 1/2 of the nasal septum, the cribriform plate and the superior
medial surface of the superior turbinate.1 This tumor
has a broad histological spectrum and olfactory neuroblastoma can be
confused with other small round cell tumors and undifferentiated carcinomas.
The diagnosis becomes difficult when the cells have an epithelial appearance
with scant or absent neurofibrillary background. Immunohistochemistry
is of great value in differentiating ONB from other sinonasal tract
malignancies.
Left nasal
polyp biopsy of a 57 year old male patient with 3 year history of left
sided nasal obstruction was received in the department of pathology.
The patient presented with anosmia, headache and loss vision in the
left eye with worsening of symptoms over the last 3 months. CT scan revealed
a left paranasal sinus lesion occupying the left maxillary, ethmoidal
region with intraorbital and intracranial extensions. MRI revealed a
left nasal mass extending and eroding the olfactory groove at the anterior
base with erosion of the left orbital wall. Biopsy was
done and sent for histopathological examination.
Gross
Features: Multiple
irregular grey white soft tissue bits were received with a large polypoidal
bit measuring 3x1.5x1 cm. Cut section was grey white with hemorrhagic
areas.
Microscopy: Microscopic
examination showed an intact respiratory mucosa with a tumor in the
submucosa. The tumor cells were seen predominantly arranged in large
lobules, separated by fibrovascular stroma with few areas of solid nests,
cribriform pattern and comedo necrosis.(Fig.1) Occasional tumor
lobules showed Homer – Wright rosettes (Fig 2)
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| Figure 1:
Photomicrograph showing tumor cells in lobules with comedo necrosis (H&E X
100) |
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Figure 2:
Photomicrograph showing Homer-wright rosettes (H&E X 400) |
The cells
were small to intermediate sized having vesicular nucleus with single
nucleolus and scanty to moderate amount of acidophilic cytoplasm (Fig
3). Atypical mitosis of 2-3/10 HPF was noted. No neuro fibrillary
background was seen. The stroma showed extensive areas of haemorrhage
with mild lymphocytic infiltration.
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Figure 3: Photomicrograph showing tumor cells with vesicular
nucleus with nucleoli & moderate amount of cytoplasm (H&E X 400) |
IHC was strongly
positive for NSE, CK cocktail and S-100. S-100 showed positivity of
sustentacular cells along the periphery of the tumor lobules with few
cells within the tumor Lobules (Fig 4 & 5).
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| Figure 4:
Photomicrograph of immunohistochemical staining showing S-100 positivity
of the sustentacular cells in the periphery as well as within
the lobiules (X 400) |
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Figure 5:
Photomicrograph of immunohistochemical staining of the tumor cells showing
Non Specific Esterase positivity (X 400) |
The
final diagnosis of olfactory neuroblastoma grade IV was made.
Debulking
surgery was done.The patient had an uneventful post operative period
and was subjected for radiotherapy.
Olfactory
neuroblastoma is an uncommon malignant neoplasm, representing 2-3%
of sinonasal tract tumors with an incidence of approximately 6 %.1,2 These tumors arise from the olfactory neuro epithelium,
which extends from the roof of the nose to the area of the superior
turbinate and a portion of the nasal septum.2
There is
no gender predilection;1
some authors report a male to female ratio of 2:1.3 It
occurs in all age groups with a bimodal peak in the 2nd and
6th decade.4
The usual
clinical symptoms are unilateral nasal obstruction, epistaxsis, anosmia,
headache, pain and ocular disturbances.1 ONB has a tendency
to spread sub mucosally to involve the paranasal sinuses, nasal cavity
and other structures like oral cavity, orbits and the brain.3
Olfactory
neuroblastoma of grade I & II (Hyams grade) can be easily recognized
as they resemble other neuroblastomas with small round cells arranged
in rosettes and an abundant neuro fibrillary background.1
Grade III & IV (Hyams grade) have little or no neuro fibrillary
background with few or no rosette formations making morphological diagnosis
difficult. In our case, some of the cells had an epithelial appearance
with vesicular nucleus and comedo necrosis closely resembling undifferentiated
sinonasal carcinoma. The other differential diagnosis of small cell
neuroendocrine carcinoma was considered as the tumor cells showed rosette
arrangement with absent neuro fibrillary background. Some areas showed
cribriform pattern which is known to occur in ONB and neuroendocrine
carcinoma favoring differentiation rather than existence of two separate
neoplasms.4 With confounding histomorphology, the diagnosis
will be difficult which requires immunohistochemistry for confirmation.
ONB shows
variable positivity for Cyto keratin, synaptophysin and chromogranin.
One of the studies showed cytokeratin (4/11), synaptophysin (7/11) and
chromogranin A (1/11).5
The main
mode of treatment is complete craniofacial resection of the tumor followed
by radiotherapy.6 The prognosis depends on the grade and
stage of the tumor. The 5 year survival Rate is 40% for high grade tumors
with a high local recurrence (15-70%).1
Olfactory
neuroblastoma is an uncommon neoplasm of the sinonasal tract. Histologically,
the tumor can have varied cytomorphological appearance with absence
of neurofibrillary matrix and occasional areas with rosette formation.
IHC will be of immense help in differentiating it from the undifferentiated
sinonasal carcinoma.
- Christopher DM Fletcher.
Tumors of the upper respiratory tract. In Christopher DM Fletcher.
Diagnostic histopathology of tumors. 3rd ed. Elsevier, 2007. pp
112-118.
- Ghaffar,
Shehzad, Salahuddin, et al. Olfactory neuroblastoma: a case report and
review of the literature. Ear, Nose and Throat Journal. 2005 March;84(3):150-52.
- Arafat Ayoub,
Mohammed Ali Hiari. Olfactory neuroblastoma: A Case Report. Journal
of Research in Medical Sciences. Dec 2005;12(2):48-50.
- Roasi J. Respiratory
tract. In Rosai J, ed Ackerman’s Surgical Pathology. 9th
ed. Elsevier 2004;Vol 1. pp 316-17.
- Frierson HF Jr,
Ross GW, Mills SE,et al. Olfactory neuroblastoma. Additional immunohistochemical
characterization. Am J Clin pathol. 1991 Jun;95(6):903.
- Mills, Stacey
EMD, Frierson, et al. Olfactory neuroblastoma: A clinicopathologic study
of 21 cases. The Am J of Surg Pathol 1995;9:317-327.
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