Indian Journal of Cancer Medknow Publications on behalf of Indian Cancer Society ISSN: 0019-509X EISSN: 1998-4774 Vol. 48, Num. 1, 2011, pp. 115-116

Indian Journal of Cancer, Vol. 48, No. 1, January-March, 2011, pp. 115-116

Letter to Editor

Olfactory neuroblastoma with central nervous system metastasis-A rare report

B Rai, AK Pandey, R Aggarwal, K Dimri

Department of Radiotherapy, Government Medical College, Chandigarh - 160 030, India

Correspondence Address:
B Rai
Department of Radiotherapy, Government Medical College, Chandigarh - 160 030
India
bhavrai@yahoo.com

Code Number: cn11022

Sir,

A 23-year-old male presented to the radiotherapy clinic with a two-month-old history of swelling over the upper part of the nose, obstruction, and frequent headaches. He had undergone an excision of a nasal mass two years ago, the histopathology of which was reported as olfactory neuroblastoma. However, he did not report for adjuvant treatment. Over last two weeks he had developed progressive weakness of both lower limbs with parasthesias in the gluteal region. An MRI of the brain showed a large expansile area of the bilateral anterior ethmoid cells and frontal sinuses extending into superior aspect of the nasal cavity, without intraorbital or intracranial extension. [Figure - 1]. Magnetic resonance imaging (MRI) of the lumbar spine showed a partial collapse of the L4 vertebra, with marrow edema at L4, 5, and S1, and an anterior epidural soft tissue mass causing marked thecal sac compression at L4-5. [Figure - 2].

A diagnosis of olfactory neuroblastoma with Kadish stage C was made. The patient was started on radiotherapy to the lumbar sacral region (L3-S2). A dose of 30 Gray in ten fractions, over two weeks, was planned, by a single posterior field treated at a depth by Cobalt-60. He was planned for chemotherapy with cisplatin and etoposide followed by radiotherapy to the primary site. However, he refused further treatment after two cycles of chemotherapy. At the last follow-up, after eight months, he had 20 - 25% subjective response at the local site and had no neurological deficit. He was lost to follow-up thereafter.

Esthesioneuroblastoma or olfactory neuroblastoma is a rare neuroepithelial tumor originating in the cribriform plate or upper nasal cavity and accounts for 1 - 5% of cancers of the nasal cavity. ^[1] The tumor can spread locally through direct extension or along the olfactory nerves. Local recurrence occurs in two-thirds of the patients who undergo surgery and is the most common cause of death. Metastasis occurs in about 20% of the patients and cervical lymph nodes are the frequent site of metastasis. Metastasis to the central nervous system (CNS) is rare and in the spinal cord nearly 80% of metastasis occurs in the cauda equine. ^[2] This is thought to occur when the tumor cells violate the ependymal epithelium of the ventricles to gain access to the cerebrospinal fluid (CSF). The tumor cells then disseminate through the CSF pathway to different sites. The tumor is positive for neuroendocrine markers ^[3] and the presence of tumor necrosis is the only histological feature of prognostic significance. ^[4]

In a literature review and report by Shaari et al, Parenchymal CNS metastasis to the cerebral hemispheres was more common as compared to spinal cord metastasis. ^[1] The analysis suggested that tumor cells harbored in areas of large CSF cisterns, and therefore, cauda equine (the most dependant spinal cistern) was the most frequent site of spinal cord metastasis. Most of the CNS metastasis developed in patients with advanced disease and the prognosis in these patients was poor with survival of less than two years. The treatment recommended was surgical resection of the metastasis followed by radiation and / or chemotherapy. In a study by Yoh et al, the response rate of 83% with chemotherapy was seen in young patients with unresectable or recurrent olfactory neuroblastoma. The drugs commonly used were cisplatin, cyclophosphamide, docetaxel, and doxorubicin. ^[5]

In conclusion, the standard treatment for olfactory neuroblastoma is aggressive surgical resection followed by adjuvant radiotherapy. However, in patients with unresectable, recurrent or metastatic disease, chemotherapy may be considered.

References

1.	Shaari CM, Catalano PJ, Sen C, Post K. Central nervous system metastasis from neuroblastoma. Otolaryngologol-Head Neck Surg 1996;114:808-12.  Back to cited text no. 1
2.	Turakhia S. Patel K. Esthesioneuroblastoma. Indian J Radiol Imaging 2006;16:669-72.  Back to cited text no. 2
3.	Rinaldo A, Ferlito A, Shaha AR, Wei Wi, Lund VJ. Esthesioneuroblastoma and cervical lymph node metastasis: Clinical and therapeutic implications. Acta Otolaryngol 2002;122:215-21.  Back to cited text no. 3  [PUBMED]
4.	Ramsay AD. Oxford Textbook of Pathology. In: James OD, McGee SP, Wright NA, editors. Pathology of systems. Chap-15.2, Vol. 2A. Oxford New York Tokyo: Oxford University Press; 1992. P. 1112.  Back to cited text no. 4
5.	Yoh K, Tahara M, Kawada K, Mukai H, Nakata M, Itoh K, et al. Chemotherapy in the treatment of advanced or recurrent olfactory neuroblastoma. Asia Pacif J Clin Oncol 2006;2:180-4.  Back to cited text no. 5

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