British Journal of Neurosurgery

ISSN: 0268-8697 (Print) 1360-046X (Online) Journal homepage: http://www.tandfonline.com/loi/ibjn20

Multiple intradural spinal metastases of esthesioneuroblastoma: A case report Sandeep Mohindra, Manjul Tripathi, Satyawati Mohindra, Amey Savardekar & Bishan D. Radotra To cite this article: Sandeep Mohindra, Manjul Tripathi, Satyawati Mohindra, Amey Savardekar & Bishan D. Radotra (2015) Multiple intradural spinal metastases of esthesioneuroblastoma: A case report, British Journal of Neurosurgery, 29:4, 579-581, DOI: 10.3109/02688697.2015.1016889 To link to this article: http://dx.doi.org/10.3109/02688697.2015.1016889

Published online: 26 Mar 2015.

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Date: 29 September 2015, At: 11:42

British Journal of Neurosurgery, August 2015; 29(4): 579–581 © 2015 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2015.1016889

SHORT REPORT

Multiple intradural spinal metastases of esthesioneuroblastoma: A case report Sandeep Mohindra1, Manjul Tripathi1, Satyawati Mohindra2, Amey Savardekar1 & Bishan D. Radotra3 1Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India,

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2Department of Otolaryngology, Postgraduate Institute of Medical Education and Research, Chandigarh, India, and 3Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Patient underwent extended bifrontal craniotomy and total excision of tumor along with anterior skull base dura. Dural defect was repaired using pericranial graft. Postoperatively, the patient remained asymptomatic and received 30 Gy of radiotherapy at the operative site. At 3-month follow-up, he was asymptomatic, with Karnofsky Performance Scale of 90%. At one year of follow-up, he presented with 3-day history of spastic paraplegia, with bowel–bladder involvement. Contrast MRI scans of craniospinal axes revealed no primary site recurrence, while there were intradural extramedullary mass lesions at T7 and at cauda equina. Diffuse multiple nodules abutting against the sheaf of cauda equina were seen (Fig. 2). Patient underwent excision of both the masses (T7 and L3) along with excision biopsy of carpeting lesions. Pathological examination confirmed the spinal mass lesions as metastases from anterior cranial ENB (Fig. 2). Nasopharyngeal tumor showed lobular architecture in some foci containing poorly differentiated cells, suggestive of olfactory neuroblastoma. This was of grade-3 histology in view of lack of anaplastic features such as pleomorphic nuclei with prominent nucleoli. Even though mitosis and necrosis were present, they were not extensive. Occasional foci of neurofibrillary background were seen. This tumor was negative for pancytokeratin and cytokeratin 8/18, and was positive for S100 in sustentacular cells thus excluding diagnosis of sinonasal undifferentiated carcinoma. The biopsy from conus medullaris tumor contained a few necroses in the diffuse sheets of cells, indicating Hyams’ grade-3 morphology1. There were small blue round cells containing scanty cytoplasm and hyperchromatic nuclei without any fibrillary background. Immunostaining for neuron-specific enolase (NSE) showed positivity in the tumor cells. (Fig. 2). Postoperatively, spinal radiotherapies of 32 Gy and 10 Gy at lumbar spine were administered. There was a remarkable improvement in neurological status of both the lower limbs, and patient was ambulatory within 8 weeks of surgery. After

Abstract Spinal metastases from esthesioneuroblastoma (ENB) might have variable presentations. Discrete lesions, even when multiple, warrant radical excision, followed by radiotherapy. The authors present a case of anterior skull base ENB, metastasizing to spine at muliple levels. Clinical and radiological pictures are described with intraoperative findings. Keywords: esthesioneuroblastoma; metastases; Spine; MRI scan

Introduction Olfactory neuroblastoma or esthesioneuroblastoma (ENB), a neuroectodermal tumor of olfactory neuroepithelium,1 rarely metastasizes via cerebrospinal fluid (CSF) pathways. A few cases describing metastases at cauda equina are available,2 while a single case with thoracic-site intradural– extramedullary metastasis has been reported.2 Contrary to its described not-so-aggressive clinical behavior,3 we present a young male patient with widespread spinal metastases (thoracic and cauda equina), which occurred within one year of radical excision of primary neoplasm, with no recurrence at the primary site.

Case report A 23-year-old male presented with repeated attacks of epistaxis, headache, and vomiting of 3-week duration. On anterior rhinoscopy, there was grayish, polypoidal mass within anterior nasal cavity. Radiological investigations were suggestive of an extensive anterior skull base mass, extending on both sides of skull base. Computerized tomography (CT) scan showed a well-defined polypoidal mass within both the ethmoidal sinuses, extending intracranially, destroying the bony ethmoidal roofs. Contrast-enhanced magnetic resonance imaging (MRI) scans showed a well-encapsulated mass lesion on both sides of skull base (Fig. 1).

Correspondence: Sandeep Mohindra, MCh, FRCSEd, Assistant Professor, Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, India. Tel: 0172-2693124. Fax: 0172-2744401. E-mail: [email protected] Received for publication 19 February 2013; accepted 31 January 2015

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Fig. 1. Contrast-enhanced MRI scan: axial (a), coronal (b), and sagittal section (c) showing a mass lesion in paranasal sinuses, invading bifrontal lobes (right ⬎ left). Coronal section T2-weighted scan (d) showing the same mass lesion, with intense bifrontal cerebral edema.

6 months of spinal exploration, the patient is ambulatory with support, with mild spasticity, but poor bladder control.

Discussion ENB was first described in 1924.2 Although the exact cell of origin remains debatable, most evidence supports a neuronal–neural crest origin. Basal progenitor cell of the olfactory epithelium is considered as the cell of origin for ENB.1,2 Histopathological diagnosis is based on results of

antigen expression detected through a panel of antibodies by immunohistochemistry. As distant metastases are rare,4,5 investigations including spinal radiology are not routinely performed at our place. Still, all patients operated for ENBs and having clinical symptomatology pertaining to spine should be evaluated with contrast MRI scans. Metastases of ENB can occur up to 10 years after initial diagnosis. Common sites of metastases are cervical lymph nodes, lungs, abdominal solid viscera, long bones, and pelvis.3 ENB metastases to spine or spinal

Fig. 2. (a, b) Contrast-enhanced T1-weighted and T2-weighted MRI scans showing intradural, extramedullary ENB metastasis at the level of T7. (c, d) Contrast-enhanced T1-weighted and T2-weighted MRI scans showing ENB carcinomatosis at cauda equina. (e) Intraoperative picture showing a brown mass lesion (left), with sheaf of roots scalloping over it and yellow-colored stagnated CSF collection (right) at the lower end. (f ) Nasopharyngeal tumor showing lobular architecture with some foci containing poorly differentiated cells. Excised metastasis shows a few necroses in the diffuse sheets of cells, indicating Hyams’ grade-3 morphology. There are small blue round cells containing scanty cytoplasm and hyperchromatic nuclei without any fibrillary background. Immunostaining for NSE shows positivity for tumor cells.

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Multiple, spine, metastases, esthesioneuroblastoma cord are extremely rare. With less than 20 cases reported worldwide, the pattern of metastases may range from leptomeningeal spread, to discrete nodular compressive lesions detected preoperatively or at autopsy.2 Multiple lesions (intradural), extradural lesions, or bony spinal metastases have also been described. Discrete lesions, even when multiple, warrant radical excision. As clean surgical margins are difficult to obtain, radiotherapy is often recommended. Even when the exact role of chemotherapy in treatment protocol remains unclear, a few reports describing the utility of cisplatin, etoposide, and temozolomide have been published.5 The present case highlights the aggressive behavior of ENB, necessitating adjuvant therapy (in the form of radiotherapy/ chemotherapy) for all ENB primary-site surgeries. Survival according to treatment modalities was 65% for surgery plus radiotherapy. The histopathological grading according to Hyams and the presence of cervical lymph node metastases emerged as prognostic factors.2 ENB is considered locally aggressive, but having significant propensity for distant metastases (22–40%). We propose that patients with Kadish’s grade-3 clinical staging along with higher Hyams’ pathological grading should be followed up

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closely for distant metastases.1 Radical surgical excision of primary ENB along with local-site radiotherapy seems to be an optimal management strategy. Prophylactic craniospinal irradiation is not advocated, but spinal metastases should be dealt with by radical excision and radiotherapy. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

References 1. Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001;2:683–90. 2. Arnold PM, Habib A, Newell K, Anderson KK. Esthesioneuroblastoma metastatic to the thoracic intradural and extradural space. Spine J 2009;9:e1–5. 3. Hyams VJ, Batsakis JG, Michaels L. Tumors of the upper respiratory tract and ear. 2nd series, fascicle 25. In: Hyams VJ, ed. Washington: Armed Forces Institute of Pathology, 1988:240–8. 4. Mori R, Sakai H, Kato M, et al. Olfactory neuroblastoma with spinal metastasis: case report. No Shinkei Geka 2007;35:503–8. 5. Ranjan D, Hennessy RG. Esthesioneuroblastoma: cerebral and spinal metastases without direct cranial invasion. J Neurooncol 1986;4:71–4.

Multiple intradural spinal metastases of esthesioneuroblastoma: A case report.

Spinal metastases from esthesioneuroblastoma (ENB) might have variable presentations. Discrete lesions, even when multiple, warrant radical excision, ...
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