|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 416-417
Primary cervical primitive neurectodermal tumor with supratentorial metastasis
Hrushikesh Kharosekar, Jasmeet Singh, Vernon Velho
Department of Neurosurgery, Grant Medical College, Sir J Group of Hospitals, Mumbai, Maharashtra, India
|Date of Web Publication||15-May-2015|
Department of Neurosurgery, 4th Floor, Grant Medical College, Sir J Group of Hospitals, Byculla (E), Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kharosekar H, Singh J, Velho V. Primary cervical primitive neurectodermal tumor with supratentorial metastasis. Med J DY Patil Univ 2015;8:416-7
Primitive neurectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuro-ectodermal origin. Cranial PNETs are commonly located in the cerebellum, especially in pediatric age group, and are rarely supratentorial. Most of the spinal PNETs are caused by a drop metastasis, in which the malignant cells from the cranial PNET drop into the spine along with the cerebrospinal fluid (CSF), however, the reverse is seldom reported.  Primary spinal PNETs account for a small percentage of the PNETs and are a rare entity. Brain metastasis from primary spinal PNET is still rare with only few cases reported in so far. We report a rare case of primary spinal PNET metastasizing to the brain after a long interval.
A 41-year-old female presented to us with the complaints of neck pain associated with a tingling sensation and progressive weakness in right upper limb since 2 months and bowel and bladder dysfunction. On examination, power in right upper limb was 2/5 (Medical Research Council grading) in proximal muscle groups and 4/5 in distal muscle groups; with decreased sensations in C5-C6 dermatome. Her higher mental functions and cranial nerve examination were normal. Magnetic resonance imaging (MRI) of the cervical spine was suggestive of an enhancing, globular, extradural lesion at C3-C4 level [Figure 1]. A provisional diagnosis of neurofibroma was considered. MRI brain with contrast was done which did not reveal any abnormality. Patient was operated for C2-C5 laminotomy and gross total tumor resection was done. Intraoperatively, a firm reddish colored encapsulated lesion with both extradural and intradural component was seen. Areas of hemorrhage were seen within the tumor. Postoperative course was uneventful; power in right upper limb improved to 4/5. Tumor was subjected to histopathological examination, which showed highly cellular tumor, with cells infiltrating the surrounding meningeal and fibrocollagenous tissue, arranged in small sheets, highly suggestive of PNET; the diagnosis was further confirmed with immunohistochemistry (IHC). IHC stains for tumor cells showed membranous positivity for CD99. Patient was given radiotherapy and chemotherapy. Follow-up MRI of the cervical spine after 3 months showed no recurrence. Patient again presented to us with a headache associated with vomiting 6 months after surgery. Patient was thoroughly evaluated and investigated. MRI brain and whole spine with gadolinium contrast were done, which showed metastatic lesion in suprasellar location [Figure 2]. MRI spine was done again, which did not reveal any abnormality. The CSF examination revealed presence of malignant cell. Hence based on the radiological and CSF picture, patient was referred for radiotherapy.
|Figure 1: Magnetic resonance imaging cervical spine showing the lesion (axial and sagittal images)|
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|Figure 2: Magnetic resonance imaging (MRI) cervical spine at 6 months showing no local recurrence but MRI brain showing suprasellar metastasis|
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Intracranial metastasis from primary spinal PNET is rare, and there are a few reports available in the literature. The average age at presentation is 17.4 years with female preponderance. , The primary location of the spinal PNETs is varied, with a slight predilection for thoracic spine. There are no specific protocols for treatment of intracranial metastasis from spinal PNET, as most of the published cases are single case reports. Most centers use surgery, radiotherapy, and chemotherapy for their treatments.  For multiple intracranial metastases, chemotherapy and radiotherapy are routinely given. Recently, high dose chemotherapy with autologous stem cell support is being used for better results. Surgery is restricted to solitary intra cranial metastasis, ranging from biopsy to gross total resection. , Adjunctive immunotherapy is currently being investigated as a possible therapy.  Prognosis for patients with intra cranial metastasis from spinal PNETs is poor with a median survival of 1-year. 
Our case is one of the rare cases of primary cervical PNET with intra cranial metastasis with CSF dissemination. Our case signifies the importance of CSF examination in proven PNET cases with metastases, as the outcome and plan of treatment tilt in favor of adjuvant therapies; provided there is no mass effect resulting from the intra cranial metastases.
| References|| |
Ghanta RK, Koti K, Ghanta VS, Teegala R. Intracranial metastasis from primary spinal primitive neuroectodermal tumor. Asian J Neurosurg 2013;8:42-7.
Cai CQ, Zhang QJ, Shen CH, Hu XL. Primary intraspinal primitive neuroectodermal tumor: A case report and review of literature. J Pediatr Neurosci 2008;3:154-6.
Virani MJ, Jain S. Primary intraspinal primitive neuroectodermal tumor (PNET): A rare occurrence. Neurol India 2002;50:75-80.
[Figure 1], [Figure 2]