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Primary spinal intramedullary primitive neuroectodermal tumor.

Harbhajanka A, Jain M, Kapoor SK - J Pediatr Neurosci (2012)

Bottom Line: Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual.The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery.The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Lady Hardinge Medical College, New Delhi, India.

ABSTRACT
Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual. The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery. The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

No MeSH data available.


Related in: MedlinePlus

Microphotograph showing a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 (inset)
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Figure 3: Microphotograph showing a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 (inset)

Mentions: An 18-year-old female presented with complaints of low back ache and weakness involving bilateral lower extremities for 3 months along with bowel bladder involvement for 1 week. Examination revealed tenderness in lower back with hypotonic and paraplegia (0/5 Medical Research Council grade) in bilateral lower limbs. Ninety percent sensory loss to all modalities of sensation was present with no evidence of sacral sparing. Magnetic resonance imaging (MRI) of the spine revealed an intramedullary lesion, predominantly isointense on TIW MRI with homogenous contrast enhancement and interspersed cystic/necrotic areas from D11-L2 [Figures 1 and 2]. A differential diagnosis of common intramedullary tumors namely ependymoma (myxopapillary variant), paraganglioma, other glial neoplasms, metastases or any inflammatory pathology was considered. Laminectomy and tumor decompression was performed. . Histopathology revealed a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 [Figure 3]. Bone scan and an intracranial MRI were done which did not reveal any evidence of tumor elsewhere and a diagnosis of primary intramedullary PNET was made. Post-operative course was uneventful with minimal improvement in power and the patient is awaiting adjuvant chemoradiotherapy.


Primary spinal intramedullary primitive neuroectodermal tumor.

Harbhajanka A, Jain M, Kapoor SK - J Pediatr Neurosci (2012)

Microphotograph showing a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 (inset)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3401662&req=5

Figure 3: Microphotograph showing a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 (inset)
Mentions: An 18-year-old female presented with complaints of low back ache and weakness involving bilateral lower extremities for 3 months along with bowel bladder involvement for 1 week. Examination revealed tenderness in lower back with hypotonic and paraplegia (0/5 Medical Research Council grade) in bilateral lower limbs. Ninety percent sensory loss to all modalities of sensation was present with no evidence of sacral sparing. Magnetic resonance imaging (MRI) of the spine revealed an intramedullary lesion, predominantly isointense on TIW MRI with homogenous contrast enhancement and interspersed cystic/necrotic areas from D11-L2 [Figures 1 and 2]. A differential diagnosis of common intramedullary tumors namely ependymoma (myxopapillary variant), paraganglioma, other glial neoplasms, metastases or any inflammatory pathology was considered. Laminectomy and tumor decompression was performed. . Histopathology revealed a highly cellular tumor, consisting mainly of small round to oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of Homer-Wright pseudorosettes; the tumor cells being immunopositive for CD 99 [Figure 3]. Bone scan and an intracranial MRI were done which did not reveal any evidence of tumor elsewhere and a diagnosis of primary intramedullary PNET was made. Post-operative course was uneventful with minimal improvement in power and the patient is awaiting adjuvant chemoradiotherapy.

Bottom Line: Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual.The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery.The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Lady Hardinge Medical College, New Delhi, India.

ABSTRACT
Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual. The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery. The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

No MeSH data available.


Related in: MedlinePlus