Spinal cord tumors seminar presentation.pptx

Spinal cord tumor

1. PRIMARY MALIGNANT SPINAL CORD TUMORS Dr PRANEETH PREM KUMAR

2. Uncommon 2 – 8% of all CNS tumors of adults. Majority are benign and < 1/3 are malignant. Difficult to deal and associated with significant morbidity and mortality

3. CLASSIFICATION Divided as Intradural & Extradural. Intra Dural, intra medullary(IM) – 5 to 10% of spinal cord tumours in adults and 35% in children. Primary glial tumors – 80% of IM tumors that include Astrocytoma Ependymoma Least common – Gangliogliomas , oligodendrogliomas, sub ependymoma Ependymomas ,most common IM tumors , 50 – 70 % in adults, rest – astrocytoma In chidren < 10 years, this is vice versa.

4. Malignant ependymomas – rare Astrocytoma most malignant Intra Medullary tumors . Age at presentation is 35 – 39 years males > females. MPNST and malignant meningiomas – high grade are rare may be a/w NF 1.

5. MALIGNANT ASTROCYTOMAS Intra Medullary lesion, astrocytic origin. Most are low grade 10 – 15% - high grade ( Grade 3/4) Anaplastic astrocytoma (Grade 3), majority GBM < 0.2% (Grade - IV) Symptoms – non specific and takes several years, if malignant, rapid progression Focal back pain, localised to the spinal segments over the tumor , worse at night while lying down d/t increased venous engorgement and mass effect.

6. Sensory / motor tract deficits in half of patients & signs of myelopathy and UMN disease – spasticity, hyper reflexia , extensor plantar Bowel and bladder – if involved, more advanced diseased, can be seen earlier if conus medullaris. Hydrocephalus - less with low grade lesions and may be seen with high grade lesion. Malignant astrocytoma – disseminated disease by leptomeningeal spread at a rate of 47 – 58 % - reduced survival. Localised anaplastic astrocytoma has 25 fold increase in survival time

7. Children – hard to recognise symptoms, alteration of gait, increased clumsiness, more frequent falls, scoliosis development Young children – motor regression .

8. IMAGING MRI – asymmetrical, focal, fusiform expansion of cord - span < 4 vertebral segments. May be cystic and may be a/w synringomyelia . Well circumscribed – low grade lesions. Infiltrative – higher grade. Iso to hypo intense on T1 and hyper intense in T2. Contrast enhancement + , high grade – enhance, hemorrhage , necrosis. DD – multiple sclerosis, transvere myelitis, cord infarction or dural AV fistula.

9. MANAGEMENT Treatment – ranges from biopsy with an expansile duraplasty to gross total excision. Multilevel laminectomy over the site of tumor , midline durotomy. Intra op USG – guide the exposure and localisation. Superficial lesions can be entered directly and deeper lesions – careful myelotomy is needed to reduce deficits. Tumor removed to safe maximal extent. GTE is often not feasible d/t lack of clear dissection plane.

10. If residual bulk is left behind – an expansile duraplasty with a patch graft allows expansion of cord as tumor progresses. For recurrent malignant astrocytomas – en bloc resection of spinal segment has been described in case report - This is only advised if there is existing neurological deficit. Adjuvant treatment- RT and CT is advised d/t high rates of recurrence both local and distant. RT – modest survival improvement and local tumor control. CT – Temozolamide , commonly used – reserved for progression after sx and RT, unclear role

11. GENTICS AND PROGNOSIS Under developed for spinal astrocytomas IDH 1 – lower in spinal astrocytoma is < than cranial counterpart. Histone (H3A) – malignant astrocytomas of midline neural structures – a/w high grade spinal astrocytomas . Poor prognosis - 1 year – 67%, 2 year – 40%, 5 year – 23% Anaplastic astrocytoma is slightly better than glioblastoma multiforme MA – better in adults than children, median survival of 9 months in pediatric population inspite of aggressive multidoality tx .

12. ANAPLASTIC EPENDYMOMA Majority of Intra Medullary SC tumors Low grade WHO Grade I or II Well circumscribed Intra medullary lesion Myxo papillary – distinct enhancing mass a/w filum terminale . Sporadic usually, spinal ependymomas are prone for NF 2 gene deletions Anaplastic are WHO grade 3 – rare < 2 % of intraspinal ependymomas. Similar presentation to ependymomas – low back at night.

13. They present with dysesthesia, not sensory loss d/t central location os tumors and damage of crossing spinothalamic tracts MRI - Hypo on T1 and Hyper in T2 and FLAIR and enhance homogenously with contrast. Treated by surgical resection and curable completely in case of low grade with GTE. High grade – maximal safe resection as plane allows. Post op RT for anaplastic astrocytoma regardless of dissection in adults and children. CT – yet to be elucidated. 1 & 2 year– 92% & 82% overall survival, 5 y and 10 yr survival rate – 50 – 100%

14. ANAPLASTIC ODG AND ANAPLASTIC GANGLIOGLIOMAS Only 1 – 2 % of spinal cord tumors Benign mostly and aggressive malignant variants are described too. Presentation depend on their location with in spinal cord. Gangliogliomas – mixed type composed of neuronal + glial elements. Mostly in children and rare after 40 years. Benign and indolent and can transform to anaplastic ganglioglioma WHO gradeIII , Presentation – paraparesis 50 % or segmental pain 46%

15. Imaging – variable on T1 – hypo, iso, hyper FLAIR and T2– Hyper more reliable They involve spinal cord assymetrically and can be diffuse with more vertebral segment involvement GG – treated by maximal surgical resection and GTE is possible in > 80% than in cranial tumors . Better survival > 90% at 5 years. More recurrent than the supratentorial of upto 30 – 47%.

16. Routine Radio therpy is not advised but can be used in recurrence and malignant transformation to higher grade lesion. Primary anaplastic ganglioglioma- rare In adults – unfavorable and better outcomes in children. GTE is advised for Intramedullary anaplastic gangliogliomas

17. Spinal Intra Medullary ODG – very rare only < 50 cases have been reported Leptomeningeal spread and elevated ICP and may have fluctuating symptoms d/t intratumoral hemorrhage MRI – Iso on T1 Hyper on T2 with heterogenous contrast ehancement Complete resection of ODG is difficult d/t surrounding parenchyma infiltration and GTE was achieved in < 50%.

18. ANAPLASTIC OLIGODENDROGLIOMA Garde 3 Poor prognosis only few survive more than 3 years Good results were noted by sub total resection + adjuvant RT in patient who didn’t had leptomeningeal spread. Temozolomide useful in patients with 1p/19q co deletion.

19. MALIGNANT PERIPHERAL NERVE SHEATH TUMORS Rare soft tissue sarcomas that arise de novo/ as degeneration from previously existing plexiform neurofibromas Can be found through out the body and can involve spinal roots in 2 to 3%. They are found in patients with NF1, 3 – 5 % of incidence And 0.001% in general population Radiation is another risk factor at 4- 11%. Pain, weakness or sensory deficits.

20. Rapid onset of symptoms – malignancy. MRI – Hyper on T2 and iso to mxed on T1 Fat suppression needed to delineate the nerve in the extra spinal fat lesions Contrast – heterogenous enhancement. They are a/w Larger diameter > 5cm, irregular lobulated margins, indistinct boundarieson MRI and osteolytic bone destruction on CT When compared to benign tumors and no specific findings

21. They are resistant to chemo like soft tissue sarcoma and used only in patients with metastatic disease. Poor prognosis despite the aggressive treatment. Local recurrence , leptomeningeal spread and systemic metastasis. 5 year survival rate 16 to 52%.

22. HISTOLOGICAL DD cellular schwannoma, benign and uncommon – 100 % survival rate Malignant triton tumor – highly aggressive with rhabdomyoblastic differentiation 5% of MPNST, MTT of spinal nerve is rare, aggressive rapid progression 5 year survival rate 11% only.

23. schwannoma

24. MALIGNANT MENINGIOMA 2 nd most common intradural extramedullary Slow growing tumors – benign and a/w low morbidity and mortality Malignant meningiomas of spine are WHO grade III. Very rare rhabdoid and rhabdoid papillary are reported. Malignant transformation of low grade tumor is possible but very uncommon. Unclear treatment, few suggested post op RT in case of malignancies.

25. PRIMARY SPINAL CORD MELANOMA Melanocytes – neural crest cells and can be found in uvea, cerebral parenchyma, mucus membrane and leptomeninges. Primary CNS melanoma is rare < 1 % of all melanoma And spinal melanoma is even rare, majority of these lesions are intra medullary they have also been found extra medullary extension. Criteria for diagnosis – Hayward Presence of melanoma lesion with out any evidence of tumor outside of CNS

26. MRI – T1 hyper intense Iso to hypo on T2. Mild enhancement with contrast. ½ of thes are to be found in the thoracic spine. Surgical resection is mainstay of treatment Surgery + adjuvant treatment had better outcome that surgery alone and has been a/w decreased chance of subsequent metastasis. Median survival of 6 years and 5 year survival rate of 70%.

27. NCCN

28. PSCT 2

29. PSCT 3

30. PSCT 4

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