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Tumours of the spine and spinal cord represent nearly 15% of primary CNS tumours. Most of them are benign and they tend to constrict rather than invade surrounding structures.
Based on the compartment involved they can be subdivided into three groups:

  • Extradural [ED]
  • Intradural extra-medullary [ID-EM]
  • Intramedullary spinal cord tumours [IMSCT]

 

Extra-Dural Spinal Cord Tumors

They represent 55% of all CNS spinal cord tumors and they arise outside cord in epidural tissue or vertebral bodies and may be subdivided in:

Secondary spinal tumours (more common)

  • Osteolytic metastatic tumours
    • Carcinoma (lung, breast, prostate)
    • Lymphoma
    • Plasmacytoma
    • Multiple myeloma
  • Metastatic tumours that may be osteoblastic
    • Prostate carcinoma
    • Breast carcinoma

Primary spinal tumours (very rare)

  • Benign
    • Osteoma osteoid and osteoblastoma

Details of Osteoma osteoid and osteoblastoma

They are both osteogenic tumour and are histologically identical but they differ for size: while osteoma has 1 cm maximum diameter, osteoblastoma has at least 1,5cm of diameter. They occur especially in young male (under 20 years). They prefer posterior element of the vertebra and they may be associated to scoliosis. They are characterized by night pain which responds to NSAID and aspirin.

    • Osteochondroma

Details of Osteochondroma

It is a tumour which origins by cartilage tissue. It prefers cervical level and occurs most frequently in young male (20-30 years). It is usually asymptomatic but it may occasionally manifests itself with symptom and signs of compression.

    • Vertebral hemangiomas

Details of Vertebral hemangiomas

They represent the most common benign primary spinal tumour. They are vascular tumours which incidence peak of 10%. They arise as two types: cavernous (most common) and capillary (rare). They are commonly asymptomatic (>98,8%); symptoms may include compression fracture, disc herniation and very rarely neural compression

    • Aneurysmal bone cyst

Details of Aneurysmal bone cyst

It represent 15% of all spine tumours . It is a tumour-like osteolytic lesion consisting of highly vascular honeycomb of blood-filled cavities separated by connective tissue septa and surrounded by thin cortical bone. It may arise from pre-existing tumour or following acute fracture. It prefers young female ad usually occurs in sacral-lumbar level. It is commonly symptomatic (pain or painful fractures)

    • Giant cell tumours (AKA osteoclastoma)

Details of Giant cell tumours (AKA osteoclastoma)

They are tumours that arise from osteoclasts. They prefer knee and wrist but occasionally can occur in skull and column (nearly 4% occurs in sacrum)

    • Eosinophilic granuloma (AKA Unifocal Langerhans cell hystiocytosis)

Eosinophilic granuloma (AKA Unifocal Langerhans cell hystiocytosis)

It is a eosinophilic rich lesion which is more frequent in young male (under 10 years). It may be solitary or multiple and it usually occurs in cervical level. It can be symptomatic (night pain sensitive to NSAID). It is highly radiosensitive

  • Malignant
    • Chordomas

Characteristics of Chordomas

They are rare tumours of the remnant of the primitive notochord. They are generally slow-growing tumours which can occur both in skull (clivus) and columns (sacral level). They are characterized by high recurrence rate (85%) following surgery

    • Osteosarcoma

Characteristics of Osteosarcoma

It is a malignant bone tumour that may origin ex novo or as a result of benign bone tumour involution. It may also origin from pre-radiated tissue or in patient with Paget disease. It is very aggressive and it destroy bone tissue and surrounded ones. Local pain is the most common symptom at the onset. 

    • Chondrosarcoma

Characteristics of Chondrosarcoma

It is a malignant tumour of cartilage. It prefers male and occurs commonly in adulthood (5th-6th decade). Local pain and neural compression signs and symptoms are frequent at the onset. 

    • Ewing’s sarcoma

Characteristics of Ewing’s sarcoma

It is a malignant mesenchimal tumour with unknown origin. It prefers male and it is typical of pediatric age. More than 50% occurs in sacral level and they prefer anterior element of the vertebra (on the contrary of benign tumour). Treatment aim is palliative and it consists of radical excision followed by RTX and chemotherapy. 

    • Plasmacytoma

Characteristics of Plasmacytoma

It is an ematologic tumour which occurs adulthood and prefers male. 

    • Multiple myeloma

Characteristics of Multiple myeloma

It is a tumour (1% of all) which origins from plasma cell. It represents 1% of all ematologic tumours. It frequently afflicts bone and causes osteolysis. Clinically they are characterized by pain and fractures.

    • Lymphoma (very rare, more frequent represents spread of systemic disease)

 

Intra-Dural Extra-Medullary Spinal Cord Tumors

They constitute 40% of all CNS spinal tumours and arise in leptomeninges or roots and include

  • Meningiomas (40%)

Features of Meningiomas

They are more frequent in adulthood (peak age 40-70 years) and they prefer female (F:M ratio = 4:1) more likely due to hormonal influences, that’s why they may rapidly grow during pregnancy.
Meningiomas most frequently occur at thoracic level followed by cervical and lumbar ones.
They origin from arachnoid cells and they are benign (WHO grade I) in most cases. They arise as solitary lesion or less frequently as multiple ones. They usually appear as round masses that constrict spinal cord without invading it and that’s why the onset key symptom is pain. 

  • Neurinomas and neurofibromas (50%)

Features and classifications of Neurinomas and neurofibromas

They are more frequent in adulthood but they also can afflict young people (especially in case of neurofibromas among Neurofibromatosi I e II). Not detectable gender difference. 
Neurinomas (AKA Schwannomas) origin from Schwann’s cell while neurofibromas origin from mesenchymal cell belonging to fibroblastic series.
They frequently arise from dorsal (sensitive) rootlets (75%) and they may grow along the longitudinal axis of the nerve involved. They are capsulated and well circumscribed. They can appear as:

  • Entirely Intradural (most frequently)
  • Completely extradural
  • Combination of intra and extradural
  • Dumbbell tumours: they are tumours that develop an “hourglass” shape as a result of an anatomic barrier encountered during growth.

The are subdivided according to Asazuma classification system as following:
Type I = both intradural and extradural but restricted to spinal canal
Type II = extradural and foraminal constriction
Type III = both intradural and extradural with foraminal constriction
Type IV = extradural and intravertebral
Type V = extradural and extralaminar with laminar invasion
Type VI = Extradural with multidirectional bone erosion

Symptoms, diagnosis and treatment

  • Pain is the most common symptom at onset, followed by other symptoms and signs according to level and extent involvement of spinal cord
  • MRI is the gold standard for diagnosis
  • Elective treatment is surgical excision with different approach (anterior, lateral, posterior) based on level of involvement. Recurrence rate depends on tumour histotype (e.g. meningioma recurrence rate is approximately 7% within a minimum of 6 years).

 

Intra-Medullary Spinal Cord Tumors

They are the less common CNS spinal tumour of which they represent only 5% and arise in spinal cord substance and invade tracts and grey matter
They consist of:

  • Ependymoma (45%)

Features of Ependymoma

Ependymoma can be considered both extra-medullary due to surgical approach and intra-medullary due to histological origin from ependymal cells.
They are more frequent in adulthood (on the contrary of brain ependymomas).
They are usually situated at conus medullaris or filum terminale and they represent the most common glioma of lower cord.
They usually are solitary and of the myxopapillary subtype (WHO grade I) 

  • Astrocytoma (30%)

Features of Astrocytoma

It is common in adulthood, with peak age: 30-50 years. It is little more frequent in male.
It occurs at all levels but more frequently at thoracic one. 
Astrocytomas may be both low and high grade with ratio 3:1 

  • Miscellaneous
    • Dermoid and epidermoid
    • Hemangioma
    • Hemangioblastoma

Clinical Note

Ependymoma and astrocytoma represent more than 90% of all IMSCT in paediatrics 

Symptoms, diagnosis and treatment

  • Pain is the most common symptom at onset, followed by other symptom and signs according to level and extent involvement of spinal cord
  • MRI is the gold standard for diagnosis
  • Elective treatment is surgical excision for low grade tumours whereas it is not useful in case of high grade tumours (e.g. astrocytomas WHO grade III, glioblastomas (IV)) due to marked invasion of the spinal cord.

References

Kopelson G, Linggood RM, Kleinman GM, et al. Management of Intramedullary Spinal Cord Tumors. Radiology. 1980; 135:473–479 


Adams RD, Victor M. In: Intraspinal Tumors. Principles of Neurology. 2nd ed. New York: McGraw-Hill; 1981:638–641 


Liu JK, Brockmeyer DL, Dailey AT, Schmidt MH. Surgical management of aneurysmal bone cysts of the spine. Neurosurg Focus. 2003; 15 


Grubb MR, Currier BL, Pritchard DJ, et al. Primary Ewing's Sarcoma of the Spine. Spine. 1994; 19:309– 313

Seppala MT, Haltia MJ, Sankila RJ, Jaaskelainen JE, Heiskanen O. Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases. J Neurosurg. 1995; 83:621–626 


Conti P, Pansini G, Mouchaty H, Capuano C, Conti R. Spinal neurinomas: retrospective analysis and longterm outcome of 179 consecutively operated cases and review of the literature. Surg Neurol. 2004; 61:34–43; discussion 44 


Asazuma T, Toyama Y, Maruiwa H, Fujimura Y, Hira- bayashi K. Surgical strategy for cervical dumbbell tumors based on a three-dimensional classification. Spine. 2004; 29:E10–E14 


Dorwart RH, LaMasters DL, Watanabe TJ, Newton TH, Potts DG. In: Tumors. Computed Tomography of the Spine and Spinal Cord. San Anselmo: Clavadal Press; 1983:115–131

Helwig EB, Stern JB. Subcutaneous sacrococcygeal myxopapillary ependymoma. A clinicopathologic study of 32 cases. Am J Clin Pathol. 1984; 81:156–161

Guidetti B, Mercuri S, Vagnozzi R. Long-Term Results of the Surgical Treatment of 129Intramedullary Spinal Gliomas. J Neurosurg. 1981; 54:323–330

Tekkök IH, Açikgöz B, Saglam A, Önol B. Vertebral Hemangioma Symptomatic During Pregnancy - Report of a Case and Review of the Literature. Neurosurgery. 1993; 32:302–306 


Graham JJ, Yang WC. Vertebral Hemangioma with Compression Fracture and Paraparesis Treated with Preoperative Embolization and Vertebral Resection. Spine. 1984; 9:97–101

Vinai Kumar, Abul K. Abbas, Jon C. Aster, Robbins and Contran Pathologic Basis of Disease, Elviser Inc.

Mark S. Greenberg, Handbook of Neurosurgery, Thieme.

Guido Staffa, Elementi di Neurochirurgia, Timeo Editore.

Authors

 

Fabio Paio, MS

Medical Student
Azienda Ospedaliera Universitaria Integrata di Verona (AOUI)
University of Verona (Italy)