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|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 1 | Page : 114-116
Primary spinal extraosseous cervical chondroma in an adult
Amol Raheja1, Sachin A Borkar1, Aasma Nalwa2, Vaishali Suri2
1 Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||4-Mar-2015|
Sachin A Borkar
Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Raheja A, Borkar SA, Nalwa A, Suri V. Primary spinal extraosseous cervical chondroma in an adult. Neurol India 2015;63:114-6
A 25-year-old male patient presented with ascending spastic quadriparesis and graded sensory loss below the C4 dermatome for 10 months without any history of sphincteric involvement. His imaging revealed a cervical, intradural extramedullary lesion [Figure 1]a-c. During surgery, a fibrous, moderately vascular, greyish tumor in close proximity to the left ventrolateral dura was seen compressing the cord toward the right side. Histopathology revealed a cartilaginous tumor arranged in lobules with mild pleomorphism but without any evidence of mitosis. Immunopositivity for S100 confirmed a diagnosis of a chondroma [Figure 2]. At a 1-year follow-up, the patient was clinically stable. The follow-up scan showed a small, residual nodular enhancement [Figure 1]d-f. The patient has been kept on a regular follow-up.
|Figure 1: Axial (a), coronal (b), and sagittal (c) preoperative contrast MRI spine demonstrated a solitary left sided 40 × 35 × 25 mm intradural extramedullary lesion (arrow) at C4– 5 level with heterogeneous peripheral ring enhancement and compressing the cord to right side. Axial (d), coronal (e), and sagittal (f) postoperative contrast MRI spine demonstrated a small residual nodular enhancement (arrow) measuring 9 × 4 mm along with mild peripheral dural enhancement and decompressed cord on right side. MRI = Magnetic resonance imaging|
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|Figure 2: Demonstrates a lobular cartilaginous tumor (A; H and E, ×100), cells are showing mild pleomorphism (B; H and E, ×200) with immunopositivity for S100 (C; IHC, ×200). H and E = Hematoxylin and eosin, IHC = immunohistochemistry|
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Chondromas accounts for 4% of all primary spinal tumors, have a male predilection (2:1) and a peak incidence in the 2 nd and 3 rd decade.  They are further classified based on the site of origin into enchondromas (arising from the medullary cavity) and periosteal chondromas (arising from the surface of periosteum).  They arise from any part of the vertebrae with the neural arch being the commonest site of origin.  The patients may present with nerve root or spinal cord compression, local pain, palpable mass, and/or pathological fracture; or, may be diagnosed incidentally if asymptomatic.  Till date, only 12 cases of a cervical chondroma have been reported in literature, ,,,,,,,,,,, all having an osseous origin and an extradural compression (if at all present). Thus, they may be categorized into either an enchondroma or a periosteal chondroma. We report the first case of an intraspinal, extraosseous, intradural primary cervical chondroma in an adult.
The natural history of an osseous extradural spinal chondroma is usually benign with a late presentation of neurological symptoms and a recurrence rate of <10% after complete resection.  In contrast, spinal extraosseous intradural, extramedullary chondromas are more aggressive clinically with an early presentation and a higher chance of recurrence. This is due to the difficulties encountered in their complete resection. Based on the prevelant hypotheses for origin of dural-based chondromas in the intracranial compartment,  we postulate that the intradural variant of spinal chondromas arise from either heterotopic chondrocytes; secondary to metaplasia from meningeal fibroblasts or perivascular mesenchymal tissue; or, from meningeal connective tissue secondary to cartilaginous activation of fibroblasts by trauma or inflammation. Chondromas can undergo a sarcomatous change (especially in the setting of Ollier's or Maffucci's syndrome) , or a metaplasia to a fibrochondroma or myxochondroma.  Tumor size <7 cm and cortical destruction with a soft tissue component are the radiological hallmarks of malignant degeneration in an osseous chondroma. ,
Although not pathognomonic, well-circumscribed solitary lesions with stippled calcification, a characteristic pattern of ring-like mild peripheral enhancement with central nonenhancing area; heterogeneously hyperintense appearance on T2-weighted imaging; absence of dural tail, hemorrhage, or cystic areas; and lack of tumor blush on angiography should suggest the radiological diagnosis of a chondroma. , The differential diagnosis includes a mesenchymal chondrosarcoma, meningioma, schwannoma, hemangiopericytoma, solitary fibrous tumor, Ewing's sarcoma, and metastatic tumor.  Histologically, the chondroma has a well-differentiated cartilaginous tissue surrounded by a delicate fibrous connective tissue capsule. Bland chondrocytes with no evidence of multinucleation, mitotic activity, or pleomorphism are evident among copious chondroid matrices.  Immunostaining reveals S100 positivity and absence of epithelial markers. Cellular atypia is an indicator of possible malignant transformation.  The primary microscopic differential diagnosis is a well-differentiated chondrosarcoma. The treatment of choice is radical excision of the tumor (if feasible) with preservation of vital neurovascular structures, and maintenance of spinal stability.  Preoperative computed tomography (CT)-guided biopsy may be relevant for appropriate surgical planning.  Subtotal excision, curettage, and chemical cauterization of the tumor cavity are other viable options.  Despite a few isolated cases of intracranial chondroma showing tumor control by proton or photon beam radiotherapy,  the role of the latter modalities is limited because of the radioresistant nature of these lesions, adverse effect on adjacent neural tissue, and a higher propensity to having a subsequent malignant degeneration.
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[Figure 1], [Figure 2]
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