Spinal drop metastasis from grade I skull base chondrosarcoma

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Case Reports / Journal of Clinical Neuroscience 17 (2010) 135–137

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Spinal drop metastasis from grade I skull base chondrosarcoma Nick D.P. Hall a,*, Gavin Fabinyi a, Shahid M. Gul b, Lawrence Cher c, Norbert J. Leibsch d a

Department of Neurosurgery, Austin Hospital, PO Box 5555, Heidelberg, Victoria 3084, Australia Department of Neurosurgery, Austin Hospital, Heidelberg, Victoria, Australia c Department of Neuro-Oncology, Austin Hospital, Heidelberg, Victoria, Australia d Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA b

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Article history: Received 14 October 2008 Accepted 24 February 2009

Keywords: Chondrosarcoma Metastasis Spinal

a b s t r a c t Chondrosarcoma of the skull base is a rare tumour with a good prognosis following surgical resection. We describe a patient with low-grade chondrosarcoma of the skull base with intradural extramedullary spinal metastases. A 31-year-old female with grade 1 chondrosarcoma involving the cavernous sinus, sphenoid wing and clivus presented at age 19. The tumour was subtotally excised at initial surgery and over the following 4 years, 3 subsequent resections were undertaken for tumour progression followed by proton beam radiotherapy to the residual tumour. The patient re-presented with cervical radiculopathy 7 years later. MRI showed multiple, intradural extramedullary spinal drop metastases. Following surgical excision of the symptomatic lesion, histological diagnosis was confirmed as a mixed hyaline/myxoid grade 1 chondrosarcoma. Patients with skull base chondrosarcoma with intradural extension should have whole spine imaging as part of long-term monitoring to exclude drop metastases, particularly after intradural surgery. Ó 2009 Elsevier Ltd. All rights reserved.

1. Introduction Intracranial conventional chondrosarcoma is a rare clinical entity. It usually occurs at the skull base and is thought to arise from the cartilage of synchondroses. Patients with these lesions usually present with raised intracranial pressure or cranial nerve palsies. The histological variants can be divided into three subtypes: hyaline, myxoid or mixed. Following tumour resection the reported prognosis is variable, however after proton beam radiotherapy the prognosis is excellent. Metastases from intracranial chondrosarcoma are rarely described and are usually associated with high-grade tumours. A 31-year-old female is reported who re-presented with intradural extramedullary spinal chondrosarcoma metastases 9 years after treatment for intracranial chondrosarcoma.

2. Case report A 19-year-old female presented with diplopia and impaired memory. Clinical examination revealed a right-sided abducens palsy. MRI demonstrated a well defined, enhancing mass in the right parasellar region with extension into the cavernous sinus and clivus (Figs. 1 and 2). Subtotal resection of the parasellar tumour was performed. Histology demonstrated grade I mixed hyaline/myxoid chondrosarcoma. Six months later, the patient underwent a right orbito-zygomatic craniotomy to clear tumour from the cavernous sinus, however residual tumour was left at the superior aspect of the clivus. One year later, a further endoscopic transphenoidal approach was used to remove the residual clival tumour. Two years later, MRI demonstrated a large recurrence of tumour requiring further surgical clearance. The patient was then sent to Massachusetts General Hospital for proton beam radiotherapy. The histology was re-examined and confirmed as grade I mixed hyaline/myxoid conventional chondrosarcoma. Serial follow-up imaging did not demonstrate any evidence of intracranial disease, recurrence or progression.

* Corresponding author. Tel.: +61 3 9496 5000; fax: +61 3 9458 4779. E-mail address: [email protected] (N.D.P. Hall).

Fig. 1. Contrast enhanced axial T1-weighted MRI showing the original right parasellar tumour.

At 31 years of age, the patient re-presented with left arm pain and paraesthesia. MRI of the spine showed multiple intradural extramedullary lesions with cord compression at C3–4 level and lesions at C7, T4, T5, T10 and L4 (Fig. 3). Excision of the compressive lesion improved the symptoms. Histology confirmed a grade I mixed hyaline/myxoid chondrosarcoma, identical to the original biopsies. Given the marked clinical improvement, no further adjuvant therapy was planned. Close serial MRI monitoring of the non-compressive residual spinal metastases is indicated, with a view to consideration of further surgery or proton beam radiotherapy in the event of disease progression.

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Case Reports / Journal of Clinical Neuroscience 17 (2010) 135–137

Fig. 2. Contrast enhanced coronal T1-weighted MRI showing the original right parasellar tumour.

The largest case series of conventional chondrosarcoma reported three histological subtypes: 7.5% were hyaline, 29.5% were myxoid and 63% were the mixed hyaline/myxoid subtype.5 The types were also subclassified by tumour grade: 50.5% were grade I, 28.5% contained areas of grade I and grade II, and 21% were grade II.5 Some difficulty lies in the histopathological distinction between myxoid type chondrosarcoma and chordoma, however the distinction can usually be made using immunohistochemical staining.12 Given the difference in prognosis between these two tumour types, it is important to make this distinction.5 Prognosis is excellent for patients with conventional chondrosarcoma who undergo surgical resection followed by proton beam radiotherapy. The disease-specific survival rate is 99% over 10 years.5 In contrast, the follow-up for patients with chordoma treated with surgery and proton beam radiotherapy shows survival rates of 51% and 35% for 5 and 10 years, respectively.5 Chondrosarcoma in other parts of the body is associated with systemic metastasis, with 10% of grade II tumours resulting in metastasis and 71% of grade III tumours.16 However, metastases from intracranial chondrosarcoma have been rarely reported. In a review of 50 cases of intracranial chondrosarcoma, Hassounah et al. described six cases of metastasis, of which three were highgrade conventional chondrosarcoma and one case exhibited cerebrospinal fluid (CSF) dissemination.17 In 1969, Rengachary and Kepes described a single case of mesenchymal chondrosarcoma with bony metastatic disease, causing spinal cord compression.18 In 1971, Leedham and Swash described a case with disseminated subarachnoid spread of chondrosarcoma.19 Though extremely rare, malignant transformation of skull base chondrosarcoma has been reported, particularly with the mesenchymal subtype.20 The case presented here describes the first report of CSF dissemination in a patient with a low-grade conventional type chondrosarcoma. Based on these findings it is clear that, although rare, CSF dissemination of chondrosarcoma is a definite clinical entity. Given the excellent prognosis conferred with the combination of surgery and proton beam radiotherapy, it may be appropriate to visualise the neuraxis at the time of diagnosis to evaluate the presence of spinal metastasis. References

Fig. 3. Sagittal T1-weighted MRI with contrast showing multiple, enhancing intradural extramedullary tumours.

3. Discussion The exact incidence of chondrosarcoma arising from the cranium is unknown, but it may be as low as 0.16% of all intracranial tumours.1,2 The tumour may be associated with Maffucci’s Syndrome.3 The tumour occurs with equal sex distribution at an average age of 37 years (range: 3 months to 76 years).4 Chondrosarcoma usually arises from the skull base with 66% of cases occurring at the temporo-occipital junction, 28% at the clivus and 6% in the spheno-ethmoidal complex.5 Chondrosarcoma above the skull base has been reported to arise from the choroid plexus, dura mater or brain parenchyma.6–11 Chondrosarcoma is subclassified into conventional, de-differentiated, clear cell and mesenchymal subtypes. The most common is the conventional subtype.12,13 The clinical presentation of chondrosarcoma of the skull base has been well described. Commonly, patients present with long standing headaches and signs relating to raised intracranial pressure. Cranial nerve palsies, hemiparesis and deafness can also occur, as can dizziness and tinnitus.4,14,15

1. Berkmen YM, Blatt ES. Cranial and intracranial cartilaginous tumours. Clin Radiol 1968;19:327–33. 2. Cianfriglia F, Pompili A, Acchipinti E. Intracranial malignant cartilaginous tumours. Report of 2 cases and review of literature. Acta Neurochir 1978;45:163–75. 3. Tachibana E, Saito K, Takahashi M. Surgical treatment of a massive chondrosarcoma of the skull base associated with Maffucci’s syndrome: a case report. Surg Neurol 2000;54:165–9. 4. Korten AG, ter Berg HJ, Spincemaille GH, et al. Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry 1988;65:88–92. 5. Rosenberg AE, Nielsen GP, Keel SB. Chondrosarcoma of the base of the skull: A clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 1999;23:1370. 6. Salcman M, Scholtz H, Kristt D, et al. Extra-skeletal myxoid chondrosarcoma of the falx. Neurosurgery 1992;31:344–8. 7. Hardy Jr RW, Benjamin SP, Gardner WJ. Prolonged survival following excision of dural chondroma. Case report. J Neurosurg 1978;48:125–7. 8. Mapstone TB, Wongmongkolrit T, Roessman U, et al. Intradural chondroma: A case report and review of the literature. Neurosurgery 1983;12:111–4. 9. Ozgen T, Pamir MN, Akalan N, et al. Intracranial solitary chondroma. Case Report. J Neurosurg 1984;61:399–401. 10. Yang PJ, Seeger JF, Carmody RF, et al. Chondroma of falx: CT findings. J Comput Assist Tomo gr 1986;10:1075–6. 11. Ahyai A. Spoerri. Intracerebral chondroma. Surg Neurol 1979;11:431–3. 12. Rosenberg AE, Brown GA, Bhan AK, et al. Chondroid chordoma–a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol 1984;101:36–41. 13. Rosenberg AE, Nielsen GP, Efrid JT, et al. Base of skull chondrosarcomas. Clinicopathalogic study of 130 cases. Mod Pathol 1997;9:12A.

Case Reports / Journal of Clinical Neuroscience 17 (2010) 137–138 14. Currie J, Lubin JH, Lessell S. Chronic isolated abducens paresis from tumours at the base of the brain. Arch Neurol 1983;40:226–9. 15. Gay E, Sekhar LN, Rubenstein E, et al. Chordomas and chondrosarcomas of the cranial base: results and follow up of 60 patients. Neurosurgery 1995;36:887–97. 16. Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of bone. A clinicopathological analysis with emphasis on histologic grading. Cancer 1977;40:818–31.

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17. Hassounah M, Al-Mefty O, Akhtar M, et al. Primary cranial and intracranial chondrosarcoma: A survey. Acta Neurochir Wein 1985;78:128–32. 18. Rengachary SS, Kepes JJ. Spinal epidural metastatic ‘‘mesenchymal” chondrosarcoma. J Neurosurg 1969;30:71–3. 19. Leedham PW, Swash M. Chondrosarcoma with subarachnoid dissemination. J Pathol 1972;107:59–61. 20. Tibbs RE, Bowles AP, Raila FA. Maffucci’s syndroma and intracranial chondrosarcoma. Skull Base Surg 1997;7:49–55.

doi:10.1016/j.jocn.2009.02.033

Multiple spinal dural arteriovenous fistulae and deterioration post lumbar puncture A.M. Foote a,*, S.P.C. Bower a, R.A. Danks a,b, W. Chong a a b

Monash Medical Centre, Clayton Campus, 246 Clayton Road, Clayton, Victoria 3168, Australia Department of Surgery, Monash University, Clayton, Victoria, Australia

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Article history: Received 31 March 2008 Accepted 24 February 2009

a b s t r a c t We report a 73-year-old man presenting with bilateral leg numbness and weakness. Three spinal dural arteriovenous fistulae (SDAVF) were found after clinical deterioration post lumbar puncture (LP). This patient highlights the difficulties in the diagnosis and management of SDAVF, and the risks of LP. Crown Copyright Ó 2009 Published by Elsevier Ltd. All rights reserved.

Keywords: Spinal dural arteriovenous fistulae Lumbar puncture

1. Introduction Spinal dural arteriovenous fistulae (SDAVF) are an uncommon but serious cause of myelopathy. They account for 70% of spinal arteriovenous malformations and occur predominantly in older males.1 Without treatment, 50% of patients will be severely disabled within 3 years from onset.2 Diagnosis is often made by MRI

then confirmatory spinal angiography. Treatment is either neurosurgical ligation and/or endovascular embolisation.3

2. Case report A 73-year-old man was admitted to our hospital with a 3-month history of bilateral lower limb numbness and weakness. He denied any arm, neck or trunk symptoms, and did not have disturbance of the bladder or bowel. Examination of his lower limbs revealed mildly increased tone bilaterally, normal power, asymmetric reflexes (the left brisker than the right) and bilateral extensor plan-

Fig. 1. Sagittal T2-weighted MRI of the cervical and thoracic spine showing hyperintensity of the cervical cord (arrow) consistent with cord oedema.

* Corresponding author. Tel.: +61 3 9594 6666; fax: +61 3 9594 6111. E-mail address: [email protected] (A.M. Foote).

Fig. 2. Sagittal T1-weighted MRI contrast-enhanced fat-saturated sequence of the lower thoracic spine showing enhancing enlarged draining perimedullary veins (arrows).

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