- Email: [email protected]
Paraganglioma of the thoracic spine
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
contents, we expected to be able to perform uncomplicated dissection of the cyst wall. Although gross total excision was performed no additional neurodeficits, clinical signs or recurrence was seen on MRI at a 1-year follow-up. In conclusion, interdural cavernous dermoid tumors must be evaluated radiologically in order to develop an appropriate operative plan. Total removal should not be attempted when these lesions are tightly adherent to basal neurovascular structures. References 1. Alvord Jr EC. Growth rates of epidermoid tumors. Ann Neurol 1977;2:367–70. 2. Baxter JW, Netsky MG. Epidermoid and dermoid tumors: Pathology. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, Vol. 1. New York: McGraw-Hill; 1985. p. 655–61. 3. Conley FK. Epidermoid and dermoid tumors: Clinical features and surgical management. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, Vol.1. New York: McGraw-Hill; 1985. p. 668–73. 4. Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumors. Neurochirurgia 1976;19:104–14. 5. Abdelouafi A, Ousehal A, Gharbi A, et al. Cavernous sinus dermoid cyst with unusual parapharyngeal development. J Neuroradiol 2000;27:140–3.
823
6. DeMonte F, Al-Mefty O. Ruptured dermoid tumor of the cavernous sinus associated with the syndrome of fat embolism: case report. J Neurosurg 1992;77:312–5. 7. Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. 8. Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and late surgical results. J Neurosurg 1977;47:12–8. 9. Yasargil MG, Abernathey CD, Sarioglu AC. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561–7. 10. El-Kalliny M, Van Loveren H, Keller JT, et al. Tumors of the lateral wall of the cavernous sinus. J Neurosurg 1992;77:508–14. 11. Nakagawa K, Ohno K, Nojiri T, et al. Interdural dermoid cyst of the cavernous sinus presenting with oculomotor palsy: case report. No Shinkei Geka 1997;25:847–51. 12. Hirsh WL, Hrynshko FG, Sekhar LN, et al. Comparison of MR imaging, CT and angiography in the evaluation of the enlarged cavernous sinus. Am J Rheumatol 1988;151:1015–23. 13. Fournier D, Mercier P, Menei P, et al. Recurrent intrinsic brainstem epidermoid cyst. Childs Nerv Syst 1992;8:471–4. 14. Yamakawa K, Shitara N, Genka S, et al. Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 1989;24:568–73. 15. Akdemir G, Daglioglu E, Ergungor MF. Dermoid lesion of the cavernous sinus: case report and review of the literature. Neurosurg Rev 2004;27:294–8.
doi:10.1016/j.jocn.2006.08.018
Paraganglioma of the thoracic spine Mehmet Zileli a, Murat Kalayci b,*, Gu¨lcßin Basßdemir c a
b
Ege University, Izmir, Turkey Zonguldak Karaelmas Universitesi Rektorlugu, PK:67100 Incivez, Zonguldak, Turkey c Micropathology Laboratory, Izmir, Turkey Received 12 April 2006; accepted 23 July 2006
Abstract A 49-year-old man was admitted to hospital for treatment of a T10–11 paraspinal lesion, which was detected incidentally. He had suffered from facial flushing and palpitations associated with hypertension for many years. MRI confirmed the presence of a large, well-demarcated tumour in the left paraspinal area of the T10–11 vertebral bodies. Grossly complete excision was achieved without neurological complications. Post-operatively, the patient was normotensive with no adrenergic symptoms. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Spine tumour; Paraganglioma; Primary spine tumour
*
Corresponding author. Tel.: +90 372 257 40 10 11 12 13; fax: +90 372 257 21 40 0 372 257 50 78. E-mail address: [email protected] (M. Kalayci).
824
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
1. Introduction Paragangliomas are tumours that arise from the paraganglia cells of the autonomic nervous system, ultimately derived from the neural crest.1 The majority of paragangliomas are non-functional and benign; however, they may produce catecholamine excess or have malignant potential.2,3 Paragangliomas occur within or near the carotid body or glomus jugulare in 80–90% of cases.1,4–8 Other locations are the middle ear, thyroid, gastrointestinal tract and pancreas.7,8 Paragangliomas of the central nervous system have been observed in the pineal region, petrous ridge and sella turcica.5,7,9 Paragangliomas of the spine are uncommon lesions that usually present as intradural tumours within the cauda equina.3–5,7,9–12 Thoracic paragangliomas are distinctly unusual. We describe a case of thoracic spinal paraganglioma that was detected incidentally.
paraspinal region of the T10–11 vertebrae (Figs. 2 and 3). The tumour exhibited marked contrast enhancement with gadolinium, and multiple areas of signal void were present on T2-weighted images. A midline skin incision was made at the T9–12 level. A costotransversectomy was performed at the left T10 level. The hypervascular tumour was dis-
2. Case report A 49-year-old man was admitted to hospital for treatment of a paraspinal lesion of the T10–11 vertebral bodies, which was detected incidentally. In 2003, while the patient was undergoing evaluation because of spontaneous pneumothorax, a chest X-ray revealed an incidental left paraspinal tumour (Fig. 1). He had suffered from facial flushing and palpitations for many years. He was hypertensive, but he was not taking any medication. MRI confirmed the presence of a large, well-demarcated tumour in the left
Fig. 2. Coronal T1-weighted MRI obtained after gadolinium administration, showing a left paraspinal soft tissue mass.
Fig. 1. Chest X-ray showing a left paraspinal tumour.
Fig. 3. Contrast-enhanced axial MRI at the T10–11 level shows a left paraspinal soft tissue mass with extension into the neural foramen, without cord compression.
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
825
Fig. 4. (A) Histological image showing tumour cells with granular eosinophilic cytoplasm and ovoid nuclei (haematoxylin and eosin). Sections stained positively for synaptophysin (B), chromogranin (C), and S-100 (D). (This figure is available in colour at www.sciencedirect.com.)
sected away from the paraspinal tissues. A relatively welldefined cleavage plane was present. A T10 foraminotomy was performed. The intracanalicular component of the tumour was excised without damage to the T10 nerve root. Transient systolic blood pressure elevation up to 240 mmHg occurred during tumour manipulation, which responded well to intravenous sodium nitroprusside and
phentolamine. A histological examination yielded a diagnosis of paraganglioma (Fig. 4). Post-operatively, the patient made an excellent recovery, with no neurological deficits. At a 45-day post-operative follow-up examination, he was normotensive. Post-operative MRI did not reveal any residual tumour (Fig. 5). Abdominal and chest CT scans for multiple endocrine neoplasia syndromes yielded negative results. At an 18-month follow-up, he had no complaints of flushing, and his blood pressure was normal without antihypertensive drugs. 3. Discussion
Fig. 5. Contrast-enhanced axial MR image obtained in the second postoperative year. No residual tumour can be seen.
Pheochromocytomas are catecholamine-secreting tumours that represent an important but rare cause of hypertension, accounting for approximately 0.5% of the hypertensive population. Extra-adrenal pheochromocytomas of the neural crest-derived sympathetic ganglia are known as paragangliomas and account for 15% of all pheochromocytomas.2 Paragangliomas, unlike adrenal pheochromocytomas, are rarely functional, and 80 to 90% arise in the glomus jugulare or carotid bodies. Paragangliomas are more likely to be malignant (29–40%) than adrenal pheochromocytomas (10–15%).2 Retroperitoneal paragangliomas are more often malignant (28–50%) than those in other locations, as defined by the presence of metastatic
826
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
lesions.3 Metastases to bone, liver, lymph nodes and lungs may occur after many years (1–23 years). The local recurrence rate of paragangliomas is 12%, even if follow-up is extended to 30 years.8,14 Vertebral metastases have been noted, as have metastases to other extradural locations in the spine; some have led to spinal cord compression.6,13 Although approximately 75% of pheochromocytomas arise as sporadic tumours, they are also known to occur as part of several familial syndromes, such as multiple endocrine neoplasia type IIA, IIB or III, von Hippel-Lindau disease, von Recklinghausen’s disease, and Sturge-Weber syndrome.10,15,16 Paragangliomas are considered to be an infrequent feature of von Hippel-Lindau disease, present on average in 14% of cases.16 Patients with pheochromocytomas often present with symptoms secondary to catecholamine excess. Hypertension (either sustained or episodic in nature), headache, palpitations, sweating, and weight loss are common presenting symptoms. Such symptoms are almost always accompanied by elevated levels of one or more of the catecholamines in the blood or increased levels of their metabolites in urine.15 The clinical appearance of these tumours when they do not secrete catecholamines is nonspecific.14 The diagnosis of pheochromocytoma or functioning paraganglioma is made on the basis of measurement of the levels of catecholamines and their metabolites in plasma and urine.2,15 The location of the tumour can usually be identified with abdominal CT or MRI. The iodine-131-metaiodobenzylguanidine (123I-MIBG) scanning approach relies on nuclide uptake by the tumour and is particularly useful for locating extra-adrenal tumours.2,10,15 123I-MIBG uptake appears to correlate more with storage capacity rather than with actual secretion of catecholamines.10 The MRI features of spinal paragangliomas include well-demarcated masses with low/intermediate signal intensity on T1-weighted scans and intermediate/high signal intensity on T2-weighted scans, in comparison with neighbouring paravertebral tissues. Gadolinium administration produces intense heterogeneous enhancement, and high-velocity flow through the hypervascular tumours produces multiple serpiginous areas of signal void in all sequences.2 Because the majority of paragangliomas are benign, most patients can be treated successfully by surgical excision of the tumour. Malignant paragangliomas can also be treated surgically, but the prognosis depends primarily on whether the tumour metastasises.13,17 Spinal metastatic paragangliomas have led to pathologic fractures and spinal cord compression. The best combination of therapy for metastatic spinal paragangliomas appears to be surgical decompression followed by a course of radiotherapy.6 Jindel et al. reported 80–90% local control with radiation.18 Preoperative treatment of patients with pheochromocytomas and functioning paragangliomas is critical. At least 2 weeks of management of hypertension with an aanti-adrenergic agent is required to allow the chronically contracted extravascular space to expand.2,15 Management of hypertension with b-anti-adrenergic agent alone is con-
traindicated, because such treatment can cause a paradoxical increase in blood pressure that is attributable to the inhibition of peripheral b2 receptors.2 Gentle surgical techniques and expert anaesthesia are required to minimise intraoperative hypertension attributable to handling of the tumour.2,15 Hypertension during the course of the procedure can be managed with intravenous phentolamine, sodium nitroprusside, or deepening of the maintenance anaesthetic. Hypotension, which can occur after tumour resection, can be managed with the administration of volume or, if necessary, pressors.19 Paragangliomas have distinctive histological features. Clusters of large polyhedral chief cells are arranged in nests (Zellballen), surrounded by a delicate fibrovascular stroma. The tumour cells have granular eosinophilic cytoplasm and round or ovoid nuclei. There is positive staining for synaptophysin, chromogranin, S-100, and tumour-specific antigen.2 Malignancy cannot be determined with histological assessments but is demonstrated by metastases, recurrences and invasion.2,15,19 Paragangliomas involving the spine are rare, with approximately 90 cases reported in the literature.1–31 The vast majority of tumours are intradural and are found within the cauda equina (43%).4,10,11,16 Metastatic spinal lesions represent 35% of all spinal paragangliomas. Cervical (2%) and sacral (3%) paragangliomas are extremely rare. Thoracic paragangliomas are rare (17%). Only 13 thoracic spinal paragangliomas have been reported, and cord compression was the presenting feature in every case,1,2,8,14–16,18,25,27,29–31 10 of which were located extradurally1,2,14,15,18,27,29–31 and two of which were functional.2,15 Three of those cases involved metastases.18,29 Therefore, it has been suggested that metastatic spread and an extradural location are characteristic features of thoracic paragangliomas, compared with the cauda equina variant.1,2 The case in the present report was a thoracic paraganglioma that was extradural and functional. Our case of paraganglioma is the first to be resected by costotransversectomy. Long-term post-operative surveillance is essential, because paragangliomas have a higher rate of delayed recurrence than pheochromocytomas, and onset of symptoms likely heralds recurrence of the tumour.7,14,15 Standard follow-up includes annual measurement of blood pressure and urinary catecholamine metabolites. The selected use of CT or MRI may yield additional information.15 4. Conclusion Paraganglioma of the spine will seldom be considered in a presurgical differential diagnosis due to its rarity and nonspecific imaging features. Adequate treatment requires total surgical removal. In the present case, resection of the paraganglioma resulted in elimination of the catecholamine excess and the resulting hypertension. We present only the 14th documented case of a primary thoracic spine paraganglioma and only the third to present with adrenergic symptoms.
Case Reports / Journal of Clinical Neuroscience 15 (2008) 827–830
References 1. Houten JK, Babu RP, Miller DC. Thoracic paraganglioma presenting with spinal cord compression and metastases. J Spinal Disord Tech 2002;15:319–23. 2. Jeffs GJ, Lee GYF, Wong GTH. Functioning paraganglioma of the thoracic spine: case report. Neurosurgery 2003;53:992–5. 3. Coles CP, Alexander DI, Gross M, et al. Intraosseous paraganglioma of the sacrum: a case report. CJS 2000;43:137–9. 4. Sundgren P, Annertz M, Englund E, et al. Paragangliomas of the spinal canal. Neuroradiology 1999;41:788–94. 5. Boncoeur-Martel MP, Lesort A, Moreau JJ, et al. MRI of paraganglioma of the filum terminale. J Comput Assist Tomogr 1996;20:162–5. 6. Brodkey JA, Brodkey JS, Watridge CB. Metastatic paraganglioma causing spinal cord compression. Spine 1995;20:367–72. 7. Faro SH, Turtz AR, Koennigsberg RA, et al. Paraganglioma of the cauda equina with associated intramedullary cyst: MR findings. AJNR 1997;18:1588–90. 8. Silverstein AM, Quint DJ, McKeever PE. Intradural paraganglioma of the thoracic spine. AJNR 1990;11:614–6. 9. Lamer S, Carlier RY, Parker F, et al. Paraganglioma of the cauda equine: MR findings. One case. J Neuroradiol 1997;24:215–7. 10. Camera L, Biondi B, Daniele S, et al. Screening in von Hippel-Lindau disease: concurrent pheochromocytomas, paragangliomas and spinal hemangioblastomas revealed by helical-CT, MIBG scintigraphy and MRI in an asymptomatic patient. Eur J Radiol Extra 2003;48: 8–13. 11. Moran CA, Albores-Saavedra J, Wenig BM, et al. Pigmented extraadrenal paragangliomas. Cancer 1997;79:398–402. 12. Herman M, Pozzi-Mucelli RS, Skrap M. Paraganglioma of the cauda equina: case report and review of the MRI features. Acta Univ Polacki Olomus Fak Med 1998;141:27–30. 13. Absher KJ, Witte DA, Truong LD, et al. Aspiration biopsy of osseous metastasis of retroperitoneal paraganglioma. Acta Cytologica 2001;45:249–53. 14. Cybulski GR, Nijensohn E, Brody BA, et al. Spinal cord compression from a thoracic paraganglioma: case report. Neurosurgery 1991;28:306–9. 15. Spector JA, Willis DN, Ginsburg HB. Paraganglioma (pheochromocytoma) of the posterior mediastinum: a case report and review of the literature. J Pediatr Surg 2003;38:1114–6.
827
16. Mossner R, Keidel M. Malignant pheochromocytoma with progressive paraparesis in von Hippel-Lindau disease. Eur J Neurol 2000;7:439–42. 17. Teno S, Tanabe A, Nomura K, et al. Acutely exacerbated hypertension and increased inflammatory signs due to radiation treatment for metastatic pheochromocytoma. Endocrine J 1996;43:511–6. 18. Jindel R, Gupta AK, Mahapatra AK, et al. Extradural paraganglioma with multiple skeletal metastases. Br J Radiol 1992;65: 938–40. 19. Olson JJ, Loftus CM, Hitchon PW. Metastatic pheochromocytoma of the cervical spine. Spine 1989;14:349–51. 20. Gabriel EM, Sampson JH, Dodd LG, et al. Glomus jugulare tumor metastatic to the sacrum after high dose radiation therapy: case report. Neurosurgery 1995;37:1001–5. 21. Chiras J, Cognard C, Rose M, et al. Percutaneous injection of an alcoholic embolizing emulsion as an alternative preoperative embolization for spine tumor. AJNR 1993;14:1113–7. 22. North CA, Zinreich ES, Christenhsen WN, et al. Multiple spinal metastases from paraganglioma. Cancer 1990;66:2224–8. 23. Osborn RE, Mojtahedi S. Paraganglioma metastatic to the cervical spine. Computerized Radiol 1986;10:167–70. 24. Occhiogrosso M, De Tommasi A, Vailati G, et al. Malignant chemodectoma of the carotid body causing spinal cord compression. Surg Neurol 1985;23:14–8. 25. Solymosi L, Ferbert A. A case of spinal paraganglioma. Neuroradiol 1985;27:217–9. 26. Sankla S, Khan GM. Cauda equina paraganglioma presenting with intracranial hypertension: case report and review of the literature. Neurol India 2004;52:243–4. 27. Shin JY, Lee SM, Hwang MY, et al. MR findings of the spinal paraganglioma: report of three cases. J Korean Med Sci 2001;16:522–6. 28. Mori S, Okura T, Kitami Y, et al. A case metastatic extra-adrenal pheochromocytoma 12 years after surgery. Hypertens Res 2002;25:141–4. 29. Constantini S, Soffer S, Siegal T, et al. Paraganglioma of the thoracic spine with cerebrospinal fluid metastases. Spine 1989;14:643–5. 30. Fitzgerald L, Cech D, Goodman J. Paraganglioma of the thoracic spinal cord. Clin Neurol Neurosurg 1996;98:183–5. 31. Sato N, Imai T, Aikawa H, et al. Recurrence and pulmonary metastasis of extradural paraganglioma in thoracic vertebral canal: report of a case. Kyobu Geka 2001;54:610–3.
doi:10.1016/j.jocn.2006.07.024
Ependymal cyst in the lumbar spine associated with cauda equina compression Minori Kato a, Hiroaki Nakamura b,*, Eisuke Suzuki c, Hidetomi Terai a, Kenichi Wakasa d, Tomoko Wakasa d, Kunio Takaoka a a
Department of Orthopaedic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan b Department of Orthopaedic Surgery, Osaka City General Hospital, Osaka, Japan c Department of Orthopaedic Surgery, Osaka Prefectural Rehabilitation Center, Sakai, Japan d Department of Clinical Pathology, Osaka City University Graduate School of Medicine, Osaka, Japan Received 24 August 2006; accepted 25 December 2006
*
Corresponding author. Present address: 2-13-22, Miyakojimahondori Miyakojima-ku, Osaka 534-0021, Japan. Tel.: +81 6 6645 3851; fax: +81 6 6646 6260. E-mail address: [email protected] (H. Nakamura).
contents, we expected to be able to perform uncomplicated dissection of the cyst wall. Although gross total excision was performed no additional neurodeficits, clinical signs or recurrence was seen on MRI at a 1-year follow-up. In conclusion, interdural cavernous dermoid tumors must be evaluated radiologically in order to develop an appropriate operative plan. Total removal should not be attempted when these lesions are tightly adherent to basal neurovascular structures. References 1. Alvord Jr EC. Growth rates of epidermoid tumors. Ann Neurol 1977;2:367–70. 2. Baxter JW, Netsky MG. Epidermoid and dermoid tumors: Pathology. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, Vol. 1. New York: McGraw-Hill; 1985. p. 655–61. 3. Conley FK. Epidermoid and dermoid tumors: Clinical features and surgical management. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, Vol.1. New York: McGraw-Hill; 1985. p. 668–73. 4. Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumors. Neurochirurgia 1976;19:104–14. 5. Abdelouafi A, Ousehal A, Gharbi A, et al. Cavernous sinus dermoid cyst with unusual parapharyngeal development. J Neuroradiol 2000;27:140–3.
823
6. DeMonte F, Al-Mefty O. Ruptured dermoid tumor of the cavernous sinus associated with the syndrome of fat embolism: case report. J Neurosurg 1992;77:312–5. 7. Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. 8. Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and late surgical results. J Neurosurg 1977;47:12–8. 9. Yasargil MG, Abernathey CD, Sarioglu AC. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561–7. 10. El-Kalliny M, Van Loveren H, Keller JT, et al. Tumors of the lateral wall of the cavernous sinus. J Neurosurg 1992;77:508–14. 11. Nakagawa K, Ohno K, Nojiri T, et al. Interdural dermoid cyst of the cavernous sinus presenting with oculomotor palsy: case report. No Shinkei Geka 1997;25:847–51. 12. Hirsh WL, Hrynshko FG, Sekhar LN, et al. Comparison of MR imaging, CT and angiography in the evaluation of the enlarged cavernous sinus. Am J Rheumatol 1988;151:1015–23. 13. Fournier D, Mercier P, Menei P, et al. Recurrent intrinsic brainstem epidermoid cyst. Childs Nerv Syst 1992;8:471–4. 14. Yamakawa K, Shitara N, Genka S, et al. Clinical course and surgical prognosis of 33 cases of intracranial epidermoid tumors. Neurosurgery 1989;24:568–73. 15. Akdemir G, Daglioglu E, Ergungor MF. Dermoid lesion of the cavernous sinus: case report and review of the literature. Neurosurg Rev 2004;27:294–8.
doi:10.1016/j.jocn.2006.08.018
Paraganglioma of the thoracic spine Mehmet Zileli a, Murat Kalayci b,*, Gu¨lcßin Basßdemir c a
b
Ege University, Izmir, Turkey Zonguldak Karaelmas Universitesi Rektorlugu, PK:67100 Incivez, Zonguldak, Turkey c Micropathology Laboratory, Izmir, Turkey Received 12 April 2006; accepted 23 July 2006
Abstract A 49-year-old man was admitted to hospital for treatment of a T10–11 paraspinal lesion, which was detected incidentally. He had suffered from facial flushing and palpitations associated with hypertension for many years. MRI confirmed the presence of a large, well-demarcated tumour in the left paraspinal area of the T10–11 vertebral bodies. Grossly complete excision was achieved without neurological complications. Post-operatively, the patient was normotensive with no adrenergic symptoms. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Spine tumour; Paraganglioma; Primary spine tumour
*
Corresponding author. Tel.: +90 372 257 40 10 11 12 13; fax: +90 372 257 21 40 0 372 257 50 78. E-mail address: [email protected] (M. Kalayci).
824
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
1. Introduction Paragangliomas are tumours that arise from the paraganglia cells of the autonomic nervous system, ultimately derived from the neural crest.1 The majority of paragangliomas are non-functional and benign; however, they may produce catecholamine excess or have malignant potential.2,3 Paragangliomas occur within or near the carotid body or glomus jugulare in 80–90% of cases.1,4–8 Other locations are the middle ear, thyroid, gastrointestinal tract and pancreas.7,8 Paragangliomas of the central nervous system have been observed in the pineal region, petrous ridge and sella turcica.5,7,9 Paragangliomas of the spine are uncommon lesions that usually present as intradural tumours within the cauda equina.3–5,7,9–12 Thoracic paragangliomas are distinctly unusual. We describe a case of thoracic spinal paraganglioma that was detected incidentally.
paraspinal region of the T10–11 vertebrae (Figs. 2 and 3). The tumour exhibited marked contrast enhancement with gadolinium, and multiple areas of signal void were present on T2-weighted images. A midline skin incision was made at the T9–12 level. A costotransversectomy was performed at the left T10 level. The hypervascular tumour was dis-
2. Case report A 49-year-old man was admitted to hospital for treatment of a paraspinal lesion of the T10–11 vertebral bodies, which was detected incidentally. In 2003, while the patient was undergoing evaluation because of spontaneous pneumothorax, a chest X-ray revealed an incidental left paraspinal tumour (Fig. 1). He had suffered from facial flushing and palpitations for many years. He was hypertensive, but he was not taking any medication. MRI confirmed the presence of a large, well-demarcated tumour in the left
Fig. 2. Coronal T1-weighted MRI obtained after gadolinium administration, showing a left paraspinal soft tissue mass.
Fig. 1. Chest X-ray showing a left paraspinal tumour.
Fig. 3. Contrast-enhanced axial MRI at the T10–11 level shows a left paraspinal soft tissue mass with extension into the neural foramen, without cord compression.
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
825
Fig. 4. (A) Histological image showing tumour cells with granular eosinophilic cytoplasm and ovoid nuclei (haematoxylin and eosin). Sections stained positively for synaptophysin (B), chromogranin (C), and S-100 (D). (This figure is available in colour at www.sciencedirect.com.)
sected away from the paraspinal tissues. A relatively welldefined cleavage plane was present. A T10 foraminotomy was performed. The intracanalicular component of the tumour was excised without damage to the T10 nerve root. Transient systolic blood pressure elevation up to 240 mmHg occurred during tumour manipulation, which responded well to intravenous sodium nitroprusside and
phentolamine. A histological examination yielded a diagnosis of paraganglioma (Fig. 4). Post-operatively, the patient made an excellent recovery, with no neurological deficits. At a 45-day post-operative follow-up examination, he was normotensive. Post-operative MRI did not reveal any residual tumour (Fig. 5). Abdominal and chest CT scans for multiple endocrine neoplasia syndromes yielded negative results. At an 18-month follow-up, he had no complaints of flushing, and his blood pressure was normal without antihypertensive drugs. 3. Discussion
Fig. 5. Contrast-enhanced axial MR image obtained in the second postoperative year. No residual tumour can be seen.
Pheochromocytomas are catecholamine-secreting tumours that represent an important but rare cause of hypertension, accounting for approximately 0.5% of the hypertensive population. Extra-adrenal pheochromocytomas of the neural crest-derived sympathetic ganglia are known as paragangliomas and account for 15% of all pheochromocytomas.2 Paragangliomas, unlike adrenal pheochromocytomas, are rarely functional, and 80 to 90% arise in the glomus jugulare or carotid bodies. Paragangliomas are more likely to be malignant (29–40%) than adrenal pheochromocytomas (10–15%).2 Retroperitoneal paragangliomas are more often malignant (28–50%) than those in other locations, as defined by the presence of metastatic
826
Case Reports / Journal of Clinical Neuroscience 15 (2008) 823–827
lesions.3 Metastases to bone, liver, lymph nodes and lungs may occur after many years (1–23 years). The local recurrence rate of paragangliomas is 12%, even if follow-up is extended to 30 years.8,14 Vertebral metastases have been noted, as have metastases to other extradural locations in the spine; some have led to spinal cord compression.6,13 Although approximately 75% of pheochromocytomas arise as sporadic tumours, they are also known to occur as part of several familial syndromes, such as multiple endocrine neoplasia type IIA, IIB or III, von Hippel-Lindau disease, von Recklinghausen’s disease, and Sturge-Weber syndrome.10,15,16 Paragangliomas are considered to be an infrequent feature of von Hippel-Lindau disease, present on average in 14% of cases.16 Patients with pheochromocytomas often present with symptoms secondary to catecholamine excess. Hypertension (either sustained or episodic in nature), headache, palpitations, sweating, and weight loss are common presenting symptoms. Such symptoms are almost always accompanied by elevated levels of one or more of the catecholamines in the blood or increased levels of their metabolites in urine.15 The clinical appearance of these tumours when they do not secrete catecholamines is nonspecific.14 The diagnosis of pheochromocytoma or functioning paraganglioma is made on the basis of measurement of the levels of catecholamines and their metabolites in plasma and urine.2,15 The location of the tumour can usually be identified with abdominal CT or MRI. The iodine-131-metaiodobenzylguanidine (123I-MIBG) scanning approach relies on nuclide uptake by the tumour and is particularly useful for locating extra-adrenal tumours.2,10,15 123I-MIBG uptake appears to correlate more with storage capacity rather than with actual secretion of catecholamines.10 The MRI features of spinal paragangliomas include well-demarcated masses with low/intermediate signal intensity on T1-weighted scans and intermediate/high signal intensity on T2-weighted scans, in comparison with neighbouring paravertebral tissues. Gadolinium administration produces intense heterogeneous enhancement, and high-velocity flow through the hypervascular tumours produces multiple serpiginous areas of signal void in all sequences.2 Because the majority of paragangliomas are benign, most patients can be treated successfully by surgical excision of the tumour. Malignant paragangliomas can also be treated surgically, but the prognosis depends primarily on whether the tumour metastasises.13,17 Spinal metastatic paragangliomas have led to pathologic fractures and spinal cord compression. The best combination of therapy for metastatic spinal paragangliomas appears to be surgical decompression followed by a course of radiotherapy.6 Jindel et al. reported 80–90% local control with radiation.18 Preoperative treatment of patients with pheochromocytomas and functioning paragangliomas is critical. At least 2 weeks of management of hypertension with an aanti-adrenergic agent is required to allow the chronically contracted extravascular space to expand.2,15 Management of hypertension with b-anti-adrenergic agent alone is con-
traindicated, because such treatment can cause a paradoxical increase in blood pressure that is attributable to the inhibition of peripheral b2 receptors.2 Gentle surgical techniques and expert anaesthesia are required to minimise intraoperative hypertension attributable to handling of the tumour.2,15 Hypertension during the course of the procedure can be managed with intravenous phentolamine, sodium nitroprusside, or deepening of the maintenance anaesthetic. Hypotension, which can occur after tumour resection, can be managed with the administration of volume or, if necessary, pressors.19 Paragangliomas have distinctive histological features. Clusters of large polyhedral chief cells are arranged in nests (Zellballen), surrounded by a delicate fibrovascular stroma. The tumour cells have granular eosinophilic cytoplasm and round or ovoid nuclei. There is positive staining for synaptophysin, chromogranin, S-100, and tumour-specific antigen.2 Malignancy cannot be determined with histological assessments but is demonstrated by metastases, recurrences and invasion.2,15,19 Paragangliomas involving the spine are rare, with approximately 90 cases reported in the literature.1–31 The vast majority of tumours are intradural and are found within the cauda equina (43%).4,10,11,16 Metastatic spinal lesions represent 35% of all spinal paragangliomas. Cervical (2%) and sacral (3%) paragangliomas are extremely rare. Thoracic paragangliomas are rare (17%). Only 13 thoracic spinal paragangliomas have been reported, and cord compression was the presenting feature in every case,1,2,8,14–16,18,25,27,29–31 10 of which were located extradurally1,2,14,15,18,27,29–31 and two of which were functional.2,15 Three of those cases involved metastases.18,29 Therefore, it has been suggested that metastatic spread and an extradural location are characteristic features of thoracic paragangliomas, compared with the cauda equina variant.1,2 The case in the present report was a thoracic paraganglioma that was extradural and functional. Our case of paraganglioma is the first to be resected by costotransversectomy. Long-term post-operative surveillance is essential, because paragangliomas have a higher rate of delayed recurrence than pheochromocytomas, and onset of symptoms likely heralds recurrence of the tumour.7,14,15 Standard follow-up includes annual measurement of blood pressure and urinary catecholamine metabolites. The selected use of CT or MRI may yield additional information.15 4. Conclusion Paraganglioma of the spine will seldom be considered in a presurgical differential diagnosis due to its rarity and nonspecific imaging features. Adequate treatment requires total surgical removal. In the present case, resection of the paraganglioma resulted in elimination of the catecholamine excess and the resulting hypertension. We present only the 14th documented case of a primary thoracic spine paraganglioma and only the third to present with adrenergic symptoms.
Case Reports / Journal of Clinical Neuroscience 15 (2008) 827–830
References 1. Houten JK, Babu RP, Miller DC. Thoracic paraganglioma presenting with spinal cord compression and metastases. J Spinal Disord Tech 2002;15:319–23. 2. Jeffs GJ, Lee GYF, Wong GTH. Functioning paraganglioma of the thoracic spine: case report. Neurosurgery 2003;53:992–5. 3. Coles CP, Alexander DI, Gross M, et al. Intraosseous paraganglioma of the sacrum: a case report. CJS 2000;43:137–9. 4. Sundgren P, Annertz M, Englund E, et al. Paragangliomas of the spinal canal. Neuroradiology 1999;41:788–94. 5. Boncoeur-Martel MP, Lesort A, Moreau JJ, et al. MRI of paraganglioma of the filum terminale. J Comput Assist Tomogr 1996;20:162–5. 6. Brodkey JA, Brodkey JS, Watridge CB. Metastatic paraganglioma causing spinal cord compression. Spine 1995;20:367–72. 7. Faro SH, Turtz AR, Koennigsberg RA, et al. Paraganglioma of the cauda equina with associated intramedullary cyst: MR findings. AJNR 1997;18:1588–90. 8. Silverstein AM, Quint DJ, McKeever PE. Intradural paraganglioma of the thoracic spine. AJNR 1990;11:614–6. 9. Lamer S, Carlier RY, Parker F, et al. Paraganglioma of the cauda equine: MR findings. One case. J Neuroradiol 1997;24:215–7. 10. Camera L, Biondi B, Daniele S, et al. Screening in von Hippel-Lindau disease: concurrent pheochromocytomas, paragangliomas and spinal hemangioblastomas revealed by helical-CT, MIBG scintigraphy and MRI in an asymptomatic patient. Eur J Radiol Extra 2003;48: 8–13. 11. Moran CA, Albores-Saavedra J, Wenig BM, et al. Pigmented extraadrenal paragangliomas. Cancer 1997;79:398–402. 12. Herman M, Pozzi-Mucelli RS, Skrap M. Paraganglioma of the cauda equina: case report and review of the MRI features. Acta Univ Polacki Olomus Fak Med 1998;141:27–30. 13. Absher KJ, Witte DA, Truong LD, et al. Aspiration biopsy of osseous metastasis of retroperitoneal paraganglioma. Acta Cytologica 2001;45:249–53. 14. Cybulski GR, Nijensohn E, Brody BA, et al. Spinal cord compression from a thoracic paraganglioma: case report. Neurosurgery 1991;28:306–9. 15. Spector JA, Willis DN, Ginsburg HB. Paraganglioma (pheochromocytoma) of the posterior mediastinum: a case report and review of the literature. J Pediatr Surg 2003;38:1114–6.
827
16. Mossner R, Keidel M. Malignant pheochromocytoma with progressive paraparesis in von Hippel-Lindau disease. Eur J Neurol 2000;7:439–42. 17. Teno S, Tanabe A, Nomura K, et al. Acutely exacerbated hypertension and increased inflammatory signs due to radiation treatment for metastatic pheochromocytoma. Endocrine J 1996;43:511–6. 18. Jindel R, Gupta AK, Mahapatra AK, et al. Extradural paraganglioma with multiple skeletal metastases. Br J Radiol 1992;65: 938–40. 19. Olson JJ, Loftus CM, Hitchon PW. Metastatic pheochromocytoma of the cervical spine. Spine 1989;14:349–51. 20. Gabriel EM, Sampson JH, Dodd LG, et al. Glomus jugulare tumor metastatic to the sacrum after high dose radiation therapy: case report. Neurosurgery 1995;37:1001–5. 21. Chiras J, Cognard C, Rose M, et al. Percutaneous injection of an alcoholic embolizing emulsion as an alternative preoperative embolization for spine tumor. AJNR 1993;14:1113–7. 22. North CA, Zinreich ES, Christenhsen WN, et al. Multiple spinal metastases from paraganglioma. Cancer 1990;66:2224–8. 23. Osborn RE, Mojtahedi S. Paraganglioma metastatic to the cervical spine. Computerized Radiol 1986;10:167–70. 24. Occhiogrosso M, De Tommasi A, Vailati G, et al. Malignant chemodectoma of the carotid body causing spinal cord compression. Surg Neurol 1985;23:14–8. 25. Solymosi L, Ferbert A. A case of spinal paraganglioma. Neuroradiol 1985;27:217–9. 26. Sankla S, Khan GM. Cauda equina paraganglioma presenting with intracranial hypertension: case report and review of the literature. Neurol India 2004;52:243–4. 27. Shin JY, Lee SM, Hwang MY, et al. MR findings of the spinal paraganglioma: report of three cases. J Korean Med Sci 2001;16:522–6. 28. Mori S, Okura T, Kitami Y, et al. A case metastatic extra-adrenal pheochromocytoma 12 years after surgery. Hypertens Res 2002;25:141–4. 29. Constantini S, Soffer S, Siegal T, et al. Paraganglioma of the thoracic spine with cerebrospinal fluid metastases. Spine 1989;14:643–5. 30. Fitzgerald L, Cech D, Goodman J. Paraganglioma of the thoracic spinal cord. Clin Neurol Neurosurg 1996;98:183–5. 31. Sato N, Imai T, Aikawa H, et al. Recurrence and pulmonary metastasis of extradural paraganglioma in thoracic vertebral canal: report of a case. Kyobu Geka 2001;54:610–3.
doi:10.1016/j.jocn.2006.07.024
Ependymal cyst in the lumbar spine associated with cauda equina compression Minori Kato a, Hiroaki Nakamura b,*, Eisuke Suzuki c, Hidetomi Terai a, Kenichi Wakasa d, Tomoko Wakasa d, Kunio Takaoka a a
Department of Orthopaedic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan b Department of Orthopaedic Surgery, Osaka City General Hospital, Osaka, Japan c Department of Orthopaedic Surgery, Osaka Prefectural Rehabilitation Center, Sakai, Japan d Department of Clinical Pathology, Osaka City University Graduate School of Medicine, Osaka, Japan Received 24 August 2006; accepted 25 December 2006
*
Corresponding author. Present address: 2-13-22, Miyakojimahondori Miyakojima-ku, Osaka 534-0021, Japan. Tel.: +81 6 6645 3851; fax: +81 6 6646 6260. E-mail address: [email protected] (H. Nakamura).