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ment remain controversial. Patients with unruptured aneurysms are generally managed with medical therapy alone, including appropriate antimicrobial drugs and close clinical and imaging follow-up.3,5 There is general consensus that if the aneurysm enlarges, fails to resolve, or ruptures during antimicrobial treatment, they should be considered for surgical repair, parent vessel occlusion, or endovascular treatment.7,8 Our patient presented in a critical condition with Balamuthia amebic encephalitis after a renal transplant. Later he developed an asymptomatic fusiform aneurysm of the cavernoussupraclinoid carotid artery. Due to his poor systemic and neurologic condition we managed him conservatively with an appropriate anti-amebic regimen and serial non-invasive imaging. His aneurysmal dilatation resolved and he recovered with significant neurological disability. 4. Conclusions This report describes the unique occurrence of a mycotic aneurysm of the cavernous portion of the internal carotid artery in a renal transplant recipient with Balamuthia mandrillaris encephalitis. Anti-amebic therapy resulted in resolution of the aneurysm without vascular complications.
Acknowledgement We like to thank Dr. Huiling Liu for his help in the preparation of the images used in this manuscript. References 1. Matin A, Siddiqui R, Jayasekera S, et al. Increasing importance of Balamuthia mandrillaris. Clin Microbiol Rev 2008;21:435–48. 2. Martinez AJ, Sotelo-Avila C, Alcala H, et al. Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba. Acta Neuropathol 1980;49:7–12. 3. Yen PS, Teo BT, Chen SC, et al. Endovascular treatment for bilateral mycotic intracavernous carotid aneurysms. Case report and review of the literature. J Neurosurg 2007;107:868–72. 4. Cloud GC, Rich PM, Markus HS. Serial MRI of a mycotic aneurysm of the cavernous carotid artery. Neuroradiology 2003;45:546–9. 5. Marsot-Dupuch K, Riachi S, Berthet K, et al. Infectious aneurysms of the cavernous carotid artery. J Radiol 2000;81:891–8 [Article in French]. 6. Tipping B, de Villiers L, Candy S, et al. Stroke caused by human immunodeficiency virus-associated intracranial large vessel aneurysmal vasculopathy. Arch Neurol 2006;63:1640–2. 7. Barrow DL, Prats AR. Infectious intracranial aneurysms: comparison of groups with and without endocarditis. Neurosurgery 1990;27:562–73. 8. Kawakami K, Kayama T, Kondo R, et al. A case of mycotic ICA petrous portion aneurysm treated with endovascular surgery. No Shinkei Geka 1996;24:253–7 [Article in Japanese].
doi:10.1016/j.jocn.2010.11.032
Primary intraosseous paraganglioma of the sacrum Manish K. Kasliwal ⇑, Dinesh Rattnani, Bipin S. Walia, Sandeep Vaishya, Rana Patir Department of Neurosurgery, Max Institute of Neurosciences, Max Super Specialty Hospital, 1 Press Enclave Road, New Delhi 110017, India
a r t i c l e
i n f o
Article history: Received 20 September 2010 Accepted 21 November 2010
Keywords: Intraosseous Paraganglioma Sacrum Spinal Tumor
a b s t r a c t Primary intraosseous sacral paraganglioma is a rare case of location for spinal paragangliomas, which generally manifest as intradural extramedullary tumors of the cauda equina region. The diagnosis can be elusive considering the rarity of sacral paragangliomas. The clinical importance of recognizing this relatively benign tumor cannot be overemphasized as the outcome, extent of surgery and management differs significantly from other common bony sacral tumors which are generally malignant. We report a rare case of sacral paraganglioma that was diagnosed only after histopathological examination, along with a relevant review of the literature. Ó 2011 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Paragangliomas are tumors that most commonly involve the adrenal tissue. Only about 10% of paragangliomas occur outside the adrenal system – the carotid body or the glomus jugalare being the most common additional locations. Spinal involvement usually manifests as an intradural extramedullary (IDEM) tumor of the cauda equina region.1 A primary intraosseous sacral paraganglioma is very rare, with only five cases reported in the literature.2–6 We report a rare case of primary sacral bony paraganglioma, which was diagnosed only after histopathological examination with a pertinent review of the literature.
A 57-year-old female presented with a history of low backache for 10 years with a 1-year history of radiation of pain to the bilateral posterior thigh and gluteal regions without any motor weakness. Neurological examination revealed normal strength and sensation in her bilateral lower limbs with mild tenderness over the sacral region. MRI revealed a sacral mass that was isointense to muscle on T1-weighted MRI, and hyperintense on T2-weighted MRI with homogenous and avid contrast enhancement (Fig. 1). CT scan of the lumbosacral spine performed to delineate the bony anatomy, showed a lytic lesion in the sacrum with expansion of the S2 to S5 vertebrae (Fig. 2). A preoperative diagnosis of sacral chordoma was considered, and a wide en bloc resection with possible need of a myocutaneous flap for the reconstruction of a large sacral defect was planned. A midline incision exposed the spine from the L5 vertebrae to the
⇑ Corresponding author. Tel.: +91 9818900717. E-mail address:
[email protected] (M.K. Kasliwal).
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Fig. 1. (a) Axial plain T1-weighted, (b) sagittal T1-weighted with contrast, and (c) sagittal T2-weighted MRI of the lumbosacral spine showing a sacral tumor isointense on T1-weighted and hyperintense on T2-weighted MRI with homogenous and avid contrast enhancement.
Fig. 2. Axial CT scan of the sacrum showing a lytic lesion with expansion of the sacrum.
coccyx. The tumor was seen just below the subcutaneous tissue, and it was totally extradural, vascular, soft and friable – quite atypical for a sacral chordoma. The sacrum was involved from S2 to S5 with preservation of the anterior vertebral border. An intraoperative frozen section was examined without a conclusive diagnosis; but it was not suggestive of chordoma. A near-total excision was achieved. The final histopathological examination was diagnostic of paraganglioma (Fig. 3). The patient had an uneventful postoperative course with no new neurological deficits. In view of the benign nature and near total excision of tumor, no adjuvant treatment was considered and the patient was clinically well at 3 months follow-up.
3. Discussion Paragangliomas are neuroendocrine tumors that most frequently arise in the adrenal medulla, carotid body and glomus jugulare. They are benign, slow-growing tumors with low proliferative activity arising from paraganglia cells, which are collections of specialized neural crest cells associated with the segmentation of collateral autonomic ganglia throughout the body. Although paragangliomas can be classified in various ways, Glenner and Grimley proposed probably the most rational approach for classifying paragangliomas.7 Extra-adrenal paragangliomas occur at unusual sites such as the stomach, duodenum, uterus and lung.8–11
Fig. 3. Haematoxylin and eosin stained section of a primary intraosseous paraganglioma showing polygonal cells arranged in nests and cords in relation to numerous sinusoidal vascular channels (200). (This figure is available in colour at www.sciencedirect.com).
The central nervous system (CNS) involvement of paragangliomas has been described in the cerebellopontine angle and cerebellum.12 Spinal paragangliomas are very rare and most commonly manifest as intradural tumors in the cauda equina region.1 Extradural involvement by paragangliomas can manifest as purely extradural paragangliomas, which can be primary or metastatic, an extradural extension from an intradural paraganglioma, or as vertebral erosion as a result of compression by an adjacent tumor.13,14 Akin to the occurrence of CNS paragangliomas, the origin of primary bony paragangliomas remains conjectural,15 although various proposals have attempted to explain their development. Although primary paraganglioma has been described in the cervical, thoracic and lumbar spine, its occurrence in the sacrum has been rare (Supplementary Table 1). The differential diagnoses of primary sacral tumors include: neural tumors, primary osseous tumors, metastases and myeloma.16,17 After metastasis, chordoma remains the most common and important differential diagnosis for primary sacral malignant
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tumors. Typical imaging features of sacral chordoma include a lytic lesion centered in the midline on CT scan and an associated softtissue mass with calcification is seen in 30% to 70% of patients; the presence of intralesional calcification is very characteristic.16 Compared with skeletal muscle, typical chordomas are isointense or slightly hypointense on T1-weighted MRI, and typically hyperintense on T2-weighted MRI.16,17 The rarity of paraganglioma precludes a description of typical imaging features, but they also present as a lytic mass involving the sacrum with hyperintensity on both T1-weighted and T2-weighted MRI and avid contrast enhancement, as reported.2–6 The characteristic intralesional calcification seen in chordomas is typically absent in sacral paragangliomas and forms an important differentiating feature,2–6 but calcification is often absent in other common malignant tumors of the sacrum, which makes differentiating these tumors difficult on imaging alone.16 Considering the heterogeneity of sacral neoplasms, a biopsy, either open or CT-guided, as an initial diagnostic procedure can raise the possibility of the presence of a rare lesion, which helps in further surgical planning and management, as reported.2,4,5 Malignant tumors such as chordoma commonly involve the sacrum, which mandates avoidance of intralesional resection with wide en bloc resection. Along with the use of a myocutaneous flap for the reconstruction of large sacral defects, a radical and morbid surgery could result. Confirmation of preoperative diagnosis is therefore essential before embarking on radical resection.18 Presurgical diagnosis of benign tumors such as paraganglioma can avoid such extensive resection, and at the same time, result in a good overall clinical outcome, even with a subtotal tumor excision or radiotherapy.4–6 Total surgical excision can result in a surgical cure in patients with paraganglioma. However, recurrences in up to 4% to 10% of patients have been reported even after macroscopic total excision of spinal paragangliomas.19 Recurrences may also occur after subtotal removal of the tumor, and postoperative radiation therapy for patients with incomplete excision of spinal paragangliomas may not affect the recurrence rate.20 Coles et al. reported the first patient with a primary sacral intraosseous tumor involving S3, with an excellent outcome after preoperative embolization and complete excision of the tumor with no recurrence at the 2-year follow-up.2 Falavigna et al. reported an excellent outcome after surgical resection of a sacral paraganglioma, accomplished through a posterior midline incision.3 Although gross total removal should be the goal of surgery in patients with primary sacral paraganglioma, a good outcome has been achieved after partial resection,4 and external beam radiotherapy followed by iodine-131 metaiodobenzylguanidine treatment5 with no growth of the tumor at the 1-year and 5-year follow-up respectively. Spinal tumors are very vascular and can lead to massive blood loss during surgery, which makes preoperative embolization a useful adjunct to limit the associated blood loss and perioperative morbidity.21 Preoperative embolization was helpful in achieving more extensive resection in sacral intraosseous paraganglioma considering the hypervascular nature of the tumor in some reports.2,4
doi:10.1016/j.jocn.2010.11.032
4. Conclusion Despite their rarity, primary bony paraganglioma should be considered in the differential diagnosis of sacral tumors. Although surgery remains the treatment of choice; as literature on primary sacral paragangliomas is sparse, there has been heterogeneity of management in reported cases. An initial fine needle aspiration biopsy to diagnose this rare tumor followed by individualization of management options appears reasonable depending upon the extent and resectability of the tumor. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.11.032. References 1. Miliaras GC, Kyritsis AP, Polyzoidis KS. Cauda equina paraganglioma: a review. J Neurooncol 2003;65:177–90. 2. Coles CP, Alexander DI, Gross M, et al. Intraosseous paraganglioma of the sacrum: a case report. Can J Surg 2000;43:137–9. 3. Falavigna A, Righesso O, Volquind D, et al. Intraosseous sacral paraganglioma with extradural extension: case report. Acta Neurochir (Wien) 2010;152:475–80. 4. Laufer I, Edgar MA, Hartl R. Primary intraosseous paraganglioma of the sacrum: a case report. Spine J 2007;7:733–8. 5. Sundgren P, Annertz M, Englund E, et al. Paragangliomas of the spinal canal. Neuroradiology 1999;41:788–94. 6. Radouane B, Jidal M, Chaouir S, et al. Intraosseous paraganglioma of the sacrum: a case report. J Radiol 2008;89:596–7. 7. Glenner GG, Grimley PM. Tumors of extraadrenal paraganglion system (including chemoreceptors) In: Atlas of Tumor Pathology. Washington: Springer; 1974. 8. Prayson RA, Chahlavi A, Luciano M. Cerebellar paraganglioma. Ann Diagn Pathol 2004;8:219–23. 9. Schmid C, Beham A, Steindorfer P, et al. Non-functional malignant paraganglioma of the stomach. Virchows Arch A Pathol Anat Histopathol 1990;417:261–6. 10. Young TW, Thrasher TV. Nonchromaffin paraganglioma of the uterus. A case report. Arch Pathol Lab Med 1982;106:608–9. 11. Shibahara J, Goto A, Niki T, et al. Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study. Am J Surg Pathol 2004;28:825–9. 12. Deb P, Sharma MC, Gaikwad S, et al. Cerebellopontine angle paraganglioma – report of a case and review of literature. J Neurooncol 2005;74:65–9. 13. Constantini S, Soffer D, Siegel T, et al. Paraganglioma of the thoracic spinal cord with cerebrospinal fluid metastasis. Spine 1989;14:643–5. 14. Iliya AR, Davis RP, Seidman RJ. Paraganglioma of the cauda equina: case report with magnetic resonance imaging description. Surg Neurol 1991;35:366–7. 15. Ho KC, Meyer G, Garancis J, et al. Chemodectoma involving the cavernous sinus and semilunar ganglion. Hum Pathol 1982;13:942–3. 16. Llauger J, Palmer J, Amores S, et al. Primary tumors of the sacrum: diagnostic imaging. AJR Am J Roentgenol 2000;174:417–24. 17. Nader R, Rhines LD, Mendel E. Metastatic sacral tumors. Neurosurg Clin N Am 2004;15:453–7. 18. Schwab JH, Healey JH, Rose P, et al. The surgical management of sacral chordomas. Spine 2009;34:2700–4. 19. Strommer KN, Brandner S, Sarioglu AC, et al. Symptomatic cerebellar metastasis and late local recurrence of a cauda equina paraganglioma. Case report. J Neurosurg 1995;83:166–9. 20. Sonneland PR, Scheithauer BW, LeChago J, et al. Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure. Cancer 1986;58:1720–35. 21. Smith TP, Gray L, Weinstein JN, et al. Preoperative transarterial embolization of spinal column neoplasms. J Vasc Interv Radiol 1995;6:863–9.
