2
8. Goel A, Dange N. Immediate postoperative regression of retroodontoid pannus after lateral mass reconstruction in a patient with rheumatoid disease of the craniovertebral junction: case report. J Neurosurg Spine 2008;9:273–6. 9. Jun BY. Complete reduction of retro-odontoid soft tissue mass in osodontoideum following the posterior C1–C2 transarticular screw fixation. Spine 1999;24:1961–4. 10. Suetsuna F, Narita H, Ono A, et al. Regression of retroodontoid pseudotumors following C-1 laminoplasty: report of three cases. J Neurosurg Spine 2006;5:455–60. 11. Ito K, Sakai K, Yako T, et al. Atlantoaxial dislocation associated with a mass in the extradural craniovertebral junction unrelated to rheumatoid arthritis: case report. Neurol Med Chir (Tokyo) 2007;47:182–5. 12. Nishizawa S, Yamaguchi M, Kitahama Y. Retro-odontoid mass in high elderly patients: genesis, therapeutic strategies and post-operative care. Spinal Surg 2005;19:11–8. 13. Jun BY, Yoon KJ, Crockard A. Retro-odontoid pseudotumor in diffuse idiopathic skeletal hyperostosis. Spine (Phila Pa 1976) 2002;27:E266–70. 14. Matsuno A, Nakashima M, Murakami M, et al. Microsurgical excision of a retro- odontoid disc hernia via far-lateral approach: successful treatment of a rare cause of myelopathy. Neurosurgery 2004;54:1015–8. 15. Nishizawa S, Ryu H, Yokoyama T, et al. Myelopathy caused by retro-odontoid disc hernia. Neurosurgery 1996;39:1256–9. 16. Rosenberg WS, Rosenberg AE, Poletti CE. Cervical disc herniation presenting as a mass lesion posterior to the odontoid process: report of two cases. J Neurosurg 1991;75:954–9. 17. Yamazaki M, Okawa A, Mannoji C, et al. C1 dome-like laminectomy and posterior C1–C2 polyaxial screw-rod fixation for a patient with cervical myelopathy due to a retro-odontoid pseudotumor. J Clin Neurosci 2009;16: 99–103. doi:10.1016/j.jocn.2009.08.009 Cavernoma in the pineal region Antonio Figueiredo a , Shradha Maheshwari a , Atul Goel b, * a Lilavati Hospital and Research Centre, Bandra West, Mumbai, India b Department of Neurosurgery, Seth G.S. Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai 400012, India article info Article history: Received 2 August 2009 Accepted 4 August 2009 Keywords: Cavernoma Midbrain Pineal region abstract A 19-year-old girl presented with symptoms of severe headache and diplopia. Investigations revealed a large and partly calcified lesion in the pineal region. At surgery a large cavernoma was identified and was radically resected. Following surgery, the patient recovered from all her symptoms. Giant cavernomas in the pineal region are rare. The literature on the subject is reviewed. Ó 2009 Elsevier Ltd. All rights reserved. 1. Introduction Although brain cavernomas have been found in up to 0.5% of the population in a large autopsy and MRI series, 1,2 these lesions are not common in clinical practice and the true prevalence of caver- nomas is largely unknown. Location of cavernomas in the pineal region is particularly uncommon. 3 We report a patient with a cavernoma in the pineal region, review the literature and discuss its management. 2. Case report A 19-year-old girl had moderate headaches for 8 months. The headaches worsened acutely over 15 days and she developed dip- lopia. She had bilateral papilloedema. There was no clear gaze restriction or extraocular muscle weakness. There was no other neurological deficit. Investigations revealed a large, partly calcified, enhancing le- sion in the region of the pineal gland accompanied by moderate hydrocephalus. She underwent surgery by a supracerebellar infra- tentorial approach (Fig. 1). During the operation a large lesion was identified with multiple blood vessels over its surface and within it. There were areas suggesting old hemorrhage, and of xantho- chromia within and around the lesion. The entire lesion was dis- sected progressively from the adjoining neural structures and resected. There was a relatively well-defined plane of dissection. Histopathology of the lesion showed multiple large vascular chan- nels in various stages of hemorrhage along with thrombosis and calcification in some vessels, confirming the operative impression of a cavernoma (Supplementary Fig. 1). The patient recovered rap- idly from her symptoms, and at 2-year follow-up, she was asymp- tomatic. Repeat investigation at this time confirmed resection of the lesion (Supplementary Fig. 2). 3. Discussion Cavernomas in the region of the pineal gland are rare. Our literature search revealed 16 reported cases of cavernomas in this location, 4 the youngest patient being 4 weeks old. 5 Our patient was young and the lesion was large with areas of calcification. These clinical and tumor morphological characteristics could easily be mistaken for those of other pineal region tumors such as pineocytoma, pineoblastoma and germinoma, all of which may ex- hibit calcifications. The large lesion, presence of large blood vessels and the location close to the midbrain and other critical structures made surgery a formidable task. Surgical resection was made eas- ier by a relatively well-defined plane of dissection in this patient. The histology confirmed the lesion to be cavernous hemangioma in the majority of reported patients, 4 but mixed hemangioma has also been reported. 5,6 Total resection of the lesion was achieved in nine reported patients. 4,7 Partial resection of the tumor was re- ported to have led to repeated episodes of subarachnoid hemor- * Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: [email protected] (A. Goel). 652 Case Reports / Journal of Clinical Neuroscience 17 652–653

Cavernoma in the pineal region

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8. Goel A, Dange N. Immediate postoperative regression of retroodontoid pannusafter lateral mass reconstruction in a patient with rheumatoid disease of thecraniovertebral junction: case report. J Neurosurg Spine 2008;9:273–6.

9. Jun BY. Complete reduction of retro-odontoid soft tissue mass in osodontoideumfollowing the posterior C1–C2 transarticular screw fixation. Spine1999;24:1961–4.

10. Suetsuna F, Narita H, Ono A, et al. Regression of retroodontoid pseudotumorsfollowing C-1 laminoplasty: report of three cases. J Neurosurg Spine2006;5:455–60.

11. Ito K, Sakai K, Yako T, et al. Atlantoaxial dislocation associated with a mass inthe extradural craniovertebral junction unrelated to rheumatoid arthritis: casereport. Neurol Med Chir (Tokyo) 2007;47:182–5.

12. Nishizawa S, Yamaguchi M, Kitahama Y. Retro-odontoid mass in high elderlypatients: genesis, therapeutic strategies and post-operative care. Spinal Surg2005;19:11–8.

13. Jun BY, Yoon KJ, Crockard A. Retro-odontoid pseudotumor in diffuse idiopathicskeletal hyperostosis. Spine (Phila Pa 1976) 2002;27:E266–70.

14. Matsuno A, Nakashima M, Murakami M, et al. Microsurgical excision of a retro-odontoid disc hernia via far-lateral approach: successful treatment of a rarecause of myelopathy. Neurosurgery 2004;54:1015–8.

15. Nishizawa S, Ryu H, Yokoyama T, et al. Myelopathy caused by retro-odontoiddisc hernia. Neurosurgery 1996;39:1256–9.

16. Rosenberg WS, Rosenberg AE, Poletti CE. Cervical disc herniation presenting asa mass lesion posterior to the odontoid process: report of two cases. J Neurosurg1991;75:954–9.

17. Yamazaki M, Okawa A, Mannoji C, et al. C1 dome-like laminectomy andposterior C1–C2 polyaxial screw-rod fixation for a patient with cervicalmyelopathy due to a retro-odontoid pseudotumor. J Clin Neurosci 2009;16:99–103.

doi:10.1016/j.jocn.2009.08.009

652 Case Reports / Journal of Clinical Neuroscience 17 652–653

Cavernoma in the pineal region

Antonio Figueiredo a, Shradha Maheshwari a, Atul Goel b,*

a Lilavati Hospital and Research Centre, Bandra West, Mumbai, Indiab Department of Neurosurgery, Seth G.S. Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai 400012, India

a r t i c l e i n f o

Article history:Received 2 August 2009Accepted 4 August 2009

Keywords:CavernomaMidbrainPineal region

a b s t r a c t

A 19-year-old girl presented with symptoms of severe headache and diplopia. Investigations revealed alarge and partly calcified lesion in the pineal region. At surgery a large cavernoma was identified and wasradically resected. Following surgery, the patient recovered from all her symptoms. Giant cavernomas inthe pineal region are rare. The literature on the subject is reviewed.

� 2009 Elsevier Ltd. All rights reserved.

1. Introduction

Although brain cavernomas have been found in up to 0.5% of thepopulation in a large autopsy and MRI series,1,2 these lesions arenot common in clinical practice and the true prevalence of caver-nomas is largely unknown. Location of cavernomas in the pinealregion is particularly uncommon.3 We report a patient with acavernoma in the pineal region, review the literature and discussits management.

2. Case report

A 19-year-old girl had moderate headaches for 8 months. Theheadaches worsened acutely over 15 days and she developed dip-lopia. She had bilateral papilloedema. There was no clear gazerestriction or extraocular muscle weakness. There was no otherneurological deficit.

Investigations revealed a large, partly calcified, enhancing le-sion in the region of the pineal gland accompanied by moderatehydrocephalus. She underwent surgery by a supracerebellar infra-tentorial approach (Fig. 1). During the operation a large lesion wasidentified with multiple blood vessels over its surface and withinit. There were areas suggesting old hemorrhage, and of xantho-chromia within and around the lesion. The entire lesion was dis-sected progressively from the adjoining neural structures and

* Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435.E-mail address: [email protected] (A. Goel).

resected. There was a relatively well-defined plane of dissection.Histopathology of the lesion showed multiple large vascular chan-nels in various stages of hemorrhage along with thrombosis andcalcification in some vessels, confirming the operative impressionof a cavernoma (Supplementary Fig. 1). The patient recovered rap-idly from her symptoms, and at 2-year follow-up, she was asymp-tomatic. Repeat investigation at this time confirmed resection ofthe lesion (Supplementary Fig. 2).

3. Discussion

Cavernomas in the region of the pineal gland are rare. Ourliterature search revealed 16 reported cases of cavernomas in thislocation,4 the youngest patient being 4 weeks old.5 Our patient wasyoung and the lesion was large with areas of calcification. Theseclinical and tumor morphological characteristics could easilybe mistaken for those of other pineal region tumors such aspineocytoma, pineoblastoma and germinoma, all of which may ex-hibit calcifications. The large lesion, presence of large blood vesselsand the location close to the midbrain and other critical structuresmade surgery a formidable task. Surgical resection was made eas-ier by a relatively well-defined plane of dissection in this patient.The histology confirmed the lesion to be cavernous hemangiomain the majority of reported patients,4 but mixed hemangioma hasalso been reported.5,6 Total resection of the lesion was achievedin nine reported patients.4,7 Partial resection of the tumor was re-ported to have led to repeated episodes of subarachnoid hemor-

Page 2: Cavernoma in the pineal region

Fig. 1. (a) Sagittal T1-weighted MRI showing an isointense pineal region tumor; (b) axial T2-weighted MRI showing the lesion and the multiple flow-voids/calcifications; (c)sagittal contrast-enhanced T1-weighted MRI showing the lesion to heterogeneously enhance; and (d) axial contrast-enhanced CT scan showing the lesion and the calcifiedareas within the tumor.

Case Reports / Journal of Clinical Neuroscience 17 652–653 653

rhage and subsequent death.8 In our patient, radical resection ledto recovery from all symptoms.

Cavernomas in the pineal region are likely to be either missed ormisdiagnosed because they are rare, and as a result the patientmay receive inappropriate treatment such as stereotactic biopsyor radiation.9 Cerebrospinal fluid diversionary shunt surgery cansafely be avoided in patients with cavernomas. Successful resec-tion of an entirely benign cavernoma is curative, compared to mostother tumors in this region, most of which will need prolongedtreatment and care.

We conclude that cavernomas in the region of the pineal glandare rare but are benign, eminently resectable and curable com-pared to other lesions in the region.

Appendix A. Supplementary material

Supplementary data associated with this article can be found, inthe online version, at doi:10.1016/j.jocn.2009.08.009.

doi:10.1016/j.jocn.2009.08.009

References

1. Hayman LA, Evans RA, Ferrel RE, et al. Familial cavernous hemangioma: naturalhistory and genetic study over a period of 5 years. Am J Med Genet1982;11:147–60.

2. Rigamonti D, Hadley MN, Drayer BP, et al. Cerebral cavernous malformations:incidence and familial occurrence. N Engl J Med 1988;319:343–7.

3. Slavin KV, Dujovny M, McDonald LW, et al. Pineal region: rare location ofcavernous haemangioma. Neurol Res 1994;16:133–6.

4. Muzumdar DP, Mishra BK, Bhaduri AS. Pineal region cavernoma. Neurol Med Chir(Tokyo) 2000;40:372–9.

5. Sonntag VK, Waggener JD, Kaplan AM. Surgical removal of a hemangioma of thepineal region in a 4 week-old infant. Neurosurgery 1981;8:586–8.

6. Mirecka K. A case of angiomatous tumour localisation near the pineal body ofchild. Pol Przegl Radiol Med Nukl 1965;20:307–11. [Polish with English abstract].

7. Lombardi D, Scheithauer BW, Villani RM, et al. Cavernous hemangioma of thepineal region. Acta Neurochir (Wien) 1996;138:673–82.

8. Miller RH. Spontaneous subarachanoid hemorrhage: a presenting symptom of atumour of the third ventricle. Surg Clin North Am 1961;41:1043–8.

9. Combelles G, Blond S, Biondi A, et al. Familial forms of intracranial cavernoushemangioma. Neurochirurgie 1983;29:263–9.