Case Reports / Journal of Clinical Neuroscience 18 (2011) 721–723 721

on T1-weighted MRI could indicate intralesional blood, so that aneurological deficit might have developed but disappeared withina few days because there was no obvious mass effect.

4. Conclusion

We have described a patient with chorea due to a CM in thecontralateral caudate nucleus, and presented a review of reportsof chorea associated with CM. To our knowledge, this report isthe first to describe comparative findings of brain imaging ob-tained before and after the onset of chorea. Importantly, we de-tected no extralesional blood around the CM. We conclude thatCM in the caudate nucleus can cause chorea.

Acknowledgment

The authors would like to thank the Department of Neurosur-gery, Toho University, Omori Medical Center, for providing theMRI of the patient.

References

1. Porter PJ, Willinsky RA, Harper W, et al. Cerebral cavernous malformations:natural history and prognosis after clinical deterioration with or withouthemorrhage. J Neurosurg 1997;87:190–7.

doi:10.1016/j.jocn.2010.08.034

a r t i c l e i n f o

Article history:Received 2 April 2010Accepted 4 July 2010

Keywords:Malignant peripheral nerve-sheath tumourMPNSTSpinalTriton tumor

a b s t r a c t

Malignant peripheral nerve-sto as malignant triton tumouyear-old male patient with aType 1 presenting with sympMPNST with hemorrhage at tand a small part of the extradtumor presenting with an in

1. Introduction

Malignant triton tumours (MTT) refers to malignant nervesheath tumours (MPNST) with a rhabdomyomatous component.1

They are rare and are reported most frequently involving largeand medium-sized nerves such as the sciatic and brachial plexus.The involvement of spinal nerves is very rare with only 10 patientsreported previously.1 To our knowledge this is the first report, in a28-year-old man with stigmata of neurofibromatosis Type-1 (NF-1), of a cervical MTT with an intratumoral hemorrhage. We also re-view the existing literature.

⇑ Corresponding author. Tel.: +91 080 26995724; fax: +91 080 26564830.E-mail address: dwarakaneuro@yahoo.com (D. Srinivas).

2. Akbostanci MC, Yigit A, Ulkatan S. Cavernous angioma presenting withhemidystonia. Clin Neurol Neurosurg 1998;100:234–7.

3. Hidaka M, Shimoda M, Sato O, et al. Case report: hemiballism due to aputaminal cavernous hemangioma. No To Shinkei 1989;41:1135–9.

4. Carpay HA, Arts WF, Kloet A, et al. Hemichorea reversible after operation in aboy with cavernous angioma in the head of the caudate nucleus. J NeurolNeurosurg Psychiatry 1994;57:1547–8.

5. Carella F, Caraceni T, Girotti F. Hemichorea due to a cavernous angioma of thecaudate: case report of an aged patient. Ital J Neurol Sci 1992;13:783–5.

6. Lopez-Valdes E, Posada IJ, Munoz A, et al. Acute hemichorea caused by acavernous angioma in the caudate. Neurologia 1998;13:205–6.

7. Yakinci C, Durmaz Y, Korkut M, et al. Cavernous hemangioma in a childpresenting with hemichorea: response to pimozide. J Child Neurol2001;16:685–8.

8. Dewey Jr RB, Jankovic J. Hemiballism-hemichorea: clinical and pharmacologicfindings in 21 patients. Arch Neurol 1989;46:862–7.

9. Albin RL, Young AB, Penney JB. The functional anatomy of basal gangliadisorders. Trends Neurosci 1989;12:366–75.

10. Al-Shahi Salman R, Berg MJ, Morrison L, et al. Hemorrhage from cavernousmalformations of the brain: definition and reporting standards. Angiomaalliance scientific advisory board. Stroke 2008;39:3222–30.

11. Moriarity JL, Wetzel M, Clatterbuck RE, et al. The natural history of cavernousmalformations: a prospective study of 68 patients. Neurosurgery1999;44:1166–73.

12. Rivera PP, Willinsky RA, Porter PJ. Intracranial cavernous malformations.Neuroimaging Clin N Am 2003;13:27–40.

13. Kim DS, Park YG, Choi JU, et al. An analysis of the natural history of cavernousmalformations. Surg Neurol 1997;48:9–18.

14. Zabramski JM, Wascher TM, Spetzler RF, et al. The natural history of familialcavernous malformations: results of an ongoing study. J Neurosurg1994;80:422–32.

Malignant triton tumor of cervical spine with hemorrhage

Amrita Ghosh a, Savitr B. Sastri b, Dwarakanath Srinivas a,⇑, Anita Mahadevan a,Chandramouli B. Anandappa b, S.K. Shankar a

a Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bangalore 560 029, Indiab Department of Neurosurgery, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India

heath tumours (MPNST) with a rhabdomyomatous component are referredrs. Cervical tritons are rare, with only one previous report. We present a 28-malignant triton tumor involving the cervical spine with neurofibromatosistoms of rapidly progressive cord compression. Radiology was suggestive of ahe C2–C3 level. He underwent surgery with decompression of the intradural,ural, component. To our knowledge this is the first report of a cervical tritontratumoral bleed.

� 2010 Elsevier Ltd. All rights reserved.

2. Case report

The patient was a 28-year-old male who presented to our emer-gency service with acute onset quadriparesis along with respira-tory difficulty. On examination, the patient had stigmata of NF-1.A 6-cm-diameter, firm, well-circumscribed lump was felt in theright posterior triangle of the neck. He was tachypneic with poorrespiratory effort with a respiratory rate of 5, spastic quadriparesiswith Medical Research Council grade of 1–2/5 in the left upperlimb and 0/5 in all other limbs. Imaging revealed an intradural le-sion causing cord compression at the C2–C3 level and exiting thespinal canal via the right C2–C3 foramen with a large extraspinalcomponent anterior and posterior to the vertebra. The radiologicfeatures were suggestive of a peripheral nerve neurofibroma withhemorrhage (Fig. 1).

The patient underwent C2–C3 laminectomy, right facetectomyand decompression of the lesion. Peri-operatively, the tumor hadboth intradural and extradural components. The tumor showed asignificant well-formed blood clot within it, and it had a poor planeof division from the thinned out spinal cord. The intradural andextradural components of the tumor within the spinal canal weredecompressed and the patient was ventilated post-operatively.He did not make any significant improvement in power and diedafter 20 days in hospital.

Histology revealed a spindle cell tumour: the cells were ar-ranged in short intersecting fascicles alternating with hypocellu-lar myxoid areas with whorled arrangement of tumour cellsaround vessels (Supplementary Fig. 1A). The tumour cells werespindle shaped, with irregular buckle-shaped to blunt ended nu-clei and pale cytoplasm. The cellularity was high with prominentnuclear pleomorphism and hyperchromasia (SupplementaryFig. 1B). Mitotic activity in the tumour was brisk (5–7/high powerfield) with large zones of necrosis and showed high MIB-1 label-ling (Supplementary Fig. 1B, inset). The spindle cell component ofthe tumour focally expressed S-100 protein (SupplementaryFig. 1C) with axons coursing through the tumor highlighted bya neurofilament immunostain (Supplementary Fig. 1C inset). Scat-tered among the spindle cells were round, oval to elongatedstrap-like cells with abundant dense eosinophilic cytoplasm.These cells demonstrated strong immunolabeling with desminantibody (Supplementary Fig. 1E) that highlighted cross striationswithin cells not easily discernible on routine stains (Supplemen-tary Fig. 1E, inset). These were found in both the intradural andextraspinal portions of the tumor. In the extraspinal component,the tumor arose from nerve roots expanded and matted togetherin a plexiform growth pattern with myxoid stroma suggestive of aplexiform neurofibroma (Supplementary Fig. 1A, inset). The ext-raspinal portion of the tumour infiltrated the surrounding soft tis-sue and muscle. All the above features were characteristic of aMTT.

3. Discussion

The name ‘‘triton’’ was introduced to describe these tumours,referring to the work of Locatelli who showed induction of growthof supernumerary limbs containing bone and muscle on the backsof triton salamanders by implanting sciatic nerve into the soft tis-sues of the back. The current concept is that both cell lines origi-nate from less differentiated neural crest cells. As neural crestcontributes to the formation of mesenchyme and portions of bran-

chial cartilage, these tumours recapitulate both schwannian andthe mesenchymal potentiality of the neural crest. MPNST accountfor approximately 5% to 10% of all soft tissue sarcomas. The preva-lence of MPNST in patients with NF-1 is 4.6% in contrast to 0.001%in general population. However, NF-1 is associated with 57% of pa-tients with an MTT. When associated with NF-1, MPNST tend topresent at a younger age (28–36 years) than their sporadic coun-terpart (40–44 years). These tumors rarely (19%) show divergentdifferentiation (rhabdoid, osseous, chondroid, angiomatous).1,2

The frequency of a rhabdomyoblastic component in the tumouris uneven with variable distribution.3

Additional mesenchymal or epithelial areas (pluridirectionaldifferentiation) may be seen in 15% of MTT. In their recent re-view, James et al., listed 10 reported patients with spinal MTT.4

There have thus been seven males and four females reported,including our patient, with ages from 15 to 67 years with an aver-age age of 36.6 years. Ten patients presented with features ofspinal cord compression. Contrary to previous reports, only threepatients (including the present patient) had NF-1. However, fourpatients had previous exposure to radiation, which has been sug-gested as a possible predisposing factor. In eight patients (includ-ing the present patient) the tumour had an intradural extension.The lumbosacral spine (five patients) was the most frequently af-fected site, followed by the thoracic spine (four patients) and thecervicodorsal region (one patient). MTT behave more aggressivelythan usual MPNST, with crude 2-year (15%) and 5-year (11%) sur-vival rates, lower when compared to standard MPNST where therates are 57% and 39%, respectively.2 Large tumours (>5 cm), asso-ciation with NF-1 and total excision are the most important prog-nostic factors of MPNST. The explanation for the poor prognosis ofMPNST in neurofibromatosis has often been related to its loca-tion3 as many of the tumours occur in central locations comparedto the peripheral locations of sporadic MPNST. Brooks et al. con-cluded that neither tumour location, nor association with NF-1,can entirely explain the very poor survival in MTT. However, fre-quent high grade tumour histology with a resultant higher prolif-erative capacity can explain the aggressive natural history andearly metastasis.3 Although schwannomas/neurofibromas are rel-atively common spinal tumors, hemorrhage into them is veryrare.5

Appendix A. Supplementary material

Supplementary data associated with this article can be found, inthe online version, at doi:10.1016/j.jocn.2010.07.148.

Fig. 1. MRI of an intradural lesion in a 28-year-old male causing cord compression at the C2–C3 level and exiting the spinal canal via the right C2–C3 foramen with a largeextraspinal component both anterior and posterior to the vertebra: (A) sagittal T1-weighted MRI showing a heterogenous hypointense to isointense lesion; (B) sagittal T2-weighted MRI showing a predominantly hyperintense lesion; (C) sagittal flash two-dimensional (2D) MRI showing blooming in some areas suggestive of blood; and (D T1-weighted postcontrast MRI – on gadolinium injection the lesion enhanced almost uniformly except for the area that was blooming on flash 2D sequence.

722 Case Reports / Journal of Clinical Neuroscience 18 (2011) 721–723

Case Reports / Journal of Clinical Neuroscience 18 (2011) 723–724 723

References

1. Enzinger FM, Wiess SW. Malignant tumors of the peripheral nerves. In: EnzingerSW, Weiss SW, editors. Soft tissue tumors, Mosby. 4th ed. MO, USA: St Louis;1988. p. 1209–63.

2. Ducatman BS, Scheithauer BW, Piepgras DG, et al. Malignant peripheral nervesheath tumours: a clinicopathological study of 120 cases. Cancer 1986;57:2006–21.

doi:10.1016/j.jocn.2010.07.148

a r t i c l e i n f o

Article history:Received 26 April 2010Accepted 24 August 2010

Keywords:Basal cephaloceleEndoscopic surgeryMeningoencephaloceleTranssphenoidal cephalocele

a b s t r a c t

Transsphenoidal cephalocele iby a combined intracranial annoidal cephalocele manifestinnasal obstruction after birth. Nthe nasopharynx through thedal meningocephalocele. The lcele and its contents were pasreduced, the mucosa was incisdural sac and its contents. Theof the dural defect. The patiedemonstrated resolution of theless time consuming than the

⇑ Corresponding author. Tel./fax: +86 10 83198836.E-mail address: doctorlingfeng@163.com (F. Ling).

3. Brooks IS, Freeman M, Enerline HT. Malignant triton tumors, natural history andimmunohistochemistry of nine new cases with literature review. Cancer1985;55:2543–9.

4. James G, Crocker M, King A, et al. Malignant triton tumors of the spine. JNeurosurg Spine 2008;8:567–73.

5. Ciappetta P, D’Urso PI, Colamaria A. Giant craniovertebral junction hemorrhagicschwannoma: case report. Neurosurgery 2008;62:E1166.

An endoscopic endonasal approach for the surgical repair of transsphenoidalcephalocele in children

Ge Chen, Qiuhang Zhang, Feng Ling ⇑Department of Neurosurgery, Xuanwu Hospital Capital Medical University, 45 Changchun Street, Xuanwu District, Beijing 100053, China

s a rare lesion traditionally managed by either an intracranial approach ord extracranial approach. A two-year-old boy presented with a transsphe-g as apneic–cyanotic spells accompanied by breathing difficulties due toeuroimaging revealed a cystic mass extending from the pituitary fossa into

sphenoid sinus. Nasal endoscopy confirmed the diagnosis of transsphenoi-esion was managed with an endoscopic endonasal approach. The cephalo-sively reduced by applying bipolar cautery to the overlying mucosa. Onceed and removed from the underlying dura to allow further reduction of thedural sac was amputated partially using microscissors, followed by repair

nt developed mild diabetes insipidus after surgery. Follow-up evaluationpreoperative symptoms without lasting morbidity. This procedure is safe,

transcranial approach, and efficacious.� 2010 Elsevier Ltd. All rights reserved.

1. Introduction

A transsphenoidal cephalocele is a rare lesion that is tradition-ally managed by either an intracranial approach or a combinedintracranial and extracranial approach. Reports of repair using anendoscopicic endonasal approach are uncommon. We report a pa-tient with a transsphenoidal cephalocele that was managed usingthe endoscopic endonasal approach.

2. Case report

A two-year-old boy was admitted to the Department of Neuro-surgery due to an apneic–cyanotic spell accompanied by breathingdifficulties due to nasal obstruction after birth. Physical examina-tion revealed mild hypertelorism and strabismus. CT scans demon-strated a cystic mass extending from the pituitary fossa into thenasopharynx through the sphenoid sinus (Fig. 1A). The diagnosisof transsphenoidal meningocephalocele was further confirmed byMRI (Fig. 1B). Nasal endoscopy identified a glossy white, pulsatingmucosal mass in the nasopharynx (Fig. 1C). There was no pasthistory of meningitis or cerebrospinal fluid (CSF) rhinorrhea. Athorough endocrine evaluation was unremarkable.

2.1. Surgical procedure

The patient was placed in the supine position without the inser-tion of a lumbar drain. The nasal cavities were packed with gauze

pledgets soaked with a 1:100 solution of 5% chlorohexidine gluco-nate. The face and nose were prepared using the same solution,and then the patient was aseptically draped. Zero-degree Storz ri-gid endoscopes, 4-mm diameter and 18-cm long, were used. Afterconstricting the vessels of the nasal cavities, the endoscope wasthen inserted into the right nostril until it reached the middle tur-binate. The middle turbinate was then outfractured to increase theoperative space. Between the inferomedial aspect of the inferiorturbinate and the nasal septum, a glossy white, pulsating mucosalmass within the epipharynx was observed lying along the floor ofthe nasal cavity. The cephalocele and its contents were passivelyreduced by applying bipolar cautery to the overlying mucosa. Oncereduced, the mucosa was incised and removed from the underlyingdura, thus allowing further reduction of the dural sac and its con-tents. Subsequently, the dura was incised and CSF gushed out. Thedural sac was partially amputated using microscissors. The cepha-locele was then adequately reduced, followed by repair of the duraldefect. The repair was accomplished by re-approximating the duraledges to close the size of dural defect and then placing DuraGen(Integra LifeSciences, Plainsboro, NJ, USA) between the dura andthe mucosa. The mucosa overlying the cephalocele was re-approx-imated and closed through the application of fibrin glue. Anotherlayer of DuraGen was then applied in an overlay fashion, followedby nasal packing with iodoform gauze. The iodoform gauze was re-moved 8 days later, and no CSF leak was identified by endoscopicinspection.

The patient developed mild diabetes insipidus on postoperativeday 1 without requirement of vasopressin. He was discharged 12days after surgery. Upon discharge, his respiratory distress wasentirely relieved. Postoperative MRI revealed an obvious reduction