- Email: [email protected]
Interdural giant dermoid cyst of the petrous apex
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Case Reports / Journal of Clinical Neuroscience 16 (2009) 1498–1502
Interdural giant dermoid cyst of the petrous apex Carmelo Lucio Sturiale *, Annunziato Mangiola, Angelo Pompucci, Manuela D’Ercole, Licia Di Muro, Carmelo Anile Institute of Neurosurgery, Catholic University School of Medicine, Largo Agostino Gemelli 8, 00168, Rome, Italy
a r t i c l e
i n f o
Article history: Received 14 January 2009 Accepted 25 February 2009
Keywords: Dermoid cyst Interdural Petrous apex
a b s t r a c t Dermoid cysts account for less than 1% of all intracranial neoplasms and these are principally situated close to the midline. Those located at the petrous apex are generally in the intradural, and less frequently in the interdural, compartment. We report a 37-year-old woman with a giant interdural dermoid cyst of the petrous apex, associated with erosion of the middle fossa floor, and review the relationships of these dysembryogenic lesions, the dura mater and the neurovascular structures of this complex anatomical region, along with the relevant literature. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2.2. Operation
Dermoid cysts (DCs) are rare congenital tumours that account for less than 1% of all intracranial neoplasms and can be found in various locations, mainly close to the midline, both in the supraand infratentorial regions.1 These tumours originate from epithelial elements contained within the neural groove when it forms the neural tube (weeks 3–5 of embryonic life).2,3 Those located at the petrous apex (PA) are rare and only a few reports have been published in the English literature. At the PA, DCs are generally in the intradural compartment, arising from the PA and less frequently from the middle cranial fossa (MCF) floor; however, they may also arise from the lateral wall of the cavernous sinus (CS)4 and present interdurally. In 2007, Ammirati et al. reported the first patient with a totally extradural DC of the PA, associated with extensive erosion of the petrous bone and the MCF floor.5 We present a patient with a giant DC of the PA with interdural growth, but similarly associated with erosion of the petrous tip, reviewing the relationships of these dysembryogenic lesions, the dura mater and the neurovascular structures of this complex anatomical region, along with the relevant literature.
At surgery, after a temporal craniotomy, opening of dura mater vault revealed the temporal lobe pushed up by the large capsulated mass (Fig. 3A). Incision of this capsule produced pearly contents consistent with desquamated cell debris and black hairy fine filaments (Fig. 3B). Progressive capsule removal and debulking of the mass uncovered the free border of the tentorium and the fourth and fifth cranial nerves along their cisternal course, previously compressed and displaced by the tumour (Fig. 3D). At the end of the debulking, the skull base was exposed, covered by a dural layer interrupted at the PA, where clear bone erosion was evident (Fig. 3C). Complete removal of the tumour was limited by its adhesion to the Gasserian ganglion and the CS. Histopathological examination was consistent with a DC and the capsule was dura mater (Fig. 4).
2. Case report 2.1. History and examination A 37-year-old woman was admitted to our department with a 3-month history of dizziness and right facial paresthesia, associated with occasional ipsilateral facial pain, along the distribution area of the second division of the trigeminal nerve. Her neurological examination revealed hypoesthesia along the same territory, without further cranial nerve deficits. A brain MRI with and without gadolinium showed a large temporal mass (41 mm 38 mm), located in the right MCF, at the PA, close to the lateral wall of the CS, that displaced the ipsilateral middle cerebral artery medially and partially obliterated the temporal horn of the lateral ventricle. This mass appeared hypointense with hyperintense striations on T1-weighted MRI and hyperintense on T2-weighted MRI, without significant contrast enhancement (Fig. 1). A fine-cut CT scan defined the erosion of the right PA and the scalloping of the sphenoid wing and the MCF floor (Fig. 2).
* Corresponding author. Tel.: +39 630154120; fax: +39 630151343. E-mail address: [email protected] (C.L. Sturiale).
2.3. Postoperative course The patient’s dizziness disappeared postoperatively but mild hemifacial paresthesia persisted. Her postoperative course was otherwise uneventful. Postoperative neuroimaging confirmed a small residual tumour, adherent to the neurovascular structures of the CS. At 1-year follow-up, the patient’s facial pain and paresthesia had resolved. 3. Discussion DCs are developmental anomalies involving pluripotential embryonal cells that probably originate from ectodermal remnants and are frequently located close to the midline. These lesions are frequently seen early in life1,6,7 and are 3 to 10 times less frequent than epidermoid lesions, accounting for 0.04% to 0.07% of all intracranial tumours. Supratentorial DCs are usually located in the suprasellar, parasellar, temporal and frontobasal regions.8–10 Those located at the PA are rare with only few reports in the literature. Gacek argues persuasively that dysembryogenetic tumours of the PA originate in the area of the foramen lacerum, because it contains embryologic remnants of both ectoderm and mesoderm and thus seems the most likely origin of many of these tumours, including DCs.11 Headache, seizures, hemiparesis, visual field defects, signs of increased intracranial pressure and, less frequently, meningeal signs, exophthalmos and oculomotor palsy have been reported as clinical features.6,7,12
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1498–1502
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Fig. 1. (A) Axial T1-weighted MRI, (B) axial T1-weighted gadolinium (Gd)-enhanced MRI, (C) axial T2-weighted MRI, (D) axial fluid-attenuated inversion recovery (FLAIR) MRI, (E) coronal T1-weighted Gd-enhanced and (F) coronal T2-weighted MRI showing a large temporal mass in the right middle cranial fossa, which is hyperintense on T2weighted and FLAIR sequences and hypointense with hyperintense striations on T1-weighted sequences, without significant contrast enhancement.
The pathophysiology of these lesions is related to their slow growth and resulting compression and dislocation of nearby neurovascular structures.12 These lesions grow in a complex intracranial space, medially limited by the CS, postero-inferiorly by the PA and the Gasserian ganglion, and anterio-inferiorly by the sphenoid wing with its foraminae in which many neurovascular structures converge. Furthermore, in this region, the dura mater of the MCF floor duplicates itself as a double layer to constitute Meckel’s cave and the lateral wall of the CS.13,14
Fig. 2. (A, B) Thin section CT scan of petrous pyramids showing the erosion of the right petrous apex and the scalloping of the sphenoid wing and middle cranial fossa floor; (C, D) coronal reconstruction of CT angiograms showing an unenhanced large mass close to the ipsilateral cavernous sinus, that erodes the right cranial middle fossa floor.
Infrequently these tumours rupture spontaneously1,15–19 or following trauma,20,21 spreading their content within the subarachnoid and/or intraventricular space, causing chemical meningitis and ventriculitis, headache and seizures. Intratumoral haemorrhage is, however, rare.22–24 More usually, the symptoms occur late in the clinical course because of the slow growth of these lesions.8 Some patients may be asymptomatic or have only mild symptoms for a long time, even though upto half of supratentorial DCs have radiological or clinical evidence of rupture at diagnosis.25 Differential diagnosis of lesions of the PA includes a wide spectrum of diseases: primary tumours, metastasis, cholesterol granulomas, epidermoid cysts (ECs), mucocele, and aneurysms of the carotid artery.26 Cholesterol granulomas are the most frequent surgical lesions of the pneumatised PA (up to 60%), most likely arising from obstruction of air cells and microhaemorrhage: these are not neoplasms but granulomatous reactions to blood breakdown products, consisting primarily of cholesterol crystals. Neuroimaging, nevertheless, is not always able to distinguish these granulomas from DCs/ECs.27,28 DCs and ECs are both congenital dysembryogenic tumours that develop from inclusion of ectodermal elements during neural tube closure. This defect occurs earlier for DC formation compared to ECs and may explain the propensity of DCs rather than ECs for the midline. Failure of the surface ectoderm to separate from the underlying mesodermal structures or sequestration of surface ectoderm may both occur as embryological accidents between weeks 3 and 5 of gestation. ECs, of course, can form later in life, as a consequence of implantation. Embryological sites of dermal fusion are situated around the eyes, ears and face and explain the common sites for EC formation, mostly intradiploic and extradural, with a tendency to occur around the temporal and parietal bones, the anterior fontanelle and the superolateral orbital ridge.29 DCs, instead, are more common in the spine, in the posterior fossa around the cerebellar vermis and the fourth ventricle and less frequently, in the supraparasellar and pineal regions. Histopathologically, these are both unilocular cysts, but they produce different ectodermal remnants. DCs include a variety of dermal components,
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Fig. 3. Image taken after a right temporal polectomy showing: (A) a large mass covered by dura mater (arrow); (A, C) the cranial base after debulking of the mass covered by dura mater (asterisks); (C) bone erosion at the cranial base (arrowhead); (B) after incision of the mass, pearly contents are consistent with desquamated cell debris and black hair filaments; (D) after debulking of the mass, the fourth (white arrow) and fifth (black arrow) cranial nerves and right posterior communicating artery (white arrowhead) along their cisternal course. This figure is available in colour at www.sciencedirect.com.
Fig. 4. The histopathological sections of the surgical specimen showing dermal elements, lamellated keratinous debris and abundant sebaceous glands lined by stratified keratinizing squamous epithelium (hematoxylin and eosin stain, upper right 25; upper left 50; lower right 200; lower left 100). This figure is available in colour at www.sciencedirect.com.
including the material that makes up hair, teeth, nails and sebaceous glands. ECs, however, contain only squamous epithelium that tends to exfoliate. However, there are no trapped mesodermal elements in DCs and ECs: hence, adipose tissue, which arises from the mesodermal layer, is not a constituent of DCs and the ‘‘fatty”, cheesy content of these cysts is actually keratinaceous debris, liquid cholesterol, sebaceous and sweat secretion as well as ectodermal appendages.29 DCs tend to grow more rapidly then ECs
because of endocrine influences;30 however, the only mechanism of enlargement for ECs is squamous epithelial exfoliation into the cyst.31 Even though DCs and ECs often display the same densitometric characteristics, appearing as lobular or rounded hypodense or isodense areas on CT scans, DCs show a greater range of attenuation values than ECs (ranging from 0 to –150 HU), generally without contrast enhancement. Occasionally, however, these cysts do show contrast enhancement, frequently peripherally, with focal
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1498–1502
calcifications. Moreover, they can be associated with dermal sinus tracts and skeletal anomalies.12,32 On MRI, DCs show variable attenuation times depending on the fat content. On T1-weighted MRI they are frequently hyperintense (because of the short T1 attenuation time of lipids), and are sometimes inhomogeneous due to hair or epithelial debris; an inhomogeneous signal is also typical on T2-weighted MRI.25 Hair contained in the cysts appears as hypointense striations,33 as observed in our patient. The importance of the relationships between lesions of the PA and the dura mater in this region has not always been emphasized in the literature. Many reports claim that DCs in the parasellar region (related to the CS, Meckel’s cave or the PA) are intradural.6,16,34 Less commonly, some authors describe these tumours to be interdural.4,35–38 The interdural location of parasellar ECs is widely accepted in the international literature, because these lesions are believed to originate between the two layers of lateral wall of the CS or between the two dural layers of Meckel’s cave.13,14,39 In 1992, El-Kalliny reported a series of CS lateral wall tumours, describing the surgical anatomy of this region.14 On the basis of this study, Gharabaghi et al. have divided the ECs involving the CS into three groups: (i) extracavernous cysts that invade the CS and originate generally at the PA or in Meckel’s cave (the second type can be interdural); (ii) ECs of the CS lateral wall that originate between the thicker outer dural layer (dura propria) and the thinner inner membranous layer; and (iii) true intracavernous epidermoid tumours, which tend to encase the internal carotid artery, encircling and laterally displacing the cranial nerves.13 MRI can sometimes aid recognition of interdural lesions because of their typically smooth contours, oval shape and associated medial displacement of the CS neurovascular structures.38 With respect to DCs, North et al. reported a 4-year-old boy with an oculomotor palsy, describing a lesion located between the dural layers of the CS lateral wall, without invasion.35 Intradural and interdural growth patterns have been also been reported by Chen et al. describing dumbbell-shaped dermoids that extend from the CS to the interpeduncular cistern, but without lateral extension.36 Pure interdural DCs of the CS have been described by Nakagawa et al.37 A rare extradural location, associated with PA erosion, was first reported by Ammirati et al. in 2007. In our patient, although the lesion clearly developed between the two dural layers, similar bone erosion of the skull base was evident. In most reports a partial CS syndrome has been reported as a consequence of tumour masses in this region. In our patient, although the lesion was large, the patient complained only of a moderate neuralgia the involved the right second division of the trigeminal nerve area, probably resulting from the compression of the fifth cranial nerve, along its pathway from the Gasserian ganglion to the CS or into Meckel’s cave. Alhough total resection of intracranial DCs has been reported by many authors12,34,37 and specific neurosurgical techniques have been described to permit reflection of the dura propria of the lateral wall without entering the CS (Hakuba or Dolenc),14 some authors believe that an aggressive surgical approach results in a higher mortality.40,41 Total removal is not recommended when these lesions adhere to the CS neurovascular structures, as this is associated with high morbidity and mortality; and a successful reoperation for recurrence can be considered.6,42 4. Conclusions Despite careful preoperative evaluation by neuroradiological imaging, it can be often difficult to define the exact growth pattern of the temporal DCs. Thus, although complete resection remains the treatment of choice to limit recurrence from tumour remnants
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or capsule, conservative surgery should be considered for interdural DCs when the cyst adheres tightly to the nearby CS neurovascular structures, because of the related high morbidity and mortality. References 1. Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and surgical results. J Neurosurg 1977;47:12–8. 2. Carmel PW. Brain tumors of disordered embryogenesis. In: Youmans JR, editor. Neurological surgery. Philadelphia, PA: Saunders; 1990. 3. Baxter JW, Netsky MG. Epidermoid and dermoid tumors: pathology. In: Wilkins SS, Rengachary SS, editors. Neurosurgery, vol. 1. New York: McGraw-Hill; 1985. p. 655–61. 4. 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. 5. Ammirati M, Delgado M, Slone W, et al. Extradural dermoid tumor of the petrous apex: case report. J Neurosurg 2007;107:426–9. 6. Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. 7. Rubin G, Scienza R, Pasqualin A, et al. Cranio-cerebral epidermoid and dermoid. A review of 44 cases. Acta Neurochir (Wien) 1989;97:1–16. 8. Caldarelli M, Colosimo C, Di Rocco C. Intra-axial dermoid/epidermoid tumors of the brainstem in children. Pediatr Neurosurg 2001;56:97–105. 9. Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumors. Neurochirurgia 1976;19:104–14. 10. Hamer J. Diagnosis by computerized tomography of intradural dermoid with spontaneous rupture of the cyst. Acta Neurochir (Wien) 1980;51:219–26. 11. Gacek RR. Diagnosis and management of primary tumous of the petrous apex. Ann Otol Rhinol Laryngol 1975;18(Suppl.):1–20. 12. Yasßargil MG, Abernathey CD, Sarioglu AÇ. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurg 1989;24:561–7. 13. Gharabaghi A, Koerbel A, Samii A, et al. Epidermoid cyst of the cavernous sinus. Surg Neurol 2005;64:428–33. 14. el-Kalliny M, van Loveren H, Keller JT, et al. Tumors of the lateral wall of the cavernous sinus. J Neurosurg 1992 Oct;77:508–14. 15. van der Graaff M, Davies G. Status epilepticus due to a ruptured dermoid cyst. J Neurol Neurosurg Psychiatry 1997;62:222. 16. Wilms G, Casselman J, Demaerel P, et al. CT and MRI of ruptured intracranial dermoids. Neuroradiology 1991;33:149–51. 17. Smith AS, Benson JE, Blaser SI, et al. Diagnosis of ruptured intracranial dermoid cyst: value MR over CT. Am J Neuroradiol 1991;12:175–80. 18. Martin R, Knone A, Schuknecht B, et al. Rapid development of occlusion hydrocephalus by intraventricular fat possibly derived from a ruptured dermoid cyst. J Neurol Neurosurg Psychiatry 1989;52:134–5. 19. Nakamura M, Mizuguchi M, Momoi MY, et al. Transient cheiro-oral syndrome due to a ruptured intracranial dermid cyst. Brain Dev 2001;23:261–3. 20. Scearce TA, Shaw CM, Bronstein AD, et al. Intraventricular fat from a ruptured sacral dermoid cyst: clinical, radiographic and pathological correlation. Case report. J Neuroimag 1994;4:169–70. 21. Maravilla KR. Intraventricular fat fluid level secondary to rupture of an intracranial dermoid cyst. Am J Radiol 1977;128:500–1. 22. Mamata H, Matsumae M, Yanagimachi N, et al. Parasellar dermoid tumor with intra-tumoral hemorrhage. Case report. Eur Radiol 1998;8.. 1594–7. 23. Wakai S, Yamakawa K, Manaka S, et al. Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. Neurosurgery 1982;10:437. 24. Iwama T, Ohkuma A, Miwa Y, et al. Brain tumours manifesting as intracranial hemorrhage. Neurol Med Chir 1992;32:130. 25. Messori A, Polonara G, Serio A, et al. Expanding experience with spontaneous dermoid rupture in the MRI era: diagnosis and follow-up. European J Radiol 2002;43:19–27. 26. Chaljub G, Vrabec J, Hollingsworth C, et al. Magnetic resonance imaging of petrous tip lesions. Am J Otolaryngology 1999;20:304–13. 27. Castillo MP, Samy RN, Isaacson B, et al. Petrous apex cholesterol granuloma aeration: does it matter? Otolaryngol Neck Surg 2008;138:518–22. 28. Connor SEJ, Leung R, Natas S. Imaging of the petrous apex: a pictorial review. Br J Radiol 2008;81:427–35. 29. Jonson DG, Stemper SJ, Withers TK. Ruptured ‘‘giant” supratentorial dermoid cyst. J Clin Neurosci 2005;12:198–201. 30. Kalmes D, Gray L. Epidermoid and dermoid tumors: imaging. In: Wilkins RH, Rengachary SS, editors. Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996. p. 965–71. 31. Smirniotopoulos JG, Chiechi MV. Teratomas, dermoid, epidermoids of the head and neck. Radiographics 1995;15:1437–55. 32. Osborne DR. Epidermoid and dermoid tumours: radiology. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, vol. 1. New York: McGraw-Hill; 1985. p. 662–7. 33. Markus H, Kendall BE. MRI of a dermoid cyst containing hair. Neuroradiology 1993;35:256–7. 34. Gormley WB, Tomecek FJ, Qureshi N, et al. Craniocerebral epidermoid and dermoid tumors: a review of 32 cases. Acta Neurochir (Wien) 1994;128:115–21. 35. North KN, Anthony JH, Johnston IH. Dermoid of cavernous sinus resulting in isolated oculomotor nerve palsy. Pediatr Neurol 1993;9:221–3. 36. Chen Y-F, Liu H-M, Tu Y-K. Dermoid cyst as a dumbbell-shaped tumour of the cavernous sinus. Pediatr Radiol 2003;33:72.
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37. Nakagawa K, Ohno K, Nojiri T, et al. Interdural dermoid cyst of the cavernous sinus presenting with oculomotor palsy: case report (Jpn). No Shinkei Geka 1997;25:847-851. . 38. Tun K, Celimez RC, Okutan O, et al. Dermoid tumour of the lateral wall of the cavernous sinus. J Clin Neurosci 2008;15:820–3. 39. Tatagiba M, Iaconetta G, Samii M. Epidermoid cyst of the cavernous sinus: clinical features, pathogenesis and treatment. Br J Neurosurg Dec 2000;14: 571–5.
40. Fournier D, Mercier P, Menei P, et al. Recurrent intrinsic brainstem epidermoid cyst. Childs Nerv Syst 1992;8:471–4. 41. Ogawa T, Sekino H, Fuse T, et al. Multiple intracranial epidermoids located in the brainstem and the middle cranial fossa: case report. Neurol Med Chir (Tokyo) 1985;25:393–7. 42. 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.
doi:10.1016/j.jocn.2009.02.010
Clear cell meningioma presenting as rapidly deteriorating visual field and acuity during pregnancy D.S. Baxter a,*, P. Smith a, K. Stewart b, M. Murphy a a b
Department of Neurosurgery, St Vincent’s Hospital, Victoria Parade, Fitzroy 3065, Victoria, Australia Department of Pathology, St Vincent’s Hospital, Fitzroy, Australia
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Article history: Received 30 October 2008 Accepted 3 February 2009
Keywords: Clear cell meningioma Meningioma Pregnancy Visual field Visual acuity
a b s t r a c t Clear cell meningioma is a rare histological phenotype of meningioma. It has an atypical grade II World Health Organization classification due to a high recurrence rate. This report documents the rapid deterioration of visual field and acuity in a pregnant 32-year-old woman and the successful post-operative resolution of symptoms. We review the literature about this rare disease and identify clinical and pathological features unique to this patient. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction Clear cell meningioma (CCM) is an uncommon histological variant of meningioma. It was first reported in 1993, and since then there have been fewer than 50 reports. CCM represents about 0.2% of all meningiomas.1 They have a high recurrence rate (between 22% and 61%) and are classified as grade II of III in the World Health Organization (WHO) grading system of meningiomas.2 2. Case report A 32-year-old gravida 3 primi 2 orthoptist presented to her local doctor at 24-weeks’ gestation with a 7-week history of deteriorating vision in her left eye, and several weeks of morning headaches but no symptoms of nausea or vomiting. The pregnancy until presentation had been otherwise uneventful. Visual examination demonstrated visual acuity deterioration from 6/4 to 6/36 and an afferent pupillary defect. Visual field examination revealed a temporal hemianopia affecting the left eye. MRI, completed without gadolinium contrast due to her pregnancy, revealed a mass lesion superior to the tuberculum sellae with displacement of the left optic nerve (Fig. 1a). A right extended pterional craniotomy was performed with excision of the tumour. The patient was positioned supine with a wedge placed under her pelvis to accommodate her gravid abdomen. External foetal heart rate monitoring was used pre-operatively and post-operatively, but not intraoperatively. The postoperative course was routine with obstetric examination demon* Corresponding author. Tel.: +61 3 9288 2211; fax: +61 3 9288 3350. E-mail address: [email protected] (D.S. Baxter).
strating a viable foetus. Post-operative visual field and acuity examination revealed complete normalisation of vision. Pituitary function remained normal throughout the pre-operative and post-operative course. Histological examination confirmed a tumour showing a loosely swirling architecture of bland cells with ovoid nuclei streaming through a myxoid stroma (Fig. 2a). Some areas showed cells with clear perinuclear halos and well-defined cell borders containing periodic acid-Schiff-positive, diastase-labile glycogen droplets (Fig. 2b,c). Typical whorling architecture and psammoma bodies were not identified. There was no necrosis, mitoses or vascular endothelial proliferation. Immunohistochemistry staining was positive for epithelial membrane antigen and progesterone receptor (PR), and negative for glial fibrillary acidic protein, estrogen receptor, cytokeratin and neuroendocrine stains. The features were in keeping with the clear-cell variant of meningioma. Scans were repeated post-partum with contrast at both 5 months and 7 months post-operatively. There was evidence of a small residual extra-axial mass located anteriorly within the suprasellar cistern (Fig. 1b). There was no change in size between the scans, which therefore represented residual rather than recurrent tumour. Stereotactic radiosurgery for the residual tumour was considered, although it was decided to follow the patient with serial scans before proceeding with further surgical and/or radiosurgical treatment. 3. Discussion The five main points of interest in this report are that: (i) CCM is uncommon; (ii) the patient was pregnant; (iii) the progression of the symptoms was alarmingly rapid; (iv) the anatomical location
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1498–1502
Interdural giant dermoid cyst of the petrous apex Carmelo Lucio Sturiale *, Annunziato Mangiola, Angelo Pompucci, Manuela D’Ercole, Licia Di Muro, Carmelo Anile Institute of Neurosurgery, Catholic University School of Medicine, Largo Agostino Gemelli 8, 00168, Rome, Italy
a r t i c l e
i n f o
Article history: Received 14 January 2009 Accepted 25 February 2009
Keywords: Dermoid cyst Interdural Petrous apex
a b s t r a c t Dermoid cysts account for less than 1% of all intracranial neoplasms and these are principally situated close to the midline. Those located at the petrous apex are generally in the intradural, and less frequently in the interdural, compartment. We report a 37-year-old woman with a giant interdural dermoid cyst of the petrous apex, associated with erosion of the middle fossa floor, and review the relationships of these dysembryogenic lesions, the dura mater and the neurovascular structures of this complex anatomical region, along with the relevant literature. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2.2. Operation
Dermoid cysts (DCs) are rare congenital tumours that account for less than 1% of all intracranial neoplasms and can be found in various locations, mainly close to the midline, both in the supraand infratentorial regions.1 These tumours originate from epithelial elements contained within the neural groove when it forms the neural tube (weeks 3–5 of embryonic life).2,3 Those located at the petrous apex (PA) are rare and only a few reports have been published in the English literature. At the PA, DCs are generally in the intradural compartment, arising from the PA and less frequently from the middle cranial fossa (MCF) floor; however, they may also arise from the lateral wall of the cavernous sinus (CS)4 and present interdurally. In 2007, Ammirati et al. reported the first patient with a totally extradural DC of the PA, associated with extensive erosion of the petrous bone and the MCF floor.5 We present a patient with a giant DC of the PA with interdural growth, but similarly associated with erosion of the petrous tip, reviewing the relationships of these dysembryogenic lesions, the dura mater and the neurovascular structures of this complex anatomical region, along with the relevant literature.
At surgery, after a temporal craniotomy, opening of dura mater vault revealed the temporal lobe pushed up by the large capsulated mass (Fig. 3A). Incision of this capsule produced pearly contents consistent with desquamated cell debris and black hairy fine filaments (Fig. 3B). Progressive capsule removal and debulking of the mass uncovered the free border of the tentorium and the fourth and fifth cranial nerves along their cisternal course, previously compressed and displaced by the tumour (Fig. 3D). At the end of the debulking, the skull base was exposed, covered by a dural layer interrupted at the PA, where clear bone erosion was evident (Fig. 3C). Complete removal of the tumour was limited by its adhesion to the Gasserian ganglion and the CS. Histopathological examination was consistent with a DC and the capsule was dura mater (Fig. 4).
2. Case report 2.1. History and examination A 37-year-old woman was admitted to our department with a 3-month history of dizziness and right facial paresthesia, associated with occasional ipsilateral facial pain, along the distribution area of the second division of the trigeminal nerve. Her neurological examination revealed hypoesthesia along the same territory, without further cranial nerve deficits. A brain MRI with and without gadolinium showed a large temporal mass (41 mm 38 mm), located in the right MCF, at the PA, close to the lateral wall of the CS, that displaced the ipsilateral middle cerebral artery medially and partially obliterated the temporal horn of the lateral ventricle. This mass appeared hypointense with hyperintense striations on T1-weighted MRI and hyperintense on T2-weighted MRI, without significant contrast enhancement (Fig. 1). A fine-cut CT scan defined the erosion of the right PA and the scalloping of the sphenoid wing and the MCF floor (Fig. 2).
* Corresponding author. Tel.: +39 630154120; fax: +39 630151343. E-mail address: [email protected] (C.L. Sturiale).
2.3. Postoperative course The patient’s dizziness disappeared postoperatively but mild hemifacial paresthesia persisted. Her postoperative course was otherwise uneventful. Postoperative neuroimaging confirmed a small residual tumour, adherent to the neurovascular structures of the CS. At 1-year follow-up, the patient’s facial pain and paresthesia had resolved. 3. Discussion DCs are developmental anomalies involving pluripotential embryonal cells that probably originate from ectodermal remnants and are frequently located close to the midline. These lesions are frequently seen early in life1,6,7 and are 3 to 10 times less frequent than epidermoid lesions, accounting for 0.04% to 0.07% of all intracranial tumours. Supratentorial DCs are usually located in the suprasellar, parasellar, temporal and frontobasal regions.8–10 Those located at the PA are rare with only few reports in the literature. Gacek argues persuasively that dysembryogenetic tumours of the PA originate in the area of the foramen lacerum, because it contains embryologic remnants of both ectoderm and mesoderm and thus seems the most likely origin of many of these tumours, including DCs.11 Headache, seizures, hemiparesis, visual field defects, signs of increased intracranial pressure and, less frequently, meningeal signs, exophthalmos and oculomotor palsy have been reported as clinical features.6,7,12
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Fig. 1. (A) Axial T1-weighted MRI, (B) axial T1-weighted gadolinium (Gd)-enhanced MRI, (C) axial T2-weighted MRI, (D) axial fluid-attenuated inversion recovery (FLAIR) MRI, (E) coronal T1-weighted Gd-enhanced and (F) coronal T2-weighted MRI showing a large temporal mass in the right middle cranial fossa, which is hyperintense on T2weighted and FLAIR sequences and hypointense with hyperintense striations on T1-weighted sequences, without significant contrast enhancement.
The pathophysiology of these lesions is related to their slow growth and resulting compression and dislocation of nearby neurovascular structures.12 These lesions grow in a complex intracranial space, medially limited by the CS, postero-inferiorly by the PA and the Gasserian ganglion, and anterio-inferiorly by the sphenoid wing with its foraminae in which many neurovascular structures converge. Furthermore, in this region, the dura mater of the MCF floor duplicates itself as a double layer to constitute Meckel’s cave and the lateral wall of the CS.13,14
Fig. 2. (A, B) Thin section CT scan of petrous pyramids showing the erosion of the right petrous apex and the scalloping of the sphenoid wing and middle cranial fossa floor; (C, D) coronal reconstruction of CT angiograms showing an unenhanced large mass close to the ipsilateral cavernous sinus, that erodes the right cranial middle fossa floor.
Infrequently these tumours rupture spontaneously1,15–19 or following trauma,20,21 spreading their content within the subarachnoid and/or intraventricular space, causing chemical meningitis and ventriculitis, headache and seizures. Intratumoral haemorrhage is, however, rare.22–24 More usually, the symptoms occur late in the clinical course because of the slow growth of these lesions.8 Some patients may be asymptomatic or have only mild symptoms for a long time, even though upto half of supratentorial DCs have radiological or clinical evidence of rupture at diagnosis.25 Differential diagnosis of lesions of the PA includes a wide spectrum of diseases: primary tumours, metastasis, cholesterol granulomas, epidermoid cysts (ECs), mucocele, and aneurysms of the carotid artery.26 Cholesterol granulomas are the most frequent surgical lesions of the pneumatised PA (up to 60%), most likely arising from obstruction of air cells and microhaemorrhage: these are not neoplasms but granulomatous reactions to blood breakdown products, consisting primarily of cholesterol crystals. Neuroimaging, nevertheless, is not always able to distinguish these granulomas from DCs/ECs.27,28 DCs and ECs are both congenital dysembryogenic tumours that develop from inclusion of ectodermal elements during neural tube closure. This defect occurs earlier for DC formation compared to ECs and may explain the propensity of DCs rather than ECs for the midline. Failure of the surface ectoderm to separate from the underlying mesodermal structures or sequestration of surface ectoderm may both occur as embryological accidents between weeks 3 and 5 of gestation. ECs, of course, can form later in life, as a consequence of implantation. Embryological sites of dermal fusion are situated around the eyes, ears and face and explain the common sites for EC formation, mostly intradiploic and extradural, with a tendency to occur around the temporal and parietal bones, the anterior fontanelle and the superolateral orbital ridge.29 DCs, instead, are more common in the spine, in the posterior fossa around the cerebellar vermis and the fourth ventricle and less frequently, in the supraparasellar and pineal regions. Histopathologically, these are both unilocular cysts, but they produce different ectodermal remnants. DCs include a variety of dermal components,
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Fig. 3. Image taken after a right temporal polectomy showing: (A) a large mass covered by dura mater (arrow); (A, C) the cranial base after debulking of the mass covered by dura mater (asterisks); (C) bone erosion at the cranial base (arrowhead); (B) after incision of the mass, pearly contents are consistent with desquamated cell debris and black hair filaments; (D) after debulking of the mass, the fourth (white arrow) and fifth (black arrow) cranial nerves and right posterior communicating artery (white arrowhead) along their cisternal course. This figure is available in colour at www.sciencedirect.com.
Fig. 4. The histopathological sections of the surgical specimen showing dermal elements, lamellated keratinous debris and abundant sebaceous glands lined by stratified keratinizing squamous epithelium (hematoxylin and eosin stain, upper right 25; upper left 50; lower right 200; lower left 100). This figure is available in colour at www.sciencedirect.com.
including the material that makes up hair, teeth, nails and sebaceous glands. ECs, however, contain only squamous epithelium that tends to exfoliate. However, there are no trapped mesodermal elements in DCs and ECs: hence, adipose tissue, which arises from the mesodermal layer, is not a constituent of DCs and the ‘‘fatty”, cheesy content of these cysts is actually keratinaceous debris, liquid cholesterol, sebaceous and sweat secretion as well as ectodermal appendages.29 DCs tend to grow more rapidly then ECs
because of endocrine influences;30 however, the only mechanism of enlargement for ECs is squamous epithelial exfoliation into the cyst.31 Even though DCs and ECs often display the same densitometric characteristics, appearing as lobular or rounded hypodense or isodense areas on CT scans, DCs show a greater range of attenuation values than ECs (ranging from 0 to –150 HU), generally without contrast enhancement. Occasionally, however, these cysts do show contrast enhancement, frequently peripherally, with focal
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calcifications. Moreover, they can be associated with dermal sinus tracts and skeletal anomalies.12,32 On MRI, DCs show variable attenuation times depending on the fat content. On T1-weighted MRI they are frequently hyperintense (because of the short T1 attenuation time of lipids), and are sometimes inhomogeneous due to hair or epithelial debris; an inhomogeneous signal is also typical on T2-weighted MRI.25 Hair contained in the cysts appears as hypointense striations,33 as observed in our patient. The importance of the relationships between lesions of the PA and the dura mater in this region has not always been emphasized in the literature. Many reports claim that DCs in the parasellar region (related to the CS, Meckel’s cave or the PA) are intradural.6,16,34 Less commonly, some authors describe these tumours to be interdural.4,35–38 The interdural location of parasellar ECs is widely accepted in the international literature, because these lesions are believed to originate between the two layers of lateral wall of the CS or between the two dural layers of Meckel’s cave.13,14,39 In 1992, El-Kalliny reported a series of CS lateral wall tumours, describing the surgical anatomy of this region.14 On the basis of this study, Gharabaghi et al. have divided the ECs involving the CS into three groups: (i) extracavernous cysts that invade the CS and originate generally at the PA or in Meckel’s cave (the second type can be interdural); (ii) ECs of the CS lateral wall that originate between the thicker outer dural layer (dura propria) and the thinner inner membranous layer; and (iii) true intracavernous epidermoid tumours, which tend to encase the internal carotid artery, encircling and laterally displacing the cranial nerves.13 MRI can sometimes aid recognition of interdural lesions because of their typically smooth contours, oval shape and associated medial displacement of the CS neurovascular structures.38 With respect to DCs, North et al. reported a 4-year-old boy with an oculomotor palsy, describing a lesion located between the dural layers of the CS lateral wall, without invasion.35 Intradural and interdural growth patterns have been also been reported by Chen et al. describing dumbbell-shaped dermoids that extend from the CS to the interpeduncular cistern, but without lateral extension.36 Pure interdural DCs of the CS have been described by Nakagawa et al.37 A rare extradural location, associated with PA erosion, was first reported by Ammirati et al. in 2007. In our patient, although the lesion clearly developed between the two dural layers, similar bone erosion of the skull base was evident. In most reports a partial CS syndrome has been reported as a consequence of tumour masses in this region. In our patient, although the lesion was large, the patient complained only of a moderate neuralgia the involved the right second division of the trigeminal nerve area, probably resulting from the compression of the fifth cranial nerve, along its pathway from the Gasserian ganglion to the CS or into Meckel’s cave. Alhough total resection of intracranial DCs has been reported by many authors12,34,37 and specific neurosurgical techniques have been described to permit reflection of the dura propria of the lateral wall without entering the CS (Hakuba or Dolenc),14 some authors believe that an aggressive surgical approach results in a higher mortality.40,41 Total removal is not recommended when these lesions adhere to the CS neurovascular structures, as this is associated with high morbidity and mortality; and a successful reoperation for recurrence can be considered.6,42 4. Conclusions Despite careful preoperative evaluation by neuroradiological imaging, it can be often difficult to define the exact growth pattern of the temporal DCs. Thus, although complete resection remains the treatment of choice to limit recurrence from tumour remnants
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or capsule, conservative surgery should be considered for interdural DCs when the cyst adheres tightly to the nearby CS neurovascular structures, because of the related high morbidity and mortality. References 1. Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and surgical results. J Neurosurg 1977;47:12–8. 2. Carmel PW. Brain tumors of disordered embryogenesis. In: Youmans JR, editor. Neurological surgery. Philadelphia, PA: Saunders; 1990. 3. Baxter JW, Netsky MG. Epidermoid and dermoid tumors: pathology. In: Wilkins SS, Rengachary SS, editors. Neurosurgery, vol. 1. New York: McGraw-Hill; 1985. p. 655–61. 4. 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. 5. Ammirati M, Delgado M, Slone W, et al. Extradural dermoid tumor of the petrous apex: case report. J Neurosurg 2007;107:426–9. 6. Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. 7. Rubin G, Scienza R, Pasqualin A, et al. Cranio-cerebral epidermoid and dermoid. A review of 44 cases. Acta Neurochir (Wien) 1989;97:1–16. 8. Caldarelli M, Colosimo C, Di Rocco C. Intra-axial dermoid/epidermoid tumors of the brainstem in children. Pediatr Neurosurg 2001;56:97–105. 9. Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumors. Neurochirurgia 1976;19:104–14. 10. Hamer J. Diagnosis by computerized tomography of intradural dermoid with spontaneous rupture of the cyst. Acta Neurochir (Wien) 1980;51:219–26. 11. Gacek RR. Diagnosis and management of primary tumous of the petrous apex. Ann Otol Rhinol Laryngol 1975;18(Suppl.):1–20. 12. Yasßargil MG, Abernathey CD, Sarioglu AÇ. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurg 1989;24:561–7. 13. Gharabaghi A, Koerbel A, Samii A, et al. Epidermoid cyst of the cavernous sinus. Surg Neurol 2005;64:428–33. 14. el-Kalliny M, van Loveren H, Keller JT, et al. Tumors of the lateral wall of the cavernous sinus. J Neurosurg 1992 Oct;77:508–14. 15. van der Graaff M, Davies G. Status epilepticus due to a ruptured dermoid cyst. J Neurol Neurosurg Psychiatry 1997;62:222. 16. Wilms G, Casselman J, Demaerel P, et al. CT and MRI of ruptured intracranial dermoids. Neuroradiology 1991;33:149–51. 17. Smith AS, Benson JE, Blaser SI, et al. Diagnosis of ruptured intracranial dermoid cyst: value MR over CT. Am J Neuroradiol 1991;12:175–80. 18. Martin R, Knone A, Schuknecht B, et al. Rapid development of occlusion hydrocephalus by intraventricular fat possibly derived from a ruptured dermoid cyst. J Neurol Neurosurg Psychiatry 1989;52:134–5. 19. Nakamura M, Mizuguchi M, Momoi MY, et al. Transient cheiro-oral syndrome due to a ruptured intracranial dermid cyst. Brain Dev 2001;23:261–3. 20. Scearce TA, Shaw CM, Bronstein AD, et al. Intraventricular fat from a ruptured sacral dermoid cyst: clinical, radiographic and pathological correlation. Case report. J Neuroimag 1994;4:169–70. 21. Maravilla KR. Intraventricular fat fluid level secondary to rupture of an intracranial dermoid cyst. Am J Radiol 1977;128:500–1. 22. Mamata H, Matsumae M, Yanagimachi N, et al. Parasellar dermoid tumor with intra-tumoral hemorrhage. Case report. Eur Radiol 1998;8.. 1594–7. 23. Wakai S, Yamakawa K, Manaka S, et al. Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. Neurosurgery 1982;10:437. 24. Iwama T, Ohkuma A, Miwa Y, et al. Brain tumours manifesting as intracranial hemorrhage. Neurol Med Chir 1992;32:130. 25. Messori A, Polonara G, Serio A, et al. Expanding experience with spontaneous dermoid rupture in the MRI era: diagnosis and follow-up. European J Radiol 2002;43:19–27. 26. Chaljub G, Vrabec J, Hollingsworth C, et al. Magnetic resonance imaging of petrous tip lesions. Am J Otolaryngology 1999;20:304–13. 27. Castillo MP, Samy RN, Isaacson B, et al. Petrous apex cholesterol granuloma aeration: does it matter? Otolaryngol Neck Surg 2008;138:518–22. 28. Connor SEJ, Leung R, Natas S. Imaging of the petrous apex: a pictorial review. Br J Radiol 2008;81:427–35. 29. Jonson DG, Stemper SJ, Withers TK. Ruptured ‘‘giant” supratentorial dermoid cyst. J Clin Neurosci 2005;12:198–201. 30. Kalmes D, Gray L. Epidermoid and dermoid tumors: imaging. In: Wilkins RH, Rengachary SS, editors. Neurosurgery. 2nd ed. New York: McGraw-Hill; 1996. p. 965–71. 31. Smirniotopoulos JG, Chiechi MV. Teratomas, dermoid, epidermoids of the head and neck. Radiographics 1995;15:1437–55. 32. Osborne DR. Epidermoid and dermoid tumours: radiology. In: Wilkins RH, Rengachary SS, editors. Neurosurgery, vol. 1. New York: McGraw-Hill; 1985. p. 662–7. 33. Markus H, Kendall BE. MRI of a dermoid cyst containing hair. Neuroradiology 1993;35:256–7. 34. Gormley WB, Tomecek FJ, Qureshi N, et al. Craniocerebral epidermoid and dermoid tumors: a review of 32 cases. Acta Neurochir (Wien) 1994;128:115–21. 35. North KN, Anthony JH, Johnston IH. Dermoid of cavernous sinus resulting in isolated oculomotor nerve palsy. Pediatr Neurol 1993;9:221–3. 36. Chen Y-F, Liu H-M, Tu Y-K. Dermoid cyst as a dumbbell-shaped tumour of the cavernous sinus. Pediatr Radiol 2003;33:72.
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40. Fournier D, Mercier P, Menei P, et al. Recurrent intrinsic brainstem epidermoid cyst. Childs Nerv Syst 1992;8:471–4. 41. Ogawa T, Sekino H, Fuse T, et al. Multiple intracranial epidermoids located in the brainstem and the middle cranial fossa: case report. Neurol Med Chir (Tokyo) 1985;25:393–7. 42. 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.
doi:10.1016/j.jocn.2009.02.010
Clear cell meningioma presenting as rapidly deteriorating visual field and acuity during pregnancy D.S. Baxter a,*, P. Smith a, K. Stewart b, M. Murphy a a b
Department of Neurosurgery, St Vincent’s Hospital, Victoria Parade, Fitzroy 3065, Victoria, Australia Department of Pathology, St Vincent’s Hospital, Fitzroy, Australia
a r t i c l e
i n f o
Article history: Received 30 October 2008 Accepted 3 February 2009
Keywords: Clear cell meningioma Meningioma Pregnancy Visual field Visual acuity
a b s t r a c t Clear cell meningioma is a rare histological phenotype of meningioma. It has an atypical grade II World Health Organization classification due to a high recurrence rate. This report documents the rapid deterioration of visual field and acuity in a pregnant 32-year-old woman and the successful post-operative resolution of symptoms. We review the literature about this rare disease and identify clinical and pathological features unique to this patient. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction Clear cell meningioma (CCM) is an uncommon histological variant of meningioma. It was first reported in 1993, and since then there have been fewer than 50 reports. CCM represents about 0.2% of all meningiomas.1 They have a high recurrence rate (between 22% and 61%) and are classified as grade II of III in the World Health Organization (WHO) grading system of meningiomas.2 2. Case report A 32-year-old gravida 3 primi 2 orthoptist presented to her local doctor at 24-weeks’ gestation with a 7-week history of deteriorating vision in her left eye, and several weeks of morning headaches but no symptoms of nausea or vomiting. The pregnancy until presentation had been otherwise uneventful. Visual examination demonstrated visual acuity deterioration from 6/4 to 6/36 and an afferent pupillary defect. Visual field examination revealed a temporal hemianopia affecting the left eye. MRI, completed without gadolinium contrast due to her pregnancy, revealed a mass lesion superior to the tuberculum sellae with displacement of the left optic nerve (Fig. 1a). A right extended pterional craniotomy was performed with excision of the tumour. The patient was positioned supine with a wedge placed under her pelvis to accommodate her gravid abdomen. External foetal heart rate monitoring was used pre-operatively and post-operatively, but not intraoperatively. The postoperative course was routine with obstetric examination demon* Corresponding author. Tel.: +61 3 9288 2211; fax: +61 3 9288 3350. E-mail address: [email protected] (D.S. Baxter).
strating a viable foetus. Post-operative visual field and acuity examination revealed complete normalisation of vision. Pituitary function remained normal throughout the pre-operative and post-operative course. Histological examination confirmed a tumour showing a loosely swirling architecture of bland cells with ovoid nuclei streaming through a myxoid stroma (Fig. 2a). Some areas showed cells with clear perinuclear halos and well-defined cell borders containing periodic acid-Schiff-positive, diastase-labile glycogen droplets (Fig. 2b,c). Typical whorling architecture and psammoma bodies were not identified. There was no necrosis, mitoses or vascular endothelial proliferation. Immunohistochemistry staining was positive for epithelial membrane antigen and progesterone receptor (PR), and negative for glial fibrillary acidic protein, estrogen receptor, cytokeratin and neuroendocrine stains. The features were in keeping with the clear-cell variant of meningioma. Scans were repeated post-partum with contrast at both 5 months and 7 months post-operatively. There was evidence of a small residual extra-axial mass located anteriorly within the suprasellar cistern (Fig. 1b). There was no change in size between the scans, which therefore represented residual rather than recurrent tumour. Stereotactic radiosurgery for the residual tumour was considered, although it was decided to follow the patient with serial scans before proceeding with further surgical and/or radiosurgical treatment. 3. Discussion The five main points of interest in this report are that: (i) CCM is uncommon; (ii) the patient was pregnant; (iii) the progression of the symptoms was alarmingly rapid; (iv) the anatomical location