- Email: [email protected]
Intracranial epithelioid schwannoma – a case report
Intracranial epithelioid schwannoma
91
Department of Neurosurgery and Surgery, University of Melbourne, Royal Parade, Parkville, Victoria 3050, Australia
REFERENCES 1. Abrams H, Spiro R, Goldstein N. Metastases in carcinoma – analysis of 1000 autopsied cases. Cancer 1950; 3: 74–85. 2. Cotran RS, Kumar V, Collins T. Robbins Pathological Basis of Disease. W.B. Saunders Co., Philadelphia 1999; 991–996. 3. Figlin RA, Piantadosi S, Feld R et al. Intracranial Recurrence of Carcinoma after complete resection of Stage I, II, and III Non-Small-Cell Lung Cancer. N Engl J Med 1988; 318: 1300–1305. 4. Fukui K, Okamura K, Watanabe T et al. Choroid plexus involvement in malignant lymphoma: case report. Neurol Med Chir (Tokyo) 1990; 30: 869–873. 5. Gay P, Litchy W, Cascino T. Brain metastasis in hypernephroma. J Neuro-oncol 1987; 5: 51–56. 6. Healy JF, Rosenkrantz H. Intraventricular metastasis demonstrated by cranial computed tomography. Radiology 1980; 136: 124. 7. Iwatsuki K, Sato M, Taguchi J, Fukui T, Kiyohara H, Yoshimine T, Hayakawa T. Choroid plexus metastasis of renal cell carcinoma causing intraventricular haemorrhage: a case report. No Shinkei Geka 1999; 27(4): 359–363. 8. Jubelirer SJ. Late solitary cerebral metastasis from renal cell carcinoma: a case report and review of the literature. W Va Med J 1996; 92(1): 26–27. 9. Kart BH, Reddy SC, Rao GR et al. Choroid plexus metastasis: CT appearance. J Comput Assist Tomogr 1986; 10: 537–540. 10. Kendall B, Reider-Grosswasser I, Valentine A. Diagnosis of masses presenting within the ventricles on computed tomography. Neuroradiology 1983; 25: 11–22. 11. Khono M, Matsunami M, Sasaki T et al. Solitary metastasis to the choroid plexus of the lateral ventricle: report of three cases and a review of the literature. J Neuro-oncol 1996; 27: 47–52. 12. Kuroki K, Taguchi H, Sumida M, Daimaru Y, Onda J. Cerebral metastasis from a renal cell carcinoma more than 10 years after nephrectomy: report of two cases. No Shinkei Geka 1999; 27(1): 89–93. 13. Matsumura H, Yoshimine T, Yamamoto S, Maruno M, Hayakawa T, Ono Y, Kondoh N, Namiki M. Single solitary metastasis of the slowly progressive type of renal cell carcinoma to the choroid plexus: case report. Neurol Med Chir 1997; 37(12): 916–919. 14. Mertens R, Muhler E, Heimann G. Disseminiertes neuroblastom mit intrazerbraler metastase beim neugeborenen. Klin Padiatr 1987; 199: 424–428. 15. Mizuno M, Asakura K, Nakajima S, Sampei T, Sayama I, Kawamura S, Yasui N, Fukazawa H. Renal cell carcinoma metastasising to choroid plexus of lateral ventricle: a case report. No Shinkei Geka 1992; 20(4): 469–474. 16. Morrison G, Sobel DF, Kelley WM et al. Intraventricular Mass Lesions. Radiology 1984; 153: 435–442. 17. Nakabayashi H, Murata K, Sakaguchi M, Nakajima K, Katsuyama J. Choroid plexus metastasis from gastric cancer: case report. Neurol Med Chir (Tokyo) 1994; 34(3): 183–186. 18. Posner J, Chernik N. Intracranial metastases from systemic cancer. Adv Neurol 1978; 19: 579–592. 19. Qasho R, Tommaso V, Rocchi G, Simi U, Delfini R. Choroid plexus metastasis from carcinoma of the bladder: case report and review of the literature. J Neuro-oncol 1999; 45(3): 237–240. 20. Radley MG, McDonald JV, Pilcher WH, Wilbur DC. Late cerebral metastases from renal cell carcinoma: report of two cases. Surg Neurol 1993; 39(3): 230–234. 21. Raila F, Bottoms W, Fratkin J. Solitary Choroid Plexus Metastasis From a Renal Cell Carcinoma. Southern Med J 1998; 91(12): 1159–1162. 22. Roser F, Rosahl SK, Samii M. Single cerebral metastasis 3 and 19 years after primary renal cell carcinoma: case report and review of the literature. J Neurol Neurosurg Ps 2002; 72(2): 257–258. 23. Saitoh H, Shimbo T, Tasaka T, Iida T, Hara K. Brain metastases of renal adenocarcinoma. Tokai J Exp Med 1982; 7(3): 337–343. 24. Shigemori M, Shimamoto H, Noguchi S et al. Choroid plexus metastasis of renal cell carcinoma. CT Kenkyu 1987; 9: 603–606. 25. Suetake K, Shinya T, Takeda M. A choroid plexus metastasis of a renal cell carcinoma: a case report. Jpn J Neurosurg (Tokyo) 1994; 3: 436–441. 26. Tanimoto M, Tatsumi S, Tominaga S, Kamikawa S, Nagao T, Tamaki N, Matsumoto S. Choroid plexus metastasis of lung carcinoma: a case report. Neurol Med Chir (Tokyo) 1991; 31: 152–155.
Summary We report a case of an intracranial epithelioid schwannoma. Epithelioid schwannoma is a low-grade malignant peripheral nerve sheath tumor that occurs most commonly in the limbs and inguinal region. Association with cranial nerves has been reported to be rare and in almost all cases has only involved extracranial distal branches. ª 2003 Published by Elsevier Ltd. Journal of Clinical Neuroscience (2004) 11(1), 91–95 0967-5868/$ - see front matter ª 2003 Published by Elsevier Ltd. doi:10.1016/S0967-5868(03)00114-0
Keywords: schwannoma, brain tumor, nerve sheath tumor Received 22 January 2003 Accepted 24 January 2003 Correspondence to: Dr. Tanya Yuen, Department of Neurosurgery, Royal Melbourne Hospital, Parkville, 3050, Victoria, Australia. Tel.: +61-3-0342-8218; Fax: +61-3-9342-7273; E-mail: [email protected]
INTRODUCTION Malignant peripheral nerve sheath tumors (MPNST) can exhibit great complexity with intermingling of several tissue components.1 Epithelioid schwannoma, a term first proposed by
Intracranial epithelioid schwannoma – a case report Tanya Yuen,
MBBS
Andrew H. Kaye,
ª 2003 Published by Elsevier Ltd.
MBBS FRACS FRCS
Fig. 1 CT scan showing: (A) Hyperdense enhancing tumor mass centered on the sella turcica, (B) floor of sella eroded centrally, tumor extension into sphenoid sinus, erosion of dorsum sella and adjacent clivus.
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McCormack et al. in 1954,2 is an uncommon variant of MPNST with predominance of epithelioid cells and low- grade malignant behavior,3 found mostly in the limbs. Those involving cranial nerves are extremely rare and even then they are almost always situated extracranially.1;10 We present a case of an epithelioid schwannoma involving the intracranial portion of a cranial nerve. CASE REPORT A 72-year-old female presented with 6 weeks history of sudden onset diplopia and diffuse headache. On examination she had a left sixth nerve palsy. CT and MRI showed an enhancing lesion in the left cavernous sinus extending into the middle cranial fossa. Digital subtraction angiography showed tumor blush suggestive of meningioma. The patient underwent excision via a left pterional craniotomy. Tumor encased the left carotid artery, the optic, third, fourth, fifth and the sixth cranial nerves, preventing complete excision. Histology revealed an epithelioid schwannoma. At two years of follow up MRI showed residual tumor extending into the left sphenoid sinus and floor of the sella, encasing the left cavernous carotid artery. She was lost to follow-up for 8 years then presented again with weight loss and vomiting. On examination visual acuity in the right eye was 6/60 and reduced to hand movement only in the left eye. There were left 3rd, 4th and 6th cranial nerve palsies. CT and MRI revealed a 2.5 1.5 cm hyperdense, uniformly enhancing tumor centered on the sella turcica, extending into the cavernous and sphenoid sinuses and
Fig. 2
eroding the floor of the sella, the dorsum sella and clivus (Figs. 1 and 2). A transphenoidal approach was used to explore the tumor, which had extended through the sphenoid sinus and the pituitary fossa into the suprasellar cistern. Both carotid arteries were encased once again preventing complete resection. Abdominal fat was used to pack the emptied sinus and pituitary fossa. Six months later MRI showed extensive recurrent tumor, completely destroying the sella and adjacent clivus with cavernous carotid encasement, bilateral cavernous sinus infiltration and extension down into the nasopharynx on the right (Fig. 3). The tumor was deemed inoperable and the patient died 6 months later. In both surgical specimens, histology confirmed epithelioid schwannoma with polymorphic features, strong surface immunoreactivity for epithelial membrane antigen but no reactivity for S100 protein, cytokeratin, kappa and lambda light chain, vimentin, chromogranin and leukocyte common antigen (Figs. 4 and 5). DISCUSSION We present a case of an intracranial epithelioid schwannoma arising from a cranial nerve. According to the current WHO classification of CNS tumors6;7 the epithelioid schwannoma is a low-grade malignant peripheral nerve sheath tumor with a predominance of epithelioid cells and which histologically resembles carcinoma or amelanotic melanoma.8 Its incidence among MPNST has been estimated between 5 and 17%, a range that reflects differences in the stringency of diagnostic criteria and clinical evaluation to exclude a carcinoma or melanoma.3;4;9
MRI scan of recurrence showing: (A) Erosion of sella turcica, (B) total encasement of bilateral carotid arteries, (C) erosion of dorsum sella and clivus.
Journal of Clinical Neuroscience (2004) 11(1)
ª 2003 Published by Elsevier Ltd.
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Fig. 3 MRI scan of second recurrence showing: (A) Complete destruction of sella and adjacent clivus, (B) suprasellar extension elevating optic chiasm and invagination into inferior left frontal lobe anteriorly, (C) cavernous carotid encasement, bilateral cavernous sinus infiltration and extension down into the nasopharynx, (D) tumor bulge laterally into adjacent medial temporal lobes, (E) extension inferiorly to fill sphenoid sinus, infiltration of prevertebral musculature at skull base, nasopharynx and bulge into prepontine cistern.
Fig. 4 Histology slides showing: (A) Densely hypercellular tumor in which tumor cells with hyperchromatic nuclei and well-demarcated cytoplasmic borders are arranged in diffuse sheets (H&E X 400), (B) tumor cells with strong immunostaining for epithelial membrane antigen (EMA) (Strepavidin PAP X 400), (C) tumor cells with strong immunostaining for S-100 protein (Streptavidin PAP X 200).
ª 2003 Published by Elsevier Ltd.
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lioid cell.10 However, these antigens are not specific and are shared by other neural and non-neural tissues so that care in interpretation of the antigenic profile of atypical tumors is required.1 While earlier reports based primarily upon deeply situated limb and trunk epithelioid schwannomas indicated a high mortality rate,9 more indolent behavior was noted in the series of Laskin et al.3 In the latter, out of 16 superficial tumors, 2 recurred locally and 2 metastasised, with no disease related deaths. Of the 10 deeply seated tumors 1 recurred locally and 3 died of metastatic disease. All of the tumors in these 2 series were extracranial and the prognosis for intracranial epithelioid schwannoma is unknown. Optimal management for the epithelioid schwannoma appears to be complete resection but this was not technically possible in our case. Low-grade malignant behavior that is characteristic of the tumor was evident in this case with the patient surviving 12 years from the time of diagnosis. In the time she was lost to follow-up, disease extension was such that further surgery could only palliate. CONCLUSION Epithelioid schwannoma, a low-grade malignant peripheral nerve sheath tumor has been reported in many regions of the body. This is a rare case of the tumor occurring intracranially and involving cranial nerves. The patient survived 12 years from the time of diagnosis before succumbing with extensive intracranial tumor invasion.
Fig. 5 Electron micrographs showing: (A) Basal lamina surrounding tumor cells (X30K), (B) (X 210K).
Occurring at any age, it usually does not produce pain and does not seem to have the association with von Recklinghausens disease that is evident with the usual form of malignant schwannoma.3;4;9 The most frequently affected sites are the limbs and inguinal region with the sciatic,1 peroneal, antebrachial cutaneous and digital nerves being involved.3 A nerve of origin is identified in only half the cases. MPNST as a whole are rare in the head and neck. They rarely involve intracranial nerves. In our literature review we found one report of the epithelioid variant displaying malignant behavior.5 Epithelioid schwannomas arising in cranial nerves are usually found within the distal extracranial branches of the trigeminal or facial nerve.1;17;18 Typically, epithelioid schwannomas present as a slow growing mass that produce abrupt neurological symptoms due to rapid enlargement.3 The infiltrating tumors cause a fusiform expansion of the involved nerves and are often bulky at the time of presentation1;3 as were the operative findings in this case. The histology of the epithelioid schwannoma can be variable.11 The most characteristic appearance is that of short cords of large epithelioid cells arranged in a nodular pattern alternating with spindle fascicular areas.12 The cells usually have clear cytoplasm and large round nuclei with prominent melanoma-like nucleoli.4;9–11;13 Immunohistochemistry of the epithelioid schwannomas have been reported to be positive for S-100 protein in 80% but lack a melanoma-associated antigen and cytokeratin.3 This is in contrast with most conventional malignant schwannomas, which express S-100 protein in about 50% of cases.14 Other antigens expressed include Leu715 and EMA.16 These may be helpful when S-100 protein is absent or equivocal. In this case the original tissue was positive for EMA only. The lack of expression of S-100 protein could be a reflection of the greater differentiation of the epitheJournal of Clinical Neuroscience (2004) 11(1)
REFERENCES 1. Bigner DD, McLendon RE, Bruner JM. Russell and RubenstienÕs Pathology of tumors of the Nervous System. sixth edn. Edward Arnold, London 1998. 2. McCormack LJ, Hazard JB, Dickson. Malignant epithelioid neurilemoma (schwannoma). Cancer 1954; 7: 725–728. 3. Laskin W, Weiss S, Bratthauer G. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 1991; 15(12): 1136–1145. 4. McMenamin ME, Fletcher CDM. Expanding the spectrum of malignant change in schwannoma. Am J Surg Pathol 2001; 25(1): 13–25. 5. Kroh H, Matyja E, Marchel D. Epithelioid schwannomas of the acoustic nerve. Folia Neuropathol 2000; 38(1): 23–27. 6. Kleihues P, Burger PC, Scheithauser BW. The new WHO classification of Brain tumors. Brain Pathol 1993; 3: 255–263. 7. Kleihues P, Cavanee WK. Pathology and Genetics of Tumors of the Nervous System. WHO Classification of Tumors. IARC press, Lyon 2000. 8. Kaye AH, Laws Jr ER (eds) Brain Tumors. second edn. Churchill Livingstone, Toronto 2001. 9. DiCarlo EF, Woodruff JM, Bansal M, Erlandson RA. The purely epithelioid malignant peripheral nerve sheath tumor. Am J Surg Pathol 1986; 10: 78–490. 10. Franks AJ. Epithelioid neurilemoma of the trigeminal nerve: an immunohistochemical DNA ultrastructual study. Histopathology 1985; 9: 1339–1350. 11. Weiss SW, Goldblum JR. Enzinger and WeissÕs Soft Tissue Tumors. fourth edn. Mosby, St. Louis 2001. 12. Graham DI, Lantos P. GreenfieldÕs Neuropathology. sixth edn. Arnold and Oxford University Press co-publishers, New York 1996. 13. Taxy JB, Battifora H. Epithelioid schwannoma: diagnosis by electron microscopy. Ultrastr Pathol 1981; 2: 19–24. 14. Weiss SW, Langloss JM, Enzinger FM. Value of S-100 protein in he diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Invest 1983; 49: 299–308. 15. Perentes E, Rubenstein LJ. Immunohistochemical recognition of human nerve sheath tumors by anti-Leu7 (HNK-1) monoclonal antibody. Acta Neuropathol 1985; 68: 319–324. 16. Daimaru Y, Hashimotos H, Enjoji M. Malignant peripheral nerve sheath tumors (malignant schwannoma) an immunohistochemical study of 29 cases. Am J Surg Pathol 1985; 9: 434–444.
ª 2003 Published by Elsevier Ltd.
Surgical treatment on extensive fibrous dysplasia of the frontal bone
17. Maroun FB, Sadler M, Murray GP, Mangan M, Mathieson G, Jacob JC, Kwan A. Primary malignant tumors of the trigeminal nerve. Can J Neurol Sci 1986; 13: 146–148. 18. Mulhlbauer MS, Clark WC, Robertson JH, Gardner LG, Dohan Jr FC. Malignant nerve sheath tumor of the facial nerve: case report and discussion. Neurosurgery 1987; 21: 68–73.
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problems such as visual loss, diplopia and proptosis occur in 20– 35% of patients with FD of this region.5–7 Here we report a case of FD of left frontal bone that was successfully treated by two staged surgeries. CASE PRESENTATION
Surgery improves vision and cosmetic appearance of an adult patient with fibrous dysplasia of the frontal bone Ayataka Fujimoto1 MD, Koji Tsuboi1 MD DMS, Eiichi Ishikawa1 MD, Harumi Nose2 MD DMS, Tadao Nose2 MD DMS 1
Department of Neurological Surgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan, 2Department of Ophthalmology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan
Summary A 31-year-old woman with fibrous dysplasia (FD) of the left forehead was reported. Visual acuity impairment and diplopia were slowly progressive for 6 months associated with marked protrusion of her left forehead. Removal of left forehead lesion including the orbital ridge and total decompression of the optic canal and superior orbital fissure improved these visual symptoms dramatically. Reconstructive cranioplasty using artificial bone made by hydroxyapatite (ApaceramTM ) was very satisfactory in cosmetic appearance. Surgical indication and neuroradiological findings of cranial FD are discussed as well as a review of the literatures. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(1), 95–97 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2002.12.004
Keywords: fibrous dysplasia, optic canal decompression, hydroxyapatite Received 17 October 2002 Accepted 5 December 2002 Correspondence to: Koji Tsuboi, Department of Neurological Surgery, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennohdai, Tsukuba, Ibaraki 305-8575, Japan. Tel.: +81-298533220/3168; Fax: +81-298533214; E-mail: [email protected]
INTRODUCTION Fibrous dysplasia (FD) is a benign pathological condition of the bone in which fibrous tissue gradually expands and replaces normal one or more bones including cranium.1 Histo-pathological appearance of the affected bone is reported to be composed by fibrous tissues that may contain foci of calcified bone, hyaline, cartilage, cysts and sometimes giant cells.2 Although this pathological condition was first reported by Lichtenstein in 1938,3 its aetiology is still unknown. Clinical observations indicate that FD usually begins in childhood and chronically progresses throughout puberty and adolescence, and progression stops after adolescence in most cases.4 Among FD of the entire body, cranio-facial FD is relatively rare, and it is known that ocular ª 2003 Elsevier Ltd. All rights reserved.
A 31-year-old woman presented to us with progressive left visual acuity disturbance and diplopia associated with left forehead prominence which had existed since her childhood. Her visual acuity deteriorated gradually from 20/20 to 20/250 during the recent 6 months and she realized that diplopia on looking to the left side was also progressive. On examination, her corrected visual acuity was 1.2 on the right and 0.08 on the left. Although the position of her eyes was asymmetric, light reflex and accommodation reflex were normal. Appearance of her left optic fundi was normal including papilla. Left frontal bone was protruding and extraordinarily thick on computed tomography (CT) (Fig. 1a and b). Magnetic resonance images (MRI) demonstrated that this lesion was low intensity in both T1- and T2-weighted images with mild enhanced by gadolinium (Fig. 2a and b). Bone scintigram showed marked uptake of the isotope in this lesion. In order to improve the visual symptoms and cosmetic appearance, two staged surgeries were planned. At the initial surgery, removal of her left forehead FD, and decompression of the left superior orbital fissure (SOF) and the optic canal (OC) were performed. After bicoronal skin incision, affected extremely thick bone of the forehead was removed in en-block fashion including upper orbital edge and anterior part of the orbital roof. Then, total decompression of SOF and OC was performed extradurally in this order under microscope. Anterior part of the orbital roof was reconstructed using cranioplasty kit (CEMEXTM ) and scalp was closed. Histopathological examination of the removed lesion demonstrated proliferated fibrous formation in osteoid, which was compatible with fibrous dysplasia. Three days after the surgery, thin sliced CT scan and skull X-ray films were taken in order to make an artificial bone by hydroxyapatite (ApaceramTM ), which was designed to be symmetric to the right frontal bone and was used for cranioplasty two weeks later. Her left visual acuity improved from 20/250 to 20/50 with an improvement of diplopia. Her eye position and forehead shape became symmetrical, and was very satisfactory to the patient (Fig. 3a and b). Post-operative 3D CT demonstrated that the optic canal was opened completely (Fig. 3c). DISCUSSION In the presented case, visual symptoms progressed at the age of 31, and removal of the lesion with decompression of optic canal and superior orbital fissure followed by cranioplasty with ApaceramTM resulted in an excellent outcome. Although progression of FD is considered to be common in childhood and usually stops after adolescence, there are some exceptional cases like ours. In reviewing the literature, Katz et al. reported four cases of cranio-facial FD that occurred after adolescence (over age of 30) and they mention that some cases of cranio-facial FD can occur after adolescence and progress in adulthood.9 Although the chance of improvement of visual symptoms may be low when the optic canal or superior orbital fissure are affected by FD for a certain period, our case indicated that it can be reversible by surgical decompression even 6 months after the initiation of the symptom if the progression is slow and the optic fundus is not affected. Reviewing the literature, FD with visual compromise has two clinical courses. Chronic type manifests gradual visual deterioration caused by mechanical compression by FD itself. In contrast, acute type Journal of Clinical Neuroscience (2004) 11(1)
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Department of Neurosurgery and Surgery, University of Melbourne, Royal Parade, Parkville, Victoria 3050, Australia
REFERENCES 1. Abrams H, Spiro R, Goldstein N. Metastases in carcinoma – analysis of 1000 autopsied cases. Cancer 1950; 3: 74–85. 2. Cotran RS, Kumar V, Collins T. Robbins Pathological Basis of Disease. W.B. Saunders Co., Philadelphia 1999; 991–996. 3. Figlin RA, Piantadosi S, Feld R et al. Intracranial Recurrence of Carcinoma after complete resection of Stage I, II, and III Non-Small-Cell Lung Cancer. N Engl J Med 1988; 318: 1300–1305. 4. Fukui K, Okamura K, Watanabe T et al. Choroid plexus involvement in malignant lymphoma: case report. Neurol Med Chir (Tokyo) 1990; 30: 869–873. 5. Gay P, Litchy W, Cascino T. Brain metastasis in hypernephroma. J Neuro-oncol 1987; 5: 51–56. 6. Healy JF, Rosenkrantz H. Intraventricular metastasis demonstrated by cranial computed tomography. Radiology 1980; 136: 124. 7. Iwatsuki K, Sato M, Taguchi J, Fukui T, Kiyohara H, Yoshimine T, Hayakawa T. Choroid plexus metastasis of renal cell carcinoma causing intraventricular haemorrhage: a case report. No Shinkei Geka 1999; 27(4): 359–363. 8. Jubelirer SJ. Late solitary cerebral metastasis from renal cell carcinoma: a case report and review of the literature. W Va Med J 1996; 92(1): 26–27. 9. Kart BH, Reddy SC, Rao GR et al. Choroid plexus metastasis: CT appearance. J Comput Assist Tomogr 1986; 10: 537–540. 10. Kendall B, Reider-Grosswasser I, Valentine A. Diagnosis of masses presenting within the ventricles on computed tomography. Neuroradiology 1983; 25: 11–22. 11. Khono M, Matsunami M, Sasaki T et al. Solitary metastasis to the choroid plexus of the lateral ventricle: report of three cases and a review of the literature. J Neuro-oncol 1996; 27: 47–52. 12. Kuroki K, Taguchi H, Sumida M, Daimaru Y, Onda J. Cerebral metastasis from a renal cell carcinoma more than 10 years after nephrectomy: report of two cases. No Shinkei Geka 1999; 27(1): 89–93. 13. Matsumura H, Yoshimine T, Yamamoto S, Maruno M, Hayakawa T, Ono Y, Kondoh N, Namiki M. Single solitary metastasis of the slowly progressive type of renal cell carcinoma to the choroid plexus: case report. Neurol Med Chir 1997; 37(12): 916–919. 14. Mertens R, Muhler E, Heimann G. Disseminiertes neuroblastom mit intrazerbraler metastase beim neugeborenen. Klin Padiatr 1987; 199: 424–428. 15. Mizuno M, Asakura K, Nakajima S, Sampei T, Sayama I, Kawamura S, Yasui N, Fukazawa H. Renal cell carcinoma metastasising to choroid plexus of lateral ventricle: a case report. No Shinkei Geka 1992; 20(4): 469–474. 16. Morrison G, Sobel DF, Kelley WM et al. Intraventricular Mass Lesions. Radiology 1984; 153: 435–442. 17. Nakabayashi H, Murata K, Sakaguchi M, Nakajima K, Katsuyama J. Choroid plexus metastasis from gastric cancer: case report. Neurol Med Chir (Tokyo) 1994; 34(3): 183–186. 18. Posner J, Chernik N. Intracranial metastases from systemic cancer. Adv Neurol 1978; 19: 579–592. 19. Qasho R, Tommaso V, Rocchi G, Simi U, Delfini R. Choroid plexus metastasis from carcinoma of the bladder: case report and review of the literature. J Neuro-oncol 1999; 45(3): 237–240. 20. Radley MG, McDonald JV, Pilcher WH, Wilbur DC. Late cerebral metastases from renal cell carcinoma: report of two cases. Surg Neurol 1993; 39(3): 230–234. 21. Raila F, Bottoms W, Fratkin J. Solitary Choroid Plexus Metastasis From a Renal Cell Carcinoma. Southern Med J 1998; 91(12): 1159–1162. 22. Roser F, Rosahl SK, Samii M. Single cerebral metastasis 3 and 19 years after primary renal cell carcinoma: case report and review of the literature. J Neurol Neurosurg Ps 2002; 72(2): 257–258. 23. Saitoh H, Shimbo T, Tasaka T, Iida T, Hara K. Brain metastases of renal adenocarcinoma. Tokai J Exp Med 1982; 7(3): 337–343. 24. Shigemori M, Shimamoto H, Noguchi S et al. Choroid plexus metastasis of renal cell carcinoma. CT Kenkyu 1987; 9: 603–606. 25. Suetake K, Shinya T, Takeda M. A choroid plexus metastasis of a renal cell carcinoma: a case report. Jpn J Neurosurg (Tokyo) 1994; 3: 436–441. 26. Tanimoto M, Tatsumi S, Tominaga S, Kamikawa S, Nagao T, Tamaki N, Matsumoto S. Choroid plexus metastasis of lung carcinoma: a case report. Neurol Med Chir (Tokyo) 1991; 31: 152–155.
Summary We report a case of an intracranial epithelioid schwannoma. Epithelioid schwannoma is a low-grade malignant peripheral nerve sheath tumor that occurs most commonly in the limbs and inguinal region. Association with cranial nerves has been reported to be rare and in almost all cases has only involved extracranial distal branches. ª 2003 Published by Elsevier Ltd. Journal of Clinical Neuroscience (2004) 11(1), 91–95 0967-5868/$ - see front matter ª 2003 Published by Elsevier Ltd. doi:10.1016/S0967-5868(03)00114-0
Keywords: schwannoma, brain tumor, nerve sheath tumor Received 22 January 2003 Accepted 24 January 2003 Correspondence to: Dr. Tanya Yuen, Department of Neurosurgery, Royal Melbourne Hospital, Parkville, 3050, Victoria, Australia. Tel.: +61-3-0342-8218; Fax: +61-3-9342-7273; E-mail: [email protected]
INTRODUCTION Malignant peripheral nerve sheath tumors (MPNST) can exhibit great complexity with intermingling of several tissue components.1 Epithelioid schwannoma, a term first proposed by
Intracranial epithelioid schwannoma – a case report Tanya Yuen,
MBBS
Andrew H. Kaye,
ª 2003 Published by Elsevier Ltd.
MBBS FRACS FRCS
Fig. 1 CT scan showing: (A) Hyperdense enhancing tumor mass centered on the sella turcica, (B) floor of sella eroded centrally, tumor extension into sphenoid sinus, erosion of dorsum sella and adjacent clivus.
Journal of Clinical Neuroscience (2004) 11(1)
92
Yuen and Kaye
McCormack et al. in 1954,2 is an uncommon variant of MPNST with predominance of epithelioid cells and low- grade malignant behavior,3 found mostly in the limbs. Those involving cranial nerves are extremely rare and even then they are almost always situated extracranially.1;10 We present a case of an epithelioid schwannoma involving the intracranial portion of a cranial nerve. CASE REPORT A 72-year-old female presented with 6 weeks history of sudden onset diplopia and diffuse headache. On examination she had a left sixth nerve palsy. CT and MRI showed an enhancing lesion in the left cavernous sinus extending into the middle cranial fossa. Digital subtraction angiography showed tumor blush suggestive of meningioma. The patient underwent excision via a left pterional craniotomy. Tumor encased the left carotid artery, the optic, third, fourth, fifth and the sixth cranial nerves, preventing complete excision. Histology revealed an epithelioid schwannoma. At two years of follow up MRI showed residual tumor extending into the left sphenoid sinus and floor of the sella, encasing the left cavernous carotid artery. She was lost to follow-up for 8 years then presented again with weight loss and vomiting. On examination visual acuity in the right eye was 6/60 and reduced to hand movement only in the left eye. There were left 3rd, 4th and 6th cranial nerve palsies. CT and MRI revealed a 2.5 1.5 cm hyperdense, uniformly enhancing tumor centered on the sella turcica, extending into the cavernous and sphenoid sinuses and
Fig. 2
eroding the floor of the sella, the dorsum sella and clivus (Figs. 1 and 2). A transphenoidal approach was used to explore the tumor, which had extended through the sphenoid sinus and the pituitary fossa into the suprasellar cistern. Both carotid arteries were encased once again preventing complete resection. Abdominal fat was used to pack the emptied sinus and pituitary fossa. Six months later MRI showed extensive recurrent tumor, completely destroying the sella and adjacent clivus with cavernous carotid encasement, bilateral cavernous sinus infiltration and extension down into the nasopharynx on the right (Fig. 3). The tumor was deemed inoperable and the patient died 6 months later. In both surgical specimens, histology confirmed epithelioid schwannoma with polymorphic features, strong surface immunoreactivity for epithelial membrane antigen but no reactivity for S100 protein, cytokeratin, kappa and lambda light chain, vimentin, chromogranin and leukocyte common antigen (Figs. 4 and 5). DISCUSSION We present a case of an intracranial epithelioid schwannoma arising from a cranial nerve. According to the current WHO classification of CNS tumors6;7 the epithelioid schwannoma is a low-grade malignant peripheral nerve sheath tumor with a predominance of epithelioid cells and which histologically resembles carcinoma or amelanotic melanoma.8 Its incidence among MPNST has been estimated between 5 and 17%, a range that reflects differences in the stringency of diagnostic criteria and clinical evaluation to exclude a carcinoma or melanoma.3;4;9
MRI scan of recurrence showing: (A) Erosion of sella turcica, (B) total encasement of bilateral carotid arteries, (C) erosion of dorsum sella and clivus.
Journal of Clinical Neuroscience (2004) 11(1)
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Intracranial epithelioid schwannoma
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Fig. 3 MRI scan of second recurrence showing: (A) Complete destruction of sella and adjacent clivus, (B) suprasellar extension elevating optic chiasm and invagination into inferior left frontal lobe anteriorly, (C) cavernous carotid encasement, bilateral cavernous sinus infiltration and extension down into the nasopharynx, (D) tumor bulge laterally into adjacent medial temporal lobes, (E) extension inferiorly to fill sphenoid sinus, infiltration of prevertebral musculature at skull base, nasopharynx and bulge into prepontine cistern.
Fig. 4 Histology slides showing: (A) Densely hypercellular tumor in which tumor cells with hyperchromatic nuclei and well-demarcated cytoplasmic borders are arranged in diffuse sheets (H&E X 400), (B) tumor cells with strong immunostaining for epithelial membrane antigen (EMA) (Strepavidin PAP X 400), (C) tumor cells with strong immunostaining for S-100 protein (Streptavidin PAP X 200).
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Yuen and Kaye
lioid cell.10 However, these antigens are not specific and are shared by other neural and non-neural tissues so that care in interpretation of the antigenic profile of atypical tumors is required.1 While earlier reports based primarily upon deeply situated limb and trunk epithelioid schwannomas indicated a high mortality rate,9 more indolent behavior was noted in the series of Laskin et al.3 In the latter, out of 16 superficial tumors, 2 recurred locally and 2 metastasised, with no disease related deaths. Of the 10 deeply seated tumors 1 recurred locally and 3 died of metastatic disease. All of the tumors in these 2 series were extracranial and the prognosis for intracranial epithelioid schwannoma is unknown. Optimal management for the epithelioid schwannoma appears to be complete resection but this was not technically possible in our case. Low-grade malignant behavior that is characteristic of the tumor was evident in this case with the patient surviving 12 years from the time of diagnosis. In the time she was lost to follow-up, disease extension was such that further surgery could only palliate. CONCLUSION Epithelioid schwannoma, a low-grade malignant peripheral nerve sheath tumor has been reported in many regions of the body. This is a rare case of the tumor occurring intracranially and involving cranial nerves. The patient survived 12 years from the time of diagnosis before succumbing with extensive intracranial tumor invasion.
Fig. 5 Electron micrographs showing: (A) Basal lamina surrounding tumor cells (X30K), (B) (X 210K).
Occurring at any age, it usually does not produce pain and does not seem to have the association with von Recklinghausens disease that is evident with the usual form of malignant schwannoma.3;4;9 The most frequently affected sites are the limbs and inguinal region with the sciatic,1 peroneal, antebrachial cutaneous and digital nerves being involved.3 A nerve of origin is identified in only half the cases. MPNST as a whole are rare in the head and neck. They rarely involve intracranial nerves. In our literature review we found one report of the epithelioid variant displaying malignant behavior.5 Epithelioid schwannomas arising in cranial nerves are usually found within the distal extracranial branches of the trigeminal or facial nerve.1;17;18 Typically, epithelioid schwannomas present as a slow growing mass that produce abrupt neurological symptoms due to rapid enlargement.3 The infiltrating tumors cause a fusiform expansion of the involved nerves and are often bulky at the time of presentation1;3 as were the operative findings in this case. The histology of the epithelioid schwannoma can be variable.11 The most characteristic appearance is that of short cords of large epithelioid cells arranged in a nodular pattern alternating with spindle fascicular areas.12 The cells usually have clear cytoplasm and large round nuclei with prominent melanoma-like nucleoli.4;9–11;13 Immunohistochemistry of the epithelioid schwannomas have been reported to be positive for S-100 protein in 80% but lack a melanoma-associated antigen and cytokeratin.3 This is in contrast with most conventional malignant schwannomas, which express S-100 protein in about 50% of cases.14 Other antigens expressed include Leu715 and EMA.16 These may be helpful when S-100 protein is absent or equivocal. In this case the original tissue was positive for EMA only. The lack of expression of S-100 protein could be a reflection of the greater differentiation of the epitheJournal of Clinical Neuroscience (2004) 11(1)
REFERENCES 1. Bigner DD, McLendon RE, Bruner JM. Russell and RubenstienÕs Pathology of tumors of the Nervous System. sixth edn. Edward Arnold, London 1998. 2. McCormack LJ, Hazard JB, Dickson. Malignant epithelioid neurilemoma (schwannoma). Cancer 1954; 7: 725–728. 3. Laskin W, Weiss S, Bratthauer G. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 1991; 15(12): 1136–1145. 4. McMenamin ME, Fletcher CDM. Expanding the spectrum of malignant change in schwannoma. Am J Surg Pathol 2001; 25(1): 13–25. 5. Kroh H, Matyja E, Marchel D. Epithelioid schwannomas of the acoustic nerve. Folia Neuropathol 2000; 38(1): 23–27. 6. Kleihues P, Burger PC, Scheithauser BW. The new WHO classification of Brain tumors. Brain Pathol 1993; 3: 255–263. 7. Kleihues P, Cavanee WK. Pathology and Genetics of Tumors of the Nervous System. WHO Classification of Tumors. IARC press, Lyon 2000. 8. Kaye AH, Laws Jr ER (eds) Brain Tumors. second edn. Churchill Livingstone, Toronto 2001. 9. DiCarlo EF, Woodruff JM, Bansal M, Erlandson RA. The purely epithelioid malignant peripheral nerve sheath tumor. Am J Surg Pathol 1986; 10: 78–490. 10. Franks AJ. Epithelioid neurilemoma of the trigeminal nerve: an immunohistochemical DNA ultrastructual study. Histopathology 1985; 9: 1339–1350. 11. Weiss SW, Goldblum JR. Enzinger and WeissÕs Soft Tissue Tumors. fourth edn. Mosby, St. Louis 2001. 12. Graham DI, Lantos P. GreenfieldÕs Neuropathology. sixth edn. Arnold and Oxford University Press co-publishers, New York 1996. 13. Taxy JB, Battifora H. Epithelioid schwannoma: diagnosis by electron microscopy. Ultrastr Pathol 1981; 2: 19–24. 14. Weiss SW, Langloss JM, Enzinger FM. Value of S-100 protein in he diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Invest 1983; 49: 299–308. 15. Perentes E, Rubenstein LJ. Immunohistochemical recognition of human nerve sheath tumors by anti-Leu7 (HNK-1) monoclonal antibody. Acta Neuropathol 1985; 68: 319–324. 16. Daimaru Y, Hashimotos H, Enjoji M. Malignant peripheral nerve sheath tumors (malignant schwannoma) an immunohistochemical study of 29 cases. Am J Surg Pathol 1985; 9: 434–444.
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Surgical treatment on extensive fibrous dysplasia of the frontal bone
17. Maroun FB, Sadler M, Murray GP, Mangan M, Mathieson G, Jacob JC, Kwan A. Primary malignant tumors of the trigeminal nerve. Can J Neurol Sci 1986; 13: 146–148. 18. Mulhlbauer MS, Clark WC, Robertson JH, Gardner LG, Dohan Jr FC. Malignant nerve sheath tumor of the facial nerve: case report and discussion. Neurosurgery 1987; 21: 68–73.
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problems such as visual loss, diplopia and proptosis occur in 20– 35% of patients with FD of this region.5–7 Here we report a case of FD of left frontal bone that was successfully treated by two staged surgeries. CASE PRESENTATION
Surgery improves vision and cosmetic appearance of an adult patient with fibrous dysplasia of the frontal bone Ayataka Fujimoto1 MD, Koji Tsuboi1 MD DMS, Eiichi Ishikawa1 MD, Harumi Nose2 MD DMS, Tadao Nose2 MD DMS 1
Department of Neurological Surgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan, 2Department of Ophthalmology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan
Summary A 31-year-old woman with fibrous dysplasia (FD) of the left forehead was reported. Visual acuity impairment and diplopia were slowly progressive for 6 months associated with marked protrusion of her left forehead. Removal of left forehead lesion including the orbital ridge and total decompression of the optic canal and superior orbital fissure improved these visual symptoms dramatically. Reconstructive cranioplasty using artificial bone made by hydroxyapatite (ApaceramTM ) was very satisfactory in cosmetic appearance. Surgical indication and neuroradiological findings of cranial FD are discussed as well as a review of the literatures. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(1), 95–97 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2002.12.004
Keywords: fibrous dysplasia, optic canal decompression, hydroxyapatite Received 17 October 2002 Accepted 5 December 2002 Correspondence to: Koji Tsuboi, Department of Neurological Surgery, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennohdai, Tsukuba, Ibaraki 305-8575, Japan. Tel.: +81-298533220/3168; Fax: +81-298533214; E-mail: [email protected]
INTRODUCTION Fibrous dysplasia (FD) is a benign pathological condition of the bone in which fibrous tissue gradually expands and replaces normal one or more bones including cranium.1 Histo-pathological appearance of the affected bone is reported to be composed by fibrous tissues that may contain foci of calcified bone, hyaline, cartilage, cysts and sometimes giant cells.2 Although this pathological condition was first reported by Lichtenstein in 1938,3 its aetiology is still unknown. Clinical observations indicate that FD usually begins in childhood and chronically progresses throughout puberty and adolescence, and progression stops after adolescence in most cases.4 Among FD of the entire body, cranio-facial FD is relatively rare, and it is known that ocular ª 2003 Elsevier Ltd. All rights reserved.
A 31-year-old woman presented to us with progressive left visual acuity disturbance and diplopia associated with left forehead prominence which had existed since her childhood. Her visual acuity deteriorated gradually from 20/20 to 20/250 during the recent 6 months and she realized that diplopia on looking to the left side was also progressive. On examination, her corrected visual acuity was 1.2 on the right and 0.08 on the left. Although the position of her eyes was asymmetric, light reflex and accommodation reflex were normal. Appearance of her left optic fundi was normal including papilla. Left frontal bone was protruding and extraordinarily thick on computed tomography (CT) (Fig. 1a and b). Magnetic resonance images (MRI) demonstrated that this lesion was low intensity in both T1- and T2-weighted images with mild enhanced by gadolinium (Fig. 2a and b). Bone scintigram showed marked uptake of the isotope in this lesion. In order to improve the visual symptoms and cosmetic appearance, two staged surgeries were planned. At the initial surgery, removal of her left forehead FD, and decompression of the left superior orbital fissure (SOF) and the optic canal (OC) were performed. After bicoronal skin incision, affected extremely thick bone of the forehead was removed in en-block fashion including upper orbital edge and anterior part of the orbital roof. Then, total decompression of SOF and OC was performed extradurally in this order under microscope. Anterior part of the orbital roof was reconstructed using cranioplasty kit (CEMEXTM ) and scalp was closed. Histopathological examination of the removed lesion demonstrated proliferated fibrous formation in osteoid, which was compatible with fibrous dysplasia. Three days after the surgery, thin sliced CT scan and skull X-ray films were taken in order to make an artificial bone by hydroxyapatite (ApaceramTM ), which was designed to be symmetric to the right frontal bone and was used for cranioplasty two weeks later. Her left visual acuity improved from 20/250 to 20/50 with an improvement of diplopia. Her eye position and forehead shape became symmetrical, and was very satisfactory to the patient (Fig. 3a and b). Post-operative 3D CT demonstrated that the optic canal was opened completely (Fig. 3c). DISCUSSION In the presented case, visual symptoms progressed at the age of 31, and removal of the lesion with decompression of optic canal and superior orbital fissure followed by cranioplasty with ApaceramTM resulted in an excellent outcome. Although progression of FD is considered to be common in childhood and usually stops after adolescence, there are some exceptional cases like ours. In reviewing the literature, Katz et al. reported four cases of cranio-facial FD that occurred after adolescence (over age of 30) and they mention that some cases of cranio-facial FD can occur after adolescence and progress in adulthood.9 Although the chance of improvement of visual symptoms may be low when the optic canal or superior orbital fissure are affected by FD for a certain period, our case indicated that it can be reversible by surgical decompression even 6 months after the initiation of the symptom if the progression is slow and the optic fundus is not affected. Reviewing the literature, FD with visual compromise has two clinical courses. Chronic type manifests gradual visual deterioration caused by mechanical compression by FD itself. In contrast, acute type Journal of Clinical Neuroscience (2004) 11(1)