266 C ¸ akir et al.
Seventeen cases have been reported in the English language literature, 7 of the cases have been subtotally and the remaining 10 cases have been totally resected1–3;6–11 (Table 1). Our patient #1 showed multiple meningiomas with different histopathological types and one of these meningiomas was a CCM. A total resection of the tumour was achieved. No CCM was reported in the literature in a patient with multiple meningiomas. This makes our case more interesting. The patient showed no recurrence until that time (7 months). It is obvious that a longer follow up is necessary for this case. Because CCMs have a recurrence time of 4–23 months. The tumour in case #2 was resected subtotally and the patient died 45 days later. When compared with other meningioma operations, neither macroscopic nor surgical differences could be detected in both of the operations. The goal of the treatment is to achieve radical surgical removal as much as possible. Radiotherapy and radio-surgery might be important for patients who are not good candidates for reoperation. CONCLUSION Although the differential diagnosis of CCM with the other clear cell tumours of the CNS is possible, the diagnosis of CCM might be kept in mind in meningioma cases and close follow-up is required due to the high risk of recurrence. REFERENCES
Fig. 2 (A) Contrast enhanced axial MRI showing a homogeneously contrast enhanced left temporal mass. (B) Photomicrograph showing meningeal cells with a clear cytoplasm (H & E 200).
CCMs are usually seen in younger patients and have a predilection for canalis spinalis.2;3;6 CCM are potentially aggressive and may recur, spread locally and even metastasis.2;4;6;12 All of these features make them clinically aggressive.13 Cytopathologically CCM have a whorled syncytial architecture and spindled to polygonal, bland- appearing nuclei.3;7;13;14 The immunohistochemical features included epithelial membrane antigen and vimentin positive cytoplasm.2;11;15 Ultrastructurally, tumour cells showed conspicuous inter digitations of their plasma membranes with frequent junctional complexes and contained numerous glygocen granules in the cytoplasm and its process.4;7;8;13–15 The differential diagnosis of CCMs include intracranial renal cell carcinoma metastases, hemangioblastoma, pleomorphic xanthoastrocytoma, lipid-rich glioblastoma, and clear cell ependymoma.2;6;14 The tumour is isointense with the parenchyma on T1 and T2 weighted MRI. The MRI features of CCM are not much different from the other meningiomas. On T1 and T2 weighted MRI the tumour is isointense with the parenchyma and homogeneously enhances after gadolinium injection.13 Journal of Clinical Neuroscience (2003) 10(2)
1. Heth JA. Intraspinal familial clear cell meningioma in a mother and child. Case report. J Neurosurg Spine 2000; 93: 317–321. 2. Lee W. MR imaging features of clear cell meningioma with diffuse leptomeningeal seeding. AJNR Am J Neuroradiol 2000; 21: 130–132. 3. Pimentel J. Clear cell meningioma variant and clinical aggressiveness. Clin Neuropathol 1998; 17: 141–146. 4. Dubais A. Clear cell carcinoma of the caudoequina. Neuroradiology 1998; 40(11): 743–747. 5. Matsui H. Multifocal clear cell meningioma in the spine: a case report. Neurosurg Rev 1998; 21: 171–173. 6. Shil DF. Clear cell meningioma: a case report. Chung Hua I Hsueh Tsa Chih 1996; 57: 452–456. 7. Imlay SP. Clear Cell meningioma: diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 1998; 18: 131–136. 8. Kakita A. Clear cell variants of intracranial tumors: meningioma and epandymoma. Brain Tumor Pathol 1995; 12: 111–116. 9. Shiraishi K. Glycogen-rich meningioma. Case report and short review. Neurosurg Rev 1991; 14: 61–64. 10. Teo JG. Intraparenchymal clear cell meningioma of the brain stem in a 2-year-old child. Case report and literature review. Pediatr Neurosurg 1998; 28: 27–30, abst. 11. Zorludemir S. Clear cell meningioma. A clinicopathologic study of potentially agressive variant of meningioma. Am J Surg Pathol 1995; 19: 493–505. 12. Prinz M. Clear cell meningioma:report of a spinal case. Gen diagn Pathol 1996; 141: 261–267. 13. Ho T. A case of clear cell meningioma orginating from the cerebellar tentorium. No shinkei Geka 1998; 26: 265–270, abs. 14. G€okden M. Clear cell neoplasms and pseudoneoplastic lesions of the central nervous system. Semin Diagn Pathol 1997; 14: 253–269. 15. Kubota T. Clear cell (glycogen-rich) meningioma with special reference to spherical collagen deposits. Noshuyo Byori 1995; 12(1): 53–60.
Solitary plasmacytoma with intracranial intraorbital and, paranasal sinus extension € kalp Karaarslan, Haydar Usul, Ertugrul C ¸ akir, Go € Suleyman Baykal, Erhan Arslan ª 2003 Elsevier Science Ltd. All rights reserved.
Solitary plasmacytoma
Department of Neurosurgery, School of Medicine, Karadeniz Technical University, 61080 Trabzon, Turkey
Summary Intracranial solitary plasmacytomas (ICSPs) are extremely rare tumours in neurosurgical practice, and are often misdiagnosed preoperatively. Here we present a solitary intracranial plasmacytoma with orbital, nasal and paranasal sinus extension. A subtotal excision of the tumour was performed and the complete response was seen after postoperative radio-chemotherapy. The neuroradiological and neurosurgical features of the case are discussed with the pertinent literature. c 2003 Elsevier Science Ltd. All rights reserved.
Journal of Clinical Neuroscience (2003) 10(2), 266–268 0967-5868/03/$ - see front matter ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(02)00283-7
Keywords: intracranial plasmacytoma, central nervous system, orbita, paranasal sinuses Received 15 February 2002 Accepted 7 June 2002 Correspondence to: Ertugrul C ¸ akir MD, Department of Neurosurgery, Karadeniz Technical University, School of Medicine, 61080 Trabzon, Turkey. Tel.: +90-462-377-5410; E-mail:
[email protected]
267
INTRODUCTION Plasma cell tumours may manifest as one of three pathological entities: multiple myeloma; solitary bone plasmacytoma; or extramedullary plasmacytomas.1;2 Extramedullary plasmacytomas represent 3% of plasma cell neoplasms.1;2 CASE REPORT A 49 year old, male patient presented with left orbital pain and frontal swelling with a history of head trauma 8 months before being admitted to our outpatient clinic. Physical and neurological examination revealed left proptosis temporal deviation of the left globe, a 4 4 3 cm immobile frontal mass and a retroauricular mobile, 2 2 1 cm enlarged lymphnode. Neurological examination was unremarkable. Computed tomography revealed a large craniofacial mass with intracranial, intraorbital, frontal and maxillary sinus extension. There was prominent peritumoral oedema and the tumour was compressing the lateral ventricles and causing a midline shift. Destruction of the frontal bone was also observed (Figs. 1A and B). The tumour was hyperintense on T1 and T2 weighted magnetic resonance (MR) images and showed prominent contrast enhancement after intravenous contrast injection (Figs. 2A and B).
Fig. 1 (A) Nonenhanced cranial CT shows an extracranial frontal mass with intracranial extension and peritumoral oedema. Note the tumour compressing the left lateral ventricle and causing dilatation of the right lateral ventricle. (B) Nonenhanced CT scan shows tumoural erosion of the anterior and posterior walls of the frontal sinus.
Fig. 2 (A) Contrast enhanced sagittal MR image shows a huge intra–extra cranial mass which is invading the paranasal sinuses and orbita. There is prominent mass effect intracranially. (B) Contrast enhanced coronal MR image shows paranasal sinus, intraorbital and intracranial extension of the tumour.
ª 2003 Elsevier Science Ltd. All rights reserved.
Journal of Clinical Neuroscience (2003) 10(2)
268 Ebara et al.
A frontal craniotomy was performed. A tumour which eroded the bone and dura was encountered. The results of the frozen section were consistent with a plasma cell tumour. Decompression with subtotal excision was achieved. The histopathological diagnosis was plasmacytoma. The postoperative period was uneventful. No systemic focus for the tumour could be detected. The patient received 300 cGy/day, for a total of 30 days fractionated radiotherapy and also chemotherapy [VAD 3 (Vincristine(Oncovine) 0.4 mg/day, Adriamycin 9 mg/m2 /day, Dexamethasone 40 mg/day), CHOP 4 (Cyclophosphamide 750 mg/m2 , Oncovine 1.4 mg/m2 , Adriamycin 50 mg/m2 , methyl prednisolone 40 mg/m2 )]. There was no evidence of tumour recurrence 2 years after radio-chemotherapy. DISCUSSION ICSP is a solitary myeloma plasma cell-tumour that affects the skull, meninges and brain. There are 3 variations of plasma cell tumours; multiple myeloma, solitary bone plasmacytoma, solitary extramedullary plasmacytoma.1;3 Extramedullary plasmacytoma has a separate clinical pathological entity represents 3% of plasma cell neoplasms.1–12 Plasmacytomas are seen on CT as iso-hyperdense lesions which show homogeneous contrast enhancement. They are also iso-hyperintense on T1 weighted images and are homogeneously enhancing after administration of IV gadolinium.2;8;10;13 On cerebral angiograms, they are characterised as avascular and spaceoccupying lesions.2;8;10;13 Preoperative diagnosis most often favors meningioma.2;8 However, the skull will often have lytic lesions, which is rare in meningioma.2;8 In 1989, Widenheim et al.10 reviewing the spectrum of plasmacytic central nervous system lesions, introduced the terms plasma cell granuloma and atypical monoclonal plasma-cell hyperplasia as precursors of solitary plasmacytoma. Plasma cell granulomas are inflammatory pseudotumours with a proliferation of inflammatory cells including a mixed population of plasma cells, lymphocytes and histiocytes admixed with granulation tissue and fibrosis.10 They are polyclonal in their expression of immunoglobulin light chains. Atypical monoclonal plasma cell hyperplasia contains monoclonal plasma cells and is an ill-defined entity that probably represents a type of solitary plasmacytoma.2;10 Meningiomas with a conspicuous plasma cell and lymphocyte component also must be considered in the differential diagnosis.2;10 Amyloid association with intracranial plasmacytomas has frequently been noted.2;10 Systemic evaluation includes bone marrow evaluation, skeletal survey, and bone scan to exclude multiple myeloma. Serum protein electrophoresis demonstrates a monoclonal gammopathy (M component) in 99% of cases of multiple myeloma and occasionally in cases of plasmacytoma; however, a generalised gammopathy is seen in plasmacytic granuloma. Quantitative immunoglobulin analysis and urine protein electrophoresis are performed for cases of urinary excretion of immunoglobulin elements when the M component is not seen on serum protein electrophoresis. A drop in the level of immunoglobulin in serum or cerebrospinal fluid is expected with cure of treated solitary plasmacytoma and can be followed as a tumour marker for recurrence.1;2;4–7;10–12;14;15 Solitary intracranial plasmacytoma has a favorable prognosis. Durable, sustained remission may occur after subtotal or radical resection combined with radiation therapy.2;3;10;11;16 Corticosteroid medications may have therapeutic effects.10 Plasma cell granuloma may be successfully treated by surgery without adjunctive Journal of Clinical Neuroscience (2003) 10(2)
therapy and may not progress to multiple myeloma during the follow-up period. Atypical monoclonal plasma cell hyperplasia may also respond very effectively to radiation therapy alone.2;10 The treatment of solitary plasmacytomas is surgery with adjunctive radiochemotherapy. The goal is to achieve total surgical resection but in neurologically eloquent areas some residue might be left to decrease postoperative morbidity and mortality; because these tumours are very sensitive to radiochemotherapy as seen in our case. REFERENCES 1. Benli K, Inci S. Solitary dural plasmacytoma. Neurosurgery 1995; 36: 1206– 1209. 2. Bindal AK, Bindal RK, Van Loveren H, Sawaya R. Management of intracranial plasmacytoma. J Neurosurg 1995; 83(2): 218–221. 3. Kautzky M, Susani M, Steurer M, Youssefzadeh S. Plasmacytoma of the nose and paranasal sinuses with intracranial and orbital extension. Lryngorhinootologie 1993; 72(7): 352–355. 4. Khouja N, Aouidj L, Bahri K, Jemel H, Haonet S, Khaldi M. Solitary plasmacytoma of the cranial vault. Case report and review of the literature. Neurochirurgie 2000; 46(1): 43–46. 5. Kohli CM, Kawazu T. Solitary intracranial plasmacytoma. Surg Neurol 1982; 17: 307–312. 6. Kuzeyli K, Duru S, Ceylan S, Akt€urk F. Solitary plasmacytoma of the skull. A case report. Neurosurg Rev 1995; 18: 139–142. 7. Mancardi Gl, Mandyburr TI. Solitary intracranial plasmacytoma. Cancer 1967; 51: 212–216. 8. Mantyla R, Kinnunen J, Bohling T. Intracranial Plasmacytoma: a case report. Neuroradiology 1996; 38(7): 646–649. 9. Rivas L, Guzman JR, Mora-La Cruz E, Cardozo J. Extramedullary intracranial solitary plasmacytoma. Report of 2 cases. Invest Clin 1994; 35(3): 155–167. 10. Vaicys C, Schulder M, Wolansky LJ, Fromowitz FB. Falcotentorial plasmacytoma. Case report. J Neurosurg 1999; 9(1): 132–135. 11. Vujovic O, Fisher BJ, Munoz DG. Solitary intracranial plasmacytoma: case report and review of management. J Neurooncol 1998; 39(1): 47–50. 12. Wisniewski T, Sisti M, Inhirami G. Intracerebral solitary plasmacytoma. Neurosurgery 1990; 27: 826–829. 13. Matsumura S, Kishino M, shida T, Furukawa S. Radiographic findings for solitary plasmacytoma of the bone in the anterior wall of the maxillary sinus: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000; 89(5): 651–657. 14. Knowling MA, Harwood AR, Bergsadel DE. Comparison of extramedullary plasmacytomas with solitary and multiple plasma cell tumors of bone. J Clin Oncol 1983; 1: 255–262. 15. Wax MK, Yun KJ, Omar RA. Extramedullary plasmacytomas of the head and neck. Otolaryngol Head and Neck Surg 1993; 109(5): 877–885. 16. Soo G, Chan A, Lam D, Abdullah V, van Hasselt CA. Extramedullary nasal plasmacytoma–an unusual clinical entity. Ear Nose Throat J 1996; 75(3): 171–173.
A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele S. Ebara MD, Y. Yuzawa MD, T. Kinoshita MD, J. Takahashi MD, I. Nakamura MD, H. Hirabayashi J. Kitahara MD, M. Yamada MD, K. Takaoka MD
MD,
Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto-City, Nagano 390-8621, Japan
Summary The patient presented with neurofibromatosis and a dystrophic kyphoscoliosis around the cervico–thoracic junction.
ª 2003 Elsevier Science Ltd. All rights reserved.
