Acta Neurochir (Wien) (2007) 149: 529–532 DOI 10.1007/s00701-007-1138-9 Printed in The Netherlands
Case Report Solitary thoracic intradural extramedullary plasmacytoma I. Zazpe1 , C. Caballero2 , T. Cabada3 , D. Guerrero4 , A. Gallo-Ruiz1 , and E. Portillo1 1
Department of Neurosurgery, Hospital of Navarre, Pamplona, Spain Department of Histology and Pathology, Hospital of Navarre, Pamplona, Spain 3 Department of Radiology, Hospital of Navarre, Pamplona, Spain 4 Molecular Biology Unit, Hospital of Navarre, Pamplona, Spain 2
Received October 10, 2006; accepted February 21, 2007; published online April 4, 2007 # Springer-Verlag 2007
Summary The bodies of the vertebrae are common locations for plasma cell diseases such as multiple myeloma and solitary plasmacytoma. Secondary invasion of the epidural space is infrequent but can cause neurological symptoms. Spinal cord compression due to pure intradural plasma cell infiltration is very rare. The authors report a 25-year-old woman who developed a progressive difficulty in walking due to a solitary spinal dural plasmacytoma. This is the first reported example in the English language literature of a purely intradural spinal plasmacytoma in a patient without other myelomatous lesions. An entirely intradural solitary plasmacytoma has a relatively better prognosis. Keywords: Plasmacytoma; dura matter; spine; multiple myeloma. Abbreviations CT Computerized tomography; ESR erythrocyte sedimentation rate; Ig immunoglobulin; MM multiple myeloma; MR magnetic resonance.
clonal immunoglobulin synthesis. Solitary plasmacytoma is characterised by atypical plasma cell proliferation in a single site, without evidence of significant bone marrow plasma-cell infiltration [3]. Solitary plasmacytoma is rare, most arise from bone marrow and are known as solitary osseus plasmacytomas and account for 5% of patients with plasma cell disorders. Extramedullary plasmacytomas account for approximately 3% of all plasma cell neoplasms and arise in extra osseous sites, usually involving the submucosa of various organs in the head and neck. Solitary cranial or spinal extramedullary plasmacytomas are very rare and usually originate in the intracranial dura matter and have been designated as solitary dural plasmacytomas. Less than 30 examples of solitary dural plasmacytoma have been described in the English-language literature, most of them in the cranial compartment [1, 4]. Only one patient with a solitary spinal lesion [8] has been described but the patient also had multiple bony lesions. We report a patient who had a pure spinal solitary dural plasmacytoma without evidence of any other myelomatous lesion.
Introduction
Clinical report
Plasma cell tumours are characterised by monoclonal proliferation of Ig-secreting plasma cells. Multiple myeloma is the most frequent (incidence: 4–8 cases per 100,000 persons per year) and malignant type. It is a disseminated neoplasm characterised by the involvement of multiple bones, bone marrow infiltration and mono-
A 25-year-old woman had suffered from thoracic back pain and progressive difficulty walking for about 3 weeks before she was referred to our hospital. Neurological examination showed that both legs were moderately weak, this was more marked on the right side, with impaired proprioception. Her arms were normal. There was no
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Fig. 1. (a) Gadolinium-enhanced sagittal Spin Echo (SE) sequence T1-weighted MR image demonstrating an intense and slightly heterogeneous enhancement after contrast injection. (b) Gadolinium-enhanced axial Spin Echo (SE) sequence T1-weighted MR image showing the right intradural extramedullary localization of the mass with minimal extension towards the right foramen and with shift to the left of the spinal cord
sphincter dysfunction or radiculopathy. Blood tests were normal, except for a slightly elevated ESR (23 mm=h) and proteins (8.1 g=dl) that could be explained by an existing chronic inflammatory process. Cranial CT and MR imaging were normal. Spinal MR imaging showed a 2 cm intradural extramedullary lesion in the thoracic region (Fig. 1a). The lesion was located posterolateraly on the right at D2-D3 level (Fig. 1a) and pushed spinal cord to the left (Fig. 1b). It showed an intermediate-low homogeneous signal in both T1 and T2 sequences and homogeneous contrast enhancement (Fig. 1a, b). No dural-tail or bone alterations were seen. Somato-sensory evoked potentials showed dysfunction of the posterior columns. Cranial magnetic stimulation showed abnormality of function in the corticospinal tract to the right leg. Posterior bilateral D2-D3 laminectomy was performed. The adjacent bone and the external aspect of the dura matter appeared intact. The dura matter was thickened and after it was incised in the midline, a firm and reddish tumour was exposed, compressing the spinal cord from the right. The tumour appeared to arise from the inner aspect of the dura matter on the right side and was also attached to the D3 right nerve root (Fig. 2). It looked like a typical meningioma or schwannoma. The inner surface of the dura matter was separated from the tumour and coagulated, the tumour was dissected from the nerve root and resected completely. Microscopic examination showed a cellular tumour composed of well-differentiated plasma cells with a nodular-like pattern of growth (Fig. 3). Some tumour cells
Fig. 2. Intraoperative photograph after dura matter was opened. A reddish and firm tumour is attached to the inner aspect of the dura matter and to the Th 3 nerve root
showed histiocytic transformation and Russell bodies. Immunohistochemical analysis demonstrated typical CD138 expression by the plasma cells and a monoclonal IgG-kappa chain secretion in most of the tumour cells, consistent with the diagnosis of plasmacytoma. Plasma cells express CD 138 and do not express CD 20, which is typically expressed by B-lymphocytes. In this instance, most cells expressed CD 138 and not CD 20, which was diagnostic of plasma cell proliferation, ruling out other neoplasms such as B-cell lymphoma. The monoclonal nature of the plasma cell proliferation ruled out inflammatory processes such us plasma cell granuloma [1, 7].
Solitary thoracic intradural extramedullary plasmacytoma
Fig. 3. Photomicrographs of tumour sections showing well-differentiated plasma cells. H & E, original magnification 60
After the operation, the patient made an uneventful partial neurological recovery. Investigations into the possibility of multiple myeloma were performed in the Department of Haematology. Serum protein electrophoresis and immunoglobulins were normal with no monoclonal band (M-band) present. Urine protein and urine light chains (Bence-Jones protein) were also absent. Plain film skeletal survey and isotope bone scanning did not show any other abnormality. Bone-marrow examination showed 0.4% well-differentiated plasma cells. Cytogenetic studies on the bone-marrow samples, using fluorescence in situ hybridisation, did not show chromosomal alterations. She received 30 Gy local radiation therapy and is now in remission without evidence of myelomatous conversion. Discussion Spinal compression is the most common cause of neurological symptoms in a patient with plasma cell disease and 5–10% of patients with multiple myeloma develop paraparesis. Whereas the vertebral bodies are commonly affected in MM and regarded as the most common site for solitary osseous plasmacytomas, the spine is an exceptional location for a solitary dural plasmacytoma. Plasmacytomas arising from vertebral bodies may invade epidural space secondarily, hence causing spinal cord compression [8]. In the patient we report, neurological deterioration was the most prominent symptom and not pain as is usual when there is vertebral involvement and bone destruction. The primary progressive spinal cord compression reflected an intradural discrete accumulation of plasma cells without any leptomeningeal or bone infiltration. A solitary monoclonal plasma cell
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tumour arising from the dura matter is rare and for unknown reasons, has a predilection for the intracranial dura matter [1, 2, 4]. The mean age of patients at the time of presentation with a solitary dural plasmacytoma is 50.2 years and most are female (84%) [4]. Lebrun et al. [7], reviewed the literature and reached the conclusion that a solitary dural plasmacytoma is a different pathological entity, with a longer progressionfree survival interval, to a solitary osseous plasmacytoma. The latter occurs more often in men than women (3:1) at average age between 40 and 60 years. Solitary osseous plasmacytomas appear to have a different natural history with a high risk (more than 60%) of progressing to multiple myeloma whereas this is no so common (less than 30%) in other types of extramedullary plasmacytoma [6]. Vertebral disease has been reported to be a poor prognostic factor compared to other bone locations. In contrast, cranial dural plasmacytomas [1, 4, 6], do not appear progress to multiple myeloma [4], unless located in the base where they are bone infiltrating and associated with multiple myeloma [2]. This highlights role of bone infiltration as a bad prognostic feature. We believe that only one example of an intradural spinal solitary dural plasmacytoma has been reported previously in the English language literature [8] but this patient had multiple bony lesions. Thoracic spinal cord compression (Th 5) developed and the findings were very similar to those in our patient. Further treatment and survival were not described but it seems that spinal and cranial solitary dural plasmacytoma may have a similar benign behaviour. Dural plasmacytoma does not have specific radiological features and hence is usually found at operations performed on the suspicion of another tumour, usually meningioma. In the spine, the main differential diagnosis of an intradural extramedullary lesion lies between meningioma and schwannoma, while dural metastasis and lymphoma=leukemia are less frequent. There are no pathogenomic pathological features that differentiate between solitary plasmacytoma and multiple myeloma, so that a definitive diagnosis of plasmacytoma can be made only after systemic disease has been ruled out. Radiation therapy has been the classical treatment for a solitary plasmacytoma but there are no conclusive data in the literature on the optimum dose. Most centres use approximately 40 Gy for a spinal solitary osseous lesion and the margins should include at least one uninvolved vertebra. Local control and symptom relief are achieved in more than 80% of patients. The overall median survival time is 10 years [5]. Surgery is not indicated for a spinal
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solitary osseous plasmacytoma unless there is evidence of structural instability or neurologic compromise. For extramedullary plasmacytoma, combined treatment with surgery and radiotherapy seems to provide the best results. The most commonly used dose for local control is 40 Gy (depending on tumour size) delivered over 4– 6 weeks. The 10-year overall survival rate is 70% [9]. We conclude that solitary dural plasmacytomas are usually associated with neurological symptoms and that the diagnosis is usually not suspected before operation. Although the optimum management is not known with certainty, it appears that treatment should consist of complete resection, if possible, followed by post-operative radiation with the volume and dose of radiation determined by the clinical situation [2, 10]. References 1. Benli K, Inci S (1995) Solitary dural plasmacytoma: case report. Neurosurgery 36: 1206–1209 2. Bindal AK, Bindal RK, van Loveren H, Sawaya R (1995) Management of intracranial plasmacytoma. J Neurosurg 83: 218–221 3. Galieni P, Cavo M, Avvisati G, Pulsoni A, Falbo R, Bonelli MA, Russo D, Petrucci MT, Bucalossi A, Tura S (1995) Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different entities? Ann Oncol 6: 687–691 4. Haegelen C, Riffaud L, Bernard M, Carsin-Nicol B, Morandi X (2006) Dural plasmacytoma revealing multiple myeloma. Case report. J Neurosurg 104: 608–610 5. Hu K, Yahalom J (2000) Radiotherapy in the management of plasma cell tumors. Oncology (Williston Park) 14: 101–108 6. Knobel D, Zouhair A, Tsang RW, Poortmans P, Belkacemi Y, Bolla M, Oner FD, Landmann C, Castelain B, Ozsahin M (2006)
I. Zazpe et al.: Solitary thoracic intradural extramedullary plasmacytoma
7. 8. 9.
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Prognostic factors in solitary plasmacytoma of the bone: a multicenter Rare Cancer Network study. BMC Cancer 6: 118 Lebrun C, Chanalet S, Paquis P, Frenay M, Lagrange JL, Chatel M (1997) Solitary meningeal plasmacytomas. Ann Oncol 8: 791–795 Sod LM, Wiener LM (1959) Intradural extramedullary plasmacytoma; case report. J Neurosurg 16: 107–109 Soutar R, Lucraft H, Jackson G, Reece A, Bird J, Low E, Samson E; Guidelines Working Group of the UK Myeloma Forum; British Committee for Standards in Haematology; British Society for Haematology (2004) Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 124: 717–726 Vujovic O, Fisher BJ, Munoz DG (1998) Solitary intracranial plasmacytoma: case report and review of management. J Neurooncol 39: 47–50
Comments The authors present a unique case of a solitary thoracic intradural plasmacytoma in a 25-year old woman, with diagnosis confirmed by the systemic hematological evaluation as well as the immunochemical study. Since there is no identical presentation in the currently available literature, a publication will be a valuable contribution to include this entity in our differential diagnosis of similar findings. Kenji Ohata Abeno-ku This is a nice, well documented single case report of a rare intradural extramedullary plasmacytoma without other myelomatous lesion in a young woman showing good remission following local radiotherapy. K. A. Jellinger Vienna
Correspondence: Idoya Zazpe, Department of Neurosurgery, Hospital of Navarre, Irunlarrea 3, 31008 Pamplona, Navarre, Spain. e-mail:
[email protected]
