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      Extramedullary Plasmacytoma Presenting as a Solitary Mass in the Intracranial Posterior Fossa

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          Abstract

          A patient with a 3-month history of headache refractory to pain medication was admitted. The CT scan and MRI showed evidence of a posterior fossa mass. This was pathologically confirmed as an extra medullary plasmacytoma (EMP). He had a pathologic fracture of the left humerus 7 years ago while the radiologist was unaware at the time of diagnosis. A solitary bone plasmacytoma (SBP) was the cause of the pathologic fracture. This report includes the first description of MRI findings in a patient with a rare-incidence intracranial solitary extra medullary plasmacytoma (SEP) in Iran. There is a striking similarity between the features of intracranial SEP and meningiomas. Intracranial SEP, although rare, should be included in the differential diagnosis of brain tumors in areas where meningiomas commonly arise. The MRI findings and differential diagnosis of plasmacytoma are reviewed. Before this case report, only few cases have been reported in the literature. Nonetheless, this is the first report of posterior fossa EMP from Iran.

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          Extramedullary plasmacytoma of the head and neck region: clinicopathological correlation in 25 cases.

          Extramedullary plasmacytomas (EMP) of head and neck are rare tumours. Between 1972 and 1993, 25 cases of EMP of head and neck were seen at our institute. The clinical and pathological features and response to treatment are presented. At initial presentation, 23 (92%) patients presented with disease confined to a single extramedullary site only and two patients had in addition clinical involvement of cervical lymph nodes. All except these two patients received radiotherapy to the primary site only as initial treatment. Initial primary control of local disease was obtained in 16 of 24 (67%) patients treated with radical intent. With salvage treatment of further radiotherapy and/or chemotherapy, local disease control was achieved in 21 of 24 (88%) patients. One patient was treated with palliative intent. Conversion to multiple myeloma was seen in two patients (8%). Pathologically, the tumours were classified into low, intermediate and high grade, which correlated closely with outcome. This classification has been used for the first time in extramedullary plasmacytomas and is based on the multiple myeloma grading criteria devised by Bartl et al (1987). Fifteen of eighteen (83%) low-grade tumours and only one of six (17%) intermediate- and high-grade tumours were locally controlled after primary radiotherapy. This is statistically significant for local control (P= 0.0019) but not for overall survival (P= 0.12). The median survival and 5-year overall survival is 68 months and 58.9% respectively. We recommend consideration of adjuvant chemotherapy in patients with higher grade disease. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6
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            Clinical outcome of extramedullary plasmacytoma.

            The aim of this study was a retrospective analysis of the presenting features of extramedullary plasmacytoma, its response to therapy and its clinical course. Forty-six cases diagnosed between August 1970 and June 1993 were carefully reviewed. The follow-up was continued until June 1998 and the median observation time was 118 months. The disease was most frequently localized in the upper airways (37/46; 80%), with the mass limited to a single site in all but seven patients in whom two contiguous sites were involved. Other localizations were the lymph nodes, thyroid, skin, stomach, and brain. The clinical symptoms were related to the site of presentation, and the median time between appearance and diagnosis was 7.5 months. The median age at diagnosis was 55 years (range 16-80), with 14 patients (30%) being under 50 years old. The disorder was approximately twice as common in males as in females. Ten patients (21%) had a monoclonal component. The therapeutic strategy varied, although the most frequent form of treatment was local radiotherapy. Thirty-nine patients (85%) achieved complete remission (CR), five (11%) a partial remission (PR) and two (4%) did not respond to therapy (NR). Local recurrence (LR) or recurrence at other sites (ROS) occurred in 7.5% and 10%, respectively. Seven patients (15%) developed multiple myeloma (MM), characterized by multiple sites of osteolysis in almost all cases with soft tissue involvement in some of them. The 15 year survival rate was 78%. This review of a relatively large series of patients confirms the favorable prognosis of EMP when treated locally by irradiation and/or surgery.
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              Intracranial involvement in plasmacytomas and multiple myeloma: a pictorial essay.

              The purpose of this pictorial essay is to increase awareness of the clinical presentation, neuroradiological findings, treatment options, and neuroradiological follow-up of plasmacytomas and multiple myeloma with intracranial growth. This pictorial essay reviews the clinical features and neuroradiological findings in seven patients (four women, three men; age range at diagnosis 62-82 years) followed in two institutions. Six patients, one with IgG-kappa plasmacytoma, and five with IgG-kappa (n = 3), IgG-lambda (n = 1), and nonsecretory (n = 1) multiple myeloma, had been seen over a period of 9 years in one institution, and the other patient with IgG-kappa plasmacytoma had been seen over a period of 3.5 years in the other. Intracranial involvement is rare, most frequently resulting from osseous lesions in the cranial vault, skull base, nose, or paranasal sinuses. Primary dural or leptomeningeal involvement is rarer. Some typical findings of a dural and/or osseous plasmacytoma include iso- to hyperdensity on CT scan, T1 equal to high signal intensity and T2 markedly hypointense signal on MRI, and high vascularity possibly documented on intraarterial digital subtraction angiography. However, the neuroradiological findings generally lack specificity, since they are generally no different from those of meningioma, metastasis, lymphoma, dural sarcoma, plasma cell granuloma, infectious meningitis, and leptomeningeal carcinomatosis. The spectrum of clinical and neuroradiological evaluation shows that intracranial involvement from plasmacytoma and multiple myeloma must be taken into account in the differential diagnosis of cranial osseous and meningeal disease.
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                Author and article information

                Journal
                Iran J Radiol
                Iran J Radiol
                10.5812/iranjradiol
                Kowsar
                Iranian Journal of Radiology
                Kowsar
                1735-1065
                2008-2711
                20 November 2012
                November 2012
                : 9
                : 4
                : 223-226
                Affiliations
                [1 ]Department of Radiology, Radiotherapy and Nuclear Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
                [2 ]Neurosciences Research Center (NSRC), Tabriz University of Medical Sciences, Tabriz, Iran
                [3 ]Department of Neurosurgery, Shohada Hospital, Tabriz University of Medical Sciences, Tabriz, Iran
                [4 ]Department of Clinical Science, Veterinary Faculty, Urmia University of Medical Sciences, Urmia, Iran
                [5 ]Tabriz University of Medical Sciences, Tabriz, Iran
                Author notes
                [* ]Corresponding author: Mohammad Hossein Daghighi, Department of Radiology, Radiotherapy and Nuclear Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. Tel.: +98-4113346911, Fax: +98-4113346911, E-mail: mh_daghighi@ 123456yahoo.com
                Article
                10.5812/iranjradiol.8759
                3569557
                774e376a-e54f-44b5-ae4c-031ce61d85fd
                Copyright © 2012, Tehran University of Medical Sciences and Iranian Society of Radiology

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 November 2011
                : 11 July 2012
                : 28 July 2012
                Categories
                Neuroradiology

                Radiology & Imaging
                magnetic resonance imaging,posterior fossa,plasmacytoma
                Radiology & Imaging
                magnetic resonance imaging, posterior fossa, plasmacytoma

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