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      Frontal skull craniotomy combined with moderate-dose radiotherapy effectively ameliorate a rare case of non-secretory, multiple myeloma with orbital involvement

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      World Journal of Surgical Oncology
      BioMed Central

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          Abstract

          Background

          Orbital infiltration in patients with multiple myeloma is a rare condition, with less than 50 cases reported in the medical literature. Most patients undergo conservative treatment because multiple myeloma is a disseminated systemic disease.

          Case presentation

          A 43-year-old male subject with multiple myeloma and long-term survival presented with orbital involvement. The subject lacked the typical features and poor prognostic factors associated with multiple myeloma, such as renal failure, hypercalcemia, and paraprotein in the serum and urine. The orbital computed tomographic scan revealed the tumor encasing the optic nerve, but without prominent bony destruction. Therefore, a frontal skull craniotomy with an epidural entrance to the orbital space was performed, to completely extirpate the orbital mass. The surgical procedure was followed by moderate-dose radiation therapy. After 32 months of follow-up care, the subject is doing well with excellent local control.

          Conclusion

          Although the effectiveness and applicability of this approach remains to be determined, this case report demonstrates that accurate and early detection combined with local surgical treatment and appropriate radio/chemotherapy, can be applied to effectively extend an orbital multiple myeloma patient's life.

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          Most cited references21

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          Multiple myeloma: review of 869 cases.

          R A Kyle (1974)
          A review of 869 cases of multiple myeloma seen at the Mayo Clinic from 1960 through 1971 revealed that 98% of patients were 40 years of age or older and that 61% of them were males. Inital findings were bone pain in 68% of patients, anemia in 62%, renal insufficiency in 55%, hypercalcemia in 30%, a palpable liver in 21%, and a palpable spleen in 5%. Proteinuria was noted in 88% and Bence Jones proteinuria was identified in 49%. Skeletal roentgenographic abnormalities were seen in 79%. Serum protein electrophoresis showed a spike in 76%, hypogammaglobulinemia in 9%, and minor or no abnormalities in 15%, and a globulin spike was seen 75% of the urinary electrophoretic patterns. Immunoelectrophoresis of the serum revealed a monoclonal heavy chain in 83% and a monoclonal light chain in the serum, in 8% (Bence Jones proteinemia). Three patients had no monoclonal protein in the serum or the urine ("nonsecretory"). Amyloidosis was found in 7% of the patients. Follow-up information was obtained in 99.7% ; 82% of the 869 patients have died. Infection and renal insufficiency were the most common specific causes of death. The median survival was 20 months; 66% of the patients were alive at 1 year and 18% at 5 years.
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            From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit.

            The histologic spectrum of nonosseous tumors and tumorlike lesions of the extraocular orbit in children differs from that in adults, and the appearance of these lesions at imaging reflects their pathologic features. Rhabdomyosarcoma is the most common extraocular orbital tumor in children. This neoplasm usually manifests in young children, grows quite rapidly, and is fairly vascular. Vasculogenic lesions are common orbital lesions in newborns and young infants. The most prevalent of these are infantile hemangioma, a true neoplasm, and venous-lymphatic malformation, a developmental anomaly. Hemangioma is quite vascular, has a predictable course of proliferation followed by slow involution, and is distinguished on magnetic resonance images by the finding of flow voids within the mass and at its periphery. Venous-lymphatic malformation in the orbit is an anomaly of venous and lymphatic development that is characterized by unenhancing, cystic lymphatic and enhancing, solid venous components. Intralesional hemorrhage is common and frequently produces distinctive fluid-fluid levels within the cystic portions. Unlike hemangiomas, venous-lymphatic malformations grow with the patient and never involute spontaneously. Infantile fibromatosis is one of the fibromatoses and affects newborns and young infants. The tumor is nodular and composed of a zonal architecture, with frequent hemorrhage or necrosis in the central portion, characteristics that confer a target appearance at imaging. These lesions usually stop growing or spontaneously regress. All of these extraocular masses typically manifest with proptosis, and imaging differentiation is desirable because the treatments and prognoses vary greatly.
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              Extramedullary plasmacytoma of the head and neck. A clinicopathologic study of 20 cases.

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                Author and article information

                Journal
                World J Surg Oncol
                World Journal of Surgical Oncology
                BioMed Central
                1477-7819
                2009
                12 November 2009
                : 7
                : 86
                Affiliations
                [1 ]Departments of Radiation Therapy and Oncology, Taipei, Taiwan
                [2 ]Department of Neurosurgery, Shin Kong Memorial Hospital, Taipei, Taiwan
                Article
                1477-7819-7-86
                10.1186/1477-7819-7-86
                2785810
                19909529
                6477f0a3-1e20-4ae7-bf8f-2c5c490c0294
                Copyright ©2009 Ko et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 1 June 2009
                : 12 November 2009
                Categories
                Case report

                Surgery
                Surgery

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