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      CyberKnife stereotactic radiosurgery for extramedullary plasmacytoma in the external auditory canal: illustrative case

      case-report

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          Abstract

          BACKGROUND

          Plasmacytoma, a rare plasma cell disorder, often presents as a solitary or multiple tumors within the bone marrow or soft tissues, typically associated with multiple myeloma. Extramedullary plasmacytomas (EMPs), particularly those located in the external auditory canal (EAC), are exceedingly rare and pose significant treatment challenges given their location, anatomical complexity, and high risk of recurrence.

          OBSERVATIONS

          The authors report the case of a 72-year-old male with a history of multiple myeloma, presenting with recurrent left EAC plasmacytoma. After initial conventional radiotherapy for the lesion, a recurrence was documented in 7 years. The patient subsequently underwent stereotactic radiosurgery, which proved successful, leading to complete resolution of the lesion without any long-term adverse effects or radiation-related complications over a 45-month period.

          LESSONS

          This case is a unique instance of utilizing stereotactic radiosurgery for recurrent EMP in the EAC, highlighting its potential as an effective approach in managing complex plasmacytomas.

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          Most cited references15

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          Single dose radiosurgical treatment of recurrent previously irradiated primary brain tumors and brain metastases: final report of RTOG protocol 90-05

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            Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management.

            Multiple myeloma accounts for approximately 10% of hematologic malignancies.The diagnosis requires ≥10% clonal bone marrow plasma cells or a biopsy proven plasmacytoma plus evidence of one or more multiple myeloma defining events (MDE): CRAB (hypercalcemia, renal failure, anemia, or lytic bone lesions) features felt related to the plasma cell disorder, bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain (FLC) ratio ≥100 (provided involved FLC is ≥100 mg/L), or >1 focal lesion on magnetic resonance imaging. Patients with del(17p), t(14;16), and t(14;20) have high-risk multiple myeloma. Patients with t(4;14) translocation and gain(1q) have intermediate-risk. All others are considered standard-risk. Initial treatment consists of bortezomib, lenalidomide, dexamethasone (VRD). In high-risk patients, carfilzomib, lenalidomide, dexamethasone (KRD) is an alternative to VRD. In eligible patients, initial therapy is given for approximately 3-4 months followed by autologous stem cell transplantation (ASCT). Standard risk patients can opt for delayed ASCT at first relapse. Patients not candidates for transplant are treated with Rd until progression, or alternatively, a triplet regimen such as VRD for approximately 12-18 months. After ASCT, lenalidomide maintenance is considered for standard risk patients especially in those who are not in very good partial response or better, while maintenance with a bortezomib-based regimen is needed for patients with intermediate or high-risk disease. Patients with indolent relapse can be treated with 2-drug or 3-drug combinations. Patients with more aggressive relapse require a triplet regimen or a combination of multiple active agents. Am. J. Hematol. 91:720-734, 2016. © 2016 Wiley Periodicals, Inc.
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              Head and neck manifestations of plasma cell neoplasms.

              Multiple myeloma, solitary plasmacytoma of bone, and extramedullary plasmacytoma are plasma cell neoplasms. They represent distinct manifestations of a disease continuum, whereby the clinical findings are critical to diagnosis. Plasma cell neoplasms are histologically similar, and distinguishing one from the other has significant implications for treatment and survival. Plasma cell neoplasms are relatively unusual malignancies of the head and neck region. We present a case series of plasma cell neoplasms involving the skull base, paranasal sinus, larynx, and mandible as an introduction to a complete review of the literature on plasma cell neoplasms of the head and neck area.
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                Author and article information

                Journal
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                Journal of Neurosurgery: Case Lessons
                American Association of Neurological Surgeons
                2694-1902
                6 May 2024
                6 May 2024
                : 7
                : 19
                : CASE2479
                Affiliations
                [1 ]Neurosurgery Artificial Intelligence Lab, Stanford University School of Medicine, Stanford, California; and
                [2 ]Department of Neurosurgery, Stanford University School of Medicine, Stanford, California
                Author notes
                Correspondence David J. Park: Stanford University School of Medicine, Stanford, CA. djpark@ 123456stanford.edu .

                INCLUDE WHEN CITING Published May 6, 2024; DOI: 10.3171/CASE2479.

                Disclosures Dr. Veeravagu reported personal fees from Nuvasive, Medtronic, and Surgical Theater outside the submitted work. Dr. Gibbs reported honoraria for lectures from Accuray, Inc. outside the submitted work.

                Article
                CASE2479
                10.3171/CASE2479
                11076403
                38710109
                fdce06b6-de2e-489f-9a46-c8b85cbfee30
                © 2024 The authors

                CC BY-NC-ND 4.0 ( http://creativecommons.org/licenses/by-nc-nd/4.0/)

                History
                : 1 February 2024
                : 28 March 2024
                Page count
                Figures: 2, Tables: 0, References: 15, Pages: 3
                Categories
                Stereotactic-Radiosurgery, Stereotactic Radiosurgery
                Tumor, Tumor
                Technique, Technique
                Surgical-Technique, Surgical Technique
                Case Lesson

                plasmacytoma,radiosurgery,brain neoplasm,neoplasm recurrence,external auditory canal,eac = external auditory canal,emp = extramedullary plasmacytoma,mm = multiple myeloma,mri = magnetic resonance imaging,srs = stereotactic radiosurgery

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