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Skull Base Plasmacytoma
Philip R. Chapman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Solitary bone plasmacytoma (SBP)
              • Extramedullary plasmacytoma (EMP)
              • Multiple myeloma (MM)
            • Synonyms

              • Benign plasmacytoma, solitary myeloma, solitary plasmacytoma
            • Definitions

              • Solitary bone plasmacytoma
                • Monoclonal plasma cell neoplasm of bone marrow seen in absence of other criteria to fulfill diagnosis of MM
                • Generally presents as single osteolytic lesion
                • Can affect any bone of skull base, but has predilection for sphenoid bone and petrous apex
              • Extramedullary plasmacytoma
                • Solitary extraosseous plasma cell proliferation in absence of other criteria to fulfill diagnosis of MM
                • Though closely related to SBP and MM, appears to be distinct entity
                • Soft tissue mass associated with mucosal surface of sinonasal cavity or nasopharynx, with secondary skull base erosion
                • Rarely arises as primary dural based lesion
              • Multiple solitary plasmacytoma
                • Multiple sites of extramedullary or osseous lesions in absence of MM criteria
              • Malignant plasmacytoma
                • Focal intramedullary or extramedullary tumor in context of MM
                • Once diagnosis of MM is established, most no longer use term plasmacytoma

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                • Reporting Tips

                                                  Selected References

                                                  1. Kalwani N et al: Plasmacytoma of the clivus presenting as bilateral sixth nerve palsy. J Neurol Surg Rep. 76(1):e156-9, 2015
                                                  2. Patel TD et al: Sinonasal extramedullary plasmacytoma: a population-based incidence and survival analysis. Int Forum Allergy Rhinol. 5(9):862-9, 2015
                                                  3. Neelakantan A et al: Benign and malignant diseases of the clivus. Clin Radiol. 69(12):1295-303, 2014
                                                  4. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                                  5. Gagliardi F et al: Solitary clival plasmocytomas: Misleading clinical and radiological features of a rare pathology with a specific biological behaviour. Acta Neurochir (Wien). 155(10):1849-56, 2013
                                                  6. Ahn AI et al: Skull base plasmacytoma with conductive hearing loss and an external auditory canal mass. Ear Nose Throat J. 91(7):E1-5, 2012
                                                  7. Moss HE et al: Acute optic neuropathy associated with an intracranial mass in a patient with POEMS syndrome. J Neuroophthalmol. 32(1):45-7, 2012
                                                  8. Liu ZY et al: Solitary intracranial plasmacytoma located in the spheno-clival region mimicking chordoma: a case report. J Int Med Res. 38(5):1868-75, 2010
                                                  9. Soutar R et al: Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol. 124(6):717-26, 2004
                                                  10. Kingdom TT et al: Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. Am J Otolaryngol. 24(5):317-22, 2003
                                                  11. Bret P et al: [Solitary plasmacytoma of the sphenoid. A case report] Neurochirurgie. 48(5):431-5, 2002
                                                  12. Schwartz TH et al: Association between intracranial plasmacytoma and multiple myeloma: clinicopathological outcome study. Neurosurgery. 49(5):1039-44; discussion 1044-5, 2001
                                                  13. Movsas TZ et al: Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma. J Neuroophthalmol. 20(4):242-5, 2000
                                                  14. Randoux B et al: [Solitary plasmocytoma of the calvarium] J Neuroradiol. 27(4):278-81, 2000
                                                  15. Vijaya-Sekaran S et al: Solitary plasmacytoma of the skull base presenting with unilateral sensorineural hearing loss. J Laryngol Otol. 113(2):164-6, 1999
                                                  16. Cervoni L et al: Solitary plasmacytoma of the calvarium: a review of clinical and prognostic features. Neurosurg Rev. 21(2-3):102-5, 1998
                                                  17. Nofsinger YC et al: Head and neck manifestations of plasma cell neoplasms. Laryngoscope. 107(6):741-6, 1997
                                                  18. Okamoto K et al: Solitary plasmacytomas of the occipital bone: a report of two cases. Eur Radiol. 7(4):503-6, 1997
                                                  19. Provenzale JM et al: Craniocerebral plasmacytoma: MR features. AJNR Am J Neuroradiol. 18(2):389-92, 1997
                                                  20. Savas MC et al: Bulky plasmacytoma of the skull with intracranial involvement. Am J Hematol. 54(2):173, 1997
                                                  21. Mantyla R et al: Intracranial plasmacytoma: a case report. Neuroradiology. 38(7):646-9, 1996
                                                  22. Bindal AK et al: Management of intracranial plasmacytoma. J Neurosurg. 83(2):218-21, 1995
                                                  23. Prasad ML et al: Solitary intracranial plasmacytoma of the skull base. Indian J Cancer. 31(3):174-9, 1994
                                                  24. Marais J et al: Solitary plasmacytoma of the skull base. Ann Otol Rhinol Laryngol. 101(8):665-8, 1992
                                                  25. Camacho J et al: The spectrum of neurological manifestations in myeloma. J Med. 16(5-6):597-611, 1985
                                                  26. Toland J et al: Plasmacytoma of the skull base. Clin Radiol. 22(1):93-6, 1971
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Top Differential Diagnoses

                                                        • Pathology

                                                          • Clinical Issues

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Solitary bone plasmacytoma (SBP)
                                                              • Extramedullary plasmacytoma (EMP)
                                                              • Multiple myeloma (MM)
                                                            • Synonyms

                                                              • Benign plasmacytoma, solitary myeloma, solitary plasmacytoma
                                                            • Definitions

                                                              • Solitary bone plasmacytoma
                                                                • Monoclonal plasma cell neoplasm of bone marrow seen in absence of other criteria to fulfill diagnosis of MM
                                                                • Generally presents as single osteolytic lesion
                                                                • Can affect any bone of skull base, but has predilection for sphenoid bone and petrous apex
                                                              • Extramedullary plasmacytoma
                                                                • Solitary extraosseous plasma cell proliferation in absence of other criteria to fulfill diagnosis of MM
                                                                • Though closely related to SBP and MM, appears to be distinct entity
                                                                • Soft tissue mass associated with mucosal surface of sinonasal cavity or nasopharynx, with secondary skull base erosion
                                                                • Rarely arises as primary dural based lesion
                                                              • Multiple solitary plasmacytoma
                                                                • Multiple sites of extramedullary or osseous lesions in absence of MM criteria
                                                              • Malignant plasmacytoma
                                                                • Focal intramedullary or extramedullary tumor in context of MM
                                                                • Once diagnosis of MM is established, most no longer use term plasmacytoma

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Staging, Grading, & Classification

                                                                                • Gross Pathologic & Surgical Features

                                                                                  • Microscopic Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Consider

                                                                                              • Image Interpretation Pearls

                                                                                                • Reporting Tips

                                                                                                  Selected References

                                                                                                  1. Kalwani N et al: Plasmacytoma of the clivus presenting as bilateral sixth nerve palsy. J Neurol Surg Rep. 76(1):e156-9, 2015
                                                                                                  2. Patel TD et al: Sinonasal extramedullary plasmacytoma: a population-based incidence and survival analysis. Int Forum Allergy Rhinol. 5(9):862-9, 2015
                                                                                                  3. Neelakantan A et al: Benign and malignant diseases of the clivus. Clin Radiol. 69(12):1295-303, 2014
                                                                                                  4. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                                                                                  5. Gagliardi F et al: Solitary clival plasmocytomas: Misleading clinical and radiological features of a rare pathology with a specific biological behaviour. Acta Neurochir (Wien). 155(10):1849-56, 2013
                                                                                                  6. Ahn AI et al: Skull base plasmacytoma with conductive hearing loss and an external auditory canal mass. Ear Nose Throat J. 91(7):E1-5, 2012
                                                                                                  7. Moss HE et al: Acute optic neuropathy associated with an intracranial mass in a patient with POEMS syndrome. J Neuroophthalmol. 32(1):45-7, 2012
                                                                                                  8. Liu ZY et al: Solitary intracranial plasmacytoma located in the spheno-clival region mimicking chordoma: a case report. J Int Med Res. 38(5):1868-75, 2010
                                                                                                  9. Soutar R et al: Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol. 124(6):717-26, 2004
                                                                                                  10. Kingdom TT et al: Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. Am J Otolaryngol. 24(5):317-22, 2003
                                                                                                  11. Bret P et al: [Solitary plasmacytoma of the sphenoid. A case report] Neurochirurgie. 48(5):431-5, 2002
                                                                                                  12. Schwartz TH et al: Association between intracranial plasmacytoma and multiple myeloma: clinicopathological outcome study. Neurosurgery. 49(5):1039-44; discussion 1044-5, 2001
                                                                                                  13. Movsas TZ et al: Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma. J Neuroophthalmol. 20(4):242-5, 2000
                                                                                                  14. Randoux B et al: [Solitary plasmocytoma of the calvarium] J Neuroradiol. 27(4):278-81, 2000
                                                                                                  15. Vijaya-Sekaran S et al: Solitary plasmacytoma of the skull base presenting with unilateral sensorineural hearing loss. J Laryngol Otol. 113(2):164-6, 1999
                                                                                                  16. Cervoni L et al: Solitary plasmacytoma of the calvarium: a review of clinical and prognostic features. Neurosurg Rev. 21(2-3):102-5, 1998
                                                                                                  17. Nofsinger YC et al: Head and neck manifestations of plasma cell neoplasms. Laryngoscope. 107(6):741-6, 1997
                                                                                                  18. Okamoto K et al: Solitary plasmacytomas of the occipital bone: a report of two cases. Eur Radiol. 7(4):503-6, 1997
                                                                                                  19. Provenzale JM et al: Craniocerebral plasmacytoma: MR features. AJNR Am J Neuroradiol. 18(2):389-92, 1997
                                                                                                  20. Savas MC et al: Bulky plasmacytoma of the skull with intracranial involvement. Am J Hematol. 54(2):173, 1997
                                                                                                  21. Mantyla R et al: Intracranial plasmacytoma: a case report. Neuroradiology. 38(7):646-9, 1996
                                                                                                  22. Bindal AK et al: Management of intracranial plasmacytoma. J Neurosurg. 83(2):218-21, 1995
                                                                                                  23. Prasad ML et al: Solitary intracranial plasmacytoma of the skull base. Indian J Cancer. 31(3):174-9, 1994
                                                                                                  24. Marais J et al: Solitary plasmacytoma of the skull base. Ann Otol Rhinol Laryngol. 101(8):665-8, 1992
                                                                                                  25. Camacho J et al: The spectrum of neurological manifestations in myeloma. J Med. 16(5-6):597-611, 1985
                                                                                                  26. Toland J et al: Plasmacytoma of the skull base. Clin Radiol. 22(1):93-6, 1971