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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

            • Synonyms

              • Solitary plasmacytoma, skull base plasmacytoma
            • Definitions

              • Plasmacytoma in broad sense consists of localized or focal soft tissue infiltrate or mass that consists of neoplastic monoclonal plasma cells
              • Plasma cell neoplasms present in several pathologic and clinical forms and include multiple lesion variants and solitary lesion variants
              • Malignant plasmacytoma in context of multiple myeloma (MM)
                • Focal intramedullary or extramedullary tumor in context of criteria that establish diagnosis of MM
                • This is most common form of monoclonal plasma cell infiltrate or mass
                • > 90% of patients with plasma cell tumor will meet criteria for MM, and most will have multiple lesions
                • Majority of plasma cell lesions of skull base are seen in context of multiple additional bony lesions and fulfill established criteria for MM
              • Solitary plasmacytoma is rare and exists in 2 basic forms: solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP)
                • SBP
                  • Defined by presence of single lytic lesion of bone due to monoclonal plasma cell neoplasm, ± soft tissue extension
                  • Seen in absence of systemic disease, end organ damage, or other criteria to fulfill diagnosis of MM
                  • In skull base, predilection for sphenoid bone and petrous apex
                  • Accounts for 5% of plasmacytomas
                • EMP
                  • Solitary extraosseous or soft tissue 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 often associated with mucosal surface of sinonasal cavity or nasopharynx with secondary skull base erosion
                  • Rarely arises as primary dural-based lesion
                  • Accounts for ~ 2% of plasmacytomas
              • Solitary plasmacytomas can be further classified based on histologic evaluation of bone marrow aspirate remote from lesion
                • Solitary plasmacytoma without bone marrow plasmacytosis (normal bone marrow distant from lesion)
                  • Progression of solitary plasmacytomas to MM in 10% of cases
                • Solitary plasmacytoma with minimal bone marrow plasmacytosis (monoclonal infiltration < 10%)
                  • 60% of SBPs will develop into MM
                  • 20% of EMPs will transform into MM

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          • Reporting Tips

                                            Selected References

                                            1. Hillengass J et al: International myeloma working group consensus recommendations on imaging in monoclonal plasma cell disorders. Lancet Oncol. 20(6):e302-12, 2019
                                            2. Ma XJ et al: Clinical features, radiological profiles, and surgical outcomes of primary intracranial solitary plasmacytomas: a report of 17 cases and a pooled analysis of individual patient data. J Neurooncol. 142(2):263-272, 2019
                                            3. Pham A et al: Solitary plasmacytoma: a review of diagnosis and management. Curr Hematol Malig Rep. 14(2):63-9, 2019
                                            4. Caers J et al: Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. J Hematol Oncol. 11(1):10, 2018
                                            5. Grammatico S et al: Solitary Plasmacytoma. Mediterr J Hematol Infect Dis. 9(1):e2017052, 2017
                                            6. Lee J et al: Sellar and clival plasmacytomas: case series of 5 patients with systematic review of 65 published cases. Pituitary. 20(3):381-92, 2017
                                            7. Kalwani N et al: Plasmacytoma of the clivus presenting as bilateral sixth nerve palsy. J Neurol Surg Rep. 76(1):e156-9, 2015
                                            8. Patel TD et al: Sinonasal extramedullary plasmacytoma: a population-based incidence and survival analysis. Int Forum Allergy Rhinol. 5(9):862-9, 2015
                                            9. Neelakantan A et al: Benign and malignant diseases of the clivus. Clin Radiol. 69(12):1295-303, 2014
                                            10. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                            11. 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
                                            12. 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
                                            13. 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
                                            14. 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
                                            15. 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
                                            16. Kingdom TT et al: Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. Am J Otolaryngol. 24(5):317-22, 2003
                                            17. Bret P et al: [Solitary plasmacytoma of the sphenoid. A case report] Neurochirurgie. 48(5):431-5, 2002
                                            18. Schwartz TH et al: Association between intracranial plasmacytoma and multiple myeloma: clinicopathological outcome study. Neurosurgery. 49(5):1039-44; discussion 1044-5, 2001
                                            19. Movsas TZ et al: Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma. J Neuroophthalmol. 20(4):242-5, 2000
                                            20. Randoux B et al: [Solitary plasmocytoma of the calvarium] J Neuroradiol. 27(4):278-81, 2000
                                            21. 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
                                            22. Cervoni L et al: Solitary plasmacytoma of the calvarium: a review of clinical and prognostic features. Neurosurg Rev. 21(2-3):102-5, 1998
                                            23. Nofsinger YC et al: Head and neck manifestations of plasma cell neoplasms. Laryngoscope. 107(6):741-6, 1997
                                            24. Okamoto K et al: Solitary plasmacytomas of the occipital bone: a report of two cases. Eur Radiol. 7(4):503-6, 1997
                                            25. Provenzale JM et al: Craniocerebral plasmacytoma: MR features. AJNR Am J Neuroradiol. 18(2):389-92, 1997
                                            26. Savas MC et al: Bulky plasmacytoma of the skull with intracranial involvement. Am J Hematol. 54(2):173, 1997
                                            27. Mantyla R et al: Intracranial plasmacytoma: a case report. Neuroradiology. 38(7):646-9, 1996
                                            28. Bindal AK et al: Management of intracranial plasmacytoma. J Neurosurg. 83(2):218-21, 1995
                                            29. Prasad ML et al: Solitary intracranial plasmacytoma of the skull base. Indian J Cancer. 31(3):174-9, 1994
                                            30. Marais J et al: Solitary plasmacytoma of the skull base. Ann Otol Rhinol Laryngol. 101(8):665-8, 1992
                                            31. Camacho J et al: The spectrum of neurological manifestations in myeloma. J Med. 16(5-6):597-611, 1985
                                            32. 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

                                                      • Synonyms

                                                        • Solitary plasmacytoma, skull base plasmacytoma
                                                      • Definitions

                                                        • Plasmacytoma in broad sense consists of localized or focal soft tissue infiltrate or mass that consists of neoplastic monoclonal plasma cells
                                                        • Plasma cell neoplasms present in several pathologic and clinical forms and include multiple lesion variants and solitary lesion variants
                                                        • Malignant plasmacytoma in context of multiple myeloma (MM)
                                                          • Focal intramedullary or extramedullary tumor in context of criteria that establish diagnosis of MM
                                                          • This is most common form of monoclonal plasma cell infiltrate or mass
                                                          • > 90% of patients with plasma cell tumor will meet criteria for MM, and most will have multiple lesions
                                                          • Majority of plasma cell lesions of skull base are seen in context of multiple additional bony lesions and fulfill established criteria for MM
                                                        • Solitary plasmacytoma is rare and exists in 2 basic forms: solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP)
                                                          • SBP
                                                            • Defined by presence of single lytic lesion of bone due to monoclonal plasma cell neoplasm, ± soft tissue extension
                                                            • Seen in absence of systemic disease, end organ damage, or other criteria to fulfill diagnosis of MM
                                                            • In skull base, predilection for sphenoid bone and petrous apex
                                                            • Accounts for 5% of plasmacytomas
                                                          • EMP
                                                            • Solitary extraosseous or soft tissue 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 often associated with mucosal surface of sinonasal cavity or nasopharynx with secondary skull base erosion
                                                            • Rarely arises as primary dural-based lesion
                                                            • Accounts for ~ 2% of plasmacytomas
                                                        • Solitary plasmacytomas can be further classified based on histologic evaluation of bone marrow aspirate remote from lesion
                                                          • Solitary plasmacytoma without bone marrow plasmacytosis (normal bone marrow distant from lesion)
                                                            • Progression of solitary plasmacytomas to MM in 10% of cases
                                                          • Solitary plasmacytoma with minimal bone marrow plasmacytosis (monoclonal infiltration < 10%)
                                                            • 60% of SBPs will develop into MM
                                                            • 20% of EMPs will transform into MM

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  • Image Interpretation Pearls

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Hillengass J et al: International myeloma working group consensus recommendations on imaging in monoclonal plasma cell disorders. Lancet Oncol. 20(6):e302-12, 2019
                                                                                      2. Ma XJ et al: Clinical features, radiological profiles, and surgical outcomes of primary intracranial solitary plasmacytomas: a report of 17 cases and a pooled analysis of individual patient data. J Neurooncol. 142(2):263-272, 2019
                                                                                      3. Pham A et al: Solitary plasmacytoma: a review of diagnosis and management. Curr Hematol Malig Rep. 14(2):63-9, 2019
                                                                                      4. Caers J et al: Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. J Hematol Oncol. 11(1):10, 2018
                                                                                      5. Grammatico S et al: Solitary Plasmacytoma. Mediterr J Hematol Infect Dis. 9(1):e2017052, 2017
                                                                                      6. Lee J et al: Sellar and clival plasmacytomas: case series of 5 patients with systematic review of 65 published cases. Pituitary. 20(3):381-92, 2017
                                                                                      7. Kalwani N et al: Plasmacytoma of the clivus presenting as bilateral sixth nerve palsy. J Neurol Surg Rep. 76(1):e156-9, 2015
                                                                                      8. Patel TD et al: Sinonasal extramedullary plasmacytoma: a population-based incidence and survival analysis. Int Forum Allergy Rhinol. 5(9):862-9, 2015
                                                                                      9. Neelakantan A et al: Benign and malignant diseases of the clivus. Clin Radiol. 69(12):1295-303, 2014
                                                                                      10. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                                                                      11. 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
                                                                                      12. 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
                                                                                      13. 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
                                                                                      14. 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
                                                                                      15. 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
                                                                                      16. Kingdom TT et al: Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. Am J Otolaryngol. 24(5):317-22, 2003
                                                                                      17. Bret P et al: [Solitary plasmacytoma of the sphenoid. A case report] Neurochirurgie. 48(5):431-5, 2002
                                                                                      18. Schwartz TH et al: Association between intracranial plasmacytoma and multiple myeloma: clinicopathological outcome study. Neurosurgery. 49(5):1039-44; discussion 1044-5, 2001
                                                                                      19. Movsas TZ et al: Sixth nerve palsy as a presenting sign of intracranial plasmacytoma and multiple myeloma. J Neuroophthalmol. 20(4):242-5, 2000
                                                                                      20. Randoux B et al: [Solitary plasmocytoma of the calvarium] J Neuroradiol. 27(4):278-81, 2000
                                                                                      21. 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
                                                                                      22. Cervoni L et al: Solitary plasmacytoma of the calvarium: a review of clinical and prognostic features. Neurosurg Rev. 21(2-3):102-5, 1998
                                                                                      23. Nofsinger YC et al: Head and neck manifestations of plasma cell neoplasms. Laryngoscope. 107(6):741-6, 1997
                                                                                      24. Okamoto K et al: Solitary plasmacytomas of the occipital bone: a report of two cases. Eur Radiol. 7(4):503-6, 1997
                                                                                      25. Provenzale JM et al: Craniocerebral plasmacytoma: MR features. AJNR Am J Neuroradiol. 18(2):389-92, 1997
                                                                                      26. Savas MC et al: Bulky plasmacytoma of the skull with intracranial involvement. Am J Hematol. 54(2):173, 1997
                                                                                      27. Mantyla R et al: Intracranial plasmacytoma: a case report. Neuroradiology. 38(7):646-9, 1996
                                                                                      28. Bindal AK et al: Management of intracranial plasmacytoma. J Neurosurg. 83(2):218-21, 1995
                                                                                      29. Prasad ML et al: Solitary intracranial plasmacytoma of the skull base. Indian J Cancer. 31(3):174-9, 1994
                                                                                      30. Marais J et al: Solitary plasmacytoma of the skull base. Ann Otol Rhinol Laryngol. 101(8):665-8, 1992
                                                                                      31. Camacho J et al: The spectrum of neurological manifestations in myeloma. J Med. 16(5-6):597-611, 1985
                                                                                      32. Toland J et al: Plasmacytoma of the skull base. Clin Radiol. 22(1):93-6, 1971