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