Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy
Abstract Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmac...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Georg Thieme Verlag KG
2015-06-01
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| Series: | Journal of Neurological Surgery Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0035-1554930 |
| _version_ | 1811229804772458496 |
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| author | Neil Kalwani Aaron K. Remenschneider William Faquin Judith Ferry Eric H. Holbrook |
| author_facet | Neil Kalwani Aaron K. Remenschneider William Faquin Judith Ferry Eric H. Holbrook |
| author_sort | Neil Kalwani |
| collection | DOAJ |
| description | Abstract
Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmacytomas may result in cranial neuropathies and often progress to multiple myeloma more rapidly than other intracranial or skeletal plasmacytomas.
Clinical Presentation A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at 18-month follow-up. Unfortunately he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics.
Conclusion The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas. |
| first_indexed | 2024-04-12T10:20:10Z |
| format | Article |
| id | doaj.art-6005c4a093df4da6af8a9ac3f6ade96c |
| institution | Directory Open Access Journal |
| issn | 2193-6358 2193-6366 |
| language | English |
| last_indexed | 2024-04-12T10:20:10Z |
| publishDate | 2015-06-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | Journal of Neurological Surgery Reports |
| spelling | doaj.art-6005c4a093df4da6af8a9ac3f6ade96c2022-12-22T03:37:07ZengGeorg Thieme Verlag KGJournal of Neurological Surgery Reports2193-63582193-63662015-06-017601e156e15910.1055/s-0035-1554930Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve PalsyNeil Kalwani0Aaron K. Remenschneider1William Faquin2Judith Ferry3Eric H. Holbrook4Section of Otology and Laryngology, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, United StatesSection of Otology and Laryngology, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, United StatesSection of Pathology, Department of Otolaryngology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, United StatesSection of Pathology, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, United StatesSection of Otology and Laryngology, Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, United StatesAbstract Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmacytomas may result in cranial neuropathies and often progress to multiple myeloma more rapidly than other intracranial or skeletal plasmacytomas. Clinical Presentation A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at 18-month follow-up. Unfortunately he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics. Conclusion The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0035-1554930plasmacytomaclivusskull baseendoscopic sinus surgery |
| spellingShingle | Neil Kalwani Aaron K. Remenschneider William Faquin Judith Ferry Eric H. Holbrook Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy Journal of Neurological Surgery Reports plasmacytoma clivus skull base endoscopic sinus surgery |
| title | Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy |
| title_full | Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy |
| title_fullStr | Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy |
| title_full_unstemmed | Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy |
| title_short | Plasmacytoma of the Clivus Presenting as Bilateral Sixth Nerve Palsy |
| title_sort | plasmacytoma of the clivus presenting as bilateral sixth nerve palsy |
| topic | plasmacytoma clivus skull base endoscopic sinus surgery |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0035-1554930 |
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