Solitary Intracranial Extra-osseous Plasmacytoma Presenting with Ophthalmic Signs

Brannan, S; Matthews, Bethan N.; Savant, Vijay; Brown, Ray D.; Matthews, Timothy D.

doi:10.1097/00041327-200312000-00006

Cited by 15 publications

(10 citation statements)

References 8 publications

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“…Plasmacytoma can be successfully managed with involved field irradiation with 50 Gy, preceded by chemotherapy in patients with large lesions. 74 WBRT 35-45 Gy followed by a 5-15 Gy boost was proposed for CNS Hodgkin lymphoma, with a median OS from CNS involvement of 44 months. 75 …”

Section: Who Are the Best Candidates For Radiation Therapy Alone?mentioning

confidence: 99%

How I treat primary CNS lymphoma

Ferreri¹

2011

Blood

163

109

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Primary CNS lymphoma (PCNSL) is a rare malignancy with peculiar clinical and biologic features, aggressive course, and unsatisfactory outcome. It represents a challenge for multidisciplinary clinicians and scientists as therapeutic progress is inhibited by several issues. Molecular and biologic knowledge is incomplete, limiting the identification of new therapeutic targets, and the particular microenvironment of this malignancy, and sanctuary sites where tumor cells grow undisturbed, strongly affects treatment efficacy. Moreover, active treatments are known to be associated with disabling neurotoxicity, posing the dilemma of whether to intensify therapy to improve the cure rate or to de-escalate treatment to avoid sequels. The execution of prospective trials is also difficult because of the rarity of the tumor and the impaired general condition and poor performance status of patients. Thus, level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.Despite this unfavorable background, laboratory and clinical researchers are coordinating efforts to develop new ideas, resulting in the recent publication of studies on PCNSL's biology and molecular mechanisms and of the first international randomized trials. Herein, these important contributions are analyzed to provide recommendations for everyday practice and the rationale for future trials. (Blood. 2011;118(3):510-522) IntroductionPrimary CNS lymphoma (PCNSL) is an aggressive malignancy arising exclusively in the CNS, that is, the brain parenchyma, spinal cord, eyes, cranial nerves, and/or meninges. 1 This lymphoma represents 4% of intracranial neoplasms and 4%-6% of all extranodal lymphomas but some registry studies suggest that its incidence in immunocompetent patients is progressively increasing. 2 PCNSL exhibits peculiar clinical and biologic features and constitutes a diagnostic and therapeutic challenge for multidisciplinary clinicians and scientists. Its outcome remains unsatisfactory if compared with that of patients with extra-CNS lymphomas of a similar stage and histotype, and several factors prevent therapeutic progress. First, molecular and biologic knowledge is insignificant compared with other lymphomas, which limits the identification of new therapeutic targets. Second, patients with PCNSL usually exhibit impaired general condition and poor performance status (PS) more often than subjects with other lymphomas. 1 This usually interferes with the enrollment of patients in prospective trials. In fact, current therapeutic knowledge is only based on 3 randomized trials, some single-arm phase 2 trials, anecdotal meta-analyses, and a few multicenter retrospective studies. Thus, available level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.In this manuscript, I discuss available clinical and biologic data on PCNSL in immunocompetent patients and provide recommendations for everyday pract...

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Section: Who Are the Best Candidates For Radiation Therapy Alone?mentioning

confidence: 99%

How I treat primary CNS lymphoma

Ferreri¹

2011

Blood

163

109

View full text Add to dashboard Cite

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“…Isolated intracranial plasmacytoma has been described as responsible for compressive optic neuropathy at the orbital apex in a small number of single case reports. [3][4][5][6] Orbital plasmacytoma, also a rare condition, may potentially involve the optic nerve in this location. [7][8][9] Infiltration by myelomatous cells is an even more unusual mechanism of optic nerve damage.…”

Section: Discussionmentioning

confidence: 99%

Multiple Myeloma Presenting as Bilateral Posterior Optic Neuropathy

Muñoz

Acebes

Arruga

et al. 2010

Neuro-Ophthalmology

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We report a case of bilateral posterior optic neuropathy as the presenting sign of multiple myeloma. A 72-year-old woman experienced bilateral progressive visual loss leading to blindness in 1 week without optic disc swelling. Brain and orbit magnetic resonance imaging revealed thickening of both intracranial optic nerves with abnormal sheath enhancement in post-contrast sequences. Systemic work-up detected a monoclonal band in blood and urine, and skeletal survey showed lytic lesions in the skull bones and multiple collapsed vertebrae. Bone marrow biopsy revealed atypical plasma cells. On the basis of these findings the diagnosis of multiple myeloma was made even though there was neither hypercalcemia nor renal failure. The mechanism of visual loss is a presumed infiltration of the intracranial optic nerves and the adjacent meningeal coverings.

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“…Bei einem zerebralen Plasmozytom besteht der überwiegende Teil des Infiltrats aus Plasmazellen [2,3,4,6,7,8,12,13,14,15,17,18,19,21,22]. Findet sich ein heterogenes Zellbild aus Plasmazellen, Lymphozyten, Histiozyten, gelegentlich neutrophilen Granulozyten, Granulationsgewebe und Fibrose, spricht man von Plasmazellgranulomen [5,9,10,16,20]. Weidenheim et al [24] definierten 1989 erstmals eine atypische monoklonale Plasmazellhyperplasie in Duranähe, die einem Meningeom glich.…”

Section: Diskussionunclassified

“…In ähnlichen Fallberichten wurde die Erkrankung als Plasmozytom diagnostiziert [3,4,5,7,8,12,13,14,15,17,18,19,21,22].…”

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Atypische monoklonale Plasmazellhyperplasie

et al. 2006

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Atypical monoclonal plasma cell hyperplasia is extremely rare in the central nervous system without involvement of bone or other systemic disease. This neuropathological entity was first described by Weidenheim and colleagues in 1989. It is considered an inflammatory pseudotumor that can evolve into malignant myeloma. We report on an atypical monoclonal plasma cell hyperplasia with bilateral occipital and cerebellar involvement in a 38-year-old woman. The diagnosis was confirmed by brain biopsy immunohistochemical and molecular tests.

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Solitary Intracranial Extra-osseous Plasmacytoma Presenting with Ophthalmic Signs

Cited by 15 publications

References 8 publications

How I treat primary CNS lymphoma

How I treat primary CNS lymphoma

Multiple Myeloma Presenting as Bilateral Posterior Optic Neuropathy

Atypische monoklonale Plasmazellhyperplasie

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