Central nervous system myelomatosis with optic neuropathy and intramedullary spinal cord compression responding to radiation therapy

Riley, Jenny M.; Russo, James Kyle; Shipp, Allison; Alsharif, Mariam; Jenrette, Joseph M.

doi:10.1007/s11604-011-0577-7

Cited by 7 publications

(5 citation statements)

References 7 publications

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“…Whole brain irradiation therapy is indicated when possible, given the scarcity of drugs that cross the BBB, but it is associated with significant toxicity [37]. Focused radiation can be used for bulky localized disease either in the brain or spine, but its role is limited in diffuse disease [64]. Prophylactic irradiation is generally not recommended due to high risk of toxicities.…”

Section: Radiation Therapymentioning

confidence: 99%

Retracted: Overview of recent trends in diagnosis and management of leptomeningeal multiple myeloma

et al. 2014

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Neurological complications related to multiple myeloma (MM) are not uncommon; however, direct involvement of the central nervous system (CNS) is extremely rare and represents a diagnostic and therapeutic challenge. Significant survival difference has been noted with the introduction of novel therapy in patients with MM, but their effect on the incidence and their use for management of leptomeningeal myeloma (LMM) is uncertain. Analysis of published data demonstrates its recent increased incidence, median time to CNS presentation, and slight improvement in median survival after diagnosis of LMM. Less common MM isotypes have been overrepresented in LMM. CNS relapse occurred mostly in patients with Durie-Salmon stage III MM. Despite treatments, standard or experimental, the survival rates of LMM remain dismal. Monitoring high risk patients closely, even after achieving complete remission, may be useful in early detection of LMM. As we gain better understanding of LMM, we recommend that future research and clinical care focus on earlier diagnosis and development of more efficient CNS-directed therapy to improve survival in this patient population.

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Section: Radiation Therapymentioning

confidence: 99%

Retracted: Overview of recent trends in diagnosis and management of leptomeningeal multiple myeloma

et al. 2014

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“…1 Targeted radiotherapy can alleviate focal symptoms such as muscle weakness caused by intramedullary spinal cord lesions. 58 There is evidence that modern radiotherapy techniques can deliver impressive responses in parenchymal CNS-MM lesions without significant myelotoxic sequelae. 59…”

Section: Cranial or Cranial-spinal Irradiationmentioning

confidence: 99%

Multiple myeloma with central nervous system relapse

et al. 2020

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C entral nervous system involvement in multiple myeloma is a rare complication but carries a very poor prognosis. We provide a review of current literature, including presentation, treatment and survival data, and describe our experience in a regional hematologic malignancy diagnosis center where, over a 15-year period, ten cases were identified. Although the median age of onset, frequently between 50-60 years, is comparatively young, those diagnosed usually have a preceding diagnosis of multiple myeloma and often have had several lines of treatment. We discuss putative underlying factors such as prior treatment and associations including possible risk factors and features suggestive of a distinct biology. Central nervous system involvement may be challenging to diagnose in myeloma, displaying heterogeneous symptoms that can be confounded by neurological symptoms caused by the typical features of myeloma or treatment side-effects. We discuss the clinical features, imaging and laboratory methods used in diagnosis, and highlight the importance of considering this rare complication when neurological symptoms occur at presentation or, more commonly, during the disease pathway. In the absence of clinical trial data to inform an evidence-based approach to treatment, we discuss current and novel treatment options. Finally, we propose the establishment of an International Registry of such cases as the best way to collect and subsequently disseminate presentation, diagnostic and treatment outcome data on this rare complication of multiple myeloma.

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“…28 The use of radiotherapy is controversial in terms of efficacy and toxicity; focused radiotherapy is commonly used in the treatment of focal masses either in the brain or spine, but its role is limited in diffuse CNS involvement. 29 In a review, the median survival time of LMM patients treated with cranial irradiation was 3 months, compared with 0.81 months in patients without radiotherapy, 6 while in another study long-term survivors had received adjuvant radiation, suggesting that the incorporation of this modality should be further evaluated. 21 Although in our case monoclonal plasma cells could not be assayed in CSF, the clinic-radiological picture described allowed us to make the most likely diagnosis, namely, LMM.…”

Section: Discussionmentioning

confidence: 99%

Imaging findings in a case of leptomeningeal myelomatosis, a rare but critical central nervous system complication of multiple myeloma

2023

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Leptomeningeal myelomatosis is a rare complication of multiple myeloma (<1% of the patients). There was an increase in the incidence of leptomeningeal myelomatosis during the last decade; the prognosis of leptomeningeal myelomatosis remains poor, (overall median survival from the time of diagnosis of 2 months). We discuss a rare case of a monoclonal gammopathy evolving into multiple myeloma and finally into a rapidly progressing leptomeningeal disease. A 76 year-old woman in hematologic follow-up for advanced stage multiple myeloma in sixth-line treatment had an episode of generalized tonic-clonic seizure with sphincter release followed by altered state of consciousness. The unenhanced head CT scan showed a mild enlargement of the ventricular system without intra-axial or extra-axial hemorrhages nor significant changes in brain parenchyma. The subsequent contrast-enhanced brain MRI revealed a widespread nodular leptomeningeal enhancement characterized by contrast-enhancement of the pia mater extended into the subarachnoid spaces of the sulci and cisterns, involving supra- and sub-tentorial regions and the statoacoustic nerve in the inner ear canal bilaterally. The fluid-attenuated inversion recovery MRI images demonstrated an abnormally elevated signal within the sulci in the parieto-occipital regions. The radiological diagnosis of leptomeningeal myelomatosis was made. The patient died 4 days after the examination. In patients with long-lasting multiple myeloma and onset of neurological signs or symptoms, a contrast-enhanced brain MRI should be performed to assess the actual burden of central nervous system involvement in leptomeningeal myelomatosis; CT may provide a clue to the diagnosis when progressive enlargement of the ventricles over time is noted.

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Central nervous system myelomatosis with optic neuropathy and intramedullary spinal cord compression responding to radiation therapy

Cited by 7 publications

References 7 publications

Retracted: Overview of recent trends in diagnosis and management of leptomeningeal multiple myeloma

Retracted: Overview of recent trends in diagnosis and management of leptomeningeal multiple myeloma

Multiple myeloma with central nervous system relapse

Imaging findings in a case of leptomeningeal myelomatosis, a rare but critical central nervous system complication of multiple myeloma

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