Presentation
CASE REPORT: We present one female patient with non-Hodgkin's lymphoma, who had multiple cranial nerve palsies as signs of central nervous system involvement, but who had negative results in her neuroimaging studies. [ncbi.nlm.nih.gov]
Perfect for radiologists who need a comprehensive reference while working on difficult cases, it presents a complete yet concise overview of imaging applications, findings, and interpretation in every anatomic area. [books.google.com]
The presenting history is often only a few months and most patients present with a space occupying lesion or lesions producing local deficits. [thamburaj.com]
[…] estimates were 36%.(5,15) Our patient presented with simultaneous orbital and leptomeningeal involvement, perhaps a unique presentation. [djo.harvard.edu]
It is typically a non-Hodgkin lymphoma, and maybe an isolated recurrence or may be part of a systemic disease at the time of presentation 2. [radiopaedia.org]
Entire Body System
- Weakness
Extremity weakness and autonomic dysfunction occur in 75 and 55 percent of patients, respectively. 13, 14, 16 Weakness is usually symmetric and involves the legs, although it occasionally affects the arms. [aafp.org]
Facial muscle weakness. Dizziness. Unsteadiness and incoordination, hydrocephalus (increased pressure inside the brain), voice and swallowing difficulties. [neurosurgery.ucla.edu]
Patients may experience nausea and vomiting, leg and arm weakness, seizures, headaches, changes in mental alertness or confusion, facial weakness, double vision and hearing loss and/or swallowing difficulties. [lls.org]
Meanwhile, the patient experienced progressive paresthesia and weakness in both feet and worsening back pain. [ispub.com]
Weakness or paralysis of one side of the body may also occur as a result of a tumour on one side of the brain. Who does primary CNS lymphoma commonly affect? It is most common in people aged in their 50s and 60s, but can occur at any age. [leukaemia.org.au]
- Fever
Ara-C, like MTX, also may cause meningism, headache, and fever. Thiotepa causes less neurologic toxicity than MTX; the most common effect if transient limb paresthesias. Unlike MTX, thiotepa has no antidote for the resultant myelosuppression. [aboutcancer.com]
Signs and symptoms [ edit ] A primary CNS lymphoma usually presents with seizure, headache, cranial nerve findings, altered mental status, or other focal neurological deficits typical of a mass effect. [5] [6] Systemic symptoms may include fever, night [en.wikipedia.org]
The clinical signs and symptoms of intracranial lymphoma are nausea, vomiting, ataxia, changes in vision, confusion, fever, headaches due to damage of bone or meninges by the tumor, leaning to one side when walking, loss of coordination, numbness to hot [cancerjournal.net]
He had no past history of fever and head injury. He was normotensive and nondiabetic. [nnjournal.net]
Such symptoms include persistent or repeated fever, unexplained weight loss (i.e., loss of at least 10 percent of normal body weight), and/or sweating, particularly at night (known as “night sweats”). [rarediseases.org]
Face, Head & Neck
- Facial Numbness
More than one half of patients with leptomeningeal metastases have cranial nerve palsies. 22 – 24 Common symptoms include diplopia, visual loss, dysphagia, hearing loss and facial numbness. [aafp.org]
Neurologic
- Headache
[…] and cold, pain, and seizures. [5], [8], [9], [10] In our case, the patient had headache, vomiting and diminished right-sided vision. [cancerjournal.net]
Location - Sphenoid Ridge Common Symptoms - Eye-bulging, decreased vision, paralysis of eye movement, seizures, memory difficulty, personality change, headache. [neurosurgery.ucla.edu]
(Left) Contrast-enhanced CT scan shows three separate tumor nodules (arrows) in a 42-year-old woman with breast cancer who developed headaches and confusion. [aafp.org]
Signs and symptoms usually consist of headache and altered mental status, including cognitive impairment and confusion. Currently, leptomeningeal malignancy is diagnosed in about 5% of patients with cancer. [healio.com]
Mafosfamide is a form of cyclophosphamide that is active intrathecally and has little neurotoxicity aside from headaches, but only phase II trials have been conducted. Diaziquone is effective in hematologic tumors. [aboutcancer.com]
- Papilledema
Physical: Involvement of CNS is divided into the following 3 broad anatomical groups: Cerebral involvement results in headache, lethargy, papilledema, behavior changes, and gait disturbance (the latter can be due to either cerebellar or cauda equina involvement [aboutcancer.com]
Fundoscopy revealed bilateral atrophic papilledema. Hematological examination showed hemoglobin level of 11.0 g/dL, white blood cell count of 5,840/mm 3 with a normal differential and platelet count of 234,000/mm 3. [nnjournal.net]
Table TABLE 2 Neurologic Signs and Symptoms of Metastatic Brain Tumors Signs and symptoms Percentage of patients Hemiparesis 55 to 60 Impaired cognition 55 to 60 Headache 25 to 40 Focal weakness 20 to 30 Altered mental status 20 to 25 Sensory loss 20 Papilledema [aafp.org]
[…] associated with nausea, vomiting, light-headedness) Gait difficulties from weakness or ataxia Memory problems Incontinence Sensory abnormalities CNS symptoms are divided into the following 3 anatomic groups: Cerebral involvement: Headache, lethargy, papilledema [emedicine.medscape.com]
- Cranial Neuropathy
Multiple cranial neuropathies: Presenting signs of systemic lymphoma. Survey of Ophthalmology, Vol. 37, Issue. 2, p. 125. [journals.cambridge.org]
[…] inducing cranial neuropathy, especially related to the loss of myelin. [jnnp.bmj.com]
Chemotherapy-related neuropathies Paraneoplastic neuropathies Paraneoplastic disorders Inflammatory myopathies Lambert-Eaton syndrome Enhanced MRI studies can be helpful in determining the etiology of a cranial neuropathy syndrome. [aafp.org]
- Cognitive Impairment
Signs and symptoms usually consist of headache and altered mental status, including cognitive impairment and confusion. Currently, leptomeningeal malignancy is diagnosed in about 5% of patients with cancer. [healio.com]
These side effects might be referred to as ‘ cancer-related cognitive impairment ’. Some of these problems can develop as late effects months or even years after the treatment. These problems are more likely the older you are when you are treated. [lymphomas.org.uk]
- Neck Stiffness
Pain is often prominent and can present as neck stiffness, localized spinal tenderness or radicular discomfort that radiates from the spine into an arm or leg ( Table 3 ). 22 – 24 Weakness is also common and usually affects both legs in a lower motor [aafp.org]
Workup
Diagnostic procedures PCNSL should be suspected in any patient with posterior uveitis that has a negative workup for common causes of posterior uveitis. [eyewiki.aao.org]
[…] cauda equina, or subependymal areas, which extend into the sulci of the cerebrum or folia of the cerebellum MRI of the spinal cord can show nerve-root thickening, cord enlargement, intraparenchymal and subarachnoid nodules, or epidural compression See Workup [emedicine.medscape.com]
Order a workup for LC in patients presenting with the following: Neurologic signs and symptoms at more than 1 level of the neuraxis (This is present in 75% of patients with LC.) [aboutcancer.com]
Treatment
For treatment/prophylaxis of a CNS disease as part of treatment to cure acute lymphoblastic leukemia, radiation treatment may be included. It is often combined with CNS-directed chemotherapy/intrathecal treatment. [oncolex.org]
Your treatment may depend on: the symptoms you have your general health. When you are first diagnosed, you may have treatment to control any symptoms before you have treatment for the lymphoma. [macmillan.org.uk]
[…] with late effects of your treatment. [lymphomas.org.uk]
There is no standard treatment for CNS lymphoma. [lls.org]
All patients received intrathecal methotrexate treatment. [jstage.jst.go.jp]
Prognosis
PURPOSE: Non-Hodgkin's lymphoma which occasionally involves the central nervous system, occurs more often in high-grade cases and implicates a poor prognosis. Leptomeningeal metastases may present as multiple cranial nerve involvements. [ncbi.nlm.nih.gov]
The prognosis of primary cranial vault malignant lymphoma is unknown, and any involvement of cerebral structures and systemic involvement suggests an unfavorable prognosis. [nnjournal.net]
In this section: Definition | Symptoms | Diagnosis | Treatment | Prognosis MRI image showing a typical meningioma tumor A meningioma is a tumor that arises from a layer of tissue (the meninges) that covers the brain and spine. [neurosurgery.ucla.edu]
Second, neurologic deficits from central nervous system metastasis are frequently irreversible and associated with a poor prognosis. [ispub.com]
Prognosis [ edit ] In immunocompetent patients [ edit ] The initial response to radiotherapy is often excellent, and may result in a complete remission. [en.wikipedia.org]
Etiology
The etiology of primary CNS lymphoma is not well understood. [consultant360.com]
Numb chin syndrome in cancer patients: etiology, response to treatment, and prognostic significance. Neurology. 1992;42:1181–4. Copyright © 1999 by the American Academy of Family Physicians. This content is owned by the AAFP. [aafp.org]
Etiology Although there are reports of infectious agents being associated with PCNSL in non-immunocompromised patients (HHV-8, EBV), there is more compelling evidence of a viral mechanism (EBV) in AIDs associated PCNSL. [eyewiki.aao.org]
More rarely, an infectious etiology should also be considered. [djo.harvard.edu]
It is likely that while the etiology of PCNSL in the non-immunosuppressed remains obscure, in immunosuppressed individuals with reduced immune surveillance (under T cell control) the Epstein Barr virus (EBV) may be directly implicated. [thamburaj.com]
Epidemiology
[…] or spinal cord that causes cranial-nerve palsies or radiculopathies Alterations in the metabolism of nervous tissue that cause seizures, encephalopathy, or focal deficits Occlusion of blood vessels as they cross the subarachnoid, leading to infarcts Epidemiology [emedicine.medscape.com]
Epidemiology of primary CNS lymphoma. J Neurooncol. 1999; 43 :199-201 2. Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer. 2011; 105 :1414-8 3. [thno.org]
Pathophysiology
Pathophysiology Metastatic seeding of the leptomeninges may be explained by the following 6 postulated mechanisms: (1) hematogenous spread to choroid plexus and then to leptomeninges, (2) primary hematogenous metastases through the leptomeningeal vessels [emedicine.medscape.com]
Pathophysiology: Metastatic seeding of the leptomeninges may be explained by the following 5 postulated mechanisms: (1) hematogenous spread to choroid plexus and then to leptomeninges, (2) primary hematogenous metastases through the leptomeningeal vessels [aboutcancer.com]
Prevention
You may have anti-convulsant drugs to prevent seizures (fits). The main treatment for PCNSL is usually chemotherapy. It is often given with a targeted therapy drug called rituximab. [macmillan.org.uk]
It also prevents or interferes with many chemotherapy drugs passing from the blood to the brain. This protection is very important but it prevents many drugs from reaching lymphoma in the CNS. [lymphomas.org.uk]
The prime suspect in preventing systemic therapy from reaching the site of leptomeningeal disease is the blood-brain barrier. To overcome this barrier, drug administration directly to the site of CNS involvement is being explored. [healio.com]
(This barrier prevents or slows the passage of certain foreign agents, including harmful chemicals, microorganisms, particular medications, etc., from the bloodstream into the CNS. [rarediseases.org]
Intrathecal chemotherapy treats subclinical leptomeningeal deposits and tumor cells floating in the CSF, preventing further seeding. MTX is the mainstay of treatment. [aboutcancer.com]