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Malignant Meningeal Lymphoma

Malignant Lymphoma of Meninges


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]
  • These findings suggest that a leptomeningeal localisation of a low-grade malignant lymphoma had been present for ten years before systemic manifestation of the disease.[link.springer.com]
  • Although at present we are still far from a precise recognition of the basic features of lymphoproliferative processes and from a fairly general agreement on the terminology of non-Hodgkin lymphomas, there is encouraging evidence recently of some enlightment[books.google.com]
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]
Froin's Syndrome
  • However, Froin's syndrome has not been reported in hematological malignancies. Herein, we present two cases of lymphomatous leptomeningitis with Froin's syndrome.[ncbi.nlm.nih.gov]
Aspiration
  • Includes recommendations from the 2008 National Cancer Institute Thyroid Fine Needle Aspiration State of the Science Conference.[books.google.com]
  • Unlike primary CNS lymphoma , it more commonly involves the leptomeninges and is uncommonly detectable on CT/MRI, with malignant cells found on CSF aspiration.[radiopaedia.org]
  • Some tests used to stage CNS lymphoma include: CT scan PET scan Bone marrow aspiration and biopsy Slit-lamp eye exam Vitrectomy Testicular examination in men Prognostic Factors There are certain factors that affect prognosis (the chance of recovery) and[lls.org]
  • For operative procedures, including a biopsy or a needle aspiration, we generally need a copy of both the: a. Operative note, and b. Pathology report. 3.[ssa.gov]
  • […] new application of single-cell molecular profiling on a microfluidic chip for lymphoma will address questions regarding the diagnosis and treatment response of the disease not only in CSF, but also in other paucicellular samples such as fine needle aspirates[thno.org]
Hoarseness
  • One day before readmission, she noticed double vision, dysphagia, and hoarseness.[jnnp.bmj.com]
Ptosis
  • Neurological examination demonstrated bilateral ptosis, dilatation of bilateral pupils being sluggishly reactive to light, paralysis of extraocular movement, paralysis of soft palate movement, and bilateral hearing disturbances.[jnnp.bmj.com]
  • […] progression of the disease, with attention to orbital involvement, is recorded for a period of over 2.5 years, until the patient’s death. top Case Report A 46-year-old woman presented to our clinic with a complaint of intermittent proptosis, diplopia, and ptosis[djo.harvard.edu]
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]
Headache
  • Case report Clinical features: A 42-year-old female presented with headache and vomiting for 15 days. Headache was aggravated while bending down and sneezing. Patient also complained of decreased right-sided vision since one week.[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 Nerve Involvement
  • Leptomeningeal metastases may present as multiple cranial nerve involvements. Diagnosis is achieved by recognizing the clinical manifestations, followed by neuroradiologic studies and laboratory examination of the cerebrospinal fluid.[ncbi.nlm.nih.gov]
  • Cranial-nerve involvement: Impaired vision, diplopia, hearing loss, and sensory deficits, including vertigo; cranial-nerve palsies commonly involve CN III, IV, VI, VII, and VIII Spinal-root involvement: Nuchal rigidity and neck and back pain, or invasion[emedicine.medscape.com]
  • Cranial-nerve involvement presents with impaired vision, diplopia (most common), hearing loss, and sensory deficits, including vertigo. Palsies of cranial nerves III, V, and VI are most common; palsy of nerve VII is less common.[aboutcancer.com]
Cranial Neuropathy
  • neuropathy, especially related to the loss of myelin.[jnnp.bmj.com]
  • Multiple cranial neuropathies: Presenting signs of systemic lymphoma . Survey of Ophthalmology, Vol. 37, Issue. 2, p. 125.[journals.cambridge.org]
  • As discussed previously, cranial neuropathies are most commonly caused by leptomeningeal metastases within the subarachnoid space. However, tumors can also damage cranial nerves after they have exited the subarachnoid space.[aafp.org]
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]
Xanthochromia
  • [Article in Japanese] Abstract Froin's syndrome is characterized by a combination of marked coagulation, elevated protein levels, and xanthochromia of the cerebrospinal fluid (CSF).[ncbi.nlm.nih.gov]
  • Xanthochromia can occur from leptomeningeal bleeding, which is most likely in LC from a melanoma. Biochemical markers in CSF have poor sensitivity and specificity, but levels decline with successful therapy.[aboutcancer.com]

Treatment

  • All patients received intrathecal methotrexate treatment.[jstage.jst.go.jp]
  • الصفحة 162 - Small cell carcinoma of the lung: staging, paraneoplastic syndromes, treatment and survival. Cancer 33, 527-532 (1974). ‏ الصفحة 71 - Limbic encephalitis' and its association with carcinoma. Brain: 91; 481-496, 1968. ‏[books.google.com]
  • 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]

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]
  • […] mb.infoweb.ne.jp Statistics from Altmetric.com The frequency of the involvement of non-Hodgkin's lymphoma in the CNS has been reported to be less than 10%. 1-3 Moreover, as those patients have often been resistant to both chemotherapy and radiation therapy, their prognosis[jnnp.bmj.com]
  • 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]
  • 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]
Sex distribution
Age distribution

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]
  • 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]
  • 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]
  • (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]

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