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Cui et al. Diagnosis and treatment of meningeal carcinomatosis

by Siefert in 1902. This disease was uncommon at meninges directly.
that time, and usually confirmed by autopsies. In
the past few decades, 10-30% of people with solid DIAGNOSIS
tumors progress to nervous system metastasis, with
4-15% developing into MC. [6,7] The metastases most The diagnosis of MC may be confirmed on the basis
frequently come from carcinoma of the breast, lung, of the National Comprehensive Cancer Network
gastrointestinal tract, and melanoma. Adenocarcinoma guidelines. [13] The guidelines indicated that any one
is the most common histologic types. This disorder of the criteria listed below are sufficient to diagnose
[8]
is easy to be misdiagnosed because of the diverse MC; positive CSF cytology; neuroimaging findings
clinical manifestations and lack of specificity. consistent with MC, supportive clinical signs and
symptoms and a nonspecific but abnormal CSF
This article systematically reviewed the epidemiology, changes (increased white blood cell count, decreased
pathogenesis, clinical manifestation, auxiliary glucose, and high protein concentration) in patients
examination, diagnosis, treatment and prognostic suffering from tumor. Despite substantial false negative
aspects. rate, CSF cytology remains the gold diagnostic
standard. In addition to the cytological/neuroimaging/
EPIDEMIOLOGY clinical diagnosis, other CSF parameters such as
β-glucuronidase, creatine-kinase BB isoenzyme (CK-
With the development of imaging technique and BB), etc. may be regarded as adjuvant diagnosing
therapies, the survival time of patients with MC is for MC and are also used to monitor the treatment
prolonged and the incidence of MC is growing. response. Furthermore, the diagnostic value of the
MC can occur secondary to tumors have not been tumor-derived cell-free DNA in the CSF is promising
discovered and in antitumor therapy, which was most and may improve the diagnostic yield of CSF analysis.
common in older individuals. About 4-7% of patients
with solid tumor suffer meningeal metastasis, [9-11] Clinical characteristics
with lung cancer (9-25%), gastrointestinal tumor Multiple interrelated events result in clinical
(4-14%), breast cancer (2-5%) and malignant symptoms of MC, such as obstruction of CSF reflux
melanoma (23%) as the most common causes. MC leading to hydrocephalus, nutrient metabolism
can be also detected clinically in 5-15% of patients competition between neoplastic cells and normal
with hematological malignancies (lymphomatosis cells resulting in neurological function deficit, tumors
or lymphomatous) and primary brain tumors invasion of Vichow Robin Spaces. Most patients with
(gliomatosis). [12] However, there are still cancers MC first presented with headache, nausea, vomiting,
with an unknown primary (1-7%). Above all, MC is a epilepsy, cervical radicular pain, hemiplegia and
relatively late event in carcinoma process. unconsciousness. In a cohort of 60 patients with
breast cancer leptomeningeal metastases, headache
PATHOPHYSIOLOGY was the most common presenting symptoms
(55%), followed by various cranial neuropathies
In many MC cases, the damages can be seen in and epilepsy (50% and 12%, respectively). Vertigo
autopsies as brain tissue edema, enlarged cerebral presented in 12 patients (20%). [14] Classically, MC
gyrus, meningeal congestion, cerebrospinal presents with various clinical signs and symptoms
membrane greyish white in color, ventriculomegaly on in three domains of neurologic function: the cerebral
sectioning. Microscopically, tumor cells can be seen in hemispheres; the cranial nerves; and the spinal cord
the cerebrospinal membrane and subarachnoid space and associated roots. [15]
diffusely or focally, while nodules are not obtained in
cerebral parenchyma. Headache, nausea, vomiting are the most frequent
manifestations of cerebral hemisphere dysfunction.
Cancer cells may leave the primary tumor to Other signs include hemiplegia, aphasia, changes in
meningeal by following routes: malignant neoplasms mental status, seizures and cognitive impairments.
cells may shift to subarachnoid space or cerebral However, simple focal ischemic cerebral injury and
ventricles by hematological invasion, with later non-communicating hydrocephalus are uncommon.
spread to the cerebrospinal fluid (CSF). Both the Diplopia, hearing impairment, hemianopsia, and
perineural or perivascular spaces and cranial or trigeminal sensory loss are common symptoms of
radicular nerve pathway carry tumor cells to dura cranial nerve involvement with the VI cranial nerve
mater, leptomeninges, or the ependyma, leading to being the most frequently impaired, followed by
tumor deposits. Neoplastic cells may spread to the cranial nerve III and IV. The most frequent spinal
168 Neuroimmunology and Neuroinflammation ¦ Volume 4 ¦ September 18, 2017