Meningeal carcinomatosis in solid tumors

Abstract

The involvement of the leptomeninges by metastatic tumors can be observed in solid tumors, in which case it is termed meningeal carcinomatosis (MC), and in lymphoproliferative malignant disease. It is more common in breast and lung cancer, as well as melanoma, with adenocarcinoma being the most frequent histological type. MC is usually a late event, with disseminated and progressive disease already present and, it is characterized by multifocal neurological signs and symptoms. Diagnosis is based on the evaluation of clinical presentation, cerebrospinal fluid and neuroimaging studies. The better systemic disease control is observed with new therapeutic agents, and the development of neuroimaging methods is responsible for the increasing incidence of such metastatic evolution. Intrathecal chemotherapy is generally the treatment of choice, although frequently palliative. Prognosis is guarded, although a higher performance status may indicate a subgroup of patients with a more favorable outcome. Key words: meningeal carcinomatosis, cerebrospinal fluid, meninges, chemotherapy, metastases.Carcinomatose meníngea em tumores sólidos RESUMO O acometimento leptomeníngeo por metástases tumorais pode ocorrer em tumores sólidos, sendo chamado de carcinomatose meníngea (CM), e também em doenças linfoproliferativas. Tumores de mama, pulmão e melanoma são os principais responsáveis pelos casos, e adenocarcinoma é a histologia mais frequentemente encontrada. A CM é um evento tardio na evolução da doença e caracteriza-se por sinais e sintomas neurológicos multifocais. O diagnóstico se faz pela avaliação conjunta do quadro clínico, neuroimagem e estudo do líquido cefalorraquidiano. O maior controle da doença sistêmica obtido com as novas modalidades terapêuticas e a baixa penetração de drogas no sistema nervoso central, aliados ao desenvolvimento nos métodos de neuroimagem observado nas últimas décadas, são fatores que respondem por um aumento na incidência desta apresentação. A quimioterapia intratecal é o tratamento de escolha, porém, frequentemente paliativo. O prognóstico é reservado, sendo que o melhor performance status pode selecionar um subgrupo de pacientes com melhor evolução. Palavras-Chave: carcinomatose meníngea, líquido cefalorraquidiano, meninges, quimioterapia, metástase.

Clinical Presentation, Diagnosis, and Radiological Findings of Neoplastic Meningitis

“…1,2 Neoplastic meningitis is characterized by the diffuse involvement of the leptomeninges (pia and arachnoid), the subarachnoid space, and the cerebrospinal fluid (CSF) by malignant cells, and it occurs through the hematogenous invasion of the subarachnoid space and ventricles or through direct extension from bone and brain lesions, or, in some cases, with local spread through the dura along perineural and perivascular spaces. 3 The most frequent primary solid tumors associated with neoplastic meningitis are breast cancer, non-small-cell lung cancer, and malignant melanoma. 4 Neoplastic meningitis is typically a late event (70%) and is rarely (15%) a presenting finding in an undiagnosed malignancy.…”

A 56-year-old man with acute vision loss

“…3,4 It is the first manifestation of systemic cancer in only 5%-10% of patients, although it is frequently seen in patients with disseminated systemic disease. 5,6 Hematologic tumors are the most frequent cause of meningeal carcinomatosis, particularly acute lymphoblastic leukemia and non-Hodgkins lymphoma.…”

“…21 The guidelines suggest that any one of the following diagnostic criteria are sufficient to diagnose meningeal carcinomatosis; CSF positive for tumor cells (positive CSF cytology); radiologic findings in the central nervous system consistent with meningeal carcinomatosis irrespective of supportive clinical findings or alternatively and more controversial, clinical signs and symptoms consistent with meningeal carcinomatosis, and a nonspecific but abnormal CSF analysis (high white blood cell count, low glucose <60 mg/dl, and elevated protein >50 mg/dl) in a patient known to have a cancer. 4,7 Although meningeal carcinomatosis occurs in only 3%-8% of all cancer patients, it is associated with devastating neurologic complications and high mortality. 10 The median survival of patients with this disorder is 4-6 weeks without treatment; even in response to therapy, median survival in these patients is limited to 18.6 weeks.…”

“…Patients with meningeal carcinomatosis have a guarded prognosis with a mean survival rate of 3-6 months. 4,5,22 …”

Central Diabetes Insipidus in a Young Patient with Leptomeningeal Metastasis Related Colon Cancer: Case Report

“…Bir araştırmada bu oranın solid tümörler için %15'e kadar yükselebildiği belirtilmişken, başka bir çalışmada ilerlemiş sistemik kanser öyküsü bulunan bireylerin post-mortem incelemelerinde ortalama %9 civarında saptandığı rapor edilmiştir. 11,12 Genellikle LK hastalığın son evrelerinde yaygın hastalıkla birlikte ortaya çık-maktadır; ancak bu durumun, henüz saptanmamış bir tümörün ilk bulgusu olabileceği de belirtilmiş-tir (%6).…”