RESUMO
We report a case of a 54-year-old female with progressive chronic pain in lower extremity, paraplegia and loss of function of the anal sphincter. MRI revealed an expansive solid intradural and intramedullar lesion located at the T6 to T9 levels. Histology of the lesion showed melanocytes with the results of immunohistochemistry consistent with a melanocytoma. Melanocytic tumors are rare tumors which present a diagnostic and management challenge for the modern neurosurgeon and neuro-oncologist since MRI and CT features are non-specific and there is scant data to standardise best strategic therapy.
Assuntos
Melanoma/patologia , Neoplasias Meníngeas/patologia , Terapia Combinada , Incontinência Fecal/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Melanócitos/patologia , Melanoma/diagnóstico , Melanoma/radioterapia , Melanoma/cirurgia , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Pessoa de Meia-Idade , Cuidados Paliativos , Paraplegia/etiologia , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/patologia , Neoplasias da Medula Espinal/radioterapia , Neoplasias da Medula Espinal/cirurgia , Siringomielia/etiologia , Vértebras TorácicasRESUMO
In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases. A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2-3, T5-11, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma. The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case. Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area. Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.
Assuntos
Ependimoma/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Neoplasias da Medula Espinal/cirurgia , Adulto , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Quimioterapia Adjuvante , Terapia Combinada , Descompressão Cirúrgica , Ependimoma/diagnóstico , Ependimoma/tratamento farmacológico , Ependimoma/radioterapia , Seguimentos , Humanos , Laminectomia , Imageamento por Ressonância Magnética , Masculino , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/radioterapia , Recidiva Local de Neoplasia/cirurgia , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/tratamento farmacológico , Neoplasias Primárias Múltiplas/radioterapia , Exame Neurológico , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/cirurgia , Radioterapia Adjuvante , Compressão da Medula Espinal/diagnóstico , Compressão da Medula Espinal/cirurgia , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/tratamento farmacológico , Neoplasias da Medula Espinal/radioterapiaRESUMO
Intramedullary spinal cord metastases (ISCM) are uncommon and present with rapidly progressing neurological deficits. The objective of this study was to determine the rate, duration of neurological response and survival after radiation therapy. We have retrospectively reviewed the clinical outcome of six cases with a diagnosis of ISCM from primary lung cancer, non-small cell (NSCLC) (n=3) and small cell (SCLC) (n=3). Total radiation dose ranged from 27 Gy/5 fr to 40 Gy/20 fr. Ambulation was preserved in 3 patients and partially recovered in one. Five out of the six patients (83%) showed improvement in neurological signs/symptoms with a mean duration of 17.2 days (max: 40 days; min: 6 days). Median survival time was 5 months (confidence interval (CI) 95%: 0-12) for NSCLC and 5 months (CI 95%: 4-6) for SCLC. Although radiation response rate is high, the interval free of neurological progression is very short. A therapeutic approach should be considered for each individual.
Assuntos
Carcinoma Pulmonar de Células não Pequenas/secundário , Carcinoma de Células Pequenas/secundário , Neoplasias Pulmonares/patologia , Neoplasias da Medula Espinal/secundário , Idoso , Idoso de 80 Anos ou mais , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma Pulmonar de Células não Pequenas/radioterapia , Carcinoma de Células Pequenas/patologia , Carcinoma de Células Pequenas/radioterapia , Vértebras Cervicais , Intervalo Livre de Doença , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Cuidados Paliativos , Paraplegia/etiologia , Dosagem Radioterapêutica , Estudos Retrospectivos , Compressão da Medula Espinal/etiologia , Compressão da Medula Espinal/radioterapia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/radioterapia , Vértebras Torácicas , Resultado do TratamentoAssuntos
Dor nas Costas/etiologia , Neoplasias Induzidas por Radiação/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Neurilemoma/diagnóstico , Paraplegia/etiologia , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/diagnóstico , Adolescente , Ganglioneuroma/radioterapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Induzidas por Radiação/complicações , Neoplasias Induzidas por Radiação/etiologia , Segunda Neoplasia Primária/complicações , Segunda Neoplasia Primária/etiologia , Neurilemoma/complicações , Neurilemoma/etiologia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/etiologia , Neoplasias da Medula Espinal/radioterapiaRESUMO
Nine children with neuroblastoma and five with Ewing sarcoma were found at diagnosis to have epidural extension of tumor. Five children underwent laminectomy prior to referral, with good neurologic recovery in only one. Management in the other nine children did not include laminectomy. All 14 patients were given chemotherapy without radiotherapy. Rapid regression of tumor with neurologic recovery occurred in response to chemotherapy in all patients with neurologic deficits. The responses observed in these children indicate that for chemotherapy-sensitive tumors, effective chemotherapy is a feasible alternative to laminectomy and radiation therapy in the management of epidural disease.