RESUMO
INTRODUCTION: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement. CASE DESCRIPTION: We present a 33-year-old woman previously submitted to five brain surgeries to treat a posterior fossa intradural chordoma since December 2012. Currently, she presented almost with right and left hemiplegia (grade 2 bilaterally) and also left oculomotor, adbucent and facial nerve paresis. We performed neuroaxis magnetic resonance MR which disclosed stability of posterior fossa tumor which was previously irradiated. However, there were new intradural lesions at the level of C3, T11/T12 and L4/L5/S1 vertebrae. DISCUSSION: With the advent of contemponaeous surgery, radiotherapy options and even available chemotherapy to treat Chordomas (Imatinib), patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis. Our case illustrates a late (6â¯years) follow-up presentation of an initial posterior fossa intradural chordoma. It suggests that whole neuraxis involvement may be the final presentation of all patients harbouring chordomas and surviving after adequate initial treatment.
Assuntos
Cordoma/patologia , Neoplasias Infratentoriais/secundário , Neoplasias da Medula Espinal/secundário , Adulto , Cordoma/cirurgia , Feminino , Humanos , Neoplasias Infratentoriais/cirurgia , Imageamento por Ressonância MagnéticaAssuntos
Carcinoma de Células Renais/diagnóstico por imagem , Carcinoma de Células Renais/patologia , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/patologia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/secundário , Idoso , Carcinoma de Células Renais/terapia , Diagnóstico Diferencial , Humanos , Neoplasias Renais/terapia , Imageamento por Ressonância Magnética , Masculino , Neoplasias da Medula Espinal/terapia , Vértebras Torácicas , Tomografia Computadorizada por Raios XAssuntos
Descompressão Cirúrgica/métodos , Neuroblastoma/secundário , Neoplasias do Sistema Nervoso Periférico/patologia , Canal Medular/patologia , Neoplasias da Medula Espinal/secundário , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Desenvolvimento Infantil/fisiologia , Feminino , Humanos , Biópsia Guiada por Imagem/métodos , Lactente , Imageamento por Ressonância Magnética/métodos , Masculino , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/terapia , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/terapia , Prognóstico , Medição de Risco , Canal Medular/diagnóstico por imagem , Compressão da Medula Espinal/prevenção & controle , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/terapia , Resultado do TratamentoRESUMO
El neuroblastoma es el tumor maligno sólido extracraneal más común en niños. Sólo el 10 por ciento de los casos se diagnostican después de la primera década de vida. Presentamos una paciente afroamericana de 23 años, con una masa paravertebral en T3-T5, múltiples lesiones en los cuerpos vertebrales y una lesión expansiva en la región parietal derecha. El estudio inmmunohistoquímico (negativo para CD99, CD20, CD3 y desmina y positivo para cromogranina, sinaptofisina y NB84), confirmó el diagnóstico de neuroblastoma. La paciente fue sometida a 12 ciclos de quimioterapia recibiendo VAC (vincristina / doxorubicina/ cyclofosfamida) intercalada con ICE (ifosfamida/ mesna/ etoposido). La doxorubicina fue reemplazada por actinomicina en el séptimo ciclo. La paciente toleró bien la quimioterapia y está clínicamente estable.
Neuroblastoma is the most common extracranial solid malignancy in children but rarely described in adults, being 10 percent of all cases diagnosed after the first decade of life. We report a 23 year-old black woman with a mass at paravertebral region of T3-T5, multiple lesions in vertebral bodies and expanding skull-brain lesion at the right parietal region. Immunohistochemical analysis (negative for CD99, CD20, CD3 and desmin; and positive chromogranin, synaptophysin and NB84) confi rmed the diagnosis of neuroblastoma. The patient was submitted to 12 cycles of chemotherapy receiving VAC (vincristine/doxorubicin/cyclophosphamide) interspersed with ICE (ifosfamide/mesna/etoposide) and doxorubicin was replaced by actinomycin in the 7th cycle. She had good tolerance to this therapy, and has been clinically stable.
Assuntos
Feminino , Humanos , Adulto Jovem , Neoplasias Encefálicas/secundário , Neuroblastoma/patologia , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/secundário , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Neoplasias Ósseas/secundário , Neoplasias Encefálicas/terapia , Carboplatina/administração & dosagem , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Ifosfamida/administração & dosagem , Mesna/administração & dosagem , Neuroblastoma/terapia , Neoplasias da Medula Espinal/terapia , Vértebras Torácicas , Vincristina/administração & dosagemRESUMO
Neuroblastoma is the most common extracranial solid malignancy in children but rarely described in adults, being 10% of all cases diagnosed after the first decade of life. We report a 23 year-old black woman with a mass at paravertebral region of T3-T5, multiple lesions in vertebral bodies and expanding skull-brain lesion at the right parietal region. Immunohistochemical analysis (negative for CD99, CD20, CD3 and desmin; and positive chromogranin, synaptophysin and NB84) confi rmed the diagnosis of neuroblastoma. The patient was submitted to 12 cycles of chemotherapy receiving VAC (vincristine/doxorubicin/cyclophosphamide) interspersed with ICE (ifosfamide/mesna/etoposide) and doxorubicin was replaced by actinomycin in the 7th cycle. She had good tolerance to this therapy, and has been clinically stable.
Assuntos
Neoplasias Encefálicas/secundário , Neuroblastoma/patologia , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/secundário , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Neoplasias Ósseas/secundário , Neoplasias Encefálicas/terapia , Carboplatina/administração & dosagem , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Mesna/administração & dosagem , Neuroblastoma/terapia , Neoplasias da Medula Espinal/terapia , Vértebras Torácicas , Vincristina/administração & dosagem , Adulto JovemRESUMO
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 TratamentoRESUMO
We report a case of intramedullary spinal cord metastasis from thyroid cancer in a 70-year-old woman complaining for three months an intense cervical pain, without motor or sensitive deficits. Six months before the onset of symptoms, the patient underwent total thyroidectomy for a thyroid cancer. Magnetic resonance imaging with gadolinium enhancement showed an intramedullary spinal cord lesion. A partial resection of the medullar tumor was performed and the pathological findings showed an metastatic adenocarcinoma. Post operatively the patient developed a left crural monoplegia.
Assuntos
Adenocarcinoma/secundário , Neoplasias da Medula Espinal/secundário , Neoplasias da Glândula Tireoide/patologia , Adenocarcinoma/cirurgia , Idoso , Vértebras Cervicais , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Glândula Tireoide/cirurgia , TireoidectomiaRESUMO
Relata-se um caso de metástase intramedular de neoplasia da glândula tireóide em uma mulher de 70 anos queixando-se há três meses de dor cervical intensa, sem déficit motor focal nem alterações de sensibilidade. Seis meses antes do início dos sintomas, a paciente foi submetida a tireoidectomia total para ressecção de adenocarcinoma tireoideano. Ressonância magnética com contraste gadolíneo mostrou lesão intramedular. Foi realizada ressecção parcial do tumor medular que revelou ao exame anatomopatológico adenocarcinoma metastático. No pós-operatório a paciente desenvolveu monoplegia crural à esquerda.
Assuntos
Idoso , Feminino , Humanos , Adenocarcinoma/secundário , Neoplasias da Medula Espinal/secundário , Neoplasias da Glândula Tireoide/patologia , Adenocarcinoma/cirurgia , Vértebras Cervicais , Evolução Fatal , Imageamento por Ressonância Magnética , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/cirurgia , Tireoidectomia , Neoplasias da Glândula Tireoide/cirurgiaRESUMO
In order to search for candidate genes related to pituitary adenoma aggressiveness, the present investigation was intended to compare the mRNA expression profile from a pool of four nonfunctional pituitary adenomas (NFPA) with a spinal cord metastasis of a nonfunctional pituitary carcinoma (MNFPC). The metallothionein isoform 3 (MT3) gene was differentially expressed in nonfunctional adenomas in comparison to the metastasis of nonfunctional carcinoma. A microarray dataset comprising 19,881 probes was employed for comparing expression profiles of a spinal cord metastasis of a nonfunctional pituitary carcinoma with a pool of four nonfunctional pituitary adenomas. RT-qPCR confirmed the microarray findings and was used to investigate MT3 mRNA gene expression in tumor samples of a series of 52 different pituitary adenoma subtypes comprising 10 corticotropin (ACTH)-producing, 18 growth hormone (GH)-producing, 8 prolactin (PRL)-producing, and 16 nonfunctional adenomas. Microarray data analysis by GeneSifter program unveiled Gene Ontology terms related to zinc ion-binding activity closely related to MT3 function. MT3 mRNA expression was statistically significantly higher in ACTH-producing pituitary adenomas and in nonfunctional pituitary adenomas in comparison to the other pituitary adenoma subtypes. The more abundant expression of this gene in ACTH-producing pituitary adenomas suggests that MT3 could be related to distinct pituitary cell lineage regulating the activity of some transcription factor of importance in hormone production and/or secretion.
Assuntos
Adenoma/metabolismo , Hormônio Adrenocorticotrópico/metabolismo , Proteínas do Tecido Nervoso/biossíntese , Neoplasias Hipofisárias/metabolismo , Adenoma/patologia , Expressão Gênica , Hormônio do Crescimento Humano/metabolismo , Humanos , Metalotioneína 3 , Proteínas do Tecido Nervoso/genética , Análise de Sequência com Séries de Oligonucleotídeos , Neoplasias Hipofisárias/secundário , Prolactina/metabolismo , Isoformas de Proteínas/biossíntese , RNA Mensageiro/análise , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Neoplasias da Medula Espinal/secundárioRESUMO
We report the case of an eleven years-old girl with a right temporo-parietal glioblastoma multiforme. The tumor was totally resected on neurosurgery, and cranial radiotherapy was applied at next. The tumor recurred three months later, far from primary site, in the caudal portion of the spinal canal. Death occurred in less than one month later. Taking into account the data of this case, we discuss our experience in the management of such tumors and the literature on the subject.
Assuntos
Neoplasias Encefálicas/patologia , Glioblastoma/secundário , Neoplasias da Medula Espinal/secundário , Neoplasias Encefálicas/cirurgia , Criança , Evolução Fatal , Feminino , Glioblastoma/cirurgia , Humanos , Neoplasias Supratentoriais/patologia , Neoplasias Supratentoriais/cirurgiaRESUMO
Spinal cord metastases are an uncommon secondary location of a malignant neoplasm. They are rarely diagnosed during life and when that is the case, it is in the clinical setting of a disseminated cancer and very seldom as the first clinical manifestation. We report two patients, with no previous disease, who developed a progressive myelopathy. An intramedullary spinal cord tumor was diagnosed, based on the clinical picture and imaging studies. They were operated and biopsies showed spinal cord metastases whose primary tumor was a lung neoplasm. We discuss the clinical features in these patients, the diagnosis of progressive myelopathy in cancer patients, treatment and prognosis of this unusual secondary cancer location.
Assuntos
Adenocarcinoma de Células Claras/secundário , Neoplasias Pulmonares/patologia , Neoplasias da Medula Espinal/secundário , Adenocarcinoma de Células Claras/diagnóstico por imagem , Adulto , Evolução Fatal , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Radiografia , Neoplasias da Medula Espinal/diagnóstico por imagemRESUMO
OBJECTIVES: To assess the clinical response to luteinizing hormone-releasing hormone (LH-RH) antagonist cetrorelix (SB-75) in patients with advanced carcinoma of the prostate and paraplegia due to metastatic invasion of spinal cord. METHODS: Cetrorelix was given at two different dose regimens to 5 patients with prostatic cancer Stage D2 and paraplegia. Urologic and neurologic examinations, laboratory studies, radiography (myelography), and prostate ultrasonography were carried out. Prostate-specific antigen (PSA) and free testosterone were also measured. RESULTS: In all patients, the neurologic symptoms regressed. The recovery of the thermic and vibratory sensation and motility of the toes was observed. The neurologic improvement continued during the treatment and at 3 months all the patients were able to walk with the aid of a cane. In 1 patient, the myelography showed that the spinal cord compression had disappeared and prostate volume assessed by ultrasonography showed a significant decrease. The bladder function greatly improved in all 5 patients during the treatment with cetrorelix. Baseline levels of luteinizing hormone fell from 9.28 to 1.0 IU/L and those of follicle-stimulating hormone (FSH) fell from 18.28 to 12 IU/L (P < 0.05) after the first day of therapy with cetrorelix. Mean levels of free testosterone were reduced from 52.4 to 14.7 pmol/L (P < 0.005) at 12 hours and to 13.1 pmol/L (P < 0.005) 3 days after the first injection of cetrorelix. A persistent inhibition of gonadotropins and testosterone was maintained during the subsequent 3 months of therapy. The high levels of PSA gradually decreased. CONCLUSIONS: Our results show that LH-RH antagonist cetrorelix causes an immediate lowering of the serum testosterone levels in patients with prostate cancer and metastases in the spinal cord, in whom the LH-RH agonists cannot be used as single drugs because of the possibility of flare-up and appears to be appropriate for long-term therapy.
Assuntos
Hormônio Liberador de Gonadotropina/análogos & derivados , Hormônio Liberador de Gonadotropina/antagonistas & inibidores , Paraplegia/etiologia , Neoplasias da Próstata/tratamento farmacológico , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/tratamento farmacológico , Neoplasias da Medula Espinal/secundário , Idoso , Hormônio Liberador de Gonadotropina/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Antígeno Prostático Específico/sangue , Neoplasias da Próstata/sangue , Neoplasias da Próstata/patologia , Neoplasias da Medula Espinal/complicações , Testosterona/sangueRESUMO
Los síndromes agudos de compresión medular en la gran mayoría de los casos (88 por ciento) se deben a tumores espinales, de los cuales dos tercios corresponden a metástasis. El 76 por ciento de los casos presentan un período prodrómico previo a la aparición de déficit neurológicos, caracterizados por dolores, mientras que sólo pocos casos inician su sintomatología con trastornos de la función motora o sensitiva. Aparte de las radiografías simples de columna y la mielografía, la tomografía computarizada y la resonancia magnética ocupan hoy en día una posición destacada en el diagnóstico de esta enfermedad. Independientemente del hecho si se trata de un tumor primario o una metástasis los resultados operatorios son más favorables en aquellos casos que presentan los menores déficit preoperatorios, lo cual subraya la importancia de una interpretación cuidadosa de los síntomas iniciales
Assuntos
Humanos , Masculino , Feminino , Compressão da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/secundário , Compressão da Medula Espinal/cirurgia , Compressão da Medula Espinal/fisiopatologia , Laminectomia , Dor Lombar , Anamnese , Metástase Neoplásica/diagnóstico , Desempenho Psicomotor , Tomografia Computadorizada de Emissão , Resultado do TratamentoRESUMO
We evaluated 17 children with primary intracranial neoplasms for subarachnoid metastatic disease (SAMD) using myelography with computed tomographic follow-up (Myelo + CT) and cerebrospinal fluid (CSF) histopathologic examination, as well as magnetic resonance imaging with gadolinium DTPA (MRI + Gd), between December 1988 and December 1989. There were 12 boys, and the median age was 5.7 years (range, 0.8 to 21.8 years). Tumor histology included 8 primitive neuroectodermal tumors (PNETs), 3 ependymomas, 2 low-grade astrocytomas, 1 anaplastic astrocytoma, 1 glioblastoma multiforme, 1 atypical rhabdoid tumor, and 1 malignant fibrous histiocytoma. Thirteen tumors originated in the posterior fossa, 2 were supratentorial, and 2 were in the spinal cord. The median interval between the 2 diagnostic tests was 2 days. MRI + Gd was positive in 11 (65%), Myelo + CT in 8 (47%), and CSF in 5 (29%) cases. MRI + Gd was superior in delineating spinal cord nodules and "sugar coating" whereas Myelo + CT more readily revealed nerve root sleeve filling defects. There was no case in which Myelo + CT was positive that MRI + Gd did not reveal SAMD. MRI + Gd is a safe, noninvasive test that should be used as the initial imaging modality for the presence of SAMD.
Assuntos
Gadolínio , Imageamento por Ressonância Magnética , Mielografia , Neoplasias da Medula Espinal/diagnóstico , Espaço Subaracnóideo , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/secundárioRESUMO
Between 1980 and 1984, of 107 patients receiving 16 mg/d of dexamethasone for spinal cord compression, three (2.8%) developed gastrointestinal (GI) perforation and two (1.9%) GI bleeding; of 226 being tapered from 100 mg/d of dexamethasone, perforation occurred in six (2.7%) and GI bleeding in eight (3.5%). Of 125 patients with GI perforations treated between 1979 and 1986, 41 (33%) were on steroids, 24 for neurologic disease. Median duration of steroid therapy was 24 days; 20 (91%) of the neurologic patients perforated within 30 days. The steroid group had more free peritoneal involvement (p less than 0.00001), but fewer signs and symptoms of peritonitis (p less than 0.000001) than the nonsteroid group. Seventeen patients were receiving steroids for cord compression; they had significantly more rectosigmoid perforations (p less than 0.014) and associated constipation (p less than 0.000001) than the 108 remaining patients. GI perforation is a less well-recognized complication of steroid therapy in neurologic patients than is GI bleeding though it occurs as frequently, is more difficult to diagnose, and far more serious. In steroid-treated patients, prevention of constipation might avert this serious complication, while early diagnosis will improve the outcome.
Assuntos
Dexametasona/efeitos adversos , Gastroenteropatias/induzido quimicamente , Perfuração Intestinal/induzido quimicamente , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Hemorragia Gastrointestinal/induzido quimicamente , Humanos , Perfuração Intestinal/patologia , Masculino , Pessoa de Meia-Idade , Peritonite/etiologia , Fatores de Risco , Compressão da Medula Espinal/tratamento farmacológico , Compressão da Medula Espinal/etiologia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/secundárioRESUMO
Se presenta un caso clínico de frecuencia poco común. Se trata de un paciente que presenta neoplasia vesical de alto grado de diferenciación, operado y en el que posteriormente se descubren metástasis a nivel de la médula espinal y el cráneo. El paciente evoluciona muy favorablemente sin tratamiento citostático o radioterápico, no presenta recidiva tumoral y se observa disminución de las células metastásicas de la médula espinal. Los resultados son evaluados a los 9 años y 6 meses de operado. Realizamos un breve recordatorio anatomopatológico de la neoplasia vesical
Assuntos
Pessoa de Meia-Idade , Humanos , Masculino , Neoplasias Cranianas/secundário , Neoplasias da Medula Espinal/secundário , Neoplasias da Bexiga UrináriaRESUMO
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.