RESUMEN
Intramedullary dermoids arising within the conus medullaris are rare. We report a rare association of an intramedullary dermoid cyst located in a low lying conus tethered by a thickened, fatty filum terminale and discuss the embryological implications of this association.
Asunto(s)
Cauda Equina/embriología , Quiste Dermoide/embriología , Defectos del Tubo Neural/embriología , Neoplasias de la Médula Espinal/embriología , Adulto , Cauda Equina/patología , Cauda Equina/cirugía , Quiste Dermoide/diagnóstico , Quiste Dermoide/patología , Quiste Dermoide/cirugía , Diagnóstico Diferencial , Humanos , Laminectomía , Vértebras Lumbares/embriología , Vértebras Lumbares/patología , Vértebras Lumbares/cirugía , Imagen por Resonancia Magnética , Masculino , Microcirugia , Defectos del Tubo Neural/diagnóstico , Defectos del Tubo Neural/patología , Defectos del Tubo Neural/cirugía , Sacro/embriología , Sacro/patología , Sacro/cirugía , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/patología , Neoplasias de la Médula Espinal/cirugíaRESUMEN
Children born with a tail-like appendage have a rare malformation that is frequently associated with abnormalities of the spine and spinal cord. A contiguous fibrolipoma is usually seen extending from the subcutaneous portion of the tail into the inferior spinal cord, resulting in tethered cord syndrome. We present the case of a child born with a tail and intraspinal lipoma that were not contiguous with each other, and were separated by an intact layer of lumbosacral fascia. The tail and lipoma were removed and the spinal cord untethered, and the child is neurologically normal 2 years after surgery. The absence of a contiguous lipoma from the tail to the spinal cord suggests that this condition may be principally caused by a disorder of secondary neurulation and/or regression of the normal embryonic tail bud. The embryology of the lower spine is reviewed and possible etiologies discussed.
Asunto(s)
Lipoma/patología , Lipoma/cirugía , Defectos del Tubo Neural/patología , Defectos del Tubo Neural/cirugía , Neoplasias de la Médula Espinal/patología , Neoplasias de la Médula Espinal/cirugía , Femenino , Humanos , Recién Nacido , Lipoma/embriología , Vértebras Lumbares , Defectos del Tubo Neural/embriología , Sacro , Neoplasias de la Médula Espinal/embriologíaRESUMEN
Exstrophic bladder and intramedullary teratomas are rare congenital anomalies. To the authors' knowledge, the coexistence of these 2 anomalies has not been reported previously. The authors report on a newborn with thoracal intramedullary teratoma and exstrophic bladder. The possible embryogenetic background also is discussed.
Asunto(s)
Extrofia de la Vejiga/complicaciones , Neoplasias de la Médula Espinal/congénito , Teratoma/congénito , Anomalías Múltiples , Extrofia de la Vejiga/embriología , Calcinosis/congénito , Consanguinidad , Epispadias , Humanos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Recién Nacido , Masculino , Neoplasias de la Médula Espinal/complicaciones , Neoplasias de la Médula Espinal/embriología , Teratoma/complicaciones , Teratoma/embriología , Vértebras Torácicas/patología , Derivación VentriculoperitonealRESUMEN
The early development of the neural tube has been well studied in animals and humans. After axial determinants have been accomplished the processes of primary and secondary neurulation take place. Successful completion results in a spinal cord that has arisen from primary neurulation and a lower sacro-coccygeal portion from secondary neurulation. The latter region is the site of numerous skin-covered clinical lesions, which include tumors and malformations. A listing of selected features in 764 cases of skin-covered sacrococcygeal lesions is presented. The manner in which these lesions arise and the potential for genetic factors being responsible is discussed.
Asunto(s)
Defectos del Tubo Neural/diagnóstico , Neoplasias de la Médula Espinal/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Región Lumbosacra/anomalías , Región Lumbosacra/embriología , Región Lumbosacra/patología , Masculino , Defectos del Tubo Neural/embriología , Defectos del Tubo Neural/genética , Evaluación de Resultado en la Atención de Salud , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Médula Espinal/anomalías , Médula Espinal/embriología , Médula Espinal/patología , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/genética , Factores de TiempoRESUMEN
Experience of treatment of 36 patients with extramedullary spinal cord tumors is summarized. Pigmented macules, papillomas or the spinous processes painfulness while irritation were determined on skin in accordance to tumor localization in the innervation zone of one or two segments.
Asunto(s)
Melanoma/etiología , Recurrencia Local de Neoplasia/etiología , Neurilemoma/etiología , Neoplasias de la Médula Espinal/etiología , Adulto , Anciano , Terapia Combinada , Femenino , Humanos , Melanoma/embriología , Melanoma/cirugía , Recurrencia Local de Neoplasia/embriología , Recurrencia Local de Neoplasia/cirugía , Neurilemoma/embriología , Neurilemoma/cirugía , Cuidados Posoperatorios , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/cirugíaRESUMEN
Spinal lipomas account for 5% of the tumors of the spinal cord, frequently present already at birth. Most commonly they are associated with forms of dysraphism, but lipomas without bony involvement are considered dysembriogenetic lesions too. Children with lipoma frequently have intact neurological functions, but may become symptomatic later on. Diagnosis is possible also in neurologically intact patients because of skin lesions or subcutaneous masses. Many surgeons suggest early surgery to prevent injury to neural structures from traction due to cord tethering; others prefer to wait for the rise of any symptom before considering surgery. However, neurological recovery after surgery is rarely observed, and, when present, is always partial; the primary goal of surgery is to stop the clinical progression through the detethering of the cord.
Asunto(s)
Lipoma , Neoplasias de la Médula Espinal , Niño , Preescolar , Humanos , Lipoma/diagnóstico , Lipoma/embriología , Lipoma/patología , Lipoma/terapia , Defectos del Tubo Neural/complicaciones , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/patología , Neoplasias de la Médula Espinal/terapiaRESUMEN
The germinoma represents a less malignant form of germ cell tumor. Depending on the individual's age, this neoplasm constitutes approximately 0.1% to 3.4% of all intracranial tumors. The embryologic origin remains a mystery; however, current theories implicate an aberration in primordial germ cell migration. Clinical presentation depends on tumor location and may involve endocrine, hypothalamic, visual, and cognitive dysfunction. In evaluating midline intracerebral masses, it is imperative that one be aware of the various radiologic appearances, endocrinologic changes, and chemical markers that help to distinguish germinomas from other neoplasms that appear in the pineal, suprasellar, and periventricular regions. Only through the careful evaluation of all available studies can the physician institute appropriate therapies such as biopsy, radiation, and chemotherapy. This article focuses on the epidemiology, embryology, clinical presentation, means of diagnosis, treatment, and outcome of this rare neoplasm.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Disgerminoma/diagnóstico , Neoplasias Encefálicas/embriología , Neoplasias Encefálicas/terapia , Disgerminoma/embriología , Disgerminoma/terapia , Humanos , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/terapiaRESUMEN
Leptomyelolipoma (lumbosacral lipoma) is a common form of spinal dysraphism. The deficits produced include sensory, motor, bowel, and bladder dysfunction, and vary in incidence between the pediatric and adult populations. Twenty patients treated surgically at the Mount Sinai Hospital between 1972 and 1988 are reviewed. Fifty percent were 12 years of age or less and 50% were older than 18 years of age. The surgical approach was designed to accomplish untethering of the conus medullaris, debulking of the lipomatous mass compressing the cord, reconstruction of the dural canal, and reapproximation of the paraspinal muscles and lumbosacral fascia to prevent future trauma. Postoperatively, no patient experienced deterioration of neurological function. Of the symptomatic patients. 67% displayed dramatic improvement or became asymptomatic and 33% experienced stabilization of their deficits. The symptoms most resistant to surgical correction were orthopedic foot deformities and bowel dysfunction, whereas bladder dysfunction, motor weakness, and radiculopathies were most amenable to surgical therapy. Early surgical repair is recommended in these cases to forestall irreversible neurological damage.
Asunto(s)
Lipoma , Región Lumbosacra , Neoplasias de la Médula Espinal , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Lipoma/clasificación , Lipoma/diagnóstico , Lipoma/embriología , Lipoma/fisiopatología , Lipoma/cirugía , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Reflejo Anormal , Neoplasias de la Médula Espinal/clasificación , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/fisiopatología , Neoplasias de la Médula Espinal/cirugía , Tomografía Computarizada por Rayos XAsunto(s)
Neoplasias Encefálicas , Neurofibromatosis 1 , Neoplasias del Sistema Nervioso Periférico , Neoplasias de la Médula Espinal , Adolescente , Adulto , Factores de Edad , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/embriología , Neoplasias Encefálicas/genética , Niño , Preescolar , Femenino , Asesoramiento Genético , Humanos , Lactante , Masculino , Persona de Mediana Edad , Neurofibromatosis 1/complicaciones , Neurofibromatosis 1/embriología , Neurofibromatosis 1/genética , Neoplasias del Sistema Nervioso Periférico/complicaciones , Neoplasias del Sistema Nervioso Periférico/embriología , Neoplasias del Sistema Nervioso Periférico/genética , Trastornos de la Pigmentación/etiología , Grupos Raciales , Factores Sexuales , Neoplasias de la Médula Espinal/complicaciones , Neoplasias de la Médula Espinal/embriología , Neoplasias de la Médula Espinal/genéticaRESUMEN
A case of primary rhabdomyosarcoma in the brain stem is described in an 8 year old girl. The clinical data showed a right side hemiplegia then a total paralysis of the left sixth cranial nerve and a paraplegia which became lethal in 3 months. The necrospy revealed a tumoral nodule in the left medulla oblongata and pons with diffuse subarachnoidal extension from the cranial nerves to the cauda equine roots. Histologically the tumor appeared to be polymorph with numerous rhabdomyoblasts which had a clear cross striation and which were sometimes less differentiated without any neuronal or glial elements. Perivascular tumoral cells and blood vessels were closely linked, the Virchow-Robin spaces were clearly involved. The electron microscopic study confirmed that the less differentiated cells were of a rhabdomyoblastic nature. A review of the litterature indicates that these malignant neoplasias are highly exceptional, and can be classified within the group of primary tumors of the neuraxis with muscular elements. The histogenetic origin of these tumors appears to be the ectomesenchyme of neural crests.
Asunto(s)
Neoplasias Encefálicas/patología , Tronco Encefálico/patología , Rabdomiosarcoma/patología , Neoplasias de la Médula Espinal/patología , Neoplasias Encefálicas/embriología , Diferenciación Celular , Niño , Femenino , Ganglios/embriología , Humanos , Bulbo Raquídeo/patología , Meninges/patología , Puente/patología , Rabdomiosarcoma/embriología , Neoplasias de la Médula Espinal/embriologíaRESUMEN
A study of 2072 children who developed cerebral or spinal cord tumours of varying degrees of malignancy before 15 years of age has shown that there is equally good representation of fatal and non-fatal cases in official registrations. Attack rates are higher for boys than girls and the prognosis is better for girls than boys. The risk of an early death is negatively correlated with age at diagnosis, and the risk of a late death shows the opposite relationship. These observations and a relatively high incidence of hindbrain tumours are suggestive of an embryonic origin for most of the cases.