Tumor dermóide intramedular associado a diastematomielia: relato de caso

Abstract

RESUMO -A associação de tumor dermóide intramedular e diastematomielia é rara, descrita anteriormente em 8 casos. Descrevemos novo caso, de um adulto portador desta associação que apresentou paraparesia crural progressiva e síndrome sensitiva nos membros inferiores, além de cifoescoliose tóraco-lombar. A exploração cirúrgica confirmou a ocorrência de um tumor dermóide intramedular além da diastematomielia identificados na ressonância magnética. Discutemos aspectos fisiopatológicos e clínicos e as características radiológicas destas patologias destacando a importância do diagnóstico precoce.PALAVRAS-CHAVE: diastematomielia, disrafismo espinhal, tumor espinhal. Intraspinal dermoid tumor associated with diastematomyelia: case reportABSTRACT -Intraspinal dermoid tumor and diastematomyelia is a rare combination of pathologies and only eight cases were priorly published. We report a new case in an adult with clinical presentation of progressive paresis and sensitive deficit in the lower limbs, associated with a thoraco-lombar kyphoscoliosis. The diagnosis based on the CT scan and MR images was confirmed at surgery. Pathophysiologic and clinical aspects as well as the radiological characteristics of these pathologies are dicussed and the importance of the early diagnosis is emphasized.KEY WORDS: diastematomyelia, spinal dysraphism, spinal tumor.Os tumores dermóides constituem de 1 a 2% de todos os tumores intrarraquidianos em todas as idades, apresentando localização intramedular em cerca de metade dos casos 1 . A associação de tumor dermóide com diastematomielia é rara, descrita anteriormente em 8 casos [1][2][3][4] . Neste relato apresentamos o caso de um paciente de 32 anos que apresentava déficit motor progressivo nos membros inferiores associado a deformidade da coluna tóraco-lombar. A investigação por imagem evidenciou processo expansivo intradural associado a alterações relacionadas a disrafismo espinhal incluindo diastematomielia. CASOJCA, masculino, 32 anos, natural de Cunha -SP, apresentou-se com queixa de 2 anos de déficit motor progressivo nos membros inferiores, associado a dor em queimação acometendo território de raízes L4 e L5 e alteração progressiva da curvatura da coluna lombar, notada desde o nascimento. No exame físico a estatura do paciente era 1, 44 m e, ao exame da região dorsal, notava-se lesão cicatricial radiada paravertebral esquerda com cerca de 8 cm de diâmetro e lesão puntiforme na linha mediana através da qual o paciente referia drenagem de secreção, não caracterizada, de forma intermitente, ao longo dos anos. O exame mostrava ainda anestesia tátil-térmica-dolorosa na face anterior da perna esquerda associada a hiperestesia na face anterior da coxa ipsilateral.

Dorsal intramedullary giant dermoid tumor

“…In addition, in the analysis by Arseni et al, which included 12 cases between 1935 and 1976, no difference of gender was observed, 59% were associated with congenital malformations [12,13]. The predilection for the lumbosacral region is explained by the later closure of the caudal neuropore, being the last to close in the formation of the neural tube [14].Dermoid tumors are due to an incomplete separation between the ectoderm and the neuroectoderm, causing the presence of ectodermal inclusions between these two layers, which may be congenital or acquired [5,14]. This error usually occurs between the third and fifth weeks of embryonic development [4], and subsequent differentiation of this fragment would originate the dermoid tumor [5].…”

“…The predilection for the lumbosacral region is explained by the later closure of the caudal neuropore, being the last to close in the formation of the neural tube [14].Dermoid tumors are due to an incomplete separation between the ectoderm and the neuroectoderm, causing the presence of ectodermal inclusions between these two layers, which may be congenital or acquired [5,14]. This error usually occurs between the third and fifth weeks of embryonic development [4], and subsequent differentiation of this fragment would originate the dermoid tumor [5]. The insertion of cutaneous residues during surgical procedures in patients with dysraphism has also been suggested as a possible explanation for the appearance of this type of pathology [5,6].…”

“…This error usually occurs between the third and fifth weeks of embryonic development [4], and subsequent differentiation of this fragment would originate the dermoid tumor [5]. The insertion of cutaneous residues during surgical procedures in patients with dysraphism has also been suggested as a possible explanation for the appearance of this type of pathology [5,6]. It has a thin wall with stratified squamous epithelium, having a content composed of viscous fluid material, keratin, epithelial remains and some epidermal appendages, such as sebaceous and sweat glands, and may also have teeth [2,7].…”