Neurocutaneous melanosisに脊髄腫瘍を合併した1例

Neurocutaneous Melanosis Associated with Spinal Cord Tumor. Case Report

　今回われわれは，neurocutaneous melanosisに脊髄腫瘍を合併した極めて稀な1例を経験した．症例は4歳，男児．出生時より全身に黒色母斑ならびに頻回の痙攣発作を認め，本症と診断された．歩行障害と左手巧緻運動障害が増悪したため当科を紹介され受診した．MRI，脊髄造影にて頚髄から胸髄にわたる巨大な腫瘍を認め，手術を施行，腫瘍を全摘し得た．組織所見はchondrolipomatous hamartomaであった．術後6年の現在経過良好であるが，再発による死亡例の報告もあり，今後も厳重な経過観察が必要である．

　We present a case of neurocutaneous melanosis (NCM) associated with a spinal cord tumor in a 4-year-old boy who presented progressive gait disturbance and left hand clumsiness. Myelography and magnetic resonance imaging revealed a huge spinal cord tumor extending from the mid-cervical to upper thoracic levels. Although the tumor adhered to the dura matar, total removal of the tumor was achieved. Pathological examinations established a diagnosis of chondrolipomatous hamartoma, which contained cartilage and fat tissue. His neurology improved and is maintained at 6 years postoperatively. NCM is a rare phacomatosis characterized by the presence of large melanocytic nevi in combination with excessive numbers of malanocytes in the leptomeninges. Prognosis of juvenile NCM associated with spinal cord tumor has been extremely poor and most children died within several years after birth. Careful observation, therefore, is necessary for this case even if total resection of the tumor was attained.