소아에서 발생한 악골의 다발성 각화낭성 치성종양의 치험례
TREATMENT OF MULTIPLE JAW KERATOCYSTIC ODONTOGENIC TUMOR IN CHILDREN'S JAW BONE : A CASE REPORT
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초록
악골에서 다수로 발생하는 낭은, 단독으로 발생하는 낭에 비해 매우 드물게 나타난다. 증후군을 동반하지는 않고, 악골 내 에 다수의 낭이 발생한 경우를 다발성 악골낭 이라고 하는데, 소아에서 발생하는 다발성 악골 낭의 대부분은 각화낭성 치성종 양(Keratocystic odontogenic tumor, KCOT)이다. 소아에서 나타나는 악골의 다발성 각화낭성 치성종양은, 기저세포모반증후군의 한 증상으로 발생할 수 있으므로, 악골의 방사선 사진에서 다발성 낭이 발견되면, 증후군을 의심해보고 임상 및 병리 검사를 하게 된다. 본 증례는, 다발성 악골 낭을 주소로 내원한 소아환자들로, 기저세포모반증후군을 의심하였으나 현재까지 증후군의 다른 증상들은 보이고 있지 않으며, 단지 수년간에 걸쳐서 종양의 재발과 수술을 반복하고 있는 경우이다. 악골의 다발성 낭 이외 의 기저세포모반증후군의 증상들이 나타나지 않더라도, 나이가 들면 증후군의 다른 증상들이 나타난 경우의 보고도 있으므 로, 소아에서 이러한 다발성 치성종양이 관찰되면, 지속적으로 방사선 및 임상 검사를 시행하여 증후군으로의 진행 여부를 관 찰하는 것이 중요한 것으로 사료된다.
Abstract
Occurrence of multiple cysts in jaw bone is rare compared to solitary cysts. numerous cysts occurring in jaw bone which not accompany any syndromes are defined as multiple jaw cysts, and most of these cases in children are keratocystic odontogenic tumor (KCOT) Multiple KCOT occurring in children are often associated with basal cell nevus syndrome(BCNS), so if multiple cysts are found on the radiograph, we suspect this syndrome and pursue clinical and pathological tests. In this case, a pediatric patient, reporting with multiple cysts in the jaw was suspected of BCNS, but hasn't shown any other symptoms of this syndrome up to date, and has kept repeating surgical operation and recurrence of the tumor. Although no symptoms besides multiple jaw cysts is present, it is often reported that other symptoms appear late in the patient’s age. Therefore, in cases where multiple odontogenic tumors are found in children, continuous radiographic and clinical follow-ups in order to check the progress of the syndrome is considered important.
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