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선천성 낭선종성 기형이 의심되었던 환아에서 진단된 흉막폐모세포종 1례
우성일 ( Sung Il Woo ),김효빈 ( Hyo Bin Kim ),송준섭 ( Joon Sup Song ),구현령 ( Hyun Lyoung Koo ),임호준 ( Ho Jun Lim ),홍수종 ( Soo Jong Hong ) 대한소아알레르기호흡기학회 2006 소아알레르기 및 호흡기학회지 Vol.16 No.2
Congenital cystic adenomatoid malformation(CCAM) is one of the most common congenital lung lesions. Clinical manifestations that show are neonatal respiratory distress, recurrent respiratory infection, pneumothorax, and hemothorax. But, there are patients who are asymptomatic until mid-childhood. The treatment of asymptomatic CCAM is controversial. There is a possibility to resolve it spontaneously, but late complications such as recurrent pulmonary infection, pneumothorax, hemothorax, and cancer, which includes bronchoalveolar carcinoma and rhabdomyocarcinoma, pleuropulmonary blastoma still remain. Some investigators advocate routine surgery for all cases of CCAM that are apparent at birth. A previously healthy 16-months-old girl who had suffered from a cough for 2 weeks was transferred to Asan Medical Center with CCAM. Due to a chest CT and fever, we first thought that she had CCAM with infection. After we treated her with antibiotics for one week, we performed surgery to confirm the diagnosis and to prevent further complication. But by surgical wedge resection, a pleuropulmonary blastoma was found. There were no evidence of metastasis and adjacent involvement. She started her chemotherapy with vincristine, actinomycin D and cyclophosphamide, and is now continuing maintenance chemotherapy with etoposide, vincristine, and Ifosfamide. We report pleuropulmonary blastoma that presented as CCAM. So we recommend surgical resection in asymptomatic CCAM to confirm the diagnosis and to prevent its malignant transformation, even not accompanied by symptoms. [Pediatr Allergy Respir Dis(Korea) 2006;16:177-182]
두개저와 상부 경추를 침범한 고어햄씨병의 보존적 치료 및 방사선 치료에 대한 경험 -증례보고-
장인석 ( In Seok Jang ),박준범 ( Jun Bum Park ),나영신 ( Young Shin Ra ),김정훈 ( Jeong Hoon Kim ),구현령 ( Hyun Lyoung Koo ),강신광 ( Shin Kwang Khang ) 대한뇌종양학회 2007 대한뇌종양학회지 Vol.6 No.1
A 10-year-old boy was admitted at our institute with neck pain, limiting the neck movement and chin point deviation to the right. Which confirmed the suspected diagnosis of Gorham`s disease, the patient underwent biopsy on the 3rd cervical spine. Gorham`s disease was confidently diagnosed with the support of present clinical and radiographic findgings by a regional histopathologist. In order to diminish neck pain and ensure radiation field uniformity, a halo-vest was recommended. The patient received 4,000 cGy of radiation to the upper cervical osteolytic lesion. There has been no radiographic evidence of progression of the disease at the MR follow-up of post-radiotherapy 23 months. At present, the patient is remarkably doing well with no evidence of disease progression. The authors suggest that application of a halo-vest and early radiation therapy can be a good choice to management of Gorham`s disease involving skull base and upper cervical spine. However, long term follow-up will be essential to confirm the remission of disease.