• Tuberculosis and Respiratory Diseases
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• 제67권4호
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• pp.345-350
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• 2009

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

• 대한골관절종양학회지
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• 제2권1호
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• pp.38-46
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• 1996

The traditional methodology in Ewing's sarcoma was chemotherapy and radiotherapy. Recently surgery is reemerging as an important therapeutic tool and some paper report increased survival with it. The purpose of this nonrandomized study is to evaluate our result of Ewing's sarcoma with surgery, retrospectively. We experienced 30 cases for seven years and among them 6 were extraskeletal. In location, axial was 10 cases and peripheral was 20. By Enneking's classification, state IIB was 26 cases and IIIB was 4. Eighteen patients took operation, chemotherapy and/or radiotherapy, and remaining twelve took chemotherapy and/or radiotherapy only. Type of operation was limb salvage in 16 cases and amputation 2. Average dosage of radiation was 45.1Gy. Six kinds of chemotherapeutic regimen were used, but among them main protocols were Ifosfamide-Adriamycin(17 cases) and IESS(Cytoxan, Adriamycin, Methotrexate, Vincristine:8 cases). Complications were as follows. In operation group, there were 3 local recurrence and one case of nonunion. In nonoperated group, one local recurrence and one pancytopenia resulting in death. Average follow up was 29.7 months. Kaplan-Meier's ten year actuarial survival rate for the whole 30 cases was 26.8%. Significant difference in survival exists between central and peripheral lesions(p=0.05, by log rank test). Types of chemotherapy and surgery itself showed no significance. But surgery is important in function and local control. More intensive chemotherapeutic regimen to prevent distant metastasis and combined surgery and radiotherapy may be needed in Ewing's sarcoma.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제18권4호
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• pp.734-740
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• 1996

본과에 내원하여 LCH로 진단된 13증례를 고찰해 보면, 10세 미만의 남아에서 호발되었으며, 주증상은 부종 및 동통이었고, 임상적으로 악성종양과의 감별이 중요하며, 국소적 소파 및 약물 치료로 대체로 좋은 예후를 보였다. 그러나, 이는 악골에서만의 경우를 보았을 때이며, LCH는 기본적으로 망상내피계가 존재하는 신체내 어느 곳에서나 생길 수 있으며, 실제 본과의 증례에서도 폐에서 LCH의 소견이 확인된 증례가 있었으며, 다른 증례의 환자에서도, 백내장, 결핵, 중이염 등의 병력이 있었던 바 이것이 LCH의 또다른 병소이였을 가능성을 배재할 수 없다. LCH는 비교적 좋은 예후를 기대할 수는 있으나, 좀더 장기간의 추적조사 및 전신적 검토, 관련 타과와의 협조가 필요하다.

• Archives of Plastic Surgery
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• 제33권3호
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• pp.371-375
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• 2006

Frontometaphyseal dysplasia is a rare genetic syndrome first described by Gorlin and Cohen in 1969. This disease affects the skeletal system and connective tissue, and could be characterized by hyperostosis of the skull, prominence of supraorbital ridges, additional skeletal and extraskeletal abnormalities. It is believed that the condition is an X-linked dominant trait with severe manifextations in males and extreme variability in females.We described a case of 15-year-old boy manifested a pronounced supraorbital ridge associated with exorbitism. He also had bilateral progressive hearing deficit, thoracic spine scoliosis, chest wall deformity, bilateral maxillary sinusitis and both 5th finger arachnodactyly.The patient underwent a fronto-temporo-orbital cranioplasty through a coronal incision. The frontal bone including supraorbital region, orbital lateral rim and temporal bone were extensively contoured with burr. And then, burring of the medial aspect of lateral orbital wall was made to increase both orbital volume for correction of exorbitism. Postoperative results show well corrected prominent supraorbital ridge, hyperostosis of frontotemporal bone and exorbitism. The patient was satisfied with the improved appearance. No recurrence occurred during the 6 months of follow-up period. We report this case as it shows esthetically good result without any complication.

• 대한골관절종양학회지
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• 제5권4호
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• pp.201-207
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• 1999

Malignant tumors of the hand are very rare. Between 1989 and 1998, 17 patients with malignant tumors of the hand were evaluated for clinical features, prevalence, treatment and results. The mean age was 45 years and median duration of follow-up was 38 months. A painless mass was the most common symptom and the most common lesion was the digit. Fifteen cases of soft tissue and 2 cases of bone tumor were diagnosed. On pathology, soft tissue tumors were comprised of 8 cases of malignant melanoma, 2 cases of angiosarcoma and 1 case each of malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, extraskeletal chondrosarcoma, and squamous cell carcinoma. Bone tumors were metastatic tumors and each originated from the rectal cancer or lung cancer. Lymph node involvements were noted in 4 cases of malignant melanoma and 1 case of squamous cell carcinoma. Six cases of metacarpo-phalangeal joint disarticulation and 4 cases of phalanx amputation were performed. Wide excision, ray amputation and below-elbow amputation were also performed. Three cases expired due to metastasis and progression of the original lesion. Among the surviving 14 cases, a malignant melanoma had metastasis on the axillary lymph node and 13 cases showed no local recurrence or metastasis during the follow-up.

• 대한두개안면성형외과학회지
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• 제20권6호
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• pp.421-424
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• 2019

Osteomas are benign, slow-growing osteogenic lesions frequently found in the craniomaxillofacial region. They can be classified as peripheral, central, or extraskeletal. Reactive mechanisms to trauma or infection, as well as muscle traction, are thought to play a major role in the development of peripheral osteomas. In the present report, a 41-year-old woman presented with a slowgrowing, painless mass on her left eyebrow. She had suffered trauma 15 years prior. In the computed tomography scan, a 2.5×2×0.7-cm radio-opaque tumor was detected just medial to the left supraorbital foramen, and a peripheral osteoma was clinically diagnosed. An elective operation under general anesthesia was planned. Following a suprabrow incision, subcutaneous and intramuscular dissection was performed. In the surgical plane deep to the corrugator muscles and superficial to periosteum, a branch of the supratrochlear nerve was encountered and preserved using a vessel loop. The osteoma beneath the periosteum was extracted in multiple fragments using a chisel and mallet to minimize trauma to the nerve. Contour and facial symmetry were corrected. To use a suprabrow incision, the surgeon must understand neighboring anatomical structures, including the course of the supratrochlear and supraorbital neurovascular bundles. When these structures are located adjacent to tumor lesions, careful surgical maneuvers should be performed to preserve them.

• Journal of Yeungnam Medical Science
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• 제17권1호
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• pp.87-92
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• 2000

저자들은 영남대학교 의과대학 부속병원에서 간엽성 연골육종으로 진단받은 3례를 대상으로 임상 병리학적 특징을 분석하여 다음과 같은 결과를 얻었다. 환장의 연령은 25세에서 32세였고 평균연령은 28세였다. 종양의 평균 크기는 7.3cm였다. 조직학적으로 종괴는 이형성의 소세포로 구성된 고밀도의 부분과 분화가 좋은 초자양의 연골 조직이 섞여 있었다. 3례에서 종양 세포들은 S-100단백과 NSE에 양성이었고 desmin에 모두 음성이었다.

• Childhood Kidney Diseases
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• 제12권2호
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• pp.239-244
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• 2008

말기 신부전으로 투석을 받는 환자의 결막과 각막에 골 외 석회 침착이 발생할 수 있다. 결막과 각막의 석회 침착은 대부분 장기간 혈액 투석을 받은 성인에서 보고되고 있으며, 신대체 요법의 기간, 높은 혈청 Ca$\times$P 등과 연관되는 것으로 알려져 있다. 저자들은 31개월째 복막 투석을 받고 있는 10세 여아에서 발생한 심한 결막과 각막의 석회 침착이 각막 주변부 상피세포 제거와 EDTA 투여로 호전된 경험을 하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제37권3호
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• pp.286-291
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• 2004

원발성 지방육종은 종격동에서 매우 드물게 발생하는 악성 종양으로 수술적 절제가 가장 효과적인 치료로 알려져 있다. 24세 남자 환자가 1개월 전부터 시작된 호흡곤란과 흉부 불편감으로 타 병원에서 좌측 혈흉을 의심하여 좌측 흉관삽관술을 시행받았다. 본원 입원 후 흉부 단순촬영에서 종격동 편위소견을 보였고 빈맥이 진행하였으며, 중심정맥압이 상승하는 양상보여 진단 및 혈괴제거를 위해 응급으로 좌측 개흉술을 시행하여 다량의 점액성의 혈성 흉수를 제거하였고, 조직검사 결과 골격계외 점액성 연골육종으로 진단되었다. 2주 후 좌측 개흉술로 횡격막 및 심낭의 일부를 포함하는 흉막외 좌측 전폐적출술과 함께 종격동 종양을 절제하였다. 조직검사에서 점액성/원형세포 지방육종으로 진단되었으며, 입원 54일만에 합병증 없이 퇴원하였다. 술 후 2개월째 1회의 항암화학요법(Ifosfamide+Adriamycin: IA) 후 종격동, 좌측 견갑골 부위 및 종격동 임파선에 재발하여, 약제를 바꾸어 (Etoposide+Ifosfamide+Cisplatin: VIP) 항암화학요법을 시행하였으나 복강전이로 술 후 10개월째 사망하였다. 종격동에 발생하는 원발성 지방육종은 매우 드문 질환으로 수술적 절제 및 항암요법 후 추적관찰한 1예를 문헌고찰과 함께 보고하는 바이다.

• Archives of Plastic Surgery
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• 제38권6호
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• pp.894-898
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• 2011

Purpose: Coverage of full-thickness large flank defect is a challenging procedure for plastic surgeons. Some authors have reported external oblique turnover muscle flap with skin grafting, inferiorly based rectus abdominis musculocutaneous flap, and two independent pedicled perforator flaps for flank reconstruction. But these flaps can cover only certain portions of the flank and may not be helpful for larger or more lateral defects. We report a case of large flank defect after resection of extraskeletal Ewing's sarcoma which is successfully reconstructed with reverse latissimus dorsi myocutaneous flap. Methods: A 24-year-old male patient had $13.0{\times}7.0{\times}14.0$ cm sized Ewing's sarcoma on his right flank area. Department of chest surgery and general surgery operation team resected the mass with 5.0 cm safety margin. Tenth, eleventh and twelfth ribs, latissimus dorsi muscle, internal and external oblique muscles and peritoneum were partially resected. The peritoneal defect was repaired with double layer of Prolene mesh by general surgeons. $24{\times}25$ cm sized soft tissue defect was noted and the authors designed reverse latissimus dorsi myocutaneous flap with $21{\times}10$ cm sized skin island on right back area. To achieve sufficient arc of rotation, the cephalic border of the origin of latissimus dorsi muscle was divided, and during this procedure, ninth intercostal vessels were also divided. The thoracodorsal vessels were ligated for 15 minutes before divided to validate sufficient vascular supply of the flap by intercostal arteries. Results: Mild congestion was found on distal portion of the skin island on the next day of operation but improved in two days with conservative management. Stitches were removed in postoperative 3 weeks. The flap was totally viable. Conclusion: The authors reconstructed large soft tissue defect on right flank area successfully with reverse latissimus dorsi myocutaneous flap even though ninth intercostal vessel that partially nourishes the flap was divided. The reverse latissimus dorsi myocutaneous flap can be used for coverage of large soft tissue defects on flank area as well as lower back area.