A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement / Journal of the Korean Cancer Association, 대한암학회지

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of bilateral lower limb pain. Left perirenal soft-tissue infiltration had been found incidentally two years earlier. No progression of the lesion or deterioration of renal function was observed for a period of two years. At admission, plain radiography and magnetic resonance imaging of the patient's lower limbs showed patchy osteosclerosis. Biopsy of the tibia revealed histiocytic infiltration, which was found to be positive for CD68 and negative for CD1a. This report describes an unusual case of Erdheim-Chester disease involving a stationary course of disease with no specific treatment for a long period of time.

Reconstruction with Replantation of the Resected Bone after Low Heat Treatment for Malignant Pelvic Bone Tumors / 대한정형외과학회잡지

PURPOSE: This study evaluated the oncological and functional results of a surgical treatment for malignant pelvic bone tumors using a low-heat-treated autologous bone graft. MATERIALS AND METHODS: Eleven patients with malignant pelvic bone tumors who were followed-up for more than one year were enrolled in this study. There were six males and five females. The mean age was forty-one years and the mean follow-up period was thirty months. Nine patients had primary bone tumors and two patients had metastatic tumors with various histological origins. A surgical resection was carried out according to the anatomic location (Type I/II 3 cases, Type II 2 cases, Type II/III 6 cases). The surgical methods used were a wide resection, a low-heat-treated autologous bone graft, total hip arthroplasty and rigid internal fixation. The ISOLS score was used to determine the oncological outcome. RESULTS: The mean ISOLS score was 61.2% at the final follow-up. The index of pain and emotional acceptance showed high scores, but functional ability, support, walking ability and gait showed relatively low scores. Bone union was achieved at a mean post-operative 6 months. The post-operative complications were one case of a local recurrence, two cases of infection and one case of a dislocation of the total hip arthroplasty. CONCLUSION: A wide resection and reconstruction with a low-heat-treated autologous bone graft in malignant pelvic bone tumors were satisfactory oncologically as well as functionally in the brief period. However, a longer follow-up and an examination of more cases will be needed.

Treatment of Spinal Deformities with Neurofibromatosis / 대한척추외과학회지

STUDY DESIGN: A retrospective study. OBJECTIVES: By analyzing the patients of neurofibromatosis with spinal deformities, to identify presence of dystrophic changes, progression of deformity and associated factors, and treatment results according for sagittal curve pattern and operative methods. SUMMARY OF LITERATURE REVIEW: A single thoracic curve involving four, five, or six vertebrae is recognized as the most common pattern. Risk factors for progression of curve were anterior vertebral scalloping, particularly in younger patients, three or more penciled ribs, abnormal kyphosis, etc. It has been stated that the most effective management for dystrophic curves is early and aggressive surgery. MATERIALS AND METHODS: Thirty nine patients with neurofibromatosis and spinal deformities were reviewed with chart and radi-ographic review from 1977 to 1999. RESULTS: Four of thirty nine patients were nondystrophic type, and all patients were treated nonoperatively. Thirty five of thirty nine patients were dystrophic type, and twenty seven patients were treated operatively. Eight of these patients had been in progress till operation with 7.9 degrees/year progression rate, and their commonest pattern of deformity is a single curve in lower thoracic area with dystrophic changes such as vertebral scalloping, wedging, pencilling of average four ribs, particularly. Forty three percent of dystrophic type has sagittal plane deformities. The pedicle screw system was most excellent among the instrumentations. The complications of surgery were 6 progression of curve, 2 metal failure. Reoperation was done in 5 of 27 operated patients. CONCLUSIONS: Nondystrophic type had good results with nonoperative treatment, but dystrophic type mostly required surgical intervention and had rapid progression. The treatment should be done by rigid fixation after considering sagittal plane deformi-ties and long term follow-up was needed for progressions of curve.