Background. Revision of the femoral component in total hip arthroplasty in the presence of severe bone loss is a complex problem. Proximal femoral allograft stems have been utilized with some success but are limited by graft-host mismatch, lack of graft viability, and complexity of further revision. Impaction allografting with collarless, polished, tapered stems has been used successfully in cases of mild to moderate defects, but use in severe defects is limited by stem design and a high complication rate. The use of collared, textured stems with the impaction grafting technique may be a potential means of restoring femoral integrity and stability in these massive defects and may carry advantages to other methods of revision. Methods. We present a retrospective case review of 18 hips in 17 patients having undergone revision total hip arthroplasty with impaction allograft- ing and collared, textured stems. These revisions were selected from the entire case log of the primary surgeon because they met the selection crite- ria of having underwent a revision arthroplasty with impaction grafting, having Endo-Klinik grade 2 or worse femoral defects, requiring 150 cc or more cancellous allograft during femoral reconstruction, and having at least two years of follow-up. These hips were assessed with Harris hip scores and serial radiographs to measure clinical outcomes and radiographic signs of stem subsidence and failure. Bone scintigraphy and SPECT were per- formed on five hips during follow-up to examine graft activity Results. Patients were followed for an average of 38 (24-56) months. Average Harris hip scores increased from 50 (17-73) preoperatively to 75 (40-100) at most recent follow-up. Seventeen hips were stable and func- tioning well at the time of most recent follow-up and exhibited no signs of stem subsidence or aseptic loosening on radiographs. There was one case of failure requiring further revision that was associated with visible subsi- dence and radiolucent lines on radiographs. SPECT analyses performed at an average of 37 (31-50) months postoperatively indicated active graft reorganization in all cases. Conclusions. Impaction allografting with collared, textured stems may be successfully performed in cases of massive revision total hip arthro- plasty and demonstrate good intermediate-term clinical results. The ab- sence of stem subsidence on radiographs correlates with a successful outcome. This technique avoids the limitations encountered with proximal femoral allograft stems and collarless, polished, tapered stems and may be successfully utilized in the presence of severe femoral bone loss.
Rothmund-Thomson syndrome is an autosomal recessive disorder characterized by poikilodermatous skin changes that develop in infancy. Associated manifestations include juvenile cataracts, sparse hair, short stature, skeletal defects, dystrophic nails and teeth, and hypogonadism. An increased incidence of malignancy, including osteosarcoma, has been reported in patients with Rothmund-Thomson syndrome. The molecular basis of the disorder is not known. This report describes a patient with Rothmund-Thomson syndrome in whom two primary osteosarcomas developed 12 years apart. The presentation, diagnosis, and treatment of osteosarcoma in this patient with Rothmund-Thomson syndrome are described. Cytogenetic and molecular analysis of peripheral blood and skin fibroblasts had low level mosaicism for trisomy of chromosomes 2 and 8. Although several patients have been described with mosaic trisomy 8 and i(2q) (mosaic isochromosome for the long arm of chromosome 2), the patient described here is the first to have mosaic trisomy for the entire chromosomes 2 and 8. The cytogenetic findings in this patient are consistent with an underlying defect in chromosomal stability.
A retrospective analysis of 40 patients diagnosed with melanoma of unknown primary site (MUP) was undertaken to analyze the etiology and clinical behavior of this presentation.The patient records were located by a computer search of the Pigmented Lesion Clinic data base at the University of Pennsylvania. With the Cox proportional hazards model, the survival of the MUP patients with lymph node presentation was compared with that of patients with lymph node disease and a known concurrent primary melanoma.Sixty-five percent of the patients presented with lymph node metastasis only, 28% presented with visceral lesions, and 8% presented with subcutaneous nodules. The prevalence of dysplastic nevi was 22.5%. The overall 4-year survival rate for the 40 MUP patients was 55% +/- 9%. The 4-year survival (57% +/- 12%) of patients with lymph node presentation was compared with that of patients presenting with lymph node disease and a known concurrent primary melanoma (19 +/- 6%). Survival was significantly different between the groups (P = 0.008). This survival difference remained significant (P = 0.02) even after adjustments for number of positive lymph nodes, year of diagnosis, and age at diagnosis.This analysis revealed that MUP patients with lymph node metastasis survived significantly longer than patients diagnosed with lymph node metastasis concurrent with a known cutaneous primary melanoma. The prevalence of dysplastic nevi in the MUP patient series was intermediate between that reported among primary melanoma patients and that reported among population controls, suggesting the likelihood of a primary cutaneous origin for the metastatic melanoma.
'%3e%3cpath fill='%23012169' d='M0 0v30h60V0z'/%3e%3cpath stroke='%23fff' stroke-width='6' d='m0 0 60 30m0-30L0 30'/%3e%3cpath stroke='%23C8102E' stroke-width='4' d='m0 0 60 30m0-30L0 30' clip-path='url(%23b)'/%3e%3cpath stroke='%23fff' stroke-width='10' d='M30 0v30M0 15h60'/%3e%3cpath stroke='%23C8102E' stroke-width='6' d='M30 0v30M0 15h60'/%3e%3c/g%3e%3c/svg%3e)