Search

The spectrum of mesenchymal tumors associated with rearrangements of the EWSR1 gene has been growing in recent years due to progress in molecular detection techniques. Originally identified as the gene involved in the pathogenesis of Ewing sarcoma, the EWSR1 gene is now known to be rearranged in diverse clinical and histopathological entities. The NFATC2 gene is one of the many translocation partners of EWSR1 in gene fusions in a morphologically typical, albeit rare, subgroup of mesenchymal tumors. Little is known about the clinical characteristics of tumors containing NFATC2 gene rearrangements since most of the few reports published describe molecular rather than clinical aspects. In the current study, we report three patients with tumors carrying the EWSR1-NFATC2 gene translocation, including one rare primary tumor of soft tissues. Another patient with a benign-appearing bone tumor with a unique FUS-NFATC2 gene translocation is described. In various mesenchymal tumors (e.g., myxoid/round cell liposarcoma, low-grade fibromyxoid sarcoma, or angiomatoid fibrous histiocytoma), the FUS gene, as a member of the TET family, may be alternatively rearranged instead of the EWSR1 gene without any noticeable influence on the microscopical appearance or clinical outcome. This fact seems not to apply to mesenchymal tumors with the involvement of the NFATC2 gene because both in our experience and according to the extensive literature review, they have different properties on the morphological and molecular level. Both ESWSR1-NFATC2 and FUS-NFATC2 fusion-carrying tumors do not show microscopical or clinical features of Ewing sarcoma.

This article reviews the methods recommended in the literature for treating sacral tumors, paying particular attention to the most common sacral tumors and those of uncertain malignancy. These recommendations for treatment are compared and analyzed in the light of the authors' own results in the treatment of 30 sacral tumors.

The typical radiologic finding of a "non-ossifying fibroma" of the distal femoral metaphysis was seen in a 17-year-old female patient with pain in the distal femur. The rapid development of this lesion was confirmed on the basis of earlier radiographs performed to clarify other complaints. Since the lesion had developed in no more than 34 months, with pronounced scintigraphic and MRI signal intensity, particularly in the late phase, and pain had prompted the patient to seek examination, the cause was assumed to be a neoplasia rather than a growth disorder. Histopathological examination revealed a proliferative fibrohistiocytic lesion which was histologically different from a malignant fibrous histiocytoma. The finding indicates that in the differential diagnosis between non-ossifying fibroma and benign fibrous histiocytoma in cases with identical localization, pain and the intensity of the scintigraphic signal may well be the key to further action: since benign fibrous histiocytomas may be regarded as genuine neoplasias, the question of malignancy should also be clarified beyond doubt.

The biopsy is the key for the exact diagnosis of bone and soft tissue sarcomas. However the biopsy implicates risks that eventually may significantly interfere with the prognosis. The steps of the biopsy can be differentiated in tactical and technical aspects. Tactical aspects include laboratory examinations (e.g. blood count, immunological, chemical tests) in order to establish the prebiopsy differential diagnosis, basic imaging procedures (bone scan, magnetic resonance imaging) and to discuss with the pathologist questions as which region of the tumor might be most informative, how should the material be preserved and whether a frozen section might be useful and allow for initiation of therapy as early as possible. Furthermore the site of the biopsy has to be carefully chosen, the compartments that need being passed through or opened up during biopsy must be considered. The technical aspects refer to the approach and tissue handling during the biopsy itself. Fine needle aspiration allows only for a cytologic, core biopsies for a histologic examination (however only of a small sample), while the open biopsy provides more material for more analyses. Any biopsy (open biopsy as well as percutaneous puncture) contaminates the path with tumor cells and increases the risk of systemic seeding. In order to keep these risks as small as possible the most careful technique and tissue handling is mandatory. The biopsy path has to be completely ('en bloc') resected later for local tumor control.

A case of Gorham-Stout disease of the shoulder girdle and cervico-thoracic spine in a 65-year-old woman is described. The patient presented with progressive neurologic symptoms, pain, and deformities of the cervico-thoracic spine as well as of her left shoulder following a traumatic shoulder luxation. Since the patient had a history of uterine carcinoma, the current disease was clinically difficult to differentiate from osteolytic metastases. The results of the clinical, radiological and histopathologic examination leading to the diagnosis of Gorham-Stout disease are described. Neither attempted surgery nor radiotherapy produced clinical improvement and the patient died 1 year after the first clinical evaluation.

In looking at the pathoanatomy of slipped capital epiphysis, it is important to appreciate that the articular cartilage ruptures at the level of the physis as the articular cartilage also covers part of the femoral neck metaphysis. As a consequence, smoothness is lost and already with mild slips the irregularities may injure the acetabular cartilage structures and may cause tears of the labrum. Principally therefore an open revision and restoration of the anatomy as perfectly as possible is needed. The technique for open reduction was developed by Dunn in 1964 and then refined by Ganz in 1997 applying new anatomical vascular studies. However, the long-term data on the "conservative" approach of only using transfixation to avoid further slipping show relatively good results and long-term studies of a more aggressive open approach will be needed to show evidence of further improvement. For the more severe cases, restoration of the correct anatomy by open reduction or improved axial relationships by more distant osteotomies have already been proven to give better results. Besides the extent of the slip, it is important to consider whether the slip is acute or chronic or acute and chronic. So far there are no data showing that the surgical treatment of slipped capital femoral epiphysis must be made on an emergency basis. However, prevention of further slips, e.g., by supine transport, is to be considered an emergency.

Three cases with destructive bone lesions of the distal end of the ulna caused by different pathologic entities (Ewing's sarcoma, osteosarcoma, rheumatoid pseudotumoral synovitis) are presented, all with similar clinical and comparable x-ray and magnetic resonance imaging features. Although the distal end of the ulna may be resected without significant functional impairment, careful evaluation of treatment strategies compatible with oncologic standards is warranted.

BACKGROUND Owing to twenty years of multicentric interdisciplinary cooperation, the COSS group has been able to collect data on a large group of osteosarcoma patients treated by neoadjuvant therapy. This paper reviews results achieved in patients with localized extremity tumors. PATIENTS AND METHODS INCLUSION CRITERIA Registration into a completed neoadjuvant COSS-Study. Histologically confirmed, primary, localized, high-grade, central osteosarcoma of an extremity; age or = 1/3 304. Definitive surgery in 903/925 cases, 443 limb salvage procedures. Good response (> 90% necrosis) in 469/806 (58.2%) evaluated tumors. Median follow-up for surviving patients: 5.42 years. Actuarial survival after 5 and 10 years: 72.5% (95%-CI 69.3-75.7) and 66.3% (62.5-70.0), relapse-free 62.1% (58.7-65.4) and 59.4% (55.8-63.0). 683/925 alive (601 first remission), 242 deceased (212 tumor progression, 30 other causes). 66.2% (97.3%) of all relapses within 2 (5) years. Prognosis correlates with tumor-size ( or = 1/3: 69.9% vs. 58.3% at 10 years) and -site (tibia: 74.2%, humerus: 54.5%) and -response (good vs. poor: 78.2% vs. 52.5%) (all p < 0.01). Actuarial 10-year survival by response grading I-VI according to Salzer-Kuntschik 80.9%, 82.8%, 71.1%, 60.7%, 47.7%, 27.3%. COSS-studies with preoperative 4-drug therapy more efficacious than less aggressive protocols. No impact of doxorubicin scheduling (sequential: rapid vs. 48 h-continuous infusion) or cisplatin scheduling (randomized: 5 h vs. 72 h-infusion) on prognosis detected. CONCLUSIONS Intensive multiagent chemotherapy and delayed surgery for localized extremity osteosarcoma led to excellent oncologic results in the COSS-studies. Tumor-size, -site, and -response as well as the intensity of upfront chemotherapy correlated with outcome. Giving doxorubicin and cisplatin by continuous infusions did not result in discernible prognostic disadvantages.

Typically an osteochondrosis dissecans occurs in the region of a convex articular surface, the most frequently localisation observed being the medial femoral condyle. Only few cases of an osteochondrosis dissecans in concave articular surfaces have been reported; these involved the tibial plateau, the distal tibia and the proximal surface of the navicular bone. Two more cases with an osteochondrosis dissecans in the proximal and distal tibial epiphysis are reported and additionally a hitherto unreported localisation in the glenoid fossa of the scapula. True osteochondrosis must be differentiated from ossification variants.

The maturation of the iliac apophysis (Risser sign) and of the bones of the hand and wrist of 34 girls with scoliosis with 46 pairs of data were analyzed to get information upon their predictive value for further growth and development of the scoliotic curve. A strong correlation was found between the Risser sign and the maturation of the hand and wrist (r = 0.74, p less than 0.005). The prognostic value of the Risser sign (r = -0.57) for further height gain is superior to chronological age only (r = -0.52), the maturation of the hand and wrist (r = -0.62) however is superior to the Risser sign. It can be assumed that this also holds for their prognostic value for the progression of the scoliotic curve. We feel that in the clinician routine for girls with scoliosis the determination of the skeletal maturity from X-rays of the hand is sufficient and with its probably unavoidable irradiation risks to the gonads the determination of the Risser sign should be abandoned.

The pre-operative diagnosis of osteoid osteoma of the first metacarpal bone was suggested by Magnetic Resonance Imaging in a 15 year old girl. As indicated in the literature, osteoid osteoma of the hand is relatively rare. The symptoms and the x-rays features of osteoid osteoma are essentially independent from the location consisting in rather unspecific pain and radiographically osteolytic nidus surrounded with sclerosis. In the few published cases with MR Imaging, the nidus shows high signal intensity surrounded by low signal intensity in the area corresponding to the radiographically visible sclerosis.

Summary Sixty-six hips of 65 patients with moderate to severe slipped capital femoral epiphysis were treated by open reduction of the femoral head. In 60 hips this procedure was combined with a cuneiform subcapital wedge resection of the femoral neck according to the technique described by Dunn (14). Avascular necrosis occurred in seven cases, and chon-drolysis was evident in eight hips. In the 48 hips with a follow-up time of more than 10 years (M = 20.6), the results were classified as good (normal hip) in 22, moderate in 16, and poor in 10 hips. Compared with the natural disease course, the longterm outcome appears to be improved by open reduction of the femoral head, with 60% of the hips free of degenerative changes and 19% with mild osteoarthrosis. Analysis of individual gliding angles and subcapital correction depending on the direction of the slip should lead to restoration of near-normal anatomic conditions for a normal hip function.

During the dissection of seven club feet of foetuses, aborted between the 25th and 37th week of gestation, an additional muscle bundle was found in a right foot. This muscle arose from both heads of the gastrocnemius muscle, continued downwards onto the posterior surface of the soleus, crossed the calcaneal tendon and assumed a position on the lateral side of the calcaneal tendon. The muscle's tendon was fixed just laterally to the calcaneal tendon in the calcaneal tuberosity with some tiny fibres branching off into the superior fibular retinaculum.

The standard treatment of acute distal femoral fractures consists of open anatomic reconstruction and fixation. Internal fixation may be accomplished with angular plates, condylar screw fixation, antegrade or retrograde nailing. Inveterated condylar fractures are rarely seen in our society and present challenging management problems. A 64-year-old man presented with a pseudarthrotic dislocated multifragmentary supracondylar fracture and a knee flexion contracture two years following the accident, mobilized only with a wheelchair. Because of the severly osteoporotic bone, degenerative articular changes and the flexion contracture, we chose resection of the fragments and implantation of a reconstruction system (MUTARS) instead of osteosynthesis. Eleven weeks postoperatively, the patient was able to walk without crutches. Nine months after surgery, he has flexion up to 90° and full extension, and has regained an active life.

Osteomyelitis sclerosans Garré is still not completely understood. In spite of the fact that it is caused by bacterial infection, in most cases no bacterial growth can be discovered in culture. Furthermore, it remains open whether the chronic process is maintained by low-grade persistent infection or is maintained by itself after sterilization of the infection. Newer studies that may prove the presence of bacteria, e. g., by PCR, may elucidate the causes in the future. Sclerosing bone disease, sometimes multifocal, which present the same clinical, radiographic and histological features as chronic sclerosing osteomyelitis may be associated with diseases such as palmoplantar pustulosis, colitis ulcerosa, Crohn's disease, etc. and are reported as SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome. In these diseases bacterial growth is almost always negative. However, temporary improvement under antibiotic therapy may be observed. A relationship between typical isolated chronic sclerosing osteomyelitis and SAPHO syndrome may exist, but the diseases should at present be differentiated. Neoplasms such as osteoid osteoma, Ewing sarcoma, or eosinophilic granuloma may simulate primary sclerosing osteomyelitis, and pathological examination of peripherally taken specimens may appear to be compatible with the diagnosis of sclerosing osteomyelitis. It should be appreciated that malignant tumors - even though this is the exception - can present a chronic, mild picture. Treatment of osteomyelitis sclerosans Garré needs be determined on an individual basis. A conservative approach with antibiotics usually leads only to temporary pain relief. The biopsy with opening of the medullary canal is sometimes sufficient for healing, while in extreme situations only segmental or even complete resection of the diseased bone case result in a permanent cure.

The Schwartz-Jampel syndrome (SJS; chondrodystrophic myotonia; McK 255800) is a recessively inherited condition defined by myotonia, short stature, and bone dysplasia. Genetic linkage between SJS and chromosomal region 1q36-34 has been observed in several families, but the gene has not yet been identified. We studied the clinical and radiological features in 81 patients from the literature and 5 own patients trying to identify distinct subgroups. In addition, we tested genetic linkage to the SJS locus on chromosome 1 in one family with two affected sibs. We found that a group of patients have mild skeletal changes which may be secondary consequences of myotonia, while another group of patients appear to have primary bone dysplasia with myotonia. Within this latter group, there are differences in age of manifestation, clinical course and pattern of bone changes. We tentatively isolate three different types of SJS: type 1A, usually recognized in childhood, with moderate bone dysplasia, corresponding to the original descriptions of Schwartz, Jampel and Aberfeld; type 1B, similar to type 1A but recognizable at birth, with more pronounced bone dysplasia resembling Kniest dysplasia; and type 2, manifest at birth, with increased mortality and bone dysplasia resembling Pyle disease. Genetic analysis of the family with two sibs affected by SJS type␣2 showed evidence against linkage to chromosome 1p36-34. Conclusions SJS is clinically and radiologically heterogeneous. The causes of heterogeneity are not known yet but are likely to include both different mutations at the SJS locus on chromosome 1 and the presence of a second SJS locus. A tentative clinico-radiological classification can be useful for the characterization of patients and the development of genotype-phenotype correlations.

Fifty-one patients with unilateral severe (gliding angles 30 degrees-60 degrees) slipped capital femoral epiphysis (SCFE) treated by intertrochanteric corrective osteotomy were reexamined after 20-29 years (average 24 years) of follow-up; 55% of the patients showed neither radiographic signs of degenerative hip disease nor clinical symptoms, whereas 28% had moderate and 17% had severe osteoarthritis. These results are definitely superior to those reported in other series of patients with comparable slips treated by bed rest or in situ fixation only. Analysis of individual gliding angles and directions of the slips shows that results can probably be further improved by correct assessment of the gliding process to allow for best use of the potentials of intertrochanteric corrective osteotomies. Correction should also be performed as early as possible to allow for maximum remodeling.

Background The purpose of this study was to analyze whether hips treated for developmental dysplasia of the hip (DDH) during infancy, which were clinically and radiologically fully normalized by walking age, may become dysplastic again during later growth. Materials and methods A total of 150 patients were randomly selected out of a collective of 386 patients treated for DDH at the Department of Orthopaedics at the University of Zurich between 1993 and 2004. Treatment was started at birth and continued for 6 months. All patients had clinically and radiographically normal hips by walking age. The patients did not suffer from other diseases, in particular, neurological or neuromuscular diseases. Results We detected four female subjects among the 150 patients who had been successfully treated for DDH, who had developed dysplastic hips in early adolescence, necessitating acetabular surgery. Conclusion The successful treatment of DDH in infancy does not ensure normal hip development; therefore, follow up into maturity may be recommended. Level of evidence Level IV.

Background: Local recurrence (LR) in osteosarcoma is associated with very poor prognosis. We sought to evaluate which factors correlate with LR in patients who achieved complete surgical remission with adequate margins. Patients and methods: We analyzed 1355 patients with previously untreated high-grade central osteosarcoma of the extremities, the shoulder and the pelvis registered in neoadjuvant Cooperative Osteosarcoma Study Group trials between 1986 and 2005. Seventy-six patients developed LR. Results: Median follow-up was 5.56 years. No participation in a study, pelvic tumor site, limb-sparing surgery, soft tissue infiltration beyond the periosteum, poor response to neoadjuvant chemotherapy, failure to complete the planned chemotherapy protocol and biopsy at a center other than the one performing the tumor resection were significantly associated with a higher LR rate. No differences were found for varying surgical margin widths. Surgical treatment at centers with small patient volume and additional surgery in the primary tumor area, other than biopsy and tumor resection, were significantly associated with a higher rate of ablative surgery. Conclusions: Patient enrollment in clinical trials and performing the biopsy at experienced institutions capable of undertaking the tumor resection without compromising the oncological and functional outcome should be pursued in the future

Summary From 1979 to 1990, 37 patients with extremity osteosarcomas, 22 of them males and 15 females, median age 19 years, received pre- and postoperative chemotherapy. The period of observation, calculated from after the primary operation, ranged from 1–114 months, median 25 months. After preoperative chemotherapy, 9 (24%) underwent a primary amputation, in 28 (76%) a limb salvage procedure was possible. 4/29 (14%) later developed local recurrences, metastases were diagnosed in 8/9 amputees and 5/28 after limb-sparing surgery. The five-year disease-free and overall survivals after amputation are 11% and 33%, respectively, compared to 68% and 75%, respectively (p =0.001 and 0.018, respectively, for long rank). Results of histologic assessment after preoperative chemotherapy are of significant prognostic impact. The earlier prognostic groups of 0%–49% necrosis versus 50%–100% necrosis were statistically no longer suitable for distinguishing useful prognostic groups. In this second analysis, patients with 0%–79% necrosis versus 80%–100% necrosis had 5-year disease free survivals of 81% versus 44% (p = 0.032) and 5-year overall survivals of 88% versus 56% (p = 0.07 log rank). In summary, 24% of our patients with extremity osteosarcomas, most of them with large primaries close to joints, had to undergo primary amputation and had only a 33% 5-yr-survival, whereas limb salvage procedures with pre and postoperative chemotherapy were associated with a 5-yr survival of 75% and thus had no adverse impact. Careful selection of patients for successful management of osteosarcomas is important; necrosis of 80% and more after preoperative chemotherapy is a prerequisite for a favourable outcome.

Purpose. Rotationplasty has proven its efficacy in the treatment of malignant bone tumors of the lower extremity in predominantly young patients. To our knowledge this procedure has not been reported in patients over 60 years before.Materials and Methods. 3 patients over 60 years with an A1-rotationplasty because of a sarcoma were included in this study. Complications and functional results were recorded. In one patient an electromyography was done.Results. Despite electromyography showing good adaptation of the muscles to the altered function, the functional results of these three patients were limited. two out of three patients needed a cane for walking distances over 200 meters. No secondary amputation was necessary.Discussion. Our study demonstrates that rotationplasty is an alternative to an above-knee amputation in older patients but with poorer functional results in comparison to younger patients. However, limb-salvage surgery should be preferred whenever possible.

The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb.

Four patients with malignant tumors of the proximal toe phalanx who had closed contact or direct involvement of the metatarsophalangeal joints or direct infiltration of the metatarsal bone were treated. Treatment included ray resection and reconstruction either by free microvascular fibula transfer, intermetatarsal bony fusion, or soft-tissue stabilization. Foot function was analyzed by Novel pedobarography. The four patients with a follow-up between 21 months and 8 years show almost normal gait. All patients have remained relapse free. If adequate margins can be achieved, ray resection and appropriate reconstruction may be an alternative to amputation.

PURPOSE: Cooperative Ewing’s Sarcoma Study (CESS) 86 aimed at improving event-free survival (EFS) in patients with high-risk localized Ewing tumor of bone. PATIENTS AND METHODS: We analyzed 301 patients recruited from January 1986 to July 1991 (60% male; median age 15 years). Tumors of volume >100 mL and/or at central-axis sites qualified patients for “high risk” (HR, n = 241), and small extremity lesions for “standard risk” (SR, n = 52). Standard-risk patients received 12 courses of vincristine, cyclophosphamide, and doxorubicin alternating with actinomycin D (VACA); HR patients received ifosfamide instead of cyclophosphamide (VAIA). Tumor sites were pelvis (27%), other central axis (28%), femur (19%), or other extremity (26%). The initial tumor volume was RESULTS: On May 1, 1999 (median time under study, 133 months), the 10-year EFS was 0.52. Event-free survival did not differ between SR-VACA (0.52) and HR-VAIA (0.51, P = .92). Tumor volume of >200 mL (EFS, 0.36 v 0.63 for smaller tumors; P = .0001) and poor histologic response (EFS, 0.38 v 0.64 for good responders; P = .0007) had negative impacts on EFS. In multivariate analyses, small tumor volumes of CONCLUSION: Fifty-two percent of CESS 86 patients survived after risk-adapted therapy. High-risk patients seem to have benefited from intensified treatment that incorporated ifosfamide.

Summary We report the case of a 12-year-old girl with a Ewing sarcoma originating from the first metatarsal of one foot. Because the girl and her parents refused amputation of the foot, we resorted to complete local resection and to reconstruction of the first and second metatarsals with a free microvascular fibular bone graft. At 3½ years after surgery, the patient continues to walk without a limp, and the mechanical function of the forefoot has been largely restored, although considerable loss of function of the big toe is apparent. Details of the surgical technique and tumor management are described.

Soft tissue sarcomas are rare tumors, in localized disease most authors report a five year survival between 40 and 60%. Patients with soft tissue sarcomas mostly present with their primary tumors; they can be rendered disease free clinically by wide resection. Since local recurrences and metastases, especially in grade II and III soft tissue sarcomas over 5 cm in size are a major therapeutic problem, combined modality treatment with radiotherapy and adjuvant chemotherapy is evaluated in prospective studies. Limb sparing procedures without jeopardizing the result may be used, if they are associated with radiotherapy and chemotherapy (Eilber 1980, Consensus Conference 1985, Eilber 1990). Several chemotherapeutic agents, most recently Ifosfamid was added to the list, are found to be effective; among the most promising regimes were Cyclophosphamid, Adriamycin, DTIC (CAD, Blum 1980) and in recent years Ifosfamid, Adriamycin, DTIC (IAD, Elias 1989).

Complete removal of the tumour without leaving microscopic disease is vital in the management of bone sarcomas. Magnetic resonance imaging now seems to be the best method of depicting the changes produced by the tumour within the bone and surrounding soft tissues. In order to define its reliability, five cases of primary bone sarcomas (Ewing's sarcoma, osteosarcoma, chondrosarcoma) are described where radiographs, bone scans and magnetic resonance images are directly compared to the pathological findings after resection. We conclude that surgical margins should be redefined with respect to the extent and borders of the tumour as depicted by magnetic resonance imaging. This will allow improvement in salvage procedures, but further experience is needed so as not to jeopardise the prognosis by incomplete removal of the tumour.

A retrospective study was performed between 1980 and 1995 on 38 recipients of proximal tibial allografts after wide resection of benign and malignant tumors. Twenty-one (55%) patients experienced one or more complications. Of the 26 patients who received chemotherapy, 15 (58%) experienced one or more complications, whereas of the 12 patients who did not receive chemotherapy, six (50%) experienced one or more complications. In the chemotherapy group, there were 12 (46%) fractures, four (15%) infections, three (12%) nonunions, and four (15%) instabilities. In the nonchemotherapy group there were three (25%) infections, two (17%) fractures, one (8%) instability, and one (8%) nonunion. These complications were managed adequately with multiple subsequent surgical procedures. Three patients underwent amputations for deep wound infections. Twelve (32%) patients underwent removal of the allograft, and the limb was salvaged by reallografting or by total knee arthroplasty. The results of both groups were 66% (25 of 38 patients) satisfactory (good or excellent). The chemotherapy group had a significantly higher incidence of fractures. All other complication rates and functional outcomes were not significantly different between these groups.

We report a 7-year 6-month-old boy with Legg-Calve-Perthes (LCP) disease in whom a magnetic resonance imaging (MRI) was normal during the early symptomatic phase when the 99mTc bone scintigraphy showed segmental hypoperfusion of the femoral head suggestive of bone infarction. Only later in the disease did the MRI also show the typical changes of LCP. The follow-up on this patient with bilateral disease leaves no doubt about the diagnosis of LCP. This sequence of a positive scintigram before positive MRI findings may be the exception, but it is important to realize that MRI is not always the most sensitive way to diagnose or exclude LCP as suggested hitherto by the literature.

To assess the general profile of metastatic bone disease from occult primary carcinoma, the records of 172 patients with skeletal metastases seen between 1965 and 1985 were reviewed. In 51 patients (30%), the origin of the primary could not be identified when bone metastases were first diagnosed. This group were predominantly male with a high incidence of spinal metastases, cord compression and pathological fractures, and a significantly shorter (P less than 0.1) survival compared with bony metastases when the primary was known. The site of the primary was established in 33 patients (65%), mostly at autopsy. Lung carcinoma was by far the most common primary tumour in 52% of the cases, while it accounted for only 7% of those with a diagnosed primary. We believe that the onset of bony metastases from an occult source must initially raise the possibility of lung carcinoma. If the primary remains undetected, it appears justifiable to assume it to be in the lung, since the probability of a missed lung tumour being responsible for the metastases is high.

The method of gamma-ray computed tomography (gamma-ray CT) bone densitometry described in the preceding article provides selective determination of trabecular bone density (TBD), the relative amount of compact bone (bone density, BD), and the total absorption (TA) within a bone cross section. Seven of nine children with chronic renal failure (CRF), and selected only on the basis of their serum creatinine value (greater than 5 mg/100 ml), had increased TBD values above the normal range, whereas the other bone mineral parameters were normal. Radiographic signs of secondary hyperparathyroidism (subperiosteal erosions, cysts) were reported in the five patients with the highest TBD values, whereas the subjective diagnosis of osteosclerosis reported in three of these five and in one other patient correlated less well with the TBD increases. However, this is the first report of an objective, non-invasive documentation of the radiological finding of osteosclerosis in CRF. It also explains why methods for bone mineral measurements used previously, such as a photon absorptiometry which provides only a parameter equivalent to TA, failed to reveal increases in bone mineral content in renal osteodystrophy even when signs of osteosclerosis were present. Thus, gamma-ray CT helps to document objectively the degree of osteosclerosis and its location.

In a retrospective study of 172 patients with disseminated carcinoma in the skeleton, 54% were shown by radiography and scintigraphy to have vertebral metastases. Breast carcinoma was the most common primary tumour, occurring in 30% of the patients, followed by lung (17%), prostate (10%) and kidney (9%). The lumbar spine was most often involved and some primary carcinomas showed a predilection for particular spinal segments. Cord compression occurred in 30% of the patients with vertebral spread and was a poor prognostic sign for long-term survival. Hypernephroma was the most common tumour to cause spinal cord involvement. The thoracic segment was the most frequent site of cord compression (43%), and pathological fracture-dislocation was the most common cause (50%).

Frozen homologous trabecular bone (FHTB) is probably the most commonly used allogeneic material for biologic replacement of bone defects [1]. FHTB has been in use at the Klinik Balgrist since 1974. The results in cases of large defects are of interest in comparison with, for example, massive allografts.

In a pilot study, hip development in 615 newborn children was assessed sonographically. Distinct dysplasia was found in 2.3%, and an intermediate "physiologically immature" hip development was noted in 13%. The number of controls and expenses would greatly increase if all physiologically immature hips were followed until they reached maturity. To reduce the number of unnecessary control and to facilitate assessment, we propose the use of "cut-off values" in a screening program for newborns.

PRL has been determined before and after TRH IV (200 ug/m2) in 23 children (11 ♀/12 ♂) with craniopharyngioma using reagents provided by the NIAMDD, Bethesda USA. As controls served 10 endocrinologically healthy children (4♀/6♂), aged 4-16 yrs, who had TRH tests for other reasons. 13 patients, aged 1-13 yrs, have been studied before surgery, 10 of them and 10 additional patients, aged 1-20 yrs, after surgery. Assuming that PRL secretion is controlled by PIF (PRL inhibiting factor), elevated PRL levels as seen before and after surgery would indicate hypothalamic disorder or disruption of the hypothalamo-pituitary axis. PRL deficiency, observed only after surgery, would reflect pituitary damage.

Plasma LH and FSH, basal (b) and after LHRH (p), testosterone (T), estradiol (E2), estrone (E1), androstenedione (Δ4A) and prolactin(PRL) were determined in 5 pubertal boys with G before, during and after 50 mg C daily for 56 days (d). Results (mean, SEM): In conclusion, C increases gonadotropins and testicular steroids. In spite of higher E1 and E2, G is reduced in most patients. This may be due to a higher T/E2 ratio or interference of C with E2 at the receptor site. Supported by the Swiss National Science Foundation (Grants No. 3.883.077 and 3.901.077).