Imaging of chronic recurrent multifocal osteomyelitis of childhood first presenting with isolated primary spinal involvement.

OBJECTIVE: Initial presentation with primary spinal involvement in chronic recurrent multifocal osteomyelitis of childhood (CRMO) is rare. Our objective was to review the imaging appearances of three patients who had CRMO who initially presented with isolated primary spinal involvement. DESIGN AND PATIENTS: The imaging, clinical, laboratory and histology findings of the three patients were retrospectively reviewed. Imaging included seven spinal MR imaging scans, one computed tomography scan, nine bone scans, two tomograms and 16 radiographs. These were reviewed by two musculoskeletal radiologists and a consensus view is reported. All three patients presented with atraumatic spinal pain and had extensive bone spinal pathology. The patients were aged 11, 13 and 12 years. There were two females and one male. RESULTS AND CONCLUSIONS: The initial patient had thoracic T6 and T8 vertebra plana. Bone scan showed additional vertebral body involvement. Follow-up was available over a 3 year period. The second patient had partial collapse of T9 and, 2 years later, of C6. Subsequently extensive multifocal disease ensued and follow-up was available over 8 years. The third patient initially had L3 inferior partial collapse and 1 year later T8 involvement with multifocal disease. Follow-up was available over 3 years. The imaging findings of the three patients include partial and complete vertebra plana with a subchondral line adjacent to endplates associated with bone marrow MR signal alterations. Awareness of the imaging appearances may help the radiologist to include this entity in the differential diagnosis in children who present with spinal pathology and no history of trauma. Histopathological examination excludes tumor and infection but with typical imaging findings may not always be necessary.

Durability and strength of Steinmann pin augmentation in cemented tibial defects.

It has been argued that Steinmann pin augmentation does not improve the biomechanics of polymethylmethacrylate reconstruction for massive defects of bone. The current authors investigated whether pin augmentation of polymethylmethacrylate in the reconstruction of noncontained defects of bone improved the biomechanical properties of the reconstruction as compared with polymethylmethacrylate alone when minimal or large bone porosity is present. Large noncontained defects were created in 10 pairs of human tibias. In Group 1, five left tibias had reconstruction with polymethylmethacrylate augmented with three, 5-mm diameter by 10-mm deep holes into lateral condyle cancellous bone. Right tibias had identical reconstruction with three, 3/16-inch threaded pins placed into the medullary canal. In Group 2, three, 10-mm diameter by 10-mm deep holes were created in both pairs. The left tibia had polymethylmethacrylate reconstruction and the right tibia had polymethylmethacrylate and pin augmentation. Specimens were subjected to 2000 compressive cycles then loaded to failure. In Group 1, cycles and load to failure were significantly lower in reconstructions without pins compared with reconstructions with pins. No significant difference was observed between reconstruction techniques in Group 2. In reconstructions without pins, large diameter holes had significantly better cyclical durability. Pins improved survival compared with no pins.

Calcifying aponeurotic fibroma features seen on fine-needle aspiration biopsy: case report and brief review of the literature.

Calcifying aponeurotic fibroma is a rare benign soft tissue proliferation, which occurs in the distal extremities in children. We describe a case of calcifying aponeurotic fibroma of the hand in a 9 year-old male diagnosed by FNA. Clinically and radiographically this mass was felt to be suspicious for sarcoma, likely alveolar rhabdomyosarcoma. Cytologic examination revealed benign appearing spindled cells, chondroid cells, multinucleated giant cells and calcific debris. These features recapitulate the classic histologic features of calcifying aponeurotic fibroma. Conservative excision was performed and histologic exam confirmed the diagnosis. To the authors' knowledge, this is the first description of the cytologic features of this entity.

MR Imaging of sports-related pseudotumor in children: mid femoral diaphyseal periostitis at insertion site of adductor musculature.

OBJECTIVE: The objective of this study was to review the imaging appearance of the femurs of five patients who had been referred from outside institutions after presenting with thigh pain and being given a preliminary diagnosis of primary malignant bone tumor. Typically, when making a diagnosis, physicians place emphasis on the characteristic appearances of diseases on MR imaging, but such appearances may be misleading. An awareness of the specific MR imaging pattern of stress-related partial muscle avulsion can lead to the correct diagnosis. CONCLUSION: Femoral diaphyseal periostitis after a sports injury to the adductor musculature in children has a characteristic imaging appearance. This condition can initially appear to be misleadingly aggressive. Knowledge of the findings-particularly of the findings on MR imaging-in the proper clinical setting can help physicians make the correct diagnosis and eliminate unnecessary biopsy or inappropriate treatment.

Functional and clinical outcomes of limb-sparing therapy for pediatric extremity sarcomas.

PURPOSE: To determine the clinical and functional outcomes of children undergoing limb-sparing therapy for extremity sarcomas. METHODS AND MATERIALS: We retrospectively reviewed 30 patients, age < or = 21 years, who were treated between l979 and l998 with external beam radiotherapy as a component of limb-sparing therapy for primary sarcomas of the extremity at UCSF. Included were patients for whom complete follow-up and functional outcome assessments were available. We assessed the patterns of failure, overall survival, disease-free survival, local control, and limb function. RESULTS: At a median follow-up of 3 years, 12 of the 30 patients recurred: 3 locally, 8 distantly, and 1 with synchronous local and distant disease as site of first progression. Eighteen patients were alive with no evidence of disease. The median overall survival was 10 years, with a median disease-free survival of 8 years. Functional outcome assessment revealed 15 patients retained excellent, 12 good, 1 fair, and 2 poor limb function. CONCLUSION: In pediatric patients receiving limb-sparing therapy, 90% maintained excellent or good limb function without compromising survival, demonstrating the validity of limb preservation in children with extremity sarcomas.

Cysticercosis of the triceps--an unusual pseudotumor: case report and review.

Cysticercosis is a disease caused by encysted larvae of the tapeworm Taenia solium. Most muscular disease presents with central nervous system involvement or multiple cysts or both. The authors report a 25-year-old woman with a rare single muscle cyst presenting as a tender hard triceps mass. Results of clinical examination and magnetic resonance imaging were indicative of a soft tissue tumor. Excision of the mass revealed purulence, and the histologic diagnosis was cysticercosis. In the differential diagnosis of isolated muscular masses, a rare isolated cysticercus presenting as a pseudotumor should be considered.

Peripheral nerve ectopic meningioma at the elbow joint.

A rare peripheral ectopic meningioma of the nerve in the elbow region is discussed. The clinical, radiological, surgical and pathological findings are reviewed.

Follow-up evaluation of resected lumbar vertebral chordoma over 11 years: a case report.

STUDY DESIGN: A case report with an 11-year follow-up assessment after resection and reconstruction for lumbar chordoma is given. The literature relevant to this topic is reviewed. OBJECTIVES: To report the long-term outcome in a case of lumbar chordoma, to review the literature on vertebral chordoma, and to outline the rationale for surgical resection in such cases. SUMMARY OF BACKGROUND DATA: Chordoma is a malignant bone tumor that grows slowly, often recurs locally, and metastasizes late. Although different treatment approaches exist, including radiation and surgery, the only curative treatment is early and complete surgical excision of the tumor. Immediate spinal stability must be achieved with appropriate replacement or bone graft with rigid fixation. METHODS: The 11-year follow-up evaluation of a 42-year-old woman with L3 and L4 vertebral body chordoma treated with complete removal, femoral shaft allograft replacement, fusion, and rigid metal fixation is reported. The patient was observed with serial physical examinations, radiographs, and laboratory studies over 11 years. RESULTS: At this writing, 11 years after the resection of the L3 and L4 chordoma, the patient is asymptomatic without evidence of recurrence or metastasis. CONCLUSIONS: As reported, vertebral chordomas are not curable, but the authors' experience contradicts this. The surgeon should aim at a wide, or at least a marginal, excision followed by a stable reconstruction.

Compliant prestress fixation in tumor prostheses: interface retrieval data.

This article reports the first available human retrieval data following the use of a new fixation system for tumor prostheses. The compliant prestress (CPS) fixation system obviates the need for long intramedullary stems. The CPS was designed to provide a stable, high-pressure, motion-free bone-implant interface that would prevent aseptic loosening and allow osseointegration at the bone-implant interface. At 10 months, the fourth patient in the human trial required amputation. Backscatter electron microscopy revealed a buttress of new bone had formed along 70% of the bone-metal interface, with excellent bony ingrowth (average: 42%) into the transverse, porous-coated titanium interface.

Prognostic value of histologic tumor necrosis assessment in osteogenic sarcoma of bone.

To assess the prognostic value of tumor necrosis in osteogenic sarcoma of bone, we designed a retrospective study of 18 patients with classic osteogenic sarcoma (OGS) in which several factors were considered as the common criteria of inclusion. Forty percent of patients with > or = 95% necrosis related to chemotherapy of their primary tumor experienced metastatic disease and/or tumor recurrence during their follow-up, while 50% of those with < 95% necrosis had a disease-free period of > or = 5 years. Tumor necrosis related to chemotherapy in OGS does not seem to represent, as a single predictor of disease-free survival, an accurate clinical prognostic indicator. Further clinical and epidemiologic studies are needed on larger series of patients with strict criteria of inclusion to confirm our results.

Fibrogenesis imperfecta ossium: imaging correlation in three new patients.

OBJECTIVE: Fibrogenesis imperfecta ossium is an extremely rare disorder that can easily be misdiagnosed. We retrospectively reviewed the clinical and imaging data of three confirmed cases of fibrogenesis imperfecta. DESIGN AND PATIENTS: The patients consisted of two men and one woman, ranging in age from 40 to 53 years. Radiography was performed in all the patients. One patient had a 3-year follow-up of the thoracolumbar spine with conventional radiography and thoracolumbar magnetic resonance (MR) imaging. Open biopsy was performed in all cases, confirming the diagnosis of fibrogenesis imperfecta ossium. RESULTS: All our cases demonstrated "fishnet" trabecular pattern by conventional radiographs, and a pelvis radiograph of one patient showed an equivocal sclerosis pattern. Multiple fractures were noted in two patients. A pseudoexostosis was present in the ilium in one patient. Thoracolumbar MR imaging demonstrated diffuse low signal intensity within the medullary space on both T1-weighed and T2-weighted images, except for a region of increased signal intensity in the L1 and L2 vertebral bodies on T2-weighed images due to edema from acute collapse. CONCLUSIONS: Although uncommon, fibrogenesis imperfecta ossium should be considered in a previously healthy patient with a combination of progressive bone pain, unexplained fractures, a radiologic pattern of fishnet osteopenia and MR imaging of low signal intensity bone marrow on both T1-weighted and T2-weighted images.

Chondrosarcoma of the foot: imaging, surgical and pathological correlation of three new cases.

The foot is an uncommon location for chondrosarcoma. The presentation, diagnosis, pathological findings, surgical treatment and follow-up of three patients with chondrosarcoma in this rare location are presented. Though nonspecific, MR imaging findings were of aid in the diagnosis and treatment planning of these patients. If the diagnosis of this tumor is rapidly made, a tumor excision instead of limb amputation may be sufficient treatment at surgery.

Alveolar soft part sarcoma involving the ilium. A case report.

A report of alveolar soft part sarcoma of the hip region with extension to the ilium is presented to highlight an unusual presentation for this sarcoma and to discuss the treatment options for limb salvage for large malignant neoplasma involving the periacetabular region of the pelvis. A review of the literature for reports of alveolar soft part sarcoma extending to bone is presented.

Advances in MR imaging of pediatric musculoskeletal neoplasms.

MR imaging has evolved as the most important diagnostic test for local staging of primary bone and soft tissue tumors, for monitoring response to chemotherapy, and for detecting postoperative tumor recurrence. MR imaging provides accurate preoperative staging of local tumor extent and helps to obtain adequate safety margins, prerequisites for successful limb-salvage surgery. Postcontrast MR imaging studies are helpful for evaluating the presence or absence of tumor necrosis during chemotherapy. Dynamic MR imaging after intravenous bolus administration of Gd-DTPA or other paramagnetic contrast media is particularly useful for assessing response to chemotherapy. Diffusion-weighted MR imaging is a new technique that is potentially capable of detecting and quantitating the amount of tumor necrosis after chemotherapy or radiation therapy.

Early results of lower limb surgery for osteogenic sarcoma of bone.

Little information is available in the literature about early follow up of patients undergoing surgical procedures for osteogenic sarcoma of lower limbs as a part of their treatment protocol. In the present study the authors evaluate the functional and subjective condition of patients during a 2-year postoperative period, and analyze the nature and frequency of complications arising during that period. A scoring system was designed to represent the functional condition of the patients; compiled scores could therefore account for a given trend, which was further submitted to statistical analysis. Nature and frequency of complications following surgery were related to tumor location and to performed surgical procedures. Results of the study show that during the first 6 postoperative months, most patients have poor functional and subjective condition, which progressively improves throughout the first and second year. There is a high rate of complications during this period (46%), most frequently related to proximal femur and proximal tibia sites. Complications and functional disabilities are mainly related to mechanical disfunction of the reconstructive systems currently used.

Giant cell tumor of bone. Prognosis and treatment of pulmonary metastases.

Giant cell tumor of bone is a challenging clinicopathologic entity. Despite its benign designation, it has the capacity to recur locally and develop rare pulmonary metastases. Between 1945 and 1991, 104 patients with histologically benign giant cell tumors of bone, 5 of which metastasized to the lung, were treated at the authors' institution. In these cases, histologic materials from the lung were identical to those found in the primary bone lesion. The primary bone lesions were treated with local curettage (3), wide resection (1), and wide resection with prosthesis placement (1). The patients were observed for a mean of 12.6 years (range, 5-38 years). Four of the 5 patients experienced local recurrences (average time interval, 34 months), with 3 patients experiencing 2 or more recurrences. The average time to lung metastasis was 23 months; 1 patient presented initially with pulmonary findings. Four patients underwent surgical resection of pulmonary metastases. All 4 patients are alive with no disease progression, despite incomplete pulmonary resections in 2 patients. Locally aggressive disease and multiple recurrences appear to be risk factors for pulmonary metastases in benign giant cell tumor of bone. Pulmonary metastases occurred within the first few years after discovery of primary bone tumors. Radiographs and computed tomographs of the chest are recommended to rule out this complication in patients with local recurrences. Resection of pulmonary metastasis is recommended. Long term survival is not incompatible with persistent pulmonary lesions.

Pigmented villonodular synovitis of the shoulder after anterior capsulolabral reconstruction.

Pigmented villonodular synovitis (PVNS) is a proliferative disorder of the synovium affecting joints, bursae, or tendon sheaths. PVNS is further classified into diffuse and localized forms and rarely affects the shoulder. We report a case of nodular synovitis of the shoulder after arthroscopic and open anterior capsulolabral reconstruction. The histopathology and treatment of a nodular form of PVNS of the shoulder is discussed.