Biomedical subjects
Publications and source records attributed to G Jundt.
A 63-year-old man is presented in whom a tenosynovial giant-cell tumour had destroyed the temporomandibular joint fossa and expanded intracranially. The lesion was not diagnosed for a period of at least two years. Treatment included wide resection including the surrounding bone, dura and condyle.
A retrospective study of 68 cases of tumours and tumour-like lesions related to the scapula, included in the registry of the Bone Tumour Reference Centre at the Institute of Pathology/University Clinics, Basle, has been carried out. Each case was evaluated for lesion entity, activity and location, age and sex of the patient, and, in 49 files with available radiographic documentation (mostly plain films), for radiologic appearance, with the aim to predict the histologic diagnosis or at least the correct dignity of the lesion. Statistically most frequent were cartilaginous tumours. More than 1/3 of all cases were osteochondromas, which demonstrated mostly a typical appearance. They were encountered predominantly in the first 3 decades in males and were located most often in the body of the scapula. 1/4 of all cases were chondrosarcomas, which were prevailing in the 4th-7th decades, but were occasionally found at a younger age too. Chondrosarcomas were located mainly at the lateral scapular margin over the inferior angle and in the acromion and coracoid process and their appearance ranged from typical to falsely benign. 1/3 of the cases represented a number of other benign and malignant histological entities.
Two cases of unicystic ameloblastoma in the mandible of young children are described. Conservative surgery is justified in children due to the lower risk of recurrence compared to cases of solid ameloblastoma and the risk of growth disturbance after radical surgery. However, long-term follow-up is necessary.
A 63-year-old man is presented in whom a tenosynovail giant-cell tumor destroyed the right temporomandibular joint and fossa and showed extensive intracranial growth. Because of uncharacteristic complaints, a symptomatic treatment was performed elsewhere. The lesion was finally resected under endotracheal anesthesia. After 20 months free of recurrence the patient's outcome is very satisfying. Differential diagnosis and therapy are discussed.
Most Ewing tumors (ET), including Ewing sarcomas, peripheral primitive neuroectodermal tumors (PNET), and Askin's tumors, can be defined according to the specific chromosomal translocations t(11;22)(q24;q12) (EWS-FLI-1) or t(21;22)(q21;q12) (EWS-ERG). Detection of the chimeric RNA transcripts by reverse transcriptase-polymerase chain reaction (RT-PCR) has greatly facilitated the diagnosis of ET. Because of variable chromosomal breakpoint locations, however, the EWS gene fusions with FLI-1 and ERG genes are highly heterogenous, resulting in different sizes of the amplification products. To improve the diagnostic usefulness of the RT-PCR assay, we have developed an assay to detect chimeric mRNA transcripts by nested RT-PCR, followed by digestion of the PCR fragments with three different restriction endonucleases. This allows confirmation of the specificity of the PCR product and provides a rapid method to determine the combination of exons present in a transcript. In the 12 Ewing tumors tested, five different exon combinations were detected. In nine repeat biopsies of four patients, the case-specific translocation remained unchanged. One additional central PNET had no ET-specific translocation. In conclusion, the suggested combination of RT-PCR and restriction analysis of the PCR products allows a rapid and specific determination of ET-specific translocations.
METHODS: Combined bone scanning and immunoscintigraphy (IS) with 99mTc-monoclonal antigranulocyte antibodies were performed in two patients with suspected reactivation of chronic osteomyelitis of the lower extremity. Because bone scanning and IS were strongly positive, both patients underwent surgical intervention. RESULTS: Macroscopic findings did not show purulent infection and microbiologic results remained negative, but histology revealed unexpected ectopic bone marrow, explaining the strong uptake on IS. One patient exhibited active hematopoietic bone marrow at the former fracture site of the tibial bone. The second patient presented with interspersed bone marrow in the cortical bone of the femoral diaphysis after several intramedullary surgical procedures. CONCLUSION: Unexpected ectopic hematopoietic marrow may occur in the appendicular skeleton after trauma and repeated surgical interventions. The bone marrow shows a physiologic uptake with IS and may be misinterpreted as granulocyte accumulation due to infection. This may lead to false-positive diagnosis in cases of suspected osteomyelitis.
BACKGROUND: The reconstruction of massive osteochondral defects extending to weight-bearing joints remains a surgical challenge. Total knee joint transplantation has been performed experimentally, but these studies lacked prospective evaluation of functional outcome, graft vascularization, and graft viability. METHODS: Replantation and transplantation of vascularized knee joints was performed in dogs (n=4 per group), comparing functional and morphological results during a 6-month follow-up. RESULTS: All replant recipients and three transplant recipients survived the 6-month follow-up period. At this time, duplex sonography and angiography revealed patent anastomoses in all animals. Increases in volumetric flow rates and vascular collateralization were observed in allografts, as compared with replanted joints (100+/-16 ml/min vs. 31+/-15 ml/min at 6 months after transplantation). Bone fusion at the graft-host interface was verified by fluorography in all animals at 3 months after transplantation. Six months after transplantation, microradiographies and computerized tomographies revealed spongialization of the cortical bone and filling of the medullary space by trabecular bone in transplanted joints. Such alterations were not detectable in replanted joints. Chondrocyte viability exceeded 80% in all but one transplanted joint. Lymphocyte infiltration of synovia and arterial walls was detected in all transplanted joints, suggesting the presence of chronic rejection. Weight-bearing capacity recovered in all replanted animals (weight-bearing index before transplantation: 0.499+/-0.080; 6 months after transplantation: 0.38+/-0.16) but only in two of four transplanted animals (weight-bearing index 6 months after transplantation: 0.37, 0.28, and 0.00). CONCLUSIONS: These data demonstrate the potential of joint grafting and the critical dependence of allotransplantation on the control of rejection.
Chronic post-traumatic osteomyelitis (OM) represents a particular challenge for nuclear medicine and radiology since clinical and biochemical parameters are frequently unreliable. The aim of this study was to investigate the value of combined bone scan (BS) and immunoscintigraphy (IS) with technetium-99m labelled monoclonal antigranulocyte antibody (MAB) in patients with suspected chronic OM of the appendicular skeleton. Twenty-four patients (17 females and 7 males) with suspected chronic post-traumatic OM were evaluated with three-phase BS/99mTc-MAB-IS. The final diagnosis was established by means of bone culture and histology in 19 cases and clinical follow-up in five cases. The studies were reviewed by two independent and experienced observers; the interobserver agreement was calculated by kappa statistics. The sensitivity, specificity and accuracy of BS alone were 92%, 18% and 58%, respectively. Combined BS/99mTc-MAB-IS had a sensitivity, specificity and accuracy of 84%, 72% and 79%, respectively. Of 24 studies, 11 were true-positive, two false-negative, eight true-negative and three false-positive. Two patients presented with unexpected ectopic haematopoietic bone marrow in the appendicular skeleton that caused false-positive results. A high degree of interobserver agreement was found (kappa=0. 85). It is concluded that combined BS/99mTc-MAB-IS represents a very sensitive and reproducible method with an acceptable specificity for the investigation of chronic OM. Problems may occur in the differentiation of low-grade OM from aseptic inflammation. Another problem is ectopic marrow that may occur in the appendicular skeleton due to a chronic inflammatory stimulus. A former intramedullary intervention in the femur with displacement of haematopoietic marrow may also lead to an ectopic location.
The term primary chronic osteomyelitis covers a heterogeneous spectrum of clinical features that should be separated for therapeutic reasons. Unifocal manifestations should be distinguished from multifocal ones. While bacteria are often found in the first group, bacteriological investigations are usually negative in the second group. Additionally, multifocal forms often recur (chronic multifocal recurrent osteomyelitis), possess a long clinical course and may be combined with dermatological disorders (SAPHO syndrome). A biopsy is often necessary for tumor exclusion and obtaining material for bacteriology. Histology cannot differentiate between the respective forms of chronic osteomyelitis.
A 42-year-old male patient presented with a history of persistent pain in the right femur without trauma of 2 months, duration and an episode of bloody stools 3 months earlier with no clinical findings upon examination. X-rays and CT scan revealed a circumscribed lesion with sclerosis and periostal reaction in the right proximal femur. A three-phase bone scan showed a massive hot spot in this area. Primarily differential diagnoses included a Brodie's abscess and/or a tumor. An excisional biopsy of the area was performed and revealed the coexistence of a bone infraction and the metastasis of an adenocarcinoma of unknown origin. The lesion in the bone was resected, filled with autogenous cancellous bone and stabilized with a plate. Further intensive screening with CT of the abdomen, gastroscopy and colonoscopy led to the primary tumor, an adenocarcinoma at the rectosigmoidal junction. No other metastases were detected. This patient presented with severe pain an radiologically divergent findings: a presumably benign process on radiography, but a massive hot spot on scintigraphy. Further procedures such as a CT scan and/or MRI had to be undertaken. If the analysis includes the differential diagnosis of a malignant process, a biopsy must be obtained, and if this reveals a metastasis, the primary tumor must be sought.
Based on the hypothesis that decreasing osteoblastic function is one of the reasons for the development of osteoporosis, we have studied the proliferation and protein production of isolated bone cells of young and old human donors. The isolation procedure for bone cells is based on a combined mechanical and enzymatical treatment of human trabecular bone. Endosteal bone cells (EBC) obtained by this method developed typical osteoblast-like characteristics in culture. The most important functional feature was the dose-dependent increase of osteocalcin production following stimulation with 1.25(OH)2D3. Growth of EBC (measured as emigration time after plating of trabecular bone fragments) was equal in premenopausal women and men aged under 40 years, but was impaired in EBC of men aged over 50 years. The production of osteocalcin after stimulation with 1.25(OH)2D3 was about 60% lower in older donors (> 50 years) than in younger ones (< 40 years), regardless of gender. According to our results osteoblastic function is reduced with increasing age in human EBC of both genders as clearly shown by a diminished protein production. However, the anticipated depressive effect of age on growth of bone cells was obvious in males only. So called age related osteoblastic insufficiency does exist but it has to be considered differently for bone cell function and bone cell growth. The limited data shown in this study should enhance understanding of age and sex related changes in the EBC metabolism.
A 24-year-old patient is described who had a 4-year history of pain in the right upper arm, with distinct night pain, that responded to salicylates. From the findings on conventional radiography, bone scintigraphy and MRI a multifocal osteoid osteoma was suspected, with one focus in the cancellous region of the greater tuberosity and a second cortical focus at the proximal humeral diaphysis. The resection "en bloc" of both tumors and histological examination confirmed the diagnosis. The patient was painfree after the curative resection of the two osteoid osteomas. Osteoid osteoma is a frequently found benign bone tumor, accounting for approximately 11% of cases. In rare cases a multicentric occurrence has been described. A possible occurrence of more than one osteoid osteoma in a single bone, not verified histologically, has been reported only three times in the literature. In patients with scintigraphic and radiographic findings of two foci, discrete synchronous multifocal osteoid osteomas should be suspected.
OBJECTIVE: A clinical and histopathological comparison of 2D spin-echo (SE) and 3D gradient-echo (3DGE) sequences was undertaken for the knee joint. The purpose of the study was to evaluate the clinical results and to explain the different appearances of meniscal abnormalities on both 2DSE and 3DGE images. PATIENTS, MATERIALS AND METHODS: The clinical study comprised 45 patients with arthroscopically correlated MR imaging results. For the histopathological correlation, seven cadaveric knee joints were examined with the same 2DSE and 3DGE (FISP) imaging protocol and sliced in sagittal sections according to the MR images. Different stainings were used. RESULTS: For the detection of meniscal tears, accuracy (82.2%) and positive predictive value (70.7%) of the 3DGE sequence were limited due to a high number of false positive findings. Cartilaginous lesions were more easily visible on 3DGE than on 2DSE images (sensitivity: 63.1% vs. 52.6%, respectively). As in the clinical study, the meniscal signal abnormalities of the cadaveric knee joints were much more extensive on the 3DGE images than on the 2DSE images. The 3DGE findings correlated better with degenerative meniscal changes which were visible microscopically. CONCLUSION: The high sensitivity of the 3DGE sequence for degenerative meniscal changes explains the lack of specificity for the differentiation between meniscal degeneration and tears with this sequence. The MR grading system for meniscal lesions is of limited value for the evaluation of 3D FISP images.
The clinical and histological data of twenty-three cases of adamantinomas of the long bone collected by the Working Group on Bone Tumors at the DKFZ/FRG are reported including immunohistochemical observations in twenty-one of the cases. Females and males between 5 and 67 years (mean, 25.4 years) were affected equally (11/12). All adamantinomas were positive for cytokeratins often in coexpression with vimentin, at least focally. Although exhibiting varying histological patterns, no correlation between histology and clinical course was seen. However, sex and mode of initial therapy seem to influence an unfavorable clinical outcome. All three decreased patients were males receiving marginal or delayed surgery. This underlines the low-grade malignant character of adamantinoma. To assure the histological diagnosis pathologists should employ immunohistochemistry for demonstrating the sometimes sparse epithelial cell nests when radiology is suggestive for adamantinoma. Correct diagnosis should lead to resection with wide surgical margins.
Benign bone tumors are frequently undiagnosed since they rarely cause pain. Based on the records of the Basle Bone Tumor Registry, which contains 2194 primary benign bone tumors, the aspects of benign bone tumors in childhood and adolescence are described. Benign bone-forming lesions like osteoid osteoma and osteoblastoma occur fairly frequently in this age group; osteofibrous dysplasia is almost exclusively seen in younger patients. Benign cartilage-forming tumors are much more cellular at this age and therefore sometimes misdiagnosed as chondrosarcomas. Chondromyxoid fibroma and especially chondroblastoma frequently occur in patients younger than 20 years. Because of their pleomorphic appearance (chondromyxoid fibroma) and their cellular composition and matrix deposition (chondroblastoma) they can be confused with chondrosarcomas, giant cell tumors or osteosarcomas. Benign fibrous histiocytoma is a very rare intraosseous fibrohistiocytic tumor that also can be found during adolescence. Since no one has much experience with this lesion, patients should be carefully monitored after complete excision. Giant cell tumors of bone reach their typical epimetaphyseal location after growth plate closure. If the physis is still open, giant cell tumors are almost exclusively found in a metaphyseal location abutting on epiphyseal cartilage. With increasing closure of the growth plate, the lesion is found most frequently in an epimetaphyseal position. The transition of a primary benign bone tumor to a sarcoma is very rare and often occurs in connection with irradiation treatment. The risk of a sarcomatous change independent of therapy is very infrequent.
Langerhans cell histiocytosis is a non-neoplastic granulomalike lesion of unknown origin; it is characterized by proliferation of reticulohistiocytic structures, polynuclear eosinophils, neutrophils, lymphocytes, plasma cells, multinucleate giant cells and Langerhans cells. In recent years, the older terms "eosinophilic granuloma" and "histiocytosis X" have been replaced by the more precise expression "Langerhans cell histiocytosis". The granuloma can be solitary and multifocal, with or without soft-tissue involvement. A specific entity is Hand-Schüller-Christian disease. Characteristic lesions in the skull are combined with exophthalmus and diabetes insipidus. These symptoms occur because of the involvement of the hypophysis. A clinically malignant form of the disease is Letterer-Siwe disease. This syndrome occurs in very small children. Dysfunction of various organs causes early death of the individual. The etiology of Langerhans cell histiocytosis is probably a dysfunction of the immune system. In the 130 cases registered at the Swiss bone tumor reference center the lesions were localized in almost all bones. The mandibula, skull, ribs and femur were the most frequently involved bones. Eighty-three patients (64%) were male, 43 (36%) female. Seventy-five percent of the lesions occurred in the first three decades of life. Ninety-three lesions were monostotic and 24 polyostotic; in 5 cases there was soft-tissue involvement, 7 had Hand-Schüller-Christian, and 1 Letterer-Siwe disease. Treatment of osseous lesions should be purely surgical; there is no need for chemotherapy in such cases. If soft-tissue involvement is diagnosed, chemotherapy should be considered. Alternatively, cortisone or interferone therapy can be administered. In our opinion there is no place for radiation therapy.
Functional and morphological results of transplantation (n = 3) and replantation (n = 4) of vascularised total knee joints were compared in a canine model. Follow up time was 6 months. All vascular anastomoses remained patent and all osteotomies healed. Replanted animals displayed normal static weight bearing in contrast to the transplant recipients, which showed reduced weight bearing after 6 months compared to preoperative values. Mild to moderate signs of chronic allograft rejection were detected in all transplanted animals. Functional results were correlated with morphological outcome demonstrating the relevance of adequate control of rejection.
Eight primary leiomyosarcomas of bone were registered in the files of the Basel Bone Tumor Reference Center, Basel, Switzerland, for the period 1972 to 1990. The mean age of the patients (six males and two females) was 43.7 years (range, 11 to 87 years). The tumors were located in the long bones, the fingers, and the clavicle, and presented radiologically mainly as slightly to moderately aggressive lesions (grades IB to II according to Lodwick). They reacted immunohistochemically with antibodies against alpha-smooth muscle actin (alpha-SMA), and total muscle actins (eight of eight), vimentin (seven of eight), desmin (three of eight), keratin (four of eight), type IV collagen (six of eight), laminin (five of eight), and S-100 (one of eight). Seven patients underwent surgery (five, resection; two, amputation). Some of them had received preoperative or adjuvant chemotherapy or radiation therapy. One patient with a metastasized tumor had received chemotherapy only. Tumor recurrences were observed in two cases. Four patients developed metastases of whom two were treated with chemotherapy or tumor resection. During a follow-up period of 1 to 72 months (mean, 46.5 months) four of the eight patients survived for up to 72 months, among them the only patient with grade 3 tumor and treated metastases.