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Salmonella osteomyelitis without predisposing factors is seldom seen and thus difficult to diagnose. We report on a 14-year-old healthy boy with Salmonella osteomyelitis which occurred 2 years after trauma. Radical operative debridement is recommended. Intravenous ciprofloxacin has proved to be effective because of good tissue penetration and sensitivity towards Salmonella.
Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.
PURPOSE: To define MR imaging characteristics of primary aneurysmal bone cyst. MATERIALS AND METHODS: MR imaging studies of 38 patients with histologically proven primary aneurysmal bone cyst were reviewed with reference to morphological features, signal characteristics, and patterns of contrast-enhancement. RESULT: Most lesions were well marginated towards bone and soft tissues (95%), either surrounded by a complete (84%) or incomplete (16%) rim of low signal intensity on images of all pulse sequences. Frequent features were polycyclic margins (84%), cortical expansion (87%), cystic spaces (100%), contrast-enhancing cyst walls (100%), internal septations (89%), fluid levels (71%) and diverticula-like projections of cyst walls (68%). Solid tissue components could be identified by MR imaging in all lesions which, on pathological examination, contained larger portions of solid material (18%). Edema of surrounding soft-tissues was observed in 29% of the cases. CONCLUSIONS: Primary aneurysmal bone cysts demonstrate a relatively uniform MR imaging appearance, which reflects the patho-anatomic composition of the lesion.
The purpose of this study was to monitor radiation-induced alterations of the blood-bone marrow barrier (BMB) and the reticuloendothelial system (RES) with AMI-227-enhanced magnetic resonance imaging (MRI). Twenty New Zealand white rabbits (n = 10 following total body irradiation and n = 10 controls) underwent AMI-227-enhanced MRI. Pulse sequences included dynamic fast low-angle shot (FLASH; TR/TE 50/4 msec, flip angle 60 degrees) MRI and static T1- and T2-weighted spin-echo (SE) and turbo-SE sequences of the lumbar spine and sacrum. Bone marrow enhancement was quantified as delta signal intensity (SI) (%) =|[(SIpost - SIpre)/SIpre] x 100%| and compared with histopathology, including iron stains and electron microscopy. Dynamic bone marrow deltaSI (%) data steadily increased up to 10-15 minutes after AMI-227 administration, while blood deltaSI (%) data stayed nearly constant, histologically corresponding to iron oxide leakage into the bone marrow interstitium. This bone marrow contrast enhancement increased significantly following irradiation, corresponding to alterations of the endothelial lining of the bone marrow sinusoids. Late postcontrast images exhibited a significant positive T1 enhancement and negative T2 enhancement of the normal bone marrow, which further increased with irradiation due to increased RES activity. Irradiation-induced changes in bone marrow physiology could be reliably assessed with AMI-227-enhanced MRI.
Thirteen patients with aneurysmal bone cyst of the spine (excluding sacral lesions) were retrospectively reviewed. Treatment for aneurysmal bone cysts remains controversial, but surgical resection, irradiation, and embolization are common treatment modalities for those involving the spine. Of 102 patients with aneurysmal bone cysts, 15 had a lesion of the spine, including 2 sacral cases. Of the 13 patients with a lesion of the thoracic or lumbar spine, 9 underwent resection of the lesion, 2 curettage and cementation, and 2 only currettage. Eleven patients underwent segmental arthrodesis with instrumentation after treatment of the primary or recurrent lesion, while 2 patients underwent segmental arthrodesis using autogeneic bone. Nine patients did not develop a local recurrence after resection of the lesion. However, the 2 patients who underwent curettage alone developed local recurrences. None of 4 patients developed recurrences after curettage and cementation. After recurrence, 1 patient underwent additional resection with irradiation, and 1 patient underwent resection alone. At the final follow-up, all lesions were under control. In one patient, lumbar kyphosis developed after segmental arthrodesis with instrumentation, and arthrodesis was performed again. Radical resection of aneurysmal bone cysts of the spine with instrumentation is the optimal method of acquiring a high degree of local control and preventing spinal deformity.
BACKGROUND: The lethal midline granuloma is a heterogeneous disorder and the pathogenesis is likely to be complex. Advances in immunocytochemical phenotyping and molecular genetics suggest that in several cases the origin have been proven to be malignant lymphomas, especially of T-cell lineage. PATIENT: The present clinical and pathological cases of a 35-year-old woman shows how difficult it is to establish the precise diagnosis of lethal midline granuloma (LMG). The patient presented with recurrent infections of the paranasal sinuses. Later she developed infiltrates and ulcerous lesions of the nose, paranasal sinuses, and the base of the skull clinically consistent with a LMG. Immunohistologic studies using monoclonal antibodies led to the positive diagnosis of peripheral lymphoma of T-cells. RESULTS: Several surgical interventions and explorations revealed a diffuse infiltration and partly necrotic lesions of the tissue. After identification of a T-cell lymphoma by histological examination, the patient received megavoltage irradiation of 45 Gy. Additional chemotherapy with alkylating agents was given. Two years after treatment the patient achieved and remained in remission. CONCLUSION: The midline granuloma syndrome is a mutilating process produced by a number of diseases that progressively destroy the nose, paranasal sinuses and other regions of the midface. Infectious, neoplastic and idiopathic forms have been described. The specific diagnosis must be ascertained, as the treatment is different depending of the etiology of the disease. Sinonasal lymphomas constitute a important distinct clinicopathologic entity which may present as lethal midline granuloma. They seems to be strongly associated with Epstein-Barr virus. Aggressive therapy with radiotherapy and chemotherapy can stop the progress of the midfacial destruction. The correct treatment is still discussed controversially.
BACKGROUND: Heat shock proteins (HSPs) are produced by cells in response to a wide variety of stresses. To determine a possible relationship between hemodynamic parameters and HSP 70 in the early postoperative period after heart transplantation, we examined immunohistochemically the inducible HSP 70 (anti-HSP 72) response in human heart biopsies, as well as the effect of myocardial rejection on HSP. METHODS: A total of 105 routinely processed endomyocardial biopsies from 15 consecutive patients who underwent heart transplantation were examined. Analysis of hemodynamic and echocardiographic parameters were performed within 30 min and 12 hr after the biopsies. RESULTS: Immunohistochemically detected inducible HSP 70 was mainly located in the cytoplasm and nucleus/nucleolus of cardiomyocytes. Two specimens additionally showed HSP 70-positive interstitial cells and smooth muscle cells of arteries, whereas lymphocytes were consistently negative. There was a significant relation between the echocardiographically determined increased relaxation time and positive HSP 70 staining (P < 0.011). Patients with elevated right atrial pressure (P < 0.098), as well as those with increased left ventricular end systolic diameter (P < 0.06), showed a trend to higher HSP expression. Three patients who died of sepsis or multiorgan failure showed significantly higher cytoplasmic HSP 70 expression compared with 12 patients with stable clinical course. In case of rejection, significantly more patients showed no HSP expression. CONCLUSION: Although only five patients showed organ rejection, our results suggest an inverse relationship between HSP expression and rejection with the possibility of a role for HSP 70 as a graft marker to assess graft function.
We treated a patient with large multiple chondrosarcomas derived from multiple cartilaginous exostoses. One sarcoma originated in the left pubic bone and the other sarcoma in the posterior aspect of the greater trochanter of the left femur. Thirty months after hindquarter amputation, the patient is alive without relapse. This is the first report of a patient with synchronous multiple malignant transformation of multiple cartilaginous exostoses.
OBJECTIVE: To evaluate the conventional X-ray and MR imaging features of malignant fibrous histiocytoma (MFH) of bone. DESIGN: MRI examinations and conventional radiographs were reviewed in 39 patients with biopsy-proven MFH. Imaging characteristics were analyzed and the differential diagnoses assessed in a masked fashion by two experienced radiologists. RESULTS: Typical X-ray features included aggressive, destructive tumor growth centrally located in the metaphysis of long bones. Periosteal reactions and expansive growth were rarely seen. On MR images extraosseous tumor spread was frequently noted. On T2-weighted images and contrast-enhanced T1-weighted images most of the tumors displayed an inhomogeneous, nodular signal pattern with peripheral Gd-DTPA enhancement. CONCLUSIONS: Although several MR imaging criteria were typical for MFH none of them was specific. X-ray diagnosis of MFH may also prove difficult, with the main differential diagnosis being metastasis in the older and osteosarcoma in the younger population.
So far, only 25 melanotic progonomas have been found in the central nervous system (male/female ratio 3.5), mostly located in the cerebellum. The average age is 8 years (range 3.5 months to 69 years) with 85% becoming clinically apparent in the first decade of life; 73.7% of the patients reported succumbed to their disease at a mean age of 2.8 years, with a postoperative survival time of just 9 months. Systemic metastases were reported in 9 cases and had mostly spread via cerebrospinal fluid. In contrast to peripheral melanotic progonomas usually found in the maxilla, cerebral progonomas have a much worse outcome and have to be regarded as malignant. We present the case of a 1-year-old boy suffering from a melanotic progonoma of the pineal gland, who died at the age of 22 months with extensive spinal and abdominal metastases 10 months after partial removal of the tumor.
Retroperitoneal paraganglioma is a rare tumor, especially occurring in childhood and adolescence, with a marked tendency to become biologically malignant. It has not been possible to predict the clinical outcome of paraganglioma patients by conventional histology, hence malignancy can only be demonstrated by the occurrence of metastatic lesions. Currently, only limited information on the genetics of this tumor is available. We report on a 16-year-old girl with a large retroperitoneal paraganglioma and an osseous metastasis to the first lumbar vertebra. In addition to morphological and immunohistochemical examinations, a molecular cytogenetic analysis was performed. Comparative genomic hybridization (CGH) revealed imbalanced chromosomal aberrations with a loss of chromosome 1p and a gain of 1q, indicating isochromosome 1q. A loss of chromosome 3 as well as low-level gains of chromosomes 4, 5, 6q, 11q and 13q were detected. A PCR-based microsatellite analysis of 1p confirmed the loss of heterozygosity, including NB1 and NB2 putative tumor-suppressor gene regions. Telomerase activity, which is found in the majority of malignant tumors, could not be detected. The case presented here is the first more comprehensive molecular genetic analysis of a sporadic malignant paraganglioma.
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Osteosarcoma is one of the most commonly biopsied primary tumor of bone. High-grade osteosarcomas in particular exhibit a wide spectrum of cytogenetic changes. Molecular cytogenetic studies on osteosarcomas have shown that genomic amplification, especially of both the TP53-binding MDM2 gene and the flanking SAS gene, plays an important role in the biology of these tumors. We applied CGH in order to obtain a global view of DNA-sequence losses and gains in osteosarcoma. CGH was performed on 20 high-grade medullary osteosarcomas (13 primary tumors prior to chemotherapy, 5 tumors after chemotherapy, 2 established cell lines [MB63, HOS58]) using genomic DNA of snap-frozen tumor specimens. CGH revealed DNA copy number aberrations, mostly gains, in all the tumors studied with an average of 18.5 aberrations/tumor (range 8-32). High-level amplifications were observed in all cases (average 4.1 amplifications/tumor [range 1-10]). Amplicons affecting at least five tumors were mapped to 1p21-31 (9/20 cases), 3q25-qter (6/20), 6p12-21 (6/20), 8q12-qter (10/20), 12p11-12 (9/20), 12q12-15 (enclosing MDM2 and SAS loci, 7/20). Losses were most frequently seen at 3p, 10q, 11p and 13 (all 10/20). In conclusion, our CGH data indicated that genomic amplification plays an important role in the biology of osteosarcoma. CGH demonstrated the complexity of genetic aberrations in osteosarcomas. The detection of novel non-random DNA amplifications in our study has defined regions for further targeted molecular genetic research aimed at identifying those oncogenes that are characteristic of osteosarcoma development.
BACKGROUND: Heat shock proteins (HSP) are induced by a variety of stress and are presumed to play an important role in protecting cells from the effects of stress. Some evidence exists that HSP is involved in allograft rejection. Recently, an increase of inducible HSP 70 in heterotopic rat heart allografts was shown by quantitative Western blotting. To determine a possible mRNA induction and the localization of inducible HSP 70, we examined 19 heart transplants in rats. METHODS: Fisher F344 rat hearts were heterotopically transplanted into Lewis recipients (n=10), and nine cardiac isografts (Fisher to Fisher) were performed. The 19 native hearts of the recipients served as controls. Animals were killed on posttransplantation days 1, 3, and 5. The hearts were examined immunohistologically for inducible HSP and analyzed by a semiquantitative polymerase chain reaction for inducible mRNA. RESULTS: The level of HSP 70 mRNA in the allograft increased from day 1 to 3 and day 3 to 5 after transplantation and was significantly higher than that of time-matched isografts (0.92+/-0.49 vs. 0.49+/-0.05 and 1.14+/-0.53 vs. 0.53+/-0.15; P<0.05). The native hearts showed no elevated HSP 70 expression compared with isografts. Immunohistochemically, the majority of inducible HSP was located in cardiomyocytes adjacent to infiltrating lymphocytes, which where consistently negative. CONCLUSIONS: These results demonstrate that HSP mRNA expression in cardiac allografts is time-dependent, and its protein is expressed in cardiomyocytes.
Surgery of Ewing's sarcoma sometimes results in an inadequate surgical margin. The influence of intraoperative brachytherapy on local control of the tumor, operation time, blood loss, and surgical complications was evaluated, comparing the results of 20 patients who received brachytherapy to a series of 42 patients receiving surgery without brachytherapy. The dose of intraoperative brachytherapy ranged between 9 Gy and 21 Gy. The average operation time was longer in 20 cases with brachytherapy (7.9 h) than in 42 cases without brachytherapy (4.3 h) (P < 0.0001). The average blood loss in the groups with (3531 ml) and without brachytherapy (3515 ml) was comparable (P = 0.3840). The surgical complication rate in patients receiving brachytherapy was also similar to that of untreated patients (30% versus 31%, P = 0.7690). Local relapse developed in 1 of 20 patients who received brachytherapy and 1 of 42 patients without brachytherapy. On the basis of this analysis, it can be concluded that this procedure is safe and does not increase of the acute complication rate. The latest results of local controls are awaited.
The influence of preoperative irradiation on surgical complications in 42 patients with Ewing's sarcoma was analysed. After preoperative irradiation and chemotherapy, 35 of 40 patients showed a good histological response and 25 of 40 patients had no viable tumour cells in the resected specimen. Local relapse alone did not develop, local relapse and metastasis developed in 2 patients and metastasis alone in 15 patients. Surgical complications appeared in 12 of 42 patients: 9 of 19 central tumours (19 pelvic lesions), 1 of 13 proximal and 2 of 10 distal tumours. Surgical complications after preoperative irradiation are distributed as follows: delayed wound healing 8, hematoma 2, thrombosis 2, skin infection 1, and abscess 1. On the other hand, complications appeared in 2 of 28 patients without preoperative irradiation: none in 9 patients having central tumours including 2 pelvic lesions, 1 in 12 patients with proximal tumours, and 1 in 7 patients with distal tumours. The multivariate regression test showed that the tumour site (central) is an influencing factor in the appearance of surgical complications. In central tumours, the surgical complication rate increases after preoperative irradiation; however, it is affected by the increase of the ratio of patients with pelvic tumours.
Thirty-one patients with chondrosarcoma of the pelvis were reviewed. The median followup period of the surviving patients was 66 months. Thirteen of 23 tumors after surgery with inadequate margins (57%) and 1 of 8 after surgery with adequate margins (13%) relapsed locally. Four of 10 patients had local recurrence, 2 of 2 patients had metastasis, and 4 of 4 patients who had local recurrence and metastasis died. A cumulative 10-year overall survival of patients with pelvic chondrosarcoma was 54%. Patients who had primary or high grade chondrosarcoma had a poor prognosis. Multivariate analysis (Cox proportional hazard model) showed that tumor type was the most important prognostic factor. Eight patients underwent hindquarter amputation, 6 patients had resection alone, and 17 patients had resection and reconstruction as follows: 3 interpubic arthrodeses, 2 iliosacral arthrodeses, 3 ischiofemoral arthrodeses, 8 hip prostheses (3 prostheses alone and 5 prostheses with allograft), and 1 hip transposition. Failure of reconstruction was attributed to infection in 5 patients, local recurrence of tumor in 5, and local recurrence and fracture of implant in 1 patient.
21 years after radiotherapy for breast cancer, a 63-year-old woman developed a malignant mesenchymoma of the chest wall. The total irradiation dose was 132 Gy. The first clinical symptom of this second malignancy was a slight irregular calcification around the implanted silicon protheses observed in a conventional chest X-ray. Radiation-induced sarcoma is a very rare complication of radiotherapy. In cases of chest wall calcification after radiation therapy further investigation should be carried out, because some patients with radiation-induced sarcoma could be saved, if an early diagnosis is reached.