Biomedical subjects
Publications and source records attributed to F Bertoni.
Six cases of ameloblastic fibroma of the jaw are reported, and the radiographic and histologic features and clinical symptoms are described. A conservative surgical approach, including enucleation and mechanical curettage of the surrounding bone, appeared to be successful for these patients.
BACKGROUND: Approximately one-fourth of diffuse large B-cell lymphomas (DLCL) carry the bcl-2(MBR)/JH rearrangement caused by the t(14;18) translocation. The clinical significance of this rearrangement in patients with DLCL remains controversial. By polymerase chain reaction (PCR) we prospectively evaluated the prognostic relevance of the bcl-2 (MBR)/JH rearrangement present in circulating B-cells at the time of diagnosis. MATERIALS AND METHODS: The bcl-2 (MBR)/JH rearrangement was analysed by a nested-PCR method in peripheral blood samples of 51 HIV-negative patients with previously untreated DLCL. RESULTS: The bcl-2 (MBR)/JH rearrangement was detected in 16 cases (31%). Peripheral blood bcl-2 (MBR)/JH rearrangement detection by PCR at diagnosis was correlated with poor overall survival, lymphoma-specific survival and time to progression (log-rank P < 0.05). There was no statistically significant difference between the clinical characteristics at presentation of bcl-2/JH-positive and negative patients. CONCLUSIONS: The peripheral blood is a readily accessible tissue for this type of analysis, and this study indicates that detection of the t(14;18) translocation at presentation in the blood of patients with DLCL may presage a poor prognosis.
Intra-arterial (IA) and intravenous (IV) cisplatinum (CDP) were studied in a multiagent regimen of neoadjuvant chemotherapy for osteosarcoma of the extremities. Preoperatively two cycles of high-dose methotrexate (HDMTX) were administered, followed 5 days later by CDP and Adriamycin (ADM). MTX and ADM were administered IV, and CDP was delivered IA or IV. Postoperatively, good responders received 3 more cycles of the same drugs, while poor responders had a longer chemotherapy including ifosfamide. The rate of good histological response to chemotherapy was significantly higher in patients treated intraarterially (78% vs 46%: P < .004), while no significant differences in terms of disease-free survival were observed between patients who received CDP IA and patients who received CDP IV (55% vs 51%). In the IA group, however, there was only one local recurrence vs 5 in the IV group. The IA infusion of CDP is more active on the primary tumor than the IV infusion.
Various irradiation methods have been employed in the treatment of squamous cell carcinomas of the penis; orthovoltage, interstitial implants, molds and contact radiotherapy have been used, but megavoltage external beam therapy has became prevalent. A summary of tumor control rates achieved with different treatment techniques is presented. Radiotherapy has yielded comparable results in several institutions and ten years crude survival is 50-60% with NED survival of 85%. The tumor control is closely related to the stage of the disease and control rates are comparable for the interstitial and external photon or electron beam therapy. RT with surgery reserved for the management of local recurrence is an effective first line treatment for patients with carcinoma of the penis with acceptable morbidity and high proportion of long term survivors. Radiotherapy seems to be the treatment of choice in the limited cancer of the penis if preservation of a functioning organ is the major therapeutic aim.
Fibrous dysplasia and Ollier's disease (skeletal chondromatosis) are bone lesions that result from disorders occurring during ossification. Here we report on a patient suffering from Ollier's disease in whom polyostotic fibrous dysplasia was detected. The main problem for this 20-year-old man was a nasoethmoidorbital cartilaginous mass causing nasal obstruction, nonreducible eye proptosis, and sleep apnea. The lesion was approached by means of a combined route, ie, transfrontally and transfacially (nasal-cheek flap). The possible pathogenetic links existing between the two lesions and the problems involved in surgical treatment are discussed.
BACKGROUND: Increased levels of P-glycoprotein occur in some osteosarcomas. In this study we determined the relation between P-glycoprotein status and outcome in patients with high-grade osteosarcoma. METHODS: P-glycoprotein status was determined immunohistochemically in specimens of osteosarcoma of the extremities (stage II) from 92 patients who were treated with surgery and chemotherapy. The P-glycoprotein status was analyzed in relation to the length of event-free survival. RESULTS: The presence of increased levels of P-glycoprotein in the osteosarcoma was significantly associated with a decreased probability of remaining event-free after diagnosis (P = 0.002). In a multivariate analysis, P-glycoprotein status (P = 0.001) and the extent of tumor necrosis after preoperative chemotherapy (P = 0.04) were independent predictors of clinical outcome. The risk of adverse events was increased substantially (rate ratio, 3.37; 95 percent confidence interval, 1.60 to 7.10) among patients with increased levels of P-glycoprotein in tumor cells, as compared with patients who did not have increased levels of P-glycoprotein in tumor cells. CONCLUSIONS: In patients with high-grade osteosarcoma treated with surgery and chemotherapy, the presence of increased levels of P-glycoprotein in tumor cells is associated with a significantly increased risk of adverse events and is independent of the extent of necrosis after preoperative chemotherapy.
BACKGROUND: Osteoma of the skull and facial bones is not uncommon, is usually asymptomatic, and may be associated with Gardner's syndrome. Osteomas involving other bones are unusual. METHODS: The authors describe 14 cases (eight men and six women from age 21-66 years) of parosteal osteoma of bones other than of the skull and face. RESULTS: Thirteen lesions involved long bones, and one involved the clavicle. A long-standing (18 months to 31 years) mass was reported in seven patients. Symptoms of pain, a mass, or both were present in all except three patients who were asymptomatic. Lesions ranged from 2.5 to 20 cm in greatest dimension. Histologically, they blended with the cortex, did not infiltrate the medullary cavity, and consisted of dense sclerotic lamellar bone with haversian systems, similar to the architecture of normal cortical bone. There was no spindle cell proliferation. Nine patients underwent resection, four had biopsy, and one had debulking. With a follow-up of 1-23 years, no recurrence or metastasis was reported. CONCLUSIONS: Parosteal osteoma must be distinguished from parosteal osteosarcoma, a low grade malignant neoplasm. If radiographs do not identify areas of radiolucency and sections do not contain spindle cells, the diagnosis of parosteal osteoma is justified.
A case of multicentric metachronous GCT was presented. The clinical and radiographic features of multicentric GCT as reported in the literature and in the present case were discussed. The multiple bone involvement made therapeutic assessment difficult. The surgical stage as well as the location of each lesion were important in planning surgery.
PURPOSE: Preclinical studies showed lonidamine to potentiate the effects of x-irradiation by inhibiting the repair of potentially lethal damage. This Phase III double blind, placebo-controlled study was performed to evaluate whether lonidamine can increase the tumor control of radiotherapy in the treatment of advanced head and neck cancer without any synergistic toxic effects on the exposed normal tissues. METHODS AND MATERIALS: Ninety-seven patients with Stages II-IV squamous cell carcinoma of the head and neck were enrolled. Separate analyses were done on the 96 eligible patients and the 90 patients who completed the prescribed treatment regimen. Patients received radiotherapy up to a planned total of 60-66 Gy, in 2 daily fractions of 1.5 Gy each and either lonidamine (450 mg p.o. in three divided daily doses) or placebo, given continuously for 3 months or up to 1 month after the end of radiotherapy. RESULTS: The rate of tumor clearance was 66% (32/48) in the lonidamine group and 65% (31/48) in the placebo group, while the subsequent failure rate was 50% and 77%, respectively (p < 0.05). The 3 and 5 year locoregional control rates in the adequately treated patients achieving complete tumor clearance were 66% and 63% for lonidamine vs. 41% and 37% for placebo. The disease-free survival in adequately treated patients was significantly better in the lonidamine group (p < 0.03), with 3 and 5 year rates of 44% and 40%, respectively, vs. 23% and 19% in the placebo group. The overall survival rate for all eligible patients at both 3 and 5 years was 44% in the lonidamine group and 44% and 31%, respectively, in the placebo group. Both acute and late radiation reactions were similar in the two groups. Myalgia and testicular pain were the most frequent side effects of lonidamine with an incidence of 8.5% and 4.2%, respectively. CONCLUSION: The addition of lonidamine to hyperfractionated radiotherapy was correlated with a statistically and clinically significant proportion of long-term disease-free patients. The toxicity of radiotherapy was not aggravated by the drug and the overall tolerance of the combined regimen was acceptable.
Ploidy was studied with flow and image cytometry in 51 polyps removed endoscopically from 44 patients. Evaluation was carried out on frozen material in 34 cases and on material fixed in formalin and embedded in paraffin in the remaining 17. Data analysis showed a statistically significant correlation between polyp size and aneuploidy frequency (P > 0.05). No statistically significant correlation was found between aneuploidy frequency and histological type. The linear correlation study did, however, show a correlation tendency between histological type and aneuploidy (R = 0.42211).
Five cases of ameloblastic fibrosarcomas (AFS) are reported. The tumour was characterized histologically by a biphasic pattern: the malignant mesenchymal component had the features of an intermediate grade fibrosarcoma in 3 cases, malignant fibrous histiocytoma and osteogenic sarcoma in 2 cases. The epithelial odontogenic component had a benign appearance cytologically. In 1 patient, in the recurrence only the malignant mesenchymal component was present. AFS is a fully malignant tumour, in fact 1 patient died of the tumour after inadequate surgical treatments, and 2 patients had a recurrence after intralesional surgery. The treatment of choice was achieved when surgery with wide surgical margins was performed. As MFH and OGS features are present in the malignant mesenchymal component of this tumour we prefer to use the broad term ameloblastic sarcoma instead of AFS.
BACKGROUND AND METHODS: Of the 200 cases of ABC in the Rizzoli Institute files, 15 had solid features on both gross and histologic examination. Inasmuch as fibrous proliferation with giant cell and bone production along with fibromyxoid areas and small aneurysmal spaces were found in the solid parts of the aneurysmal bone cyst, a grossly solid and radiographically osteolytic bone lesion with these microscopic features was called a solid aneurysmal bone cyst. Some authors call the same lesion extragnathic giant cell reparative granuloma. RESULTS: Sixty percent of the patients were female. The metaphysis was the preferred location in the long bones (8/11). Radiographic appearance was not specific, and sometimes a malignant lesion was very difficult to rule out. In seven patients, the lesion was considered radiographically "aggressive." Intralesional excision (curettage) in 12 patients and marginal resection in 3 patients with diaphyseal location was effective in controlling the lesion. No recurrence was detected after a mean follow-up of 59 months. CONCLUSIONS: High proliferative activity of the benign-appearing proliferative spindle cells, often with fairly abundant mitoses, associated with benign giant cells and immature bone production are the features of this pseudosarcomatous hyperplastic lesion. It is sometimes is mistaken for a malignant tumor.
BACKGROUND: Low-grade intraosseous osteosarcoma is a rare variety of osteosarcoma and it is difficult to recognize. METHODS: A series of 10 patients with low-grade intraosseous osteosarcoma is reported. These patients were identified from the Istituto Rizzoli files, which includes approximately 1000 cases of osteosarcoma. Clinical data and radiographic and histologic features were studied. RESULTS: The radiographic appearance confirmed malignancy in five patients and suggested it in two. A benign lesion was diagnosed in three patients. Three lesions resembled parosteal osteosarcoma, two appeared similar to fibrous dysplasia, and two had features of desmoplastic fibroma. A mixed histologic pattern was found in three other tumors. Recurrence after intralesional excision in all patients indicated the aggressive nature of this lesion. The development of metastases in two patients and progression in the grade of malignancy in one of these highlighted the malignant nature of the tumor. CONCLUSIONS: The correct diagnosis would permit adequate treatment with wide surgical margins.
Of 779 patients undergoing repair of abdominal aortic aneurysms over a 7-year period (1984-1990), 40 (5.1%) had gross features of inflammatory abdominal aortic aneurysms (IAAAs). Twenty IAAAs were assessed by CT scan preoperatively and postoperatively to evaluate the outcome of the inflammatory layer of the aneurysm in 19 cases. Complete postoperative regression was observed in nine cases (47.3%), partial regression in four (21%), and stable lesions in six (31.7%). No roentgenographic progression was found. The comparison between the roentgenologic outcome and preoperative clinical features (age, sex, erythrocyte sedimentation rate, and abdominal lumbar pain), pathologic findings, and follow-up time revealed a significant correlation (p < 0.05) between the postoperative outcome and the histologic findings in the wall (cell density and cell/fibrosis ratio). Complete regression of inflammation was observed when high cell density (16 +/- 0.7 cells/2116 microns 2) and a cell/fibrosis ratio > 1 were found. On the contrary, little or no regression of inflammation occurred when a low cell density (3.4 +/- 0.3 cells/2116 microns 2) and a cell/fibrosis ratio < 1 were found. Although it is generally thought that inflammation in IAAAs regresses after surgical repair, in our study, 31.7% of the postoperative CT scans showed no change. Histologically, the variability of morphologic aspects seemed to correlate with the relative proportions of cellular infiltrate and interstitial fibrosis in the aneurysmal wall. These proportions determine the postoperative course of the inflammation layer and, most likely, the response of the latter to steroid therapy as well.
A 72-year-old woman was referred to our hospital for diarrhea, abdominal and back pain, weight loss, low serum potassium level. Pathological findings and high circulating Vasoactive Intestinal Peptide (VIP) levels allowed us to diagnose "VIPoma syndrome". The patient underwent a treatment with SMS 201-995 with improvement of symptomatology and reduction of circulating VIP levels. After a wash-out period the patient was treated with salmon calcitonin with a net improvement of diarrhea, normalization of potassium serum level and reduction of VIP level. The symptomatology recurred after calcitonin withdrawal. These data confirm the effectiveness of SMS 201-995 in the VIPoma syndrome and suggest that calcitonin may ameliorate symptomatology and reduce circulating VIP level in patients with VIPoma tumor.
Inflammatory aneurysms (IAs) have peculiar macroscopic and histological aspects which make them very different from nonspecific aneurysms (NSAs). These morphological differences seem to be determined by significant modifications of the extracellular matrix. Extracellular matrix protein component concentrations were determined biochemically in infrarenal aortic biopsies from 10 NSAs, five IAs and five non-aneurysmal aortic controls. The concentration of each wall component was expressed in % w/w (relative concentration) and in mg/wall longitudinal cm (absolute concentration) with reference to total protein recovered after hydrolysis and amino acid analysis. The biochemical results were correlated with the histological and ultrastructural features of the specimens. A significant increase in total collagen was observed in the two groups of aneurysms, with respect to the controls (NSA = 285%, IA = 382%). In contrast the 80-90% decrease in the relative concentration of elastin observed in both types of aneurysm was less marked (NSA = 55%, IA = 39%). This fall was not significant when expressed in mg/cm, although elastin derived peptide (EDP) levels in the plasma of these patients was significantly higher than in age-matched controls. The concentration of the soluble collagen fraction appeared significantly higher (Mann-Whitney, p < 0.05) in the IAs with respect to the NSAs, whilst no differences were observed between the two groups regarding the concentration of insoluble elastin and of wall and plasma EDPs. As well as providing evidence of increased elastin turnover, this study emphasises the conspicuous modifications of collagen deposition in the wall of abdominal aortic aneurysms which appeared more marked in the inflammatory group.(ABSTRACT TRUNCATED AT 250 WORDS)
We studied 12 cases of a rare primary bone neoplasm--fibrocartilaginous mesenchymoma--including five from the original report on this condition. The seven male and five female patients were 9 to 25 years old. The metaphyses of the long bones were the most common site, with the fibula accounting for a third of all cases. Histologically, the lesion contained spindle cells, bone trabeculae, and islands of cartilage. At least some of the cartilage was in the form of plates that resembled epiphyseal plates. Intralesional excision led to a high rate of recurrence, but no metastasis or death was related to the tumor. Our results indicate that the entity described by Dahlin and co-authors is histologically distinct and that its behavior does not merit the term malignant.
We performed echo-guided percutaneous ethanol injection of the lesion in 26 cirrhotic patients with 33 hepatocellular carcinomas measuring 3 cm in diameter or less. Post-treatment lesions had disappeared in 4 patients, while in the remaining 22 patients the echo pattern of the lesion had changed and biopsies were negative. Fifteen patients (follow-up 11-30 months) remain disease-free, while recurrence occurred between 3 and 36 months in 7 cases. Percutaneous ethanol injection was successfully repeated in 4 cases; 5 patients died, 1 of HCC, 2 of esophageal variceal bleeding, and 2 of liver failure; 3 of the last four patients had Child's C disease. Percutaneous ethanol injection is a valuable treatment for hepatocellular carcinomas measuring not more than 3 cm. It has yet to be established how far this treatment influences the survival rates of patients and the natural course of hepatocellular carcinoma.