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Biomedical subjects

F Bertoni

Publications and source records attributed to F Bertoni.

At least 19 recordsLinked to original sources
PubMed2001

Aneurysmal bone cyst of the mobile spine: report on 41 cases.

STUDY DESIGN: Forty-one cases of aneurysmal bone cyst of the mobile spine were retrospectively reviewed. OBJECTIVES: To evaluate the role of surgical and nonsurgical treatment of aneurysmal bone cyst of the spine. SUMMARY OF BACKGROUND DATA: Ten to 30% of aneurysmal bone cysts arise from the mobile spine, frequently occurring in pediatric patients. The course of the disease depends on the aggressiveness of the tumor, as well as the treatment. Intralesional surgery seems to be an effective treatment, as well as radiotherapy and embolization. METHODS: All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical staging of these cases. Thirty-two patients underwent curettage (14 of them followed by radiotherapy), four were submitted to selective arterial embolization, three received radiotherapy alone, and two underwent en bloc-excision. RESULTS: All patients were found alive and disease free at final follow-up evaluation. Two recurrences followed one incomplete curettage and one embolization. The combination of curettage and radiotherapy, although effective, showed the greatest incidence of late axial deformity. Selective arterial embolization was curative in three of four cases and did not affect the possibility of surgery in case of local recurrence. CONCLUSIONS: If confirmed on larger series, selective arterial embolization seems to be the first treatment option for spine aneurysmal bone cyst, because of the low cost-to-benefit ratio. Diagnosis must be certain, based on pathognomonic radiographic pattern or on histologic study.- In case of neurologic involvement, pathologic fracture, technical impossibility of performing embolization, or local recurrence after at least two embolization procedures, complete intralesional excision would be the therapy of choice.

Adolescent↗
PubMed2001

Metalloproteinase expression and prognosis in soft tissue sarcomas.

BACKGROUND: Degradation of extracellular matrix by tumor-associated proteases can promote cell invasion and metastasis. This study assessed the prognostic role of MMP2, MMP9 metalloproteinases, and of the metalloproteinase inhibitor TIMP2, related to disease-free survival (DFS), in soft tissue sarcoma (STS) patients. MATERIALS AND METHODS: Level and distribution of MMP2, MMP9, and TIMP2 expression were evaluated on 73 biopsies by immunohistochemistry and immunoblotting. Biopsies included 29 liposarcomas, 29 synovial sarcomas, and 15 malignant peripheral nerve sheath tumors (MPNST). Association between DFS and overall survival with different variables was assessed. RESULTS: In terms of DFS, increased MMP2 reactivity and lack of TIMP2 expression were significant for poor prognosis in all samples (P = 0.0005 and P = 0.006 respectively). MMP2 correlated to histologic grade (P = 0.005). Lack of TIMP2 expression was a poor prognostic factor for DFS in synovial sarcoma (P = 0.009), while MMP2 and MMP9 correlated with metastasis (P = 0.008 and P = 0.005, respectively) and grade (P = 0.001 and P = 0.04 respectively) in liposarcoma. CONCLUSIONS: These prognostic markers that influence growth and spread of tumor cells might be useful to define tumor aggressiveness and risk of the metastasic event.

Adolescent↗
PubMed2001

A comparison of methods of loco-regional chemotherapy combined with systemic chemotherapy as neo-adjuvant treatment of osteosarcoma of the extremity.

AIM: Our experience of pre-operative intraarterial (i.a.) vs intravenous (i.v.) infusion of cisplatinum (CDP) in a multiagent neo-adjuvant chemotherapy for osteosarcoma of the extremity is reported. METHODS: Two successive randomized studies were performed. In the first, pre-operatively, CDP i.a. vs CDP i.v. was applied in combination with high-dose methotrexate (HDMTX) and adriamycin (ADM) within a three-drug regimen. In the second, a combination of HDMTX, ADM and IFO, within a four-drug regimen was tested. RESULTS: The rate of responses to chemotherapy (tumour necrosis > or = 90%) was significantly higher (P<0.04) for the 142 patients treated with the four-drug regimen than in the 79 patients treated with a three-drug regimen (76%vs 62%). According to the route of CDP infusion, in the three-drug regimen the rate of responses was significantly higher (P=0.004) in patients treated with i.a. CDP (77%) than in patients treated i.v. (46%); with the four-drug regimen the rate of response was not significantly different in patients treated i.a. (81%) and in patients treated i.v. (71%). No significant differences in the rates of limb salvages, local recurrence and event-free survival (EFS) were seen between the i.a. and the i.v. groups. CONCLUSION: In the treatment of osteosarcoma of the extremity, the i.a. infusion of CDP does not offer any significant advantage when this drug is used within an aggressive, multiagent, pre-operative four-drug regimen.

Adolescent↗
PubMed2001

Neoadjuvant chemotherapy for high grade osteosarcoma of the extremities: long-term results for patients treated according to the Rizzoli IOR/OS-3b protocol.

The results of the Rizzoli IOR/OS-3b neoadjuvant protocol for the treatment of osteosarcoma of the extremity are reported. Preoperative chemotherapy consisted of two cycles of high-dose methotrexate (HDMTX i.v.), followed by a combination of cisplatin (CDP i.a.)/ doxorubicin (ADM i.v.). Postoperatively all patients, regardless of the histologic response, received 3 more cycles of MTX, CDP/ADM alternated with 3 cycles of ifosfamide. In the study performed between January and December 1992 43 patients were enrolled and limb salvage was performed in 39 of them (91%). The histologic response to chemotherapy was good (90% or more tumor necrosis) in 24 patients (56%) and poor (less than 90% tumor necrosis) in 19 (44%). With a minimum follow-up of 7 years, 23 pts (53%) remained continuously free of disease, 19 relapsed and one died due to unrelated cause. In spite of the high number of limb salvages performed, only 2 local recurrences were registered. The 7-year event-free survival and overall survival were, respectively, 53% and 68%. The hematopoietic and extrahematopoietic toxicity experienced by the patients during the entire treatment was relatively mild. These long-term results confirm that, with neoadjuvant chemotherapy, it is possible to cure more than 60% of patients with osteosarcoma of the extremities, avoiding amputation in most of them. These results, however, are no better than those achieved in our previous study IOR/OS-3a, in which only poor responder patients received ifosfamide during the postoperative treatment.

Adolescent↗
PubMed2000

Long-term outcome for patients with nonmetastatic osteosarcoma of the extremity treated at the istituto ortopedico rizzoli according to the istituto ortopedico rizzoli/osteosarcoma-2 protocol: an updated report.

PURPOSE: To provide an estimate of long-term prognosis for patients with osteosarcoma of the extremity treated in a single institution with neoadjuvant chemotherapy and observed for at least 10 years. PATIENTS AND METHODS: Patients with nonmetastatic osteosarcoma of the extremity were preoperatively treated with high-dose methotrexate, cisplatin, and doxorubicin (ADM). Postoperatively, good responders (90% or more tumor necrosis) received the same three drugs used before surgery, whereas poor responders (less than 90% tumor necrosis) received ifosfamide and etoposide in addition to those three drugs. RESULTS: For the 164 patients who entered the study between September 1986 and December 1989, surgery was a limb salvage in 136 cases (82%) and a good histologic response was observed in 117 patients (71%). At a follow-up ranging from 10 to 13 years (median, 11.5 years), 101 patients (61%) remained continuously free of disease, 61 relapsed, and two died of ADM-induced cardiotoxicity. There were no differences in prognosis between good and poor responding patients. ADM-induced cardiotoxicity (six patients), male infertility (10 of the 12 assessable patients), and second malignancies (seven patients) were the major complications of chemotherapy. Despite the large number of limb salvages performed, only four local recurrences (2.4%) were registered. CONCLUSION: With an aggressive neoadjuvant chemotherapy, it is possible to cure more than 60% of patients with nonmetastatic osteosarcoma of the extremity and amputation may be avoided in more than 80% of them. Because local or systemic relapses, myocardiopathies, and second malignancies are possible even 5 years or more after the beginning of treatment, a long-term follow-up is recommended for these patients.

Adolescent↗
PubMed2000

Giant cell tumor of the bones of the hand and foot.

BACKGROUND: Giant cell tumor of the small bones of the hand and foot is suspected of having some peculiar features compared with giant cell tumor in other sites. Moreover, it could share some features with other giant cell rich lesions involving the hand and foot, and this may affect the differential diagnosis. The aim of this study was to analyze the features of lesions such as these in the files of the Rizzoli Orthopedic Institute. METHODS: The incidence of giant cell tumors of the bones of the hand and foot seen at the Rizzoli Orthopedic Institute over 50 years (1947-1997) was taken into consideration. There were 8 lesions of the hand and 21 of the foot. Clinical information and follow-up of the patients were studied and updated. Radiographs were studied and radiographic features analyzed. Histopathologic material was thoroughly reviewed and histologic features analyzed. RESULTS: Although the location of tumor was helpful information, radiographic features were not specific. Giant cell tumors of the small bones of the hand and foot showed a predominance in females, younger patients and more aggressive behavior than giant cell tumors of large bones. The authors did not observe multicentricity or pulmonary metastases. CONCLUSIONS: Because the radiographic features of giant cell tumor of the hand and foot overlap those of other giant cell rich lesions in these locations, histologic diagnosis is mandatory, although it may be difficult and require the establishment of diagnostic criteria for giant cell tumor. As this tumor tends to be more aggressive than other giant cell rich lesions, treatments of choice are aggressive curettage or resection.

Adolescent↗
PubMed2000

Association of gastric and Waldeyer's ring lymphoma: a molecular study.

The coexistence of Waldeyer's ring and gastrointestinal non-Hodgkin's lymphomas at presentation is well known. Moreover, localized gastrointestinal relapses following successful treatment of lymphomas of Waldeyer's ring and thyroid lymphomas occurring after a prolonged disease-free interval have also been described. We report two cases of concomitant lymphoma in Waldeyer's ring and stomach. On the basis of the molecular analysis of the immunoglobulin heavy chain gene rearrangements, two different patterns of concomitant involvement by a lymphoma in Waldeyer's ring and in the gastrointestinal region seem to exist. One is represented by the preferential dissemination of the lymphoma from one site to the other, the second by the apparently independent growth of clonally unrelated lymphomas at each site.

Complementarity Determining Regions↗
PubMed2000

Prognostic models for diffuse large B-cell lymphoma.

Prognosis of DLCL patients is variable and associated with well-defined risk factors. In the past decade several pretreatment variables have been incorporated into prognostic models to predict the death risk of individual patients. The International Prognostic Index (IPI), developed in an international consensus study, has been one of the most widely accepted of these models. In our study we applied some of the major prognostic models proposed for DLCLs in a cohort of 111 patients uniformly treated with a CHOP-like regimen in order to compare their sensitivity and specificity. We also evaluated the possibility of improving the IPI with the inclusion, from among the variables analysed, of serum beta-2 microglobulin level (beta-2M). The sensitivity, reflecting the ability to predict all failures in the cohort of patients as a whole, has improved from 45 to 73 per cent when the beta-2M-IPI model is compared with IPI, without a significant loss of specificity. Based on these results, the beta-2M-IPI may be useful for identifying the subset of patients with very poor prognoses. Therefore, the use of the serum beta-2M value in addition to the IPI may help in selection of the patients with DLCL at higher risk for treatment failure, and identification of those who may require specifically tailored therapeutic approaches.

Adult↗
PubMed2000

Elastofibroma dorsi.

It is often difficult to identify the characteristics of soft-tissue tumors of the chest wall. This paper describes the peculiarities of elastofibroma dorsi, a rare pseudotumor of this site, to exclude many dangerous misconceptions. During the period between 1987 and 1997, we treated nine patients with elastofibroma dorsi. In six patients, the lesion was unilateral and in three it was bilateral. All these lesions were excised and no evidence of local recurrence or shoulder disability was observed at the last clinical evaluation. Elastofibroma dorsi is a rare, slow-growing, pseudotumoral lesion of the soft tissues of the chest wall with a peculiar radiographic and histologic pattern.

Aged↗
PubMed2000

Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin.

Primary cutaneous B-cell lymphomas have been associated with Borrelia burgdorferi, the spirochete responsible for Lyme disease. Recently, cutaneous marginal zone B-cell lymphoma has been proposed as a distinct clinical-pathological entity. We report a case of primary cutaneous marginal zone lymphoma, associated with B burgdorferi infection. Polymerase chain reaction (PCR) amplification of the third complementarity determining region (CDR3) of the immunoglobulin heavy chain gene showed the presence of a monoclonal lymphoproliferation, therefore strengthening the histological diagnosis of a malignant process. B burgdorfer-specific hbb gene sequences were detected by PCR in the lymphoma tissue at diagnosis but not after antibiotic treatment. A nearly complete clinical and histological regression was observed after B burgdorferi eradication, with immunohistochemistry studies showing disappearance of plasma cell differentiation and a marked decline in the number of CD3+ T cells and Ki-67+ cells. Our case confirms the link between B burgdorferi and some cutaneous lymphomas. The disappearance of the microorganism accompanied by the unequivocal decrease of most indicators of active T- and B-cell immune response strongly supported a pathogenetic role for B burgdorferi in sustaining an antigen-driven development and growth of this cutaneous marginal zone lymphoma. Antibiotic therapy (analogous to Helicobacter pylori infection in gastric MALT lymphoma) might be helpful with the aim of averting or at least deferring the indication for more aggressive treatment.

Aged↗
PubMed2000

Molecular detection of circulating neoplastic cells in patients with clinically localised gastric and non-gastric mucosa-associated lymphoid tissue lymphoma.

BACKGROUND: Unlike other low-grade lymphomas, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type usually presents with localised disease. AIM: To detect peripheral blood lymphoma involvement to establish the incidence of occult lymphoma dissemination. PATIENTS AND METHODS: In a series of 18 cases, peripheral blood was analysed by polymerase chain reaction, with primers directed to the third-complementarity determining region of the immunoglobulin heavy chain gene. RESULTS AND CONCLUSION: The presence of circulating neoplastic cells was detected in 21% of clinically localised cases. Moreover lymphoma cells were detected in 2 out of 6 morphologically normal bone marrow specimens. The present data show that, combining morphological and molecular methods, occult dissemination can be found in a large proportion of cases thus stressing the need for careful staging procedures. However, it has still to be clarified whether the presence of polymerase chain reaction-detectable circulating lymphoma cells can influence the outcome of mucosa-associated lymphoid tissue lymphoma patients submitted to antibiotic treatment (for gastric localisation) or local therapy (surgery or radiation, for non-gastric tumours).

Adult↗
PubMed2000

Lack of CD95/FAS gene somatic mutations in extranodal, nodal and splenic marginal zone B cell lymphomas.

Germline CD95 (also known as FAS, APT1 and APO1) gene mutations have been associated with benign lymphoproliferative diseases and autoimmune processes. Somatic mutations have been reported in human tumours, including lymphomas. Since marginal zone B cell lymphomas usually arise in a background of chronic inflammation, often of autoimmune origin, we searched for CD95 gene mutations in an unselected series of marginal zone B cell lymphomas. The CD95/FAS full coding region, comprising exon-intron junctions, was amplified from genomic DNA by polymerase chain reaction (PCR) in 10 separate reactions. PCR products were analysed by single-strand conformation polymorphism (SSCP) and visualised by silver staining. Bands exhibiting an altered electrophoretic mobility were sequenced. Twenty-seven cases of marginal zone B cell lymphomas of whom fresh or frozen tumour material was available (18 extranodal, five splenic and four nodal) were studied. Previously described silent polymorphisms in exons 7 (C836T) and 3 (T416C) were detected in 42% and in 19% of the cases, respectively. One silent T-to-A substitution at bp 431, within exon 3, was found in one case. Our results did not reveal the presence of CD95 somatic mutations in unselected cases of marginal zone B cell lymphomas. On the basis of our data, we cannot rule out that other genes coding for proteins involved in the CD95-induced apoptotic pathway might be altered. However, this pathway does not seem to play an important role in the pathogenesis of these lymphoma subtypes.

Antigens, Neoplasm↗
PubMed2000

BCL10 gene mutations rarely occur in lymphoid malignancies.

BCL10, a gene involved in apoptosis signalling, has recently been identified through the cloning of chromosomal breakpoints in extranodal (MALT-type) marginal zone lymphomas carrying the t(1;14)(p22;q32) translocation. BCL10 was also found mutated in these cases as well as in other types of lymphoid and solid tumors, suggesting that its inactivation may play an important pathogenetic role; however, this has been questioned by recent studies showing a lack of somatic mutations in human cancers. We report the mutation analysis of exons 1-3 of the BCL10 gene in DNAs from 228 cases of lymphoid malignancies (30 B cell chronic lymphocytic leukemias, 123 B and 45 T non-Hodgkin's lymphomas and 30 multiple myelomas). Somatic mutations were detected in four cases (approximately 2%): one small lymphocytic, one follicular and two diffuse large cell lymphomas. The mutations were all within exon 3 and have not been previously reported. Our data suggest that BCL10 mutations may play only a limited role in the pathogenesis of lymphoid neoplasms.

Adaptor Proteins, Signal Transducing↗
PubMed2000

Endoscopic CO2 laser excision for tis, T1, and T2 glottic carcinomas: cure rate and prognostic factors.

We present the results of a retrospective study based on a cohort of 140 patients who underwent endoscopic CO(2) laser excision for previously untreated early glottic cancer (21 Tis, 96 T1, and 23 T2). This study used univariate analysis to review the impact on disease-free survival of 15 factors related to the host, the tumor, and the treatment. Recurrent cancer developed in 28 patients with an average interval to recurrence of 37.5 months. Retreatment consisted of different procedures, including laser excision, partial or total laryngectomy, and/or radiotherapy. The larynx was definitively preserved in 96% of patients. By the end of the study, 14 patients had died, but only 2 of them had died of the disease. Five-year overall survival and determinate survival were 93% and 98%, respectively. Ultimate local control at 5 years with CO(2) laser excision alone was 95% for Tis, 87% for T1, and 91% for T2 lesions. Only involvement of the anterior third of the vocal cord (P = 0.02), involvement of the false vocal cord (P = 0.02), and infiltration of the vocalis muscle (P = 0.004) showed a significant negative impact on disease-free survival.

Adult↗
PubMed2000

Neoadjuvant chemotherapy for Ewing's sarcoma of bone in patients older than thirty-nine years.

The aim of this study was to determine whether the behaviour of Ewing's sarcoma of bone in adult patients is the same as that observed in children and adolescents. We reviewed the clinical features and outcomes of 23 patients over the age of 39 (17 males, 6 females) who had been treated with neoadjuvant chemotherapy between 1983 and 1995 at our institution. The most common primary sites of tumor were the extremities (16 cases); tumor volume was more than 100 ml in 17 patients, and elevated serum LDH levels were found in 6 cases. The local treatment was surgery in 8 cases, surgery plus radiotherapy in 8, and radiotherapy alone in 7 cases. Chemotherapy comprised a 4-drug regimen in 10 patients, while the other 13 patients received 6 drugs. At a follow-up of 8.8 years (3.5-15) 13 patients remained continuously free of disease and 10 relapsed. The 5-year disease-free survival and overall survival rates were 53% and 59%, respectively. Clinical features, dose intensity, and toxicity of chemotherapy, as well as the outcome of these 23 patients were found to be exactly comparable to the findings observed in 327 patients younger than 40 years treated at our institution in the same period, with the same therapy. We conclude that Ewing's sarcoma of bone in adults is no different from that occurring in children, and we therefore recommend the inclusion of all adult patients in multidisciplinary treatment trials of this tumor.

Adult↗
PubMed2000

Dedifferentiated clear cell chondrosarcoma.

Dedifferentiation, a change in the histologic character and clinical behavior of a tumor to a more immature and aggressive one, occurs in approximately 11% of all chondrosarcomas. The original lesion is usually a low-grade chondrosarcoma. Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy with a preference for the ends of long bones. It is usually curable by resection. Recurrence commonly follows inadequate surgery, and metastases to lung, brain, and bones can develop. However, dedifferentiation has not yet been described in association with clear cell chondrosarcoma. Three patients are described who were initially diagnosed as having clear cell chondrosarcoma of the femur. Two were treated with en bloc resection for a clear cell chondrosarcoma. One of these had an undifferentiated sarcoma in a local recurrence after 6 years. In the second, metastasis of the clear cell chondrosarcoma developed 5(1/2) years after surgery; autopsy revealed undifferentiated sarcoma in the lung, heart, and lumbar spine. The third patient had dedifferentiated clear cell chondrosarcoma at the time of resection following the biopsy diagnosis of clear cell chondrosarcoma. All three died with metastatic disease. These three patients represent three different manifestations of dedifferentiation-at initial diagnosis, at recurrence, and at metastasis. To our knowledge, this is the first description of dedifferentiation occurring in clear cell chondrosarcoma.

Adenocarcinoma, Clear Cell↗