[Show abstract][Hide abstract] ABSTRACT: To identify the best surgical approach to atypical lipomatous tumors we reviewed 171 patients who underwent surgery at two sarcoma referral centers with different surgical policies.
Annals of Surgical Oncology 06/2014; · 4.12 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The survival of patients with axial skeletal or pelvic osteosarcoma (OS) remains poor, and the management of these patients is challenging. The object of this study is a cohort of unselected patients aged < 19 years with primary high-grade pelvic/axial OS. Patients were treated with high-dose methotrexate, doxorubicin, cisplatin, ifosfamide followed or preceded by local treatment (surgery and/or radiotherapy). Twenty patients aged 3-19 years were treated. Eight patients had pelvic OS, 8 axial OS and 4 mandible/maxilla OS. All patients received chemotherapy, after which necrosis was evaluable in 9 patients (≥90 % in 3). Sixteen patients underwent surgery. Radiotherapy was administered to 8 patients (total dose 34-60 Gy). The median follow-up was 35 months (8-276), and the 5-year disease-free survival and overall survival rates were 37 and 40 %, respectively. Six patients were alive at the time of this report: 2 with pelvic OS (both responded well to chemotherapy, one underwent hemipelvectomy and the other had non-radical surgery plus radiotherapy); 1 with axial and multicentric OS (with a good histological response and radical surgery); 3 with mandible/maxilla OS. Two patients died of secondary tumors (one bone and one breast cancer). It is worth noting that 4 patients had a p53 mutation: 1 is alive, 2 died of their OS, 1 of breast cancer. Adequacy of local treatment and pathological response influenced the prognosis for axial OS, which remained dismal. A high incidence of p53 mutation emerged in our series of patients.
Medical Oncology 04/2014; 31(4):875. · 2.14 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Major goals in translational oncology are to reduce systemic toxicity of current anticancer strategies and improve effectiveness. An extremely efficient cancer cell mechanism to avoid and/or reduce the effects of highly cytotoxic drugs is the establishment of an acidic microenvironment, an hallmark of all malignant tumors. The H + -rich milieu that anticancer drugs meet once they get inside the tumor leads to their protonation and neutralization, therefore hindering their access into tumor cells. We have previously shown that proton pump inhibitors (PPI) may efficiently counterattack this tumor advantage leading to a consistent chemosensitization of tumors. In this study, we investigated the effects of PPI in chemosensitizing osteosarcoma.
MG-63 and Saos-2 cell lines were used as human osteosarcoma models. Cell proliferation after pretreatment with PPI and subsequent treatment with cisplatin was evaluated by using erythrosin B dye vital staining. Tumour growth was evaluated in xenograft treated with cisplatin after PPI pretreatment. Subsequently, a multi-centre historically controlled trial, was performed to evaluate the activity of a pre-treatment administration of PPIs as chemosensitizers during neoadjuvant chemotherapy based on methotrexate, cisplatin, and adriamycin.
Preclinical experiments showed that PPI sensitize both human osteosarcoma cell lines and xenografts to cisplatin. A clinical study subsequently showed that pretreatment with PPI drug esomeprazole leads to an increase in the local effect of chemotherapy, as expressed by percentage of tumor necrosis. This was particularly evident in chondroblastic osteosarcoma, an histological subtype that normally shows a poor histological response. Notably, no significant increase in toxicity was recorded in PPI treated patients.
This study provides the first evidence that PPI may be beneficially added to standard regimens in combination to conventional chemotherapy.
Journal of Translational Medicine 10/2013; 11(1):268. · 3.46 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Autologous fat tissue grafting (AFTG) has been successfully used in the treatment of different sclerotic conditions, including localised scleroderma. Patients with advanced systemic sclerosis (SSc)?related perioral thickening and mouth opening limitation are candidates to this therapeutic approach. Aim of the study. AFTG of the lips was performed to improve mouth opening in patients with SSc.
We enrolled in the study 20 patients with diffuse SSc, (median age 35 +15 years and 11+10 years of disease duration). Two?ml?fractions of autologous fat drawn from trochanteric or peri?umbelical areas were injected in 8 different sites around the mouth. Baseline and after treatment mouth opening changes were assessed by measuring inter?incisal distance and oral perimeter, while skin hardness was tested by digital durometer. Pre? and post?treatment modifications of microvascular architecture were assessed by counting capillaries in the inferior lip videocapillaroscopy (VC) images, and by scoring the microvascular density (MVD) in anti?CD34/CD31 immuno?histochemical (IH) stained perioral skin biopsy sections. Similarly, histological sections were examined to evaluate dermo?epidermic junction (DEJ) modifications.
Three months after treatment, both the inter?incisal distance and oral perimeter significantly increased (p <0.001). At the same time, a significant skin neovascularization became evident, both considering the VC images (p <0.001) and MVD scores in IH sections (p <0.0001). Finally, some skin histological aspects also improved, as shown by the significant changes in DEJ flattening scores (p <0.0001).
The present study suggests that, in patients with SSc, AFTG can improve mouth opening and function, induce a neovascularization, and partially restore the skin structure.
[Show abstract][Hide abstract] ABSTRACT: Ewing sarcoma (ES) is the second most common type of primary bone malignancy, and retains a high propensity to metastasize; the prognosis of patients with disseminated disease is very poor, with an event-free survival rate of <20%. Current multimodality treatment for ES consists of combined chemotherapy before and concurrent with surgery and local radiotherapy for the involved bone. Cisplatin is one of the most widely used drugs for the treatment of bone tumors in children, but is not currently used in ES. We describe a child with multifocal ES, treated with a phase II trial including a single-drug window therapy, which displayed a dramatic response to 2 courses of cisplatin and had a favorable outcome.
Journal of Pediatric Hematology/Oncology 08/2013; 35(6):478-81. · 0.97 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We compared two chemotherapy regimens that included methotrexate (MTX), cisplatin (CDP), and doxorubicin (ADM) with or without ifosfamide (IFO) in patients with nonmetastatic osteosarcoma of the extremity.
Patients age ≤ 40 years randomly received regimens with the same cumulative doses of drugs (ADM 420 mg/m(2), MTX 120 g/m(2), CDP 600 mg/m(2), and IFO 30 g/m(2)) but with different durations (arm A, 44 weeks; arm B, 34 weeks). IFO was given postoperatively when pathologic response to MTX-CDP-ADM was poor (arm A) or given in the primary phase of chemotherapy with MTX-CDP-ADM (arm B). End points of the study included pathologic response to preoperative chemotherapy, toxicity, and survival. Given the feasibility of accrual, the statistical plan only permitted detection of a 15% difference in 5-year overall survival (OS).
From April 2001 to December 2006, 246 patients were enrolled. Two hundred thirty patients (94%) underwent limb salvage surgery (arm A, 92%; arm B, 96%; P = .5). Chemotherapy-induced necrosis was good in 45% of patients (48% in arm A, 42% in arm B; P = .3). Four patients died of treatment-related toxicity (arm A, n = 1; arm B, n = 3). A significantly higher incidence of hematologic toxicity was reported in arm B. With a median follow-up of 66 months (range, 1 to 104 months), 5-year OS and event-free survival (EFS) rates were not significantly different between arm A and arm B, with OS being 73% (95% CI, 65% to 81%) in arm A and 74% (95% CI, 66% to 82%) in arm B and EFS being 64% (95% CI, 56% to 73%) in arm A and 55% (95% CI, 46% to 64%) in arm B.
IFO added to MTX, CDP, and ADM from the preoperative phase does not improve the good responder rate and increases hematologic toxicity. IFO should only be considered in patients who have a poor histologic response to MTX, CDP, and ADM.
Journal of Clinical Oncology 05/2012; 30(17):2112-8. · 18.04 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The authors report a clinical case of a primary sternal chondrosarcoma, presented as a mass in the anterior mediastinum. The patient was treated with subtotal sternectomy and sternal transplantation followed by radiotherapy. Twelve months after surgery, the patient is in good clinical condition, without any sign of tumor relapse and with normal respiratory mechanics. Primary malignant tumors of the sternum are uncommon and a presentation mimicking thymoma is rare and unreported. The stermal replacement with a cryopreserved allograft sternum is an innovative technique that overcomes the problems related to the prosthetic biocompatibility or to the bone autograft.
Journal of Cardiothoracic Surgery 05/2012; 7:40. · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this study is the review of our data obtained from 1960 to 2010 in the Oncological Orthopaedics Unit of G. Pini Institute, evaluating appearance, anatomic site, complications, recurrences and different kinds of treatment of 126 paediatric patients affected by aneurysmal bone cyst. All cases were controlled and confirmed by clinical, histologic and radiographic examination. The average age was 11.8 years. Biopsy was made in every case. The treatment of choice was surgical: simple curettage, curettage and bone grafting, simple resection in expendable bones. Selective embolization was used in difficult or hazardous sites. The recurrence rate was 21% for the cases treated until 1976 and 8.9% for those treated from 1976 to 2010. A literature review about other methods of treatment is presented.
Archivio di Ortopedia e Reumatologia 04/2012; 123(1).
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to determine the efficacy of platelet-rich plasma (PRP) 1-injection during an Achilles tendon rat tear model. 80 male adult imbreded rats (Wistar Kyoto), underwent under surgical tendon rupture. 40 Animal (PRP group rats) were given a local injection with 0,25 mL of PRP, and 40 animal (control group) were given the same quantity of control solution. The rats were sacrified at 1, 2, 4 and 6 weeks (each time point, 20 rats of the each group) after surgical tear and tendon tissue was analysed by macroscopic aspect, histology, immunostaining and Real Time (RT)-PCR to evaluate tissue repair. PRP improved tendon remodelling by better coordination of the reconstructive process with earlier formation of tendon-like continuity only in the first week after surgery. However, after 2,4 and 6 weeks, Achilles tendons in the PRP group had no difference compared to the control group. Immunostaining and RT-PCR did not show any difference between PRP treated and untreated group. Based on these findings a single injection of PRP appear not useful for Achilles rat tendon tear.
Muscles, ligaments and tendons journal. 04/2011; 1(2):41-7.
[Show abstract][Hide abstract] ABSTRACT: There are no clear explanations for the spectrum of hip dysplasia nor for the observation that in normal and dysplastic hips, final development may be unpredictable with or without treatment. Immunohistochemical and histological studies of a three month old child's acetabulae were performed. Multiple inclusions were found in the lateral ring epiphysis and in the three flanges of the triradiate cartilage. These inclusions may represent cartilage vessel systems pre-destined to form the secondary centres of ossification. Damage to the primary acetabular growth plates may occur congenitally and help to explain the spectrum of acetabular dysplasia. Damage to one or more of these centres, whether due to instability, displacement or iatrogenic injury, may cause failure of late acetabular development. Hips at risk of damage to the acetabular cartilages should be followed up longer.
Hip international: the journal of clinical and experimental research on hip pathology and therapy 01/2011; 21(1):9-13. · 0.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Ectodermal dysplasia (ED) comprises a large heterogeneous group of inherited disorders that are characterized by primary defects in the skin, hair, nails, eccrine glands and teeth. The most characteristic findings are the reduced number of teeth. All rehabilitative programmes involve proper evaluation of skeletal relationships. Prosthetic-implantological treatment at the end of bony growth can be used. In this article a case of ED treated with Le Fort I for maxillary advancement, femur homografts, implants' insertion and immediate loading is described. In December 2007, a 38-year-old female was referred to the Maxillofacial Department of Galeazzi Hospital (Milan, Italy) who had a diagnosis of ED. Twelve implants were inserted in one-step surgical procedure. No implant was lost and all are stable. The occlusion is stable after 15 months of follow-up. The results indicate that the one-step oral rehabilitation can be performed in adults who are affected by ED. Also, this significantly reduces the time of oral and facial rehabilitation.
[Show abstract][Hide abstract] ABSTRACT: The osteoid osteoma is a benign bone lesion that accounts for 12.1% of benign tumors and 2.9% of all primary bone tumors.
The third of the proximal femur is the most frequent location, followed by the tibia, the posterior elements of the spine
and the humerus. The tumor, however, may be found in all parts of the skeleton. It affects more often males than females (2:1);
75% of patients are between 10 and 30 years of age. It is uncommon in children younger than 5 years and adults over 40 years.
The authors discuss the case of a 33-year-old man who undegoes arthroscopy of the ankle for a partial synoviectomy due to
Two years ago the patient underwent a surgical open intervention of reconstruction of the anterior talofibular ligament of
the ankle, for an anterolateral instability of II degree following an ankle strain.
After that intervention, the patient has complained about the persistence of articular swelling with marked functional limitation,
without benefit from conservative therapies.
During arthroscopy a large amount of hypertrofic synovial tissue is found, located in the anterior portion of the ankle. This
tissue is removed and a biopsy is performed.
The bioptic sample is composed by synovial membrane with chronic flogosis and emosiderinic pigments and a “nidus” of osteoblastic
benign proliferation related to an osteoid osteoma.
This patient therefore had a reactive chronic sinovitis secondary to a rare localization of osteoid osteoma.
Archivio di Ortopedia e Reumatologia 01/2010; 121(2):23-24.
[Show abstract][Hide abstract] ABSTRACT: In the last decade, several investigators have reported that autologous and homologous fresh frozen bones are effective materials to restore alveolar ridges before insertion of dental implants. Recently, we have used femur homograft derived from living donors. Here, we reported a 1-step oral rehabilitation of the severely resorbed maxilla by means of implants' insertion, Le Fort I osteotomy for maxillary advancement, grafts, and immediate loading.
Patients were treated with 1-step oral rehabilitation. Age, sex, implant length and diameter, tooth site, loading, and grafts were the investigated variables. Implant failure and peri-implant bone resorption were considered as predictors of clinical outcome. Kaplan-Meier algorithm and Cox regression were then performed to detect those variables statistically associated with the clinical outcome.
Eighty-four implants were inserted in 11 patients. Implants were inserted to replace 22 incisors, 21 cuspids, 20 premolars, and 21 molars. The mean follow-up was 17 months. Two of 84 implants were lost (ie, survival rate = 97.6%), and no differences were detected among the studied variables. By using the 82 fixtures processed with Kaplan-Meier and Cox regression, only implant site has an impact on clinical outcome, and molars have a worse outcome than incisors.
One-step oral rehabilitation can be used in selected patients. It significantly shortened the time of rehabilitation without adverse effects. Femur homograft derived from living donors is a valuable material for grafting jaw: it is safer, cheap, and available in programmed amounts and avoids a second operation field.
The Journal of craniofacial surgery 10/2009; 20(6):2205-10. · 0.81 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Achondroplasia (ACH) represents the major cause of dwarfism and is due to mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. The cellular mechanisms involved in the reduced growth have been mainly described for in vitro or in vivo models, but few data have been obtained for humans.
Thirteen children with ACH were enrolled in the study; the presence of FGFR3 mutations was determined by restriction fragment length polymorphism analysis and sequencing, whereas protein expression in cartilage biopsy was assessed by immunohistochemistry.
Chondrocytes in cartilage biopsies of ACH children were characterized by the presence of growth arrest mediated by STAT activation (both STAT1 and STAT5) and increased expression of p21 and cyclin D1, whereas no expression of either p53 or cyclin D3 could be detected. This mechanism was present in ACH children carrying the G380R mutation but also in a patient in whom no mutation could be detected in the entire coding region of the FGFR3 gene.
These data thus demonstrate the presence of a common final mechanism involving p21 and possibly leading to a block in chondrocyte proliferation.
Journal of Orthopaedic Science 09/2009; 14(5):623-30. · 0.96 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Soft tissue giant cell tumor (GCT-ST) of low malignant potential is an uncommon neoplasm, considered the soft tissue counterpart of giant cell tumor of bone. GCT-ST mainly affects young to middle-age adults and presents as a painless growing mass mainly located in the lower extremities and trunk. Histologically, this tumor is characterized by a mixture of uniformly scattered osteoclast-like multinucleated giant cells intimately admixed with short fascicles of spindled cells. Complete excision with negative surgical margins is associated with a benign clinical course in most cases.
The authors report the clinicopathological and immunohistochemical features of an unusual GCT-ST of 46 years duration previously histologically misdiagnosed as Kaposi's sarcoma.
Histologically, the tumor was characterized by a multinodular growth pattern with osteoclast-like multinucleated giant cells admixed with spindle cells partially arranged in a storiform pattern, fibrosis and foci of haemorrhage and mature bone. Immunohistochemistry revealed CD68 reactivity of the multinucleated giant cells.
GCT-ST is a rare neoplasm characterized by benign clinical course if excised adequately, as shown by our case of exceptionally long duration. Emphasis is placed on the importance of differential diagnosis with other giant cell-rich soft tissue neoplasms because clinical behaviour, prognosis and treatment significantly differ.