Aim

Patients treated with limb salvage surgery for bone sarcomas of the extremities (upper and lower) may have physical disability as a result of treatment. Goal of this study was to evaluate the quality of life after treatment (chemotherapy and conservative surgery) at long term.

Objective of this study was to analyse results of two stage revisions in infected megaprostheses in lower limb.

Material and Methods: Between April 1983 and December 2007, 1036 modular uncemented megaprostheses were implanted in 605 males and 431 females with mean age 33.5 yrs: 160 KMFTR®, 633 HMRS® prostheses, 68 HMRS® Rotating Hinge and 175 GMRS®. Sites: distal femur 659, proximal tibia 198, proximal femur 145, total femur 25, distal femur and proximal tibia 9. Histology showed 612 osteosarcomas, 113 chondrosarcomas, 72 Ewing’s sarcoma, 31 metastatic carcinomas, 89 GCT, 36 MFH, 68 other diagnoses.

Infection occurred in 80 cases (7.7%) at mean time of 4 yrs (min 1 month, max 19 yrs) in 18 KMFTR®, 47 HMRS®, 5 HMRS® Rotating Hinge, 10 GMRS®. Sites: 51 distal femurs, 21 proximal tibias, 6 proximal femurs, 1 total femur and 1 extrarticular knee resection. Most frequent bacteria causing infection were: Staphilococcus Epidermidis (39 cases), Staphilococcus Aureus (17) and Pseudomonas Aeruginosa (5). Infection occurred postoperatively within 4 weeks in 9 cases, early (within 6 months) in 12 cases, late (after 6 months) in 59 cases.

Usual surgical treatment was “two stage” (removal of implant, one or more cement spacers with antibiotics, new implant), with antibiotics according with coltures. One stage treatment was used for immediate postoperative infections, only since 1998.

Functional results after treatment of infection were assessed using the MSTS system.

Results: A two stage revision was attempted in 73 pts (91.2%): in 58 cases a new prostheses was implanted (with negative laboratory tests for infection) at mean time of 5 months (min 2, max 16 months), but in 3 pts infection recurred and they were amputated; 4 pts died before implanting a new prosthesis; 11 pts were amputated after several spacers since infection did not heal. One stage revision was performed in 4 of the 9 immediate postoperative infections, with successful results.

In 3 cases an amputation was primarily performed, to proceed with chemotherapy.

Revisions for infection were successful in 63 pts (79%), while 17 pts were amputated (21%).

Functional results evaluated in 53 revised cases were good or excellent in 43 (81.1%).

Conclusions: Two stage treatment of infected megaprostheses is successful in most cases. One stage has selected indications, mainly in postoperative immediate infections.

Introduction: Tumours of sacrum are rare. Treatment depends on malignancy or local aggressiveness: resection is indicated for malignant lesions, intralesional surgery for benign. Purpose of this study was to analyse risk of infection and its treatment after surgery for the two most common primary sacral tumours.

Material and Methods: Between 1976 and 2005, 82 patients with sacral chordoma or giant cell tumour were treated in our Institution. Demographic data, surgery and adjuvant treatments were analysed in the two histotypes. All patients were periodically checked with imaging studies. Special attention was given to the assessment of deep infections, their treatment and outcome.

Patients included 44 females and 38 males, ranging in age from 14 to 74 years. Mean follow-up was 9.5 years (min. 3, max. 27). Histopathological findings included chordomas in 55 cases and giant cell tumor (GCT) in 27. Most pts. had iv antibiotic therapy with amikacin and teicoplanin. Surgery of chordoma was resection, surgery of GCT was intralesional excision. In 6 sacral resections a miocutaneous transabdominal flap of rectus abdominis was used for posterior closure.

Results: No deep infections were observed in the GCT series. Three patients with sacral chordoma died for postoperative complications and were excluded from this analysis. Of the remaining 52 patients with chordoma, 23/52 had deep wound infection (44%), that required one or more additional operative procedures. In 16 pts. (70%) infection occurred within 4 weeks postoperatively, in 7 within 6 months. Most frequent bacteria causing infection were Enterococcus (23%), Escherichia Coli (20%), Pseudomonas Aeruginosa (18%). In 74% of cases a multiagent infection was detected. Surgical treatment consisted in 1 (52%) or more (48%) surgical debridements, combined with antibiotics therapy according to coltural results.

Mean surgical time was 14 hours for resections and 6 hours for excisions.

No significant difference was found comparing deep wound infections with levels of resection (15/33 resections proximal to S3-45% and 8/19 resections below or at S3-42%), previous intralesional surgery elsewhere (4/9 patients previous treated elsewhere-44% and 19/46 primarily treated patients-41%) and age at surgery.

Conclusions: Type of surgery was the prominent factor related with a major risk of infection. Operating procedure time correlated as well. Resection of sacral chordomas with wide margins improves survival although extensive soft-tissue resection in proximity to the rectum favours deep infections. Intralesional excision is the recommended surgical treatment for GCT of the sacrum and does not imply a significant risk of infection.

Objective: Aim of this study was to analyse the incidence of infections in primary prosthetic reconstructions of the humerus after resection for bone tumours and their treatment and results.

Material and Methods: Between 1974 and 2006 at Rizzoli 344 reconstructions of the humerus using prosthetic devices (alone or in association with allografts) were performed. Sites of reconstruction were: proximal humerus 311, distal humerus 19, diaphysis 5, total humerus 9. Histological diagnoses included 24 benign tumors, 253 malignant tumors and 67 metastatic carcinomas. Patients were followed periodically in the clinic. Informations were obtained from clinical charts and imaging studies with special attention to major complications requiring revision surgery. Univariate analysis through Kaplan-Meier actuarial curves was used in evaluating implant survival to major complications. Infections developing in the first 4 weeks were considered postoperative infections, those diagnosed in the first 6 months were judged early infections, while late infections those diagnosed after 6 months from surgery.

Results: In 20 patients (5.8%) a revision for deep infection was required. In 19 of these cases tumor was localized in the proximal humerus and in 1 in the distal humerus. There were 18 infections in prostheses and 2 in allograft prosthesis composites. Two infections were postoperatively diagnosed, seven were early infections and eleven late infections. Revision was required in 18 cemented prosthesis, 1 uncemented prostheses and 1 Coonrad-Morrey.

S. Epidermidis and S. Aureo were the most frequent bacteria causing infection (45%). Two stage treatment of infection was chosen: removal of the implant and temporary substitution with cement spacer with antibiotics (usually vancomycin) until infection healed. But a new prostheses was actually implanted in 3 cases only (at mean time of 5.7 mos), while in 17 the spacer was never removed by patients choice due to the acceptable result with the spacer. Systemic antibiotics were associated according to cultural results. Infection healed in all patients.

Conclusions: Infection is the most severe complication in prosthetic reconstructions for tumours of the humerus. Its incidence (5.8%) is lower than in lower limb. Treatment requires a team work: surgeon, microbiologist and infectious disease physician. One stage is indicated in postoperative infections, two stage is recommended in both early and late infections. Two stage surgery offered good results, although in most cases a new prosthesis was not implanted, since actually humeral megaprostheses act as a spacer and don’t provide a much better function.

Purpose: Aim of this study was to analyse our Institution experience with osteosarcoma in Paget’s disease (PD).

Methods: Twenty-six patients treated between 1961 and 2006 were retrospectively analysed. Information focusing on treatment, imaging and oncologic outcome were obtained from clinical charts.

Results Fifteen patients had previous diagnosis (mean time 9 yrs) of monostotic (80%) or polyostotic (20%) PD; in 11 sarcoma and PD were contemporarily diagnosed. The most frequents osteosarcoma histotypes observed were osteoblastic in 18 cases (69%), fibroblastic in 5 cases (19%), teleangectasic in 2 patients and chondroblastic in one case. In 6 patients surgery only was performed: 3 amputations and 3 resections. In 3 pts surgery (amputation), adjuvant chemotherapy and radio-therapy were given. In 1 pt surgery (amputation) and radiotherapy. In 12 patients surgery and chemotherapy: adjuvant in 10 cases (8 amputations and 2 resections) and neoadjuvant in 2 (both amputations). Two patients had only radiotherapy and 2 had only chemotherapy. Oncologic outcome showed 4 pts with no evidence sisease at a mean follow up of 139 months (min.42.6, max.257.4) and 22 died with disease at a mean time of 20.15 months (min.1, max. 84). One patient only of 6 (11%) treated with surgery only is NED at 10 years, while the other 5 died from disease at a mean time of 30 mos. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean follow up of 12 years, while 9 died of disease at a mean time of 24 months. All patients treated without surgery died at of 7.5 mos on average (min.1 – max.13.7).

Conclusions: Sarcomas in PD have a poor prognosis in pts treated without surgery or with surgery only. Surgery with chemotherapy – when feasible – improves prognosis.

Purpose: GSD, also known as massive osteolysis or disappearing bone disease, is rare, characterized by proliferation of vascular channels of hematic and lymphatic origin resulting in progressive distruction of bone. This study about Gorham-Stout disease is a retrospective review of the Rizzoli files with special attention given to treatment and outcome.

Materials and Methods: This study is based on a retrospective analysis of a single institution experience. In the Rizzoli files we found 15 cases of GSD from 1968 to 2008. Two were excluded for insufficient documentation. For 13 cases clinical data, imaging and histology were analysed. Histopatologically benign vascular proliferation of thin-walled endothelial capillaries surrounded by a fibrous stroma is present. Adipose involution of the bone marrow and extreme thinning of bony trabeculae represent other histopatologic features. A final diagnosis was established based on clinical, radiological and histopathologic features, as recommended in the literature. Imaging included X-rays in 11 cases and CT or MRI in 5. All lesions were lytic, with an associated sclerosis in two cases. There was one lesion in four cases, several lesions in the same bone in one, and multiple bones involved in six patients. Primary sites were proximal femur in 7 cases, pelvis in 2, hip and knee, calcaneus, humerus and cervical spine in 1 case each.

Results: Two patients had no treatment, 2 conservative treatment (cast or brace), 5 surgery, 6 medical treatment (byphosphonates, calcitonin, zoledronic acid, interferon, steroids), 1 radiotherapy, 2 selective arterial embolization. Surgery consisted of internal fixation of 4 pathologic fractures and reconstruction of the entire humerus with a double composite allograf in 1. Overall, surgery only in 2 patients, medical treatment only in 4 (1 also embolization), surgery and medical treatment in 2 (1 also embolization), radiotherapy only in 1, conservative treatment in 2. Four patients were lost at follow up. In the remaining 9 patients mean follow up was 17 ys.(min 2, max 30). These 9 patients had the following results: 2 dead, 3 healed, 3 with stable disease, 1 alive with asymptomatic disease at 24 ys.

Conclusions: No clear treatment recommendations were desumed. Surgery is indicated in pathologic fractures or reconstruction of massively destroyed bones, medical treatment and selective embolization are helpful. In the literature prostheses are mostly recommended for reconstructions due to the risk of allografts resorption.

Purpose of this study was to analyse the different techniques of prosthetic reconstruction of the humerus (also in association with bone grafts) after resection of primary tumors, discussing indications and evaluating implant survival.

Material and Methods: Between 1974 and 2006, 277 patients had prosthetic reconstruction of the humerus after tumor resection. These included 253 reconstructions of the proximal humerus: 225 cemented modular prostheses (MRS), 18 uncemented modular prostheses, 9 allograft/prostheses composites, 1 custom-made prosthesis; 2 diaphyseal reconstructions:1 intercalary prosthesis and 1 MRS; 13 reconstructions of the distal humerus: 11 uncemented modular prostheses, 1 allograft/prosthesis, 1 Coonrad-Morrey prosthesis; 9 total humerus reconstructions: 6 uncemented modular prostheses, 2 allograft/prostheses composites, 1 custom made prosthesis. The uncemented modular prosthesis used was the HMRS^® and the cemented modular was the MRS^®.

Histologically 24 were benign tumors and 253 primary malignant tumors.

All patients were periodically followed in the clinic, imaging studies and histology were reviewed and special attention given to prostheses-related complications and implant survival.

Univariate analysis through actuarial Kaplan Meier curves was used in evaluating implant survival to major complications. Functional results were assessed using the MSTS system.

Results: at a medium follow-up of 9 years 121 patients were NED, 14 NED1pm, 7 NED1lr, 1 NED1bm, 3 NED2pm, 2 NED2lr, 1 NED3pm, 102 died of disease, 19 died of other disease, 7 were lost to follow-up.

Major complications of the implants included 19 cases of deep infection (6.8%), 8 aseptic loosenings (2.9%), 4 breakages (1.4%) causing failure of the implants requiring revisions. Further complications were observed in revised cases. Actuarial curve of implant survival to major complications showed over 80% at 10 years and over 70% at 20 years.

Functional results according to the MSTS system were good or excellent (over 50%) in more than 90% of the patients, with an average score of 79%.

Conclusion: different techniques are available in reconstructions of the humerus after tumor resection, the indications depending on the type of resection and removal of soft tissues required.

Purpose of this study is to analyze the results of a modular reconstructive tumor prosthesis for the lower limb (GMRS^®) with a comparative statistical analysis of primary and secondary implants.

Material and methods: From October 2003 to September 2007 at Rizzoli 161 GMRS^® prostheses were implanted, most after resection of osteosarcoma (94 cases, 58%). It is a modular system with a rotating hinge mechanism for the knee, cemented and uncemented stems, in titanium and chromium-cobalt-molybdenum, curved and straight-fluted, with or without hydroxyapatite coating. Moreover adaptors are available to revise HMRS^® implants. This series includes 88 males and 73 females ranging in age from 9 to 80 years. Sites of reconstruction were 109 distal femurs, 19 proximal femurs, 1 total femur and 32 proximal tibias. There were 149 oncologic and 12 non oncologic diagnoses, including 96 primary reconstructions and 65 revisions after failure of previous implant. A retrospective analysis of imaging and complications was performed and functional results assessed according to MSTS system. Statistical analysis with Kaplan-Meier curves was used to study implant survival.

Results: At a mean follow up of 2 yrs. 106 patients are continuously NED, 31 are NED after treatment of one or more local recurrence or metastasis, 7 AWD, 5 DWD. There were 10 major complications: 8 infections (4,7%) (5 in primary and 3 in secondary implants, 2 previously infected) and 2 aseptic loosening (1,2%) (1 each). There were 9 minor complications (4 wound sloughs, 1 stiff joint, 3 disrupted extensor apparatus,1 patellar instability) requiring revision. Comparative statistical analysis of primary and secondary implants survival at major complications shows no statistically significant difference. Functional results were good or excellent in 95% of the evaluated patients, without any poor.

Conclusions: Middle term results are promising: good function, very low incidence of major complications, no breakage of implant components. This prosthetic reconstruction is indicated in oncological cases as well as in selected in some non oncological settings, such as challenging revisions of prosthetic failures with massive bone loss or post-radiation non unions or allografts failures. Although a higher incidence of complications was expected in secondary implants, statistical analysis shows similar survival.

Gorham-Stout disease (GSD) is rare, characterized by proliferation of vascular channels resulting in progressive distruction of bone. In the Rizzoli files we found 15 cases of GSD from 1968 to 2008. Two were excluded for insufficient documentation. For 13 cases clinical data, imaging and histology were analysed. Histopathologic features included benign vascular proliferation, vascular pattern of osteolytic angioma, fibro-connective tissue component and bony destruction. A final diagnosis was established based on clinical, radiological and histopathologic features.

Imaging included X-rays in 11 cases and CT or MRI in 5. All lesions were lytic, with associated sclerosis in two cases. There was one lesion only in 4 cases, multiple lesions in the same bone in 1 and multiple bones involved in 6. Primary sites were proximal femur in 7 cases, pelvis in 2, hip and knee, calcaneus, humerus and cervical spine in 1 case each. Two patients had no treatment, 2 conservative treatment (cast or brace), 5 surgery, 6 medical treatment (byphosphonates, calcitonin, zoledronic acid, interferon, steroids), 1 radiotherapy, 2 selective arterial embolization. Surgery consisted of internal fixation of pathologic fractures in 4 patients and reconstruction of the entire humerus with a double composite allograft in 1. Treatment was surgery only in 2 patients, medical treatment in 4 (1 also embolization), surgery and medical treatment in 2 (1 also embolization), radiotherapy only in 1, conservative treatment in 2. Four patients were lost at follow up. Mean follow up was 17 ys.(min 2, max 30) in 9 patients: 2 dead, 3 healed, 3 with stable disease, 1 alive with disease at 24 ys.

No conclusive treatment recommendations are possible; surgery is indicated in pathologic fractures or reconstruction of massively destroyed bones, medical treatment and selective embolization are helpful. In literature prosthetic reconstruction is preferred due to the risk of allografts resorption.

Osteosarcoma is a common primary bone sarcoma and distal femur its most frequent site. Between 2003 and 2008 at Rizzoli, 66 patients with osteosarcoma of the distal femur had neoadjuvant chemotherapy, resection and reconstruction with modular uncemented mega-prostheses. Series included 37 males and 29 females. Mean follow up was 2 years. To measure “subjective” outcome Karnofsky scale (KPS) was assessed for each patient pre and post-treatment. Also a functional evaluation according to the MSTS system was performed. To find out the current quality of life, a questionnaire on life at work, study and sport before and after treatment was sent to 64 alive patients.

Before treatment 7 patients had a Karnofsky index (KI) of 60%, 31 of 50%, 25 of 40% and 3 of 30%. After treatment 19 patients had a Karnofsky index performance of 90%, 28 of 80%, 11 of 70%, 5 of 50% and 1 of 40%. Two patients died of disease. The most represented index of KPS after teatment was “Able to carry on normal activity; minor symptoms”. Poor results were related with amputation (2), knee stiffness (3), infection (2), aseptic loosening (1). After treatment 91% of patients had a KI over 70%, while 89% a KI lower than 50% pre-treatment. MSTS system showed excellent or good results in 85% and fair or poor in 15% of the patients. Average score at MSTS evaluation was 22 (73%). Questionnaires (some still pending) confirm previous analysis.

KPS is simple and effective in evaluating quality of life in patients treated for distal femur osteosarcoma. In this study it confirmed the satisfactory MSTS assessed results. It is an easy method, useful and accessible for patients. The reported analysis shows that patients treated for osteosarcoma of the distal femur can have a good quality of life.

Between 1983 and 2006 at Rizzoli 669 knee modular uncemented prostheses were implanted after resection of the distal femur, total femur or proximal tibia. These prostheses include 126 KMFTR prostheses and 543 second generation HMRS prostheses. Patients were followed periodically in the clinic. Data was obtained from clinical charts and imaging studies with special attention to major complications requiring revision surgery. Revision for polyethylene wear was considered a minor complication, since it did not imply failure of the implant. Functional results were assessed according to the MSTS system. Since data could be misleading due to deaths in an oncologic population (although 2/3 of patients were cured or long survivors), to censore the implant unrelated events Kaplan-Meyer curves of implant survival were studied.

In 126 KMFTR group infection rated 13.5%, stem breakage 13%, aseptic loosening 9.5%; change of polyethylene rated 44%. In 543 HMRS prostheses infection rated 8.6%, stem breakage 3%, aseptic loosening 4.8%; revision for polyethylene wear rated 9.6%. Techniques of revisions and their outcome analysed showed about 2/3 of good results, but increased risk of further complications in revised implants. Functional results (MSTS system) were good or excellent in 80% of KMFTR prostheses and in 90% of HMRS.

Decrease of major complications in newer prosthetic design was statistically significant and possibly due to newer materials and modified stem design. Polyethylene wear also decreased significantly. Function was satisfactory in most patients without complications for both groups. Revision surgery is technically demanding and appropriate timing of revision is crucial, since early treatment can improve final outcome.

Purpose of this paper was to review the Rizzoli experience in prosthetic reconstruction of the knee after resection of bone tumors with special attention to major complications and functional outcome.

Material: 669 knee modular uncemented prostheses were implanted between 1983 and 2006 after resection of the distal femur, total femur or proximal tibia. These prostheses include 126 first generation Kotz prosthesis (KMFTR) and 543 second generation HMRS prostheses.

Methods: All patients are followed periodically in the clinic. Data for this study was obtained from clinical charts; imaging studies were reviewed with special attention to prosthesis related major complications requiring revision surgery. Revision for polyethylene wear was considered a minor complications, since it does not imply change of main prosthetic components, thus failure of the implant. Functional results were assessed according to the MSTS system.

Since abrupt data could be misleading due to the oncologic population and related deaths (although 2/3 of the patients were cured or long survivors), to censore the implant unrelated events a statistical analysis of the implant survival was performed and Kaplan-Meyer curves of implant survival were studied.

Results: In the 126 KMFTR group major complications included infection 13%, breakage of the stems 12%, aseptic loosening 8.7%, while revision for polyethylene wear rated 45%.

In 543 HMRS prostheses major complications were infection 8%, stem breakage 2%, aseptic loosening 4%, while revision for polyethylene wear components rated 7.4%.

Techniques of revisions were analyzed, as well as the outcome of revised cases, which showed that about 2/3 of the patients treated for major complications do well, although the risk for further complications is significantly incresed in revised implants.

Functional results were evaluated according to the MSTS system: in KMFTR prostheses were good or excellent in 80% of the patients, while in HMRS were good or excellent in 90% of the patients.

Discussion: The reduction of major complication rate with the newer designs of the modular prosthesis was statistically significant and this seems to confirm that newer materials and a modified stem design positively affected the implant survival. Also the wear of polyethylene component was dramatically decreased in the newer prosthetic design. Functional results were satisfactory in most of the patients that did not experience major complications, with a trend to improvement in the newer design. Revision surgery is technically demanding and sometimes newer ‘hybrid’ techniques are suggested, implying the use of bone allografts.

Appropriate timing of revisions is crucial. An early treatment of complications can improve the final outcome.