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The Bone & Joint Journal
Vol. 104-B, Issue 12 | Pages 1352 - 1361
1 Dec 2022
Trovarelli G Pala E Angelini A Ruggieri P

Aims

We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone.

Methods

The search terms used in combination were “multicentric”, “giant cell tumour”, and “bone”. Exclusion criteria were: reports lacking data, with only an abstract; papers not reporting data on multicentric GCT; and papers on multicentric GCT associated with other diseases. Additionally, we report three patients treated under our care.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVII | Pages 240 - 240
1 Sep 2012
Ruggieri P Angelini A Drago G Guerra G Ussia G Mavrogenis A Mercuri M
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Introduction

Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We review our experience to characterize its prevalence, treatment, relapse and survivorship at long term follow-up.

Methods

Eighty-seven patients aged from 4 to 60 years (mean 20 years), were treated from 1985 to 2008. Lesions affected the femur (38), humerus (20), tibia (19), fibula (4), pelvis (3), foot (2) and radius (1). Eight patients had metastatic disease at diagnosis. Seventy-eight patients were treated with neoadjuvant chemotherapy with three or more drugs according to different protocols, nine had surgery as first treatment. Limb salvage surgery was performed in 71 cases, amputation in 14 and rotationplasty in one. One patient died before surgery. Prognostic factors were evaluated with Kaplan-Meier analysis.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVII | Pages 318 - 318
1 Sep 2012
Ruggieri P Pala E Angelini A Drago G Romantini M Romagnoli C Mavrogenis A Abati C Mercuri M
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Introduction

Dedifferentiated chondrosarcoma (D.C.) has a very poor prognosis. The efficacy of chemotherapy is still debated. Aim of this study was to evaluate the survival of patients with D.C. and to evaluate possible prognostic factors.

Methods

Between 1990 and 2006, 109 patients were treated for D.C.: 55 males and 54 females, mean age of 59.6 years. In 81 cases tumor was located in the extremities and in 28 cases in the trunk. The most frequent dedifferentiation was in osteosarcoma (53.2%) followed by spindle cell sarcoma (21%), malignant fibrous histiocytoma (13.8%), fibrosarcoma (6.4%). All patients received surgery and mostly, limb salvage with tumor resection and implantation of a megaprosthesis or allograft (65 patients). Chemotherapy was given to 43 patients.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVII | Pages 186 - 186
1 Sep 2012
Ruggieri P Mavrogenis A Rossi G Rimondi E Angelini A Ussia G Mercuri M
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Aim

To present selective arterial embolization with N-2-butyl Cyanoacrylate for the palliative and/or adjuvant treatment of painful bone metastases not primarily amenable to surgery.

Material and Methods

From January 2003 to December 2009, 243 patients (148 men and 95 women; age range, 20–87 years) with painful bone metastases were treated with N-2-butyl Cyanoacrylate. Overall, 309 embolizations were performed; 56 patients had more than one embolization. Embolizations were performed in the pelvis (168 procedures), in the spine (83 procedures), in the upper limb (13 procedures), in the lower limb (38 procedures) and in the thoracic cage (21 procedures). Primary cancer included urogenital, breast, gastrointestinal, thyroid, lung, musculoskeletal, skin, nerve and unknown origin. Renal cell carcinoma was the most commonly treated tumor. In all patients, selective embolization was performed by transfemoral catheterization.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVII | Pages 492 - 492
1 Sep 2012
Ruggieri P Mavrogenis A Ussia G Angelini A Pala E Guerra G Drago G Mercuri M
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Background

There is doubt regarding resection compared to curettage for pelvic metastases. Previous studies have reported that curettage is associated with decreased survival compared with wide resection, and have justified a radical surgical approach to achieve pain palliation and tumor control.

Aim

To evaluate the role of wide en bloc resection compared to curettage/marginal resection for patients with pelvic metastases. The rationale was that wide resection does not improve survival even in patients with solitary pelvic metastases.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVII | Pages 494 - 494
1 Sep 2012
Ruggieri P Angelini A Mercuri M
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Introduction

Although various reports analyzed “en-bloc” excision of sacral tumors, there are still technical problems to improve protection of nerve roots, preserve surrounding structures and reduce intraoperative bleeding, maintaining the oncologic result. We present a new technique for sacral resection, with short term preliminary results, derived with modification from Osaka technique.

Methods

Seven patients were resected for their sacrococcygeal chordoma with the followed described technique. Two patients had previous surgery elsewhere. The sacrum is exposed by a posterior midline incision and complete soft-tissue dissection. Lateral osteotomies were performed through the sacral foramina using a threadwire saw and Kerrison rongeurs, to avoid sacral roots damage. After proximal osteotomy, the sacrum is laterally elevated and mobilized to allow dissection of presacral structures. Mean surgical time was 5 hours (range: 3 to 8). Mean blood loss was 3640 ml.


Purpose

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

Material and methods

From October 2003 to September 2007 at Rizzoli 161 GMRS(r) 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(r) 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.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 19 - 19
1 Apr 2012
Ruggieri P Rossi G Mavrogenis A Ussia G Angelini A Rimondi E Mercuri M
Full Access

Aim

To present selective arterial embolization with N-2-butyl Cyanoacrylate for the palliative and/or adjuvant treatment of painful bone metastases not primarily amenable to surgery.

Material and Methods

From January 2003 to December 2009, 243 patients (148 men and 95 women; age range, 20-87 years) with painful bone metastases were treated with N-2-butyl Cyanoacrylate. Overall, 309 embolizations were performed;

56 patients had more than one embolization. Embolizations were performed in the pelvis (168 procedures), in the spine (83 procedures), in the upper limb (13 procedures), in the lower limb (38 procedures) and in the thoracic cage (21 procedures). Primary cancer included urogenital, breast, gastrointestinal, thyroid, lung, musculoskeletal, skin, nerve and unknown origin.

Renal cell carcinoma was the most commonly treated tumour. In all patients, selective embolization was performed by transfemoral catheterization.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 67 - 67
1 Apr 2012
Ruggieri P Pala E Calabrò T Angelini A Fabbri N Mercuri M
Full Access

Aim

was to analyze infections after bone tumour surgery.

Method

1463 patients treated from 1976 to 2007 were analized: 1036 with resection and prostheses in the lower limbs, 344 with resection and prostheses in the upper limbs, 83 with surgery for sacral tumours. Infections were analyzed for time of occurrence (“postoperative” in the first 4 weeks from surgery, “early” within 6 months, and “late” after 6 months), microbic agents, treatment, outcome.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 52 - 52
1 Apr 2012
Ruggieri P Angelini A Abati C Drago G Errani C Mercuri M
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Aim

To evaluate outcome and complications of knee arthrodesis with a modular prosthetic system (MUTARS(r) Implantcast), as primary and revision implants in musculoskeletal oncology.

Method

Between 1975 and 2009, 24 prostheses were used for knee arthrodesis. Nineteen in oncologic cases: 6 osteosarcomas, chondrosarcoma, synovial sarcoma and metastatic carcinoma 3 each, 2 pigmented villonodular synovitis (PVNS), malignant fibrous hystiocitoma and giant cell tumour 1 each. Patients were grouped into: A) primary implants, B) revision implants. Group A included 9 patients: 8 arthrodeses after extra-articular resection with major soft tissue removal, 1 after primary resection following multiple excisions of locally recurrent PVNS. Group B included 15 patients: 12 arthrodeses for infection (5 infected TKAs, 7 infected megaprostheses), 2 for failures of temporary arthodesis with Kuntscher nail and cement, 1 for recurrent chondrosarcoma in previous arthrodesis.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 328 - 328
1 Jul 2011
Ruggieri P Pala E Montalti M Angelini A Ussia G Abati CN Calabrò T Mercuri M
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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.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 336 - 336
1 Jul 2011
Ruggieri P Angelini A Ussia G Montalti M Calabrò T Pala E Abati CN Mercuri M
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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.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 329 - 329
1 Jul 2011
Ruggieri P Calabrò T Abati CN Pala E Ussia G Angelini A Montalti M Mercuri M
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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.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_II | Pages 119 - 119
1 May 2011
Ruggieri P Angelini A Pala E Ussia G Calabrò T Casadei R Mercuri M
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Purpose: Aim of this study was to analyse the incidence of infection in orthopaedic oncology after major surgical procedures for bone tumors.

Materials and Methods: We included patients with primary sacral tumors treated by major surgical procedure and patients with bone tumors of the upper and lower limb treated by resection and prosthetic reconstruction. Demographic data, surgery, adjuvant treatments, type of reconstruction were analyzed. Special attention was given to the infection: incidence, classification, microbic agents, treatment and outcome. Infections in the first 4 weeks were considered “postoperative”, those in the first 6 months were judged “early”, while “late” those diagnosed after 6 months. Overall 1462 patients treated in one institution from 1076 to 2007. Were considered 1036 patients with tumors of the lower limb, 344 patients with tumors of the upper limb and 82 sacral tumors. Univariate analysis with Kaplan-Meier actuarial curves was used in evaluating risk factors and implant survival to infections.

Results: In the lower limb, infection occurred in 80 cases (7.7%). Most frequent bacteria were gram positive. Infection was postoperative in 9 cases, early in 12, late in 59 cases and generally monomicrobial. Surgical treatment was “two stage” in 73 patients, “one stage” in 4 and primary amputation in 3 cases. Revisions for infection were successful in 63 pts (79%), while 17 pts were amputated (21%).

In the upper limb, in 20 patients (5.8%) a revision for deep infection was required. Two infections were postoperative, 7 early and 11 late. S. Epidermidis and S. Aureo were the most frequent bacteria causing infection (45%). “Two stage” treatment of infection was performed, but a new prostheses was implanted in 3 cases. In 17 the spacer was never removed.

In the sacrum, no deep infections were observed after intralesional excision for giant cell tumors. In 23/52 resections (44%) for chordoma (3 pts. died postoperatively and were excluded), infection occurred: in 16 patients postoperatively, in 7 within 6 months. Bacteria causing infection were mostly gram negative: in 74% of cases infection was multiagent. Surgical treatment consisted in one or more surgical debridements with antibiotics therapy according to coltures: infection healed in all cases.

Conclusion: Infection is a severe complication in prosthetic reconstructions for tumors of the upper and lower limb. Its incidence in the extremities (7.7% and 5.8%) is lower than after sacral surgery (44%). Infections are mostly late, monomicrobial and caused by gram positive in extremities, while early, multimicrobial and caused by gram negative in the sacrum.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_IV | Pages 604 - 604
1 Oct 2010
Ruggieri P Angelini A Calabrò T Mercuri M Montalti M Pala E
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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.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 457 - 457
1 Jul 2010
Montalti M Pala E Calabrò T Angelini A Ussia G Ruggieri P
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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.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 452 - 452
1 Jul 2010
Ruggieri P Pala E Ussia G Angelini A Abati CN Calabrò T Pignotti E Casadei R Mercuri M
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From October 2003 to September 2007 at Rizzoli 161 GMRS® prostheses were implanted after resections of the lower limb. This is a modular system with a knee rotating hinge mechanism, cemented and uncemented stems, in titanium and chromium-cobalt-molybdenum, curved and straight-fluted, with or without hydroxyapatite coating. Adaptors are available to revise HMRS® prostheses with hybrid implants. This study 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 for failures of previous reconstructions. Analysis of imaging and complications was performed and function assessed according to MSTS system. Kaplan-Meier curves were used to statistically evaluate implant survival.

At a mean follow up of 2.5 yrs. 106 patients are continuously NED, 31 NED after treatment of relapse, 7 AWD, 5 DWD. There were 10 major complications: 8 infections (4.7%) (5 in primary and 3 in secondary implants) and 2 aseptic loosenings (1.2%) (1 each). There were 9 minor complications requiring minor revisions. Comparative statistical analysis of implant survival showed no statistically significant difference between primary and secondary implants. Functional results were good or excellent in 95% of patients, without any poor.

Middle term results are promising with no breakages of implant components. Besides oncological cases, there are selected indications in non oncological settings, such as revisions of prosthetic or allografts failures. While a higher complication rate was expected in secondary implants, statistical analysis shows similar survival.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 439 - 439
1 Jul 2010
Alberghini M Ruggieri P Angelini A Ussia G Gambarotti M Ferrari C Vanel D Picci P Mercuri M
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Forty-six hemangioendotheliomas (HE) of bone treated at Rizzoli from 1985 to 2004 were studied with minimum follow up of 4 years: 19 females and 27 males, mean age 37 years, mean follow-up 9 years, 35 cases unifocal at diagnosis (10 spine – 1 with lung metastasis also- 11 lower limb, 8 upper limb, 6 pelvis) and 11 with multifocal involvement. In 10 patients intralesional surgery was previously performed elsewhere. In 27 patients primarily treated at Rizzoli with unifocal localization, surgery was used in 15 cases, surgery and radiotherapy in 7, surgery with radio/chemotherapy in 1 and no surgery in 4 (2 radiotherapy, 1 radio/chemotherapy and 1 embolization). Eight unifocal patients already treated elsewhere had surgery in 3 cases, surgery and radiotherapy in 3, surgery with radio/chemotherapy in 1 and surgery plus chemotherapy in 1. Three of the unifocal cases had further bone involvement subsequently. Nine multifocal patients primarily treated at Rizzoli had surgery in 4 cases, surgery and radiotherapy in 4, surgery with radio/chemotherapy in 1. The 2 previously treated multifocal HE had 1 surgery and 1 radiotherapy.

Six patients died: 3 of disease, 1 of radio-induced osteosarcoma, 2 of different disease. Two patients are AWD. Of remaining 40 patients, 26 are NED (mean follow up 9 years), 11 NED after treatment of recurrence, 1 NED after treatment of radio-induced sarcoma. No lung metastases were diagnosed after treatment. All 10 cases previously treated intralesionally had recurrence. Two of 15 unifocal cases treated with surgery recurred (13%). None of 9 resected unifocal cases previously untreated recurred. Two of 21 pts. with radiotherapy (9.5%) had radio-induced sarcoma.

Surgery is recommended, resection when feasible. Radiotherapy, implying risk of induced sarcoma, should be reserved to multifocal or unresectable cases. Adverse prognostic factor was previous intralesional surgery.