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.

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.

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 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.

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 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.

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.

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.

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.

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.