H.W.B. Schreuder

Intercalary allograft reconstructions following resection of primary bone tumors: a nationwide multicenter study.

BACKGROUND Favorable reports on the use of massive allografts to reconstruct intercalary defects underline their place in limb-salvage surgery. However, little is known about optimal indications as reports on failure and complication rates in larger populations remain scarce. We evaluated the incidence of and risk factors for failure and complications, time to full weight-bearing, and optimal fixation methods for intercalary allografts after tumor resection. METHODS A retrospective study was performed in all four centers of orthopaedic oncology in the Netherlands. All consecutive patients reconstructed with intercalary (whole-circumference) allografts after tumor resection in the long bones during 1989 to 2009 were evaluated. The minimum follow-up was twenty-four months. Eighty-seven patients with a median age of seventeen years (range, 1.5 to 77.5 years) matched inclusion criteria. The most common diagnoses were osteosarcoma, Ewing sarcoma, adamantinoma, and chondrosarcoma. The median follow-up period was eighty-four months (range, twenty-five to 262 months). Ninety percent of tumors were localized in the femur or the tibia. RESULTS Fifteen percent of our patients experienced a graft-related failure. The major complications were nonunion (40%), fracture (29%), and infection (14%). Complications occurred in 76% of patients and reoperations were necessary in 70% of patients. The median time to the latest complication was thirty-two months (range, zero to 200 months). The median time to full weight-bearing was nine months (range, one to eighty months). Fifteen grafts failed, twelve of which failed in the first four years. None of the thirty-four tibial reconstructions failed. Reconstruction site, patient age, allograft length, nail-only fixation, and non-bridging osteosynthesis were the most important risk factors for complications. Adjuvant chemotherapy and irradiation had no effects on complication rates. CONCLUSIONS We report high complication rates and considerable failure rates for the use of intercalary allografts; complications primarily occurred in the first years after surgery, but some occurred much later after surgery. To reduce the number of failures, we recommend reconsidering the use of allografts for reconstructions of defects that are ≥15 cm, especially in older patients, and applying bridging osteosynthesis with use of plate fixation.

Aneurysmal bone cyst: The role of cryosurgery as local adjuvant treatment

Aneurysmal bone cysts (ABCs) are most often treated with intralesional surgery (curettage) and additional bone grafting. There is debate on whether or not to use adjuvant therapy to decrease the local recurrence rate. This study is done to assess the outcome of curettage and cryosurgery as a treatment of ABC.

Oncological and functional results of cryosurgical therapy of enchondromas and chondrosarcomas grade 1.

Cryosurgery using liquid nitrogen is used as adjuvant treatment after intralesional resection of bone tumours to induce cell death. It is applied to enlarge the oncological margins of resection and to reduce the local recurrence rate. The objective of this study is to analyze the oncological and functional results.

Hemicortical Resection and Inlay Allograft Reconstruction for Primary Bone Tumors A Retrospective Evaluation in the Netherlands and Review of the Literature

BACKGROUND Selected primary tumors of the long bones can be adequately treated with hemicortical resection, allowing for optimal function without compromising the oncological outcome. Allografts can be used to reconstruct the defect. As there is a lack of studies of larger populations with sufficient follow-up, little is known about the outcomes of these procedures. METHODS In this nationwide retrospective study, all patients treated with hemicortical resection and allograft reconstruction for a primary bone tumor from 1989 to 2012 were evaluated for (1) mechanical complications and infection, (2) oncological outcome, and (3) failure or allograft survival. The minimum duration of follow-up was twenty-four months. RESULTS The study included 111 patients with a median age of twenty-eight years (range, seven to seventy-three years). The predominant diagnoses were adamantinoma (n = 37; 33%) and parosteal osteosarcoma (n = 18; 16%). At the time of review, 104 patients (94%) were alive (median duration of follow-up, 6.7 years). Seven patients (6%) died, after a median of twenty-six months. Thirty-seven patients (33%) had non-oncological complications, with host bone fracture being the most common (n = 20, 18%); all healed uneventfully. Other complications included nonunion (n = 8; 7%), infection (n = 8; 7%), and allograft fracture (n = 3; 3%). Of ninety-seven patients with a malignant tumor, fifteen (15%) had residual or recurrent tumor and six (6%) had metastasis. The risk of complications and fractures increased with the extent of cortical resection. CONCLUSIONS Survival of hemicortical allografts is excellent. Host bone fracture is the predominant complication; however, none of these fractures necessitated allograft removal in our series. The extent of resection is the most important risk factor for complications. Hemicortical resection is not recommended for high-grade lesions; however, it may be superior to segmental resection for treatment of carefully selected tumors, provided that it is possible to obtain adequate margins. LEVEL OF EVIDENCE Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Self-perception and quality of life in adolescents during treatment for a primary malignant bone tumour

PURPOSE Adolescents experience physical and psychosocial changes as part of their normal development. It can be hypothesized that they have lower scores on Quality of Life (QoL) and self-perception when additional changes occur due to cancer treatment. The purpose of our study was to assess self-perception and QoL of adolescents during or up to three months after adjuvant treatment for a primary malignant bone tumour. METHODS Ten adolescent patients (median age of 15 years) were included. Every patient was matched with two healthy peers. Participants completed the dutch version of the Self Perception Profile of Adolescents (SPPA) to measure self-perception and the KIDSCREEN-52 questionnaire for QoL. For both instruments, normative data were available. RESULTS Adolescents with a bone tumour had consistently lower scores on QoL as compared to healthy peers. Significantly on domains: physical well-being (P < 0.002), autonomy (P = 0.02), social support (P = 0.04) and school environment (P = 0.02). Scores on self-perception in this group were similar in both the study and control group. CONCLUSIONS Adolescents with a primary malignant bone tumour during or up to three months after adjuvant treatment had lower scores on QoL (KIDSCREEN-52), significantly on domains of physical well-being and social functioning. Unlike most other quality of life instruments, the KIDSCREEN-52 contains different areas of social functioning and has shown to be a useful instrument in our patient group. Scores on self-perception in this group were similar in both study and control group.

Bone cysts in patients with afibrinogenaemia: a literature review and two new cases

Afibrinogenaemia is an autosomal recessive disease with an estimated prevalence of approximately one in a million. The most common symptoms of afibrinogenaemia are umbilical cord bleeding, bleeding into skin, mouth, muscles, gastrointestinal and genitourinary tracts and the central nervous system. Other recognized complications include; haemarthroses, spontaneous splenic rupture, epistaxis, menorrhagia, recurrent abortion and venous and arterial thromboembolism. Bone cysts have also been described as a rare complication of afibrinogenaemia. The aim of this study was to conduct a systematic literature review, summarize the reported cases and to report two new cases. Three electronic databases were searched for relevant publications: PubMed, Medline and EMBASE. The following search criteria were used: ‘(bone cysts OR intraosseous haematoma OR intraosseous haemorrhage) AND (afibrinogenaemia OR fibrinogen deficiency)’. The reference lists of the selected papers were searched for more relevant literature. In total, eight patients had bone cysts as complication of afibrinogenaemia and six of them suffered from pain in their extremities. Bone cysts were primarily located in the vicinity of the cortex or trabeculae in the diaphysis of the long bones, especially in the femora, tibiae and humeri. Some were regressive, probably due to reactive bone remodelling. A number of cysts were filled with serosanguinous fluid. It might be useful to check for bone cysts when patients with congenital afibrinogenaemia complain of ‘rheumatic’ pains in their extremities. Whole body magnetic resonance imaging is the diagnostic imaging technique of choice. Recurrent episodes of pain, but not radiological deterioration, appear to benefit from prophylactic therapy with fibrinogen concentrate.

The push-through total femoral prosthesis offers a functional alternative to total femoral replacement: a case series

PurposeOncologic resections or complications of segmental femoral prostheses can result in severe bone loss of the femur for which a total femoral prosthesis (TFP) is required. This study assesses whether the loss of stability and function caused by the loss of muscle attachments can be improved by using a push-through total femoral endoprosthesis (PTTF), because it saves parts of the femur and its muscle attachments.MethodsIn this retrospective case series, ten patients aged 25–77 (mean 54) who received a PTTF between 2005 and 2014 were included for baseline, complications and survival analysis with a mean follow-up of 5.3 (1.1–9.6) years. Functional outcome was assessed in six patients using the Musculoskeletal Tumor Society (MSTS) score, WHO performance scale, Toronto Extremity Salvage Score (TESS), SF36, EQ-5D, NRS pain score, fatigue score and satisfaction score.ResultsThe mean MSTS score was 64% (23–93%). Five patients had a WHO performance scale of 1, one patient of 3. Mean TESS was 69% (13–90%). SF36 was most notably limited by physical functioning (mean 48), vitality (68) and general health (67). NRS score was 1.9, 1.8 and 8.3 for pain, fatigue and satisfaction, respectively. There were four failures: two infections (one resulting in amputation and one in a minor revision) and two mechanical failures (which required one revision to a TFP and one minor revision). Patient survival was 100%, limb survival 90%, and prosthesis survival 80%.ConclusionThe push-through total femoral endoprosthesis allows preservation of muscle attachments and offers a good alternative to total femoral prostheses.

The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column

BACKGROUND The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whether chemotherapy is preferred over decompressive laminectomy. OBJECTIVE To underline, in this case series, the efficiency of initial chemotherapy before upfront surgery in the setting of high-grade spinal cord or cauda equina compression of primary Ewing sarcoma. METHODS Fifteen patients with Ewing sarcoma primarily located in the spine were treated at our institution between 1983 and 2015. Localization, neurological deficit expressed as Frankel grade, and outcome expressed as Rankin scale before and after initial chemotherapy, the recurrence rate, and overall survival were evaluated. The multidisciplinary approach of 1 case will be discussed in detail. RESULTS Nine patients (60%) were female. The age at presentation was 15.0 ± 5.5 years (range: 0.9-22.8 years). Ten patients (67%) were initially treated with chemotherapy, and 1 patient (7%) was treated primarily with radiotherapy followed by chemotherapy. The remaining 4 patients (27%) were initially treated with decompressive surgery. All patients treated primarily nonsurgically improved neurologically at follow-up, showing the importance of chemotherapy as an effective initial treatment option. CONCLUSION Adequate and quick decompression of neural structures with similar results can be achieved by chemotherapy and radiotherapy, avoiding the local spill of malignant cells.

Severity classification of Tenosynovial Giant Cell Tumours on MR imaging

AIM Current development of novel systemic agents requires identification and monitoring of extensive Tenosynovial Giant Cell Tumours (TGCT). This study defines TGCT extension on MR imaging to classify severity. METHODS In part one, six MR parameters were defined by field-experts to assess disease extension on MR images: type of TGCT, articular involvement, cartilage-covered bone invasion, and involvement of muscular/tendinous tissue, ligaments or neurovascular structures. Inter- and intra-rater agreement were calculated using 118 TGCT MR scans. In part two, the previously defined MR parameters were evaluated in 174 consecutive, not previously used, MR-scans. TGCT severity classification was established based on highest to lowest Hazard Ratios (HR) on first recurrence. RESULTS In part one, all MR parameters showed good inter- and intra-rater agreement (Kappa≥0.66). In part two, cartilage-covered bone invasion and neurovascular involvement were rarely appreciated (<13%) and therefore excluded for additional analyses. Univariate analyses for recurrent disease yielded positive associations for type of TGCT HR12.84(95%CI4.60-35.81), articular involvement HR6.00(95%CI2.14-16.80), muscular/tendinous tissue involvement HR3.50(95%CI1.75-7.01) and ligament-involvement HR4.59(95%CI2.23-9.46). With these, a TGCT severity classification was constructed with four distinct severity-stages. Recurrence free survival at 4 years (log rank p < 0.0001) was 94% in mild localized (n56, 1 recurrence), 88% in severe localized (n31, 3 recurrences), 59% in moderate diffuse (n32, 12 recurrences) and 36% in severe diffuse (n55, 33 recurrences). CONCLUSION The proposed TGCT severity classification informs physicians and patients on disease extent and risk for recurrence after surgical treatment. Definition of the most severe subgroup attributes to a universal identification of eligible patients for systemic therapy or trials for novel agents.

Cryosurgery as Additional Treatment in Tenosynovial Giant Cell Tumors

Introduction. Tenosynovial giant cell tumors (TGCT) emerge from the synovium and can behave aggressively. Surgical resection is the standard treatment. However, up to half of the patients with diffuse type show recurrences. Several additional treatments have been applied to reduce recurrences; none of these treatments was proven to be superior to surgical resection solely. This article describes the results of additional cryosurgery to surgical resection. Materials and Methods. We retrospectively evaluated 141 TGCT patients, between 1999 and 2007. Twelve patients had additional cryosurgery. The knee (n = 8), hip (n = 2), ankle (n = 1), and elbow (n = 1) were affected. Primary outcome variables were treatment indications, recurrences, and complications. Results. Indications for additional cryosurgery were extended disease, bone involvement, and locations that are difficult to surgically get disease-free such as cruciate ligaments. Five patients had recurrent disease, all of which had prior treatments. None of the primary treated patients had recurrent disease. One patient had a deep infection. Discussion. Cryosurgery may serve as an additional treatment for diffuse TCGT in selected cases. However, because of the small number of patients and the heterogeneous group we could not prove an advantage of additional cryosurgery over surgical resection only. Cryosurgery should be considered for further evaluation in a prospective study. If there is any effect it would be helpful, especially in patients with multiple TGCT recurrences.