Correlation NKX2.2 IHC and EWSR1 break-apart FISH in the diagnosis of Ewing sarcoma: Can combined NKX2.2 and CD99 immunoexpression obviate or minimize the need of FISH testing? First assessment study from Indian tertiary cancer care center.

Context: Ewing sarcoma (ES) are malignant small round cell tumors (MSRCT) characterized by rearrangements of EWSR1 gene. Although gold standard for diagnosis is detection of specific fusion genes by molecular testing, these ancillary tests are costly and only available in limited number of settings. There is a persuasive evidence for reliability of NKX2.2 immunohistochemistry (IHC) as a surrogate marker for EWSR1 gene rearrangement in ES. Aims: The aim of this study is to correlate the NKX2.2 immuno-expression with genetically confirmed ES cases and also to assess the reliability and accuracy of NKX2.2 along with combined positivity of NXX2.2 and CD99 in diagnosing ES and differentiating it from other relevant histological mimics. Settings and Design: The present study is a retrospective study conducted over a period of 6-year duration in a tertiary cancer care center. Methods and Material: We evaluated NKX2.2 immunoexpression in 35 genetically confirmed cases of ES and also in pertaining differential entities (n = 58) of ES including rhabdomyosarcoma (n = 20), lymphoblastic lymphoma (n = 14), Wilms tumor (n = 10), poorly differentiated synovial sarcoma (n = 4), small-cell osteosarcoma (n = 4), neuroblastoma (n = 5), and mesenchymal chondrosarcoma (n = 1). CD99 was performed in the category of MSRCTs showing NKX2.2 positivity to evaluate combined specificity for the diagnosis of ES. Results: Of the 35 genetically confirmed cases of ES, 29 cases (83%) showed NKX2.2-positive expression (83% sensitivity). Compared to ES, NKX2.2 was positive in only 05% cases (3/58 cases) of non-ES MSRCT. Only two of five cases of neuroblastomas and one case of mesenchymal chondrosarcoma showed NKX2.2 positivity. CD99 positivity was seen in 100% of ES and in the single case of mesenchymal chondrosarcoma. All five cases (100%) of neuroblastoma were negative for CD99. Conclusions: The presented study, which is the first from an Indian oncology center, showed NKX2.2 IHC is quite reliable in diagnosis of ES in the right clinicopathological context. With remarkable sensitivity and specificity of NKX2.2 IHC for diagnosis of ES, we propose that combined positivity of CD99 and NKX2.2 IHC can obviate or minimize the need of EWSR1 gene rearrangement molecular testing for diagnosis of ES.

Analysis of Clinical Outcomes of Patients with Soft Tissue Sarcoma.

The aim of this study was to evaluate the outcome of patients with soft tissue sarcoma of the extremity and abdominal wall. This is the retrospective analysis of patients from a prospectively maintained data base from a single institute. We identified 79 patients with intermediate- to high-grade soft tissue sarcomas who were treated at our institute between Jan 2015 and July 2018. Low-grade tumors were excluded. There were 60 males and 19 females with a mean age of 44.6 years. Of the 79 sarcomas, 50 were in the lower limb and 24 in the upper limb and 5 were in abdominal wall. The commonest subtypes were undifferentiated pleomorphic sarcoma (n = 21) and synovial sarcoma (n = 19). Only 9 patients had metastatic disease at presentation. All 79 patients underwent surgical resection with an intent to achieve clear margins. Amputation was done in 19 patients while wide excision of the tumor was done in 60 patients. Adjuvant radiotherapy was given in 49 patients while adjuvant chemotherapy was given in 35 patients. At last follow-up (73 patients), 48 patients are alive without disease, 9 are alive with disease, 12 patients had died of disease, and 4 patients died due to other causes. Overall survival (OS) for 3 year is 77.6%, and estimated mean survival is 55.05 months. Relapse-free surviva (RFS)l at 3 year is 74.3%, and estimated mean RFS is 51.78. The only independent factor that affected the OS was the dimension of primary tumor (p = 0.02). For disease-free survival, the independent factors that affected outcome were stage at presentation (p = 0.04) and dimension of the tumor (p = 0.04). Short-term results shown by this study shows good outcome in patient with intermediate- to high-grade sarcomas when multidisciplinary approach is utilized for the management. Patients who had metastatic disease at presentation did worse than patients who did not.

Angiomatoid fibrous histiocytoma: Report of two cases, initially construed as sarcoma with unusual clinico-pathological features.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of uncertain differentiation with low metastatic potential, most commonly occurring in children, adolescents, and young adults, involving extremities. Due to its rare nature and diverse presentation, both clinically and morphologically, it is often misdiagnosed. It becomes important to correctly diagnose this lesion, given its distinct therapeutic implications. Here, we present the clinical, radiologic, and pathologic findings of two rare cases of AFH. Since AFH is a rare soft tissue tumor with low malignant potential, both pathologists and clinicians should be aware of this entity, when encountered with a soft tissue mass in extremities of a child or adolescent, so as to accord appropriate treatment in such cases.

Multifocal primary pseudomyogenic hemangioendothelioma of bone managed with denosumab: A rare case with diagnostic and therapeutic challenge.

Pseudomyogenic hemangioendothelioma (PMH) is a rare tumor of vascular origin with intermediate malignant potential which commonly presents as a subcutaneous and soft-tissue mass with or without concurrent bone involvement. However, PMH presenting as primary multifocal bone lesions is rare. Histomorphologically, it mimicks other epithelioid tumors and cytokeratin expression in PMH can prompt an erroneous diagnosis of metastatic carcinoma, especially in an elderly patient. Diligent histopathological examination and judicious immunohistochemistry panel can guide to the correct diagnosis. Due to its rarity, the optimal therapeutic strategy has not been established till date. We present a rare case of PMH of primary bone with multifocal bony disease in a 23-year-old male who presented with severe bone pains. The patient has been managed with four weekly denosumab, and the disease is stable with symptomatic relief after 6 months.

Primary Alveolar Soft Part Sarcoma of Bone: A Rare Occurrence in 8 Patients, Posing a Diagnostic Challenge.

CONTEXT.­: Alveolar soft part sarcoma is a rare soft tissue tumor involving mainly deep soft tissue of the lower extremities. Primary bone involvement is extremely rare. OBJECTIVE.­: To discuss histopathologic and immunohistochemical features of alveolar soft part sarcoma along with challenges in diagnosis and management in the context of primary bone origin. DESIGN.­: Our study constituted 8 patients. Data were collected from the Tata Memorial Hospital, Mumbai, India, during a 10-year period. RESULTS.­: Five patients in our study were female and 3 were male. Their ages ranged from 13 to 56 years. Primary bone involvement was seen in the humerus, tibia, fibula, radius, calcaneus, and rib. Radiologic impression was that of a primary malignant bone tumor in all patients. Conventional histopathologic features were seen in 7 of 8 patients. Positive immunohistochemical expression for TFE3 was demonstrated in 6 patients. All patients had distant metastasis either at presentation or later in the course of the disease. Surgical excision of the primary tumor and adjuvant chemotherapy formed the basis of treatment. Four patients succumbed to disease and 2 were alive with disease. CONCLUSIONS.­: Rare occurrences of primary bone alveolar soft part sarcoma posed a diagnostic challenge in the form of differential diagnoses of metastatic tumors resembling it either morphologically or immunohistochemically. The combined application of clinical and radiologic features along with characteristic histomorphology and immunohistochemistry helps to arrive at a definite diagnosis of alveolar soft part sarcoma. Aggressive behavior of this tumor and its refractoriness to conventional chemotherapy were evident from our series.

Epithelioid haemangioma of bone: A series of four cases with a revision of this contentious entity.

Epithelioid hemangioma of bone is a rare and locally aggressive vascular neoplasm of bone associated with a good prognosis. Because of its worrisome histomorphologic features and aggressive clinicoradiologic findings, at times with multifocal presentation, they tend to simulate malignant tumors. We report a series of four cases of epithelioid hemangioma of bone with their clinicopathologic characteristics. All had adjacent soft tissue involvement and two had multifocal bone disease. Microscopically, all cases had a tumor in lobular configuration, composed of epithelioid endothelial cells with the formation of well-formed vessels or grew in solid sheets. The tumor cells lacked significant cytologic atypia, necrosis, and increased mitosis. All cases were immunohistochemically positive for vascular markers CD34, CD31, ERG1, whereas negative for CK. Two of the cases were treated with excision, and the other two underwent curettage. None had local recurrence or metastasis on follow-up. This study highlights the importance of recognizing histomorphological and clinicoradiological features for distinguishing epithelioid hemangiomas from malignant vascular neoplasms of bone because of their distinct therapeutic implications and clinical outcomes.

Reliability and Role of Mutation-specific H3F3A (Histone 3-3) G34W Immunohistochemistry to Differentiate Giant Cell Tumor of Bone From its Clinicoradiologic and Histologic Mimics: An Institutional Study.

Giant cell tumor of bone (GCTB) is a benign neoplasm, which can sometimes be a diagnostic challenge, especially in small biopsies, due to its histologic benign and malignant mimics. We evaluated the role of H3.3 G34W immunohistochemistry (IHC) antibody in diagnosing GCTB and its role in differentiating it from its close histologic mimics. A total of 120 cases (80 cases of GCTB and 40 cases of histologic mimics) were retrieved and subjected to IHC. Of 80 cases of GCTB, 72 cases showed a positive nuclear immunoexpression, while all 40 cases of histologic mimics of GCTB showed a negative staining for H3.3 G34W IHC. Sensitivity and specificity of this mutation-specific antibody for diagnosis of GCTB was 90% and 100%, respectively, while, the positive predictive value and the negative predictive value were 100% and 83.3%, respectively. A positive expression of H3.3 G34W was seen in all 5 cases of GCTB, postdenosumab therapy, as well as, in all 3 cases of malignant giant cell tumor. The presented study showed that H3.3 G34W mutation-specific IHC is a reliable and specific marker for GCTB and can help distinguish it from the histologic mimics due to distinct therapeutic implications.

Parosteal osteosarcoma with high-grade transformation and lung metastasis after 12 years: a rare presentation.

Parosteal osteosarcoma represents a low-grade, well-differentiated type of cortical surface osteosarcoma with a relatively better prognosis. Systemic metastasis is rare overall and occurs in 10-15% of patients only. Delayed recurrences (>5 years) of parosteal osteosarcoma are even rarer and only few isolated case reports of local recurrence with systemic metastasis occurring beyond 10 years exist in the literature. We present here a case of femoral parosteal osteosarcoma, successfully treated previously, that recurred with high-grade transformation locally and with lung metastasis after 12 years. The patient was managed by lung wedge resection followed by type A1 rotationplasty. Histopathology report confirmed evidence of high-grade transformation (de-differentiation) both locally and at the metastatic site, in view of strong CDK4 and MDM2 nuclear immuno-expression. The present report highlights the importance of long-term follow-up, even for a seemingly less aggressive osteosarcoma variant with identification of a novel pattern of lung metastasis.

Elbow reconstruction after excision of proximal ulna tumors: Challenges and solutions.

Most malignant bone tumors are treated with surgical excision, adhering to oncologic principles, followed by reconstruction to preserve form and function whenever feasible. Primary bone tumors around the elbow are rare accounting for <1% of all skeletal tumors. They pose a reconstructive challenge, due to the complex interplay between the osseous & capsulo-ligamentous structures which is essential for elbow stability and function. Tumors affecting the proximal ulna are rare and reconstruction of the defects following these tumors is extremely challenging. Various reconstruction options like arthrodesis, autogenous bone grafts, allografts, re-implantation of sterilized tumor bone, pseudoarthrosis, and endoprosthesis have been tried with variable success. However, due to lack of standardization and the rarity of the site, surgeons are often in a dilemma to choose the correct option. This can lead to suboptimal functional outcomes and long-term failures. In this article, we reviewed the published literature on proximal ulnar tumors and noted the pros and cons of various reconstructive procedures. We have also attempted to formulate reconstruction recommendations based on the level of resection of proximal ulna.

Primary Fibular Sarcomas: Do They Behave Differently? An Institutional Review of 14 Patients.

Surgical resection with wide margins is pivotal for sarcoma treatment but achieving the same for fibular sarcomas is a surgical challenge. Thus, we decided to evaluate our own institutional database of primary fibular sarcomas for surgical treatment, margins and pattern of relapse. From July 2014 to October 2018, we identified fourteen patients with histologically confirmed fibular sarcomas. Limb salvage surgery (LSS) was performed in thirteen patients included in our study. One patient treated with definitive radiotherapy was excluded from final survival and functional analyses. The proximal third fibula was the most common site of involvement (85.7%). Osteosarcoma was the histological diagnosis in eight (57.1%) and Ewing's in the remaining six (42.9%). All patients with proximal fibular tumours underwent Malawer type II resection. Margins were reported as free in twelve and involved in one case. The mean follow-up period was 37.15 months. In the operated group (n = 13), distant relapse occurred in 3 patients, combined relapse in 1 patient and 10 patients are alive and disease free until the last follow-up. The Kaplan-Meier survival analyses revealed the EFS (event-free survival-local/distant relapse) probability as 72.7% at 24 months and 53% at the end of 42 months. The OS (overall survival) probability at 24 months was 75.5% and 57.5% at the end of 42 months. Although it is difficult to achieve conventional wide margins in fibular sarcomas, our results suggest no increased incidence of local recurrence rates as compared to sarcomas at other common sites as reported in literature. Our series helps in understanding site-specific behaviour of sarcomas while contributing to the available data.

Adamantinoma of Pelvis: A Rare Tumor at an Uncommon Site, with Diagnostic Implications.

Adamantinoma is a low-grade malignant bone tumor with metastatic potential in the range of 15-20%, commonly affecting mid-diaphyseal tibial region and jaw. Numerous cases of adamantinoma affecting the appendicular skeleton have been reported but only three in the pelvis till date. We present the case of a 24-year-old male, who was initially reported as fibrous dysplasia for a lesion in pelvis. On review, a cellular tumor was noted, including areas resembling fibrous dysplasia, along with cords and nests of polygonal cells, which displayed positive immunoexpression with cytokeratin and p40. Subsequently, he underwent wide local resection (internal hemipelvectomy), along with mesh pseudoarthrosis was done. Diagnosis of adamantinoma was further confirmed, with clear resection margins. Currently, the patient is on a regular clinical and radiological follow-up. Careful assessment of key histomorphologic features, coupled with immunohistochemical stains and clinico-radiological correlation, is helpful in identifying this uncommon tumor at a rather rare site.

Clear cell chondrosarcoma of proximal femur: a rare entity with diagnostic dilemma.

Clear cell chondrosarcoma is an extremely rare malignant neoplasm. The rarity and slow-growing nature of this tumour often lead to prolonged symptoms and also initial misdiagnosis with benign lesions such as chondroblastoma. It can also be confused with avascular necrosis of the femoral head when the lesion is located in the femoral head, as was in the case we report. The patient was kept on observation and conservative treatment for almost 9 years before the correct diagnosis and appropriate treatment. Wide local resection with negative margins forms the mainstay of treatment since intralesional procedures predispose to high local recurrence rate. A prolonged follow-up is recommended since late local recurrences and metastases are common.

Surgical management of pelvic bone sarcoma with internal hemipelvectomy: Oncologic and Functional outcomes.

INTRODUCTION: The management of pelvic sarcoma is challenging and goals of surgery are adequate oncologic local control, maintenance of optimum function with good quality of life. METHODS: We have evaluated the results of internal hemipelvecotmy including age, type of resection, reconstruction, radiotherapy or chemotherapy. From 2010 to 2016, 23 patients with pelvic bone tumors (13 with Ewing's sarcoma, 9 with Osteosarcoma, 1 with chondrosarcoma) were treated by surgical resection. RESULTS: The mean follow-up was 18 months (0.5-5) years. In 12 patients reconstruction was performed and 11 were without reconstruction. A total of 3 patients (13%) had an infection develop at a mean follow up of 1 month. Surgical debridement's and antibiotics in three patients led to complete recovery. One patient had sciatic nerve injury.One patient had injury to femoral vein; was treated with femoral vein reconstruction. Two patients (9%) developed a local recurrence and were treated with best supportive treatment. Distal pulmonary metastases were seen in four patients and treated with supportive treatment. Five-year disease-specific survival rates of all patients were 83%. The mean functional MSTS score was 18(14-24). CONCLUSIONS: Proper selection of patients, preopertive planning and wide surgical margins with reconstruction provides good functional outcomes following internal hemipelvectomy. The surgical site infection and flap necrosis tend to be minor complication and can be managed leading to optimal outcomes and justifies the need for this complex surgery. The oncological and functional outcome after internal hemipelvectomy suggests that it's an effective method for treatment of patients with pelvic sarcomas.

Giant cell tumor of distal radius treated with ulnar translocation and wrist arthrodesis.

INTRODUCTION: The aim is to analyze the functional outcomes of patients of giant cell tumor (GCT) of distal radius treated with ulnar translocation and wrist arthrodesis. METHODS: Study included 25 patients of aggressive GCT of distal radius, resected and reconstructed using ulnar translocation and wrist arthrodesis. The ulna-carpal radius fixation was performed with plate and screws. The patients were followed to bony union and minimum follow-up was 1 year. RESULT: Twenty-two patients were of Campanacci grade 3 and three patients were of Campanacci grade2. The mean follow-up was of 23 months (12-36). All patients had an excellent range of pronation and supination. The mean Musculoskeletal Tumor Society score was 24 (range 22-28). Grip strength of affected hand compared to the contra lateral hand was found good in 17 cases and average in 7 cases. The mean bone union time at ulna to radius junction was at 6.5 (5-8) months and ulna to carpal junction at 4.5 (4-6) months. The complications were surgical site infection (one case), recurrence (one case) and failure of union (one case), and ulna graft fracture with implant failure in (two cases). CONCLUSION: Reconstruction of distal end of radius using ulnar translocation and wrist arthrodesis provides excellent functional outcomes with preservation of rotational movement of forearm and hand function. Reconstruction of the distal radius by ulnar translocation without complete detachment from surrounding soft tissues functions like vascularized graft without use of microvascular techniques.

Acetabular fractures labelled poor surgical choices: Analysis of operative outcome.

PURPOSE: We report the surgical outcome in 52 patients with acetabular otherwise considered as poor surgical choices. METHODS: 43 male and 9 female patients were operated at a mean age of 43 years and followed up for a mean duration of 60.3 months. There were 22 elementary fractures and 31 associated ones according to Letournal and Judet classification. Osteosynthesis was attempted in 48 patients whereas a primary total hip arthroplasty was performed in 4 patients. Outcome was assessed radiologically and functionally employing Harris Hip Score (HHS). RESULTS: Average HHS in osteosynthesis group was 82.56 ± 12.4 with excellent to good results in 59.6% of the cases. Symptomatic osteoarthritis occurred in 13.5% of cases, avascular necrosis and severe heterotopic ossification in 7.7% each, infection and nerve palsy in 11.5% each. CONCLUSION: Although the complication rates in this series is marginally more than that reported in literature, we recommend that the indications of surgical fixation in acetabular fractures need to be extended to those which were considered poor surgical choices.

Outcome of surgical resection of pelvic osteosarcoma.

BACKGROUND: The aim of the following study is to evaluate the morbidity, oncologic results and functional outcome in nonmetastatic patients with primary osteosarcoma of the pelvis treated with surgical resection. MATERIALS AND METHODS: Twelve cases of nonmetastatic osteosarcoma of pelvis were operated as part of their multimodality treatment regime between November 2003 and May 2011. There were 5 males and 7 females with a median age of 22 years (range 8-39 years). Ten patients underwent limb sparing resections while 2 had a hindquarter amputation. All 10 cases of limb sparing surgery included resection of the acetabulum. A pseudarthrosis was carried out in 7 cases. Extracorporeal radiation therapy and reimplantation of the bone followed by fixation with plates was used in 1 case and an ischiofemoral arthrodesis was carried out in 2 cases. RESULTS: Surgical margins were free in 11 patients. Seven patients had a poor histological response to chemotherapy while 4 patients had a good response to chemotherapy. In the patient reconstructed with radiated auto bone graft, the histological response to chemotherapy could not be assessed. Surgery related complications were seen in 8 out of 12 patients (67%). Three of these patients (25%) required additional surgical intervention for their complications. All patients were available for followup. The median followup of survivors was 56 months (range 24-102 months). Four patients (33%) developed a local recurrence. At 5 years, overall survival was 67%. Patients with a good response to chemotherapy had a better overall survival when compared with patients with a poor response to chemotherapy. The mean Musculoskeletal Tumor Society functional score was 22 (range12-27). CONCLUSIONS: Though complex and challenging, surgery provides good local control and oncologic outcomes with acceptable function in patients with osteosarcoma of the pelvis treated with appropriate surgical resection as part of their multimodality treatment.

Oncological and functional outcome of periosteal osteosarcoma.

BACKGROUND: Periosteal osteosarcoma is an uncommon variant of osteosarcoma which constitutes less than 2% of all osteosarcomas. Whereas adequate surgical excision remains the cornerstone of treatment, the role of chemotherapy in this tumor is still unclear. Existing literature contains very few single center studies on the outcomes for periosteal osteosarcomas and any additional information will help in better understanding of these uncommon lesions. This study aims to evaluate the oncologic and functional outcomes of treatment of periosteal osteosarcoma treated at our institute. MATERIALS AND METHODS: A retrospective analysis of 18 cases of periosteal osteosarcoma treated between January 2001 and December 2010 was carried out. There were 12 males and 6 females. The mean age at presentation was 16.3 years (range 5-26 years). Tibia and femur were the most common sites (n = 8). 16 of 18 patients received chemotherapy, 16 had limb sparing resection, one had an amputation and one had rotationplasty. Of the 16 patients with limb salvage, conventional wide excision was done in 11 cases. In 5 cases tumor was excised with hemicortical excision. Of the 11 cases treated with wide excisions, 4 patients underwent an osteoarticular resection and in 7 patients a joint preserving segmental intercalary resection was done. RESULTS: All patients were available for followup. Surgical margins were free in all patients. A good response to chemotherapy was seen in 4/11 cases and poor in 6/11 cases. In one case the histological response was not discernible due to predominant chondromyxoid nature of the tumor. The median followup was 61 months (range: 18-130 months). There were two local recurrences (11%) at 9 and 18 months postsurgery. Pulmonary metastasis subsequently occurred in 4 cases (22%). Fourteen patients are currently alive and continuously disease free. Disease free survival at 5 years was 77.8% and overall survival (OVS) was 83.3%. Patients without marrow involvement had a better OVS at 5 years when compared with patients with marrow involvement (90% vs. 75%) (P = 0.23). CONCLUSION: Surgical excision remains the mainstay of treatment. Intramedullary involvement may suggest aggressive disease biology. The role of chemotherapy is still debatable and multicenter studies are needed to provide guidelines.

Outcome of Kienböck's disease in twelve cases: a mid-term follow-up study.

INTRODUCTION: No single study has established the superiority of one treatment of Kienböck's disease over the other. Pooled outcome data is presently considered the best way to add to the knowledge and understanding of Kienböck's disease. METHODS: A total of 12 patients (9 male and 3 female) with Kienböck's disease were included in the present case series. The mean age of the 12 patients was 28 years. One patient presented in Lichtman stage I, five in Lichtman stage II, five in Lichtman stage IIIa, and one in Lichtman stage IV. Univariate and multivariate analyses of the obtained data were performed to identify any correlations. RESULTS: The mean follow-up time was 62 months, and the mean modified Mayo wrist score improved from the preoperative value of 29.5 to the final value of 89.6. Lichtman stage at presentation showed moderate positive correlation with the duration of symptoms (r = 0.56), and a strong negative correlation with the preoperative and final modified Mayo scores (r = -0.89 and r = -0.77, respectively). The final modified Mayo score showed moderate negative correlation with the duration of the symptoms (r = -0.55). There was a significant difference in the preoperative modified Mayo scores of patients who presented in stage II and those of patients who presented in stage IIIa (p = 0.03). However, the difference in the final modified Mayo scores of the patients in these stages was not significant (p = 0.14). CONCLUSION: Lichtman's stage is moderately related to the duration of symptoms, suggesting natural progression of the disease. The final outcomes of stages II and IIIa were the same irrespective of the surgical treatment (radial shortening and/or vascularised bone grafting).

Results of surgical resection in pediatric pelvic tumors.

Thirty nonmetastatic patients with malignant tumors of the pelvis underwent surgical resection between November 2000 and December 2009. The diagnosis included Ewing's sarcoma (22), osteogenic sarcoma (four), chondrosarcoma (two), and synovial sarcoma (two). Eighteen resections included the acetabulum and 12 did not. Twenty-seven patients were available for follow-up. Follow-up ranged from 4 to 138 months (mean 43 months). Nineteen patients are currently alive. There were two local recurrences. The overall survival was 68% at 5 years. The Musculoskeletal Tumor Society Score ranged from 22 to 29. Surgery provides good local control and oncologic outcomes with acceptable function in these patients.

Primary cement spacers: a cost-effective, durable limb salvage option for knee tumors.

Of a total of 818 limb sparing resections in the lower limb requiring reconstruction between December 2002 and April 2010 at our centre, primary cement spacers were used in 15 cases. In three cases they were used as joint sparing intercalary reconstructions and in 12 cases knee arthrodesis was done. Implants used to provide stability to the construct included stacked intramedullary Kuntscher nails in four, an interlocking nail in one, plates in two and a combination of nail with plate in eight. Mean length of bone resected was 18 cm. Mean follow-up was 26 months (10-87 months). There were no local recurrences and none of the spacers needed revision for mechanical failure. The Musculoskeletal Tumor Society score for patients ranged from 20 to 29 with a mean of 24 (80%). Patients with intercalary resection had better functional scores than those with arthrodesis. The construct was successfully revised to a vascularised fibula arthrodesis or prosthesis with good eventual function in three cases. Cement spacers are a suitable cost-effective, durable reconstruction modality in selected patients with good functional outcomes. They are an option to amputation in patients with financial constraints and those that present with large volume or infected fungating tumors.