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Introduction: conventional parosteal osteosarcoma is an uncommon malignant bone tumor, comprising 4% of all osteosarcomas. Although rare, parosteal osteosarcoma is the most common type of osteosarcoma of the bone surface. We present the clinical, histological and imaging characteristics of a rare histologic variant of a parosteal osteosarcoma, review the literature and emphasize the importance of radio-pathological correlation as well as the interpretation of a representative biopsy in order to obtain the correct diagnosis., Case Report: a 36-year old woman began her condition one year prior to admission to the hospital with increased volume in the left knee and pain. Image studies showed a juxtacortical heterogeneous tumor localized on the posterior surface of the distal femoral metaphysis. An incisional biopsy was performed, with the diagnosis of a Parosteal Osteosarcoma and a wide surgical resection was undertaken. According to the findings of the surgical specimen, the diagnosis of a Parosteal Osteosarcoma with low grade chondrosarcoma and liposarcoma components was made. The knowledge of this rare parosteal osteosarcoma variant can lead the orthopedic oncologists to avoid overlooking the adipose component and provide adequate surgical margins., Conclusion: we present the clinical, histological and imaging characteristics of a Parosteal Osteosarcoma with low grade liposarcoma and chondrosarcoma components.

Introduction: Rhabdomyosarcoma (RMS) is a malignant solid tumor of mesenchymal origin. It is the most com mon soft-tissue sarcoma in childhood and adolescence. 65% of cases are diagnosed before the age of 6. Histological subtypes include embryonal, alveolar, pleomorphic, and fused-cell RMS. The embryo nal subtype is more frequent in children, while the alveolar one is more frequent in adolescents and adults., Objective: To describe the clinical presentation of primary alveolar rhabdomyosarcoma in a schoolgirl., Clinical Case: 7-year-old schoolgirl with one-month history of progressive pain in her left thigh. X-ray shows a lytic lesion in the left femur diaphysis. A study was performed with 2 biopsies, immunohistochemistry, and PAX-FOXO1 studies which were compatible with alveolar RMS. Con clusion: Primary alveolar rhabdomyosarcoma of the bone is rare, but it should be considered within the differential diagnosis of primary small-round-blue cell bone tumors. Despite presenting a poor prognosis cytogenetic, this type of tumor seems to have better biological behavior, which for a successful treatment makes necessary to have a high index of suspicion in order to install a multimodal therapy in the context of a national protocol.

Rationale: Osteosarcoma is the most common primary malignant bone tumor in children. The prognosis of osteosarcoma has improved with the use of aggressive systemic chemotherapy in addition to surgery. The relapse of osteosarcomas is usually as lung metastasis observed within 2 to 3 years after the initial treatment. A relapse is rarely observed at >10 years., Patient Concerns: We report the case of a 51-year-old Japanese man who was treated for high-grade osteosarcoma of the femur at 13 years old. He was referred to our hospital with a suspicion of primary lung cancer based on back pain, respiratory distress, and an abnormal mass on chest radiograph., Diagnoses: Computed tomography-guided biopsy confirmed the lung lesion as a metastatic recurrence of high-grade osteosarcoma without local recurrence., Interventions: Chemotherapy was planned, but the patient's general condition rapidly deteriorated and thus palliative therapy was provided., Outcomes: The patient died 2 months after the initial consultation., Lessons: The survival durations of osteosarcoma patients have been prolonged by recent progress in multimodality therapy, and thus clinicians as well as osteosarcoma patients should always keep in mind the possibility of very late relapse.

Osteosarcoma arising from cortical surface is classified into parosteal, periosteal and high-grade surface osteosarcoma. Along the spectrum, parosteal osteosarcoma occupies the well-differentiated end. It is a relatively rare disease entity, comprised only 4% of all osteosarcomas and barely reported in the literature. The objective of this study is to describe cases of parosteal osteosarcoma as well as a variety of treatment options amenable to such entity. Six cases of parosteal osteosarcoma were identified based on histopathological reports in a tertiary referral hospital in Jakarta, Indonesia between January 2001 and December 2019. The mean age was 29.8 years old; four of them (66.7%) were male. Distal end of femur was the most commonly involved bone (five cases, 83.3%). The patients were treated with wide excision followed by several different reconstruction methods: replacement with endoprosthesis, extracorporeal irradiation, knee arthrodesis, or prophylactic fixation. One of our patients presented with dedifferentiated component, and therefore was treated by limb ablation. While two cases died of pulmonary metastasis, other patients reported fair to excellent functional outcome., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2020 Yogi Prabowo et al.)

Introduction: The capacity of the hydroxyapatite-coated trochanteric screwed plates used with modular hip implants to restore abductor system efficacy after proximal femoral tumor resection has never been assessed. We therefore conducted a retrospective study aiming to: (1) quantitatively evaluate abduction conservation on dynamometry according to use of digastric reinsertion, conserving continuity between the gluteus medius and vastus lateralis muscles, or not, and of standard versus small-offset; (2) assess radiographic trochanteric plate fixation; (3) assess functional scores; and (4) assess complications., Hypothesis: Trochanteric reinsertion better conserves abduction strength when reinsertion is digastric., Patients and Methods: Thirty-one patients undergoing proximal femoral tumor resection between 2006 and 2016 with reconstruction by Stanmore METS™ modular implant with trochanteric plate were included. Twenty-one had digastric fixation between the gluteus medius and vastus lateralis and 10 had simple trochanteric fixation without digastric continuity. Abduction strength was compared between sides on dynamometer. Sixteen patients had full assessment of muscle strength, by a single observer; there were 8 deaths, 5 patients lost to follow-up, and 2 cases of material removal., Results: Abduction strength conservation versus the contralateral side was 55.2±23.3% (range, 5.8-86.1%): 66.6±13.0% (46.4-86.1) with versus 36.0±24.7% (5.8-63.2%) without digastric continuity (p=0.01); severe limp rate was 4/21 when digastric continuity was preserved (19%) versus 6/10 (60%) (p=0.04), and radiologic trochanteric reinsertion stability rate was 19/21 (90%) versus 4/10 (40%) (p=0.005). Standard femoral offset conserved greater abduction strength: 64.9±20.0% versus small-offset 45.4±23.2% (p=0.05). Toronto Extremity Salvage Score (TESS) was 89±9.4%, and Musculoskeletal Tumor Society (MSTS) score 75.4±5.4%. There were 6 complications (19%): 4 infections, 1 dislocation, and 1 plate removal; the single dislocation (3%) was in the digastric conservation group. TESS (90.7±7.8% vs 88.3±4) and MSTS score (75.6±4.0% vs 75.1±3.7) and complications [4/21 (19%) vs 2/10 (20%)] did not differ according to digastric or non-digastric reinsertion (p=1)., Conclusions: Abduction strength with a modular implant using a hydroxyapatite-coated trochanteric plate was better conserved by digastric trochanteric reinsertion, resulting in less limping, although the complications rate and functional scores were unaffected. Longer term assessment is needed to confirm this conservation of abduction strength., Level of Evidence: IV, retrospective study without control group., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Aim: The aim of this study was to assess outcomes of biological (nonvascularized fibula grafts and extracorporeal irradiated autologous bone grafts) methods used for reconstruction of intercalary defects after resection of femoral diaphyseal tumors., Materials and Methods: This study included 28 patients who had undergone intercalary resection in femoral diaphyseal tumors between 2011 and 2016. The mean follow-up period was 24 months (range 12-57 months)., Results: The mean union time for diaphyseo-diaphyseal union was 10.5 and 11 months in nonvascularized fibula group and extracorporeal radiotherapy (ECRT) group, respectively. The mean union time for metaphyseo-diaphyseal union was 6.5 months in both nonvascularized fibula and ECRT groups. Six patients had distant metastasis, and one patient had local recurrence. The mean Musculoskeletal Tumor Society score was 28 at the last follow-up. Two patients had surgical site infection in the nonvascularized fibula group. Implant failure was found in one patient of the ECRT group requiring revision surgery. Three patients had nonunion (two from the nonvascularized fibula group and one from the ECRT group)., Conclusion: The present study indicates that the biological reconstruction modalities provide good functional outcomes in diaphyseal tumors of femur. Nonvasularized fibula and ECRT-treated autografts reconstruction provides good results, and union timing is comparable. The outcomes of the current study are promising as compared to the results in the reviewed literature. The reconstruction method depends on the resources available at the oncological center and the conversance with the method of the treating surgeon.

Background: The proximal femuris is an uncommon site of osteosarcoma. The unusual manifestations at this site may lead to diagnostic and therapeutic mistakes. We therefore performed a retrospective study to estimate the proportions of patients with imaging study findings and/or clinical manifestations typical for osteosarcoma and/or inappropriate treatment decisions., Hypothesis: Proximal femoral osteosarcoma often produces atypical clinical and radiological presentations., Material and Methods: Consecutive patients who underwent surgery at our center to treat proximal femoral osteosarcoma were included. For each patient, we collected the epidemiological characteristics, clinical symptoms, imaging study findings, treatment, and tumor outcome. Proportions were computed with their confidence intervals., Results: Twelve patients had surgery for proximal femoral osteosarcoma between 1986 and 2015. Imaging findings were typical in 1 (8%) patient; they consisted of ill-defined osteolysis in 11/12 (92%) patients, a periosteal reaction in 1/12 (8%) patient, soft tissue involvement in 7/12 (58%) patients, and immature osteoid matrix in 11/12 (92%) patients. No patient had the typical combination of pain with a soft tissue swelling. Management was inappropriate in 2/12 (17%) patients, who did not undergo all the recommended imaging studies before surgery and were treated in another center before the correct diagnosis was established. At last follow-up, 4 patients had died (after a mean of 7 years) and 8 were alive (after a mean of 4 years)., Conclusion: Proximal femoral osteosarcoma is uncommon and rarely produces the typical clinical and imaging study findings. The atypical presentation often results in diagnostic errors and inappropriate treatments. Ill-defined osteolysis on standard radiographs should prompt computed tomography or magnetic resonance imaging of the proximal femur. Treatment in a specialized center is imperative., Level of Evidence: IV, retrospective study., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: The aim of this study was to compare the functional outcomes and complication rates after distal femoral replacement (DFR) performed with the modular Munich-Luebeck (MML) modular prosthesis (ESKA/Orthodynamics, Luebeck, Germany) in patients being treated for malignant disease or failed total knee arthroplasty., Methods: A retrospective review of patient charts and a functional investigation (involving Musculoskeletal Tumor Society Score [MSTS], American Knee Society Score [AKSS], Oxford Knee Score [OKS], Western Ontario and McMaster Universities Osteoarthritis Index [WOMAC], Toronto Extremity Salvage Score [TESS], the 12-Item Short-Form [SF-12] Health Survey, and a failure classification system developed by Henderson et al.) of DFR cases from 2002 to 2015 were conducted. The indications for DFR were malignant tumor resection in the femur (n = 20, group A) or failure of revision total knee arthroplasty without a history of malignant disease (n = 16, group B)., Results: One-hundred and twenty-nine patients were treated during the study period. Of these, 82 were analyzed for complications and implant-survival. Further, 36 patients were available for functional assessment after a mean follow-up of 86 months (range: 24-154). There were 75 complications in total. The overall failure rate for DFR was 64.6% (53/82 patients). The most common failure mechanisms were type III (mechanical failure), followed by type I (soft tissue) and type II (aseptic loosening). The mean MSTS score (out of 30) was 17 for group A and 12 for group B. All the clinical outcome scores revealed an age-dependent deterioration of function., Conclusion: DFR is an established procedure to restore distal femoral integrity. However, complication rates are high. Post-procedure functionality depends mainly on the patient's age at initial reconstruction.

Background: The aim of this retrospective study was to investigate the frequency of intra-articular osteoid osteoma (iaOO) in a large study cohort and to demonstrate its clinical relevance as an important differential diagnosis of non-specific mono-articular joint pain., Methods: We searched the registry for bone tumours of the University Medical Centre Hamburg-Eppendorf for osteoid osteomas in the last 42 years. Herein, we present three selected iaOO which were detected in the three major weight-bearing joints. Computed tomography (CT) or magnetic resonance imaging (MRI) scans were performed for initial diagnosis., Results: Out of a total of 367 osteoid osteomas, 19 (5.2 %) tumours were localized intra-articularly. In all three presented tumours, a history of severe mono-articular pain was reported; however, the mean time to correct diagnosis was delayed to 20.7 months. Clearly, the nidus seen in CT and MRI images in combination with inconsistent salicylate-responsive nocturnal pain led to the diagnosis of iaOO., Conclusions: Rarely, osteoid osteoma can occur in an intra-articular location. In cases of diffuse mono-articular pain, iaOO should be considered both in large and smaller joints to avoid delays in diagnosis and therapy of this benign bone tumour.

Review current treatments of metastatic lesions in the proximal femur.We reviewed published literature related to diagnosis and surgical treatments and summarized current treatment options.Surgical management mainly consist of internal fixation, hip replacement, and percutaneous femoroplasty (PFP) which has been newly applied in clinical practice.An appropriate series of treatments is necessary for patients to avoid the occurrence of paraplegia and prolong survival time.

Background: Adamantinoma arising in the femur is extremely rare. We report a case of an adamantinoma occurring in the right medial femoral condyle that was diagnosed 5 years after the primary surgery., Case Presentation: A 74-year-old Asian woman first complained of right knee pain without any cause. Radiographs demonstrated a 4×4.5 cm osteolytic lesion in her medial femoral condyle. Magnetic resonance imaging revealed a lesion which showed low signal on both T1 and T2-weighted image, and enhanced signal with gadolinium contrast administration. She underwent a wide resection of the lesion and was reconstructed with a tumor endoprosthesis. On histological examination, the tumor showed clusters of spindle-shaped and squamoid epithelial cells among the fibrous stroma. Adamantinoma was considered, however, the diagnosis was inconclusive due to the unusual localization and her age. Moreover, it was difficult to exclude metastatic carcinoma. Five years later, she was diagnosed with an abnormal shadow occupying the upper lobe of her right lung in a routine physical examination. She subsequently underwent a resection of the lung mass which histologically showed proliferation of spindle-shaped and squamoid epithelial cells. The histological similarity of the lung tumor and the femoral tumor led to the diagnosis of adamantinoma arising in her right medial femoral condyle with metastasis to the upper lobe of her right lung., Conclusion: In this case report, we report the clinical, radiographic, and histological features of an adamantinoma arising in the distal femur with a review of the literature.

Carcinoembryonic antigen related cell adhesion molecule 1 (CEACAM1) is a trans-membrane multifunctional cell adhesion molecule associated with tumor cell proliferation, apoptosis, angiogenesis, invasion, and migration during tumor development. In the present study, we evaluated serum CEACAM1 level in osteosarcoma patients to explore its diagnostic and prognostic value for this particular malignancy. Sera from 113 patients with primary osteosarcoma, 98 patients with benign bone tumors and 126 healthy controls were obtained. Serum CEACAM1 level was measured with ELISA and correlation with clinicopathological characteristics was further analyzed. Receiver operating curves (ROC), Kaplan-Meier curves, and log-rank analyses as well as Cox proportional hazard models were used to evaluate diagnostic and prognostic significance. The results revealed that serum CEACAM1 level was significantly higher in osteosarcoma patients compared to benign bone tumors and healthy controls (455.2 ± 179.9 vs 287.4 ± 103.2, 260.8 ± 109.7 pg/ml, respectively). Osteosarcoma patients with larger tumors, later-tumor stages, low tumor grades, and distant metastases had much higher CEACAM1 compared to those with smaller tumors, earlier tumor stages, high tumor grades and non-distant metastases (P < 0.05 for all). Multivariate logistic regression analysis confirmed that high serum CEACAM1 level was an independent risk factor for distant metastases (OR = 3.02, 95%CI 1.65-4.17). To distinguish osteosarcoma patients from those with benign bone tumor and healthy controls, ROC/AUC analysis indicated an AUC of 0.81 (sensitivity 0.61; specificity 0.89) and an AUC of 0.77 (sensitivity 0.57; specificity 0.92), respectively. Osteosarcoma patients with higher CEACAM1 had relatively lower survival compared to those with low CEACAM1 (P < 0.01), and multivariate analyses for overall survival revealed that high serum CEACAM1 level was an independent prognostic factor for osteosarcoma (HR = 1.56, 95%CI 1.23-3.28). The present study suggested that elevated serum CEACAM1 level might be a novel diagnostic and prognostic biomarker for osteosarcoma patients.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterised by severe hypophosphataemia and osteomalacia, with renal phosphate wasting that occurs in association with tumour. The epidemiology likewise aetiology is not known. The clinical presentation of TIO includes bone fractures, bone and muscular pains, and sometimes height and weight loss. TIO may be associated with mesenchymal tumours which may be benign or malignant in rare cases. Mesenchymal tumour itself may be related to fibroblast growth factor 23 (FGF23), which is responsible for hypophosphataemia and phosphaturia occurring in this paraneoplastic syndrome. Hypophosphataemia, phosphaturia and elevated alkaline phosphatase are the main laboratory readings that may lead to more precise investigations and better diagnosis. Finding the tumour can be a major diagnostic challenge and may involve total body magnetic resonance imaging, computed tomography and scintigraphy using radiolabelled somatostatin analogue. The treatment of choice for TIO is resection of a tumour with a wide margin to insure complete tumour removal, as recurrences of these tumours have been reported. We provide here an overview on the current available TIO case reports and review the best practices that may lead to earlier recognition of TIO and the subsequent treatment thereof, even though biochemical background and the long-term prognosis of the disease are not well understood. This review also includes a 4-year-long history of a patient that featured muscular pains, weakness and multiple stress fractures localised in the hips and vertebra with subsequent recovery after tumour resection. Because the occurrence of such a condition is rare, it may take years to correctly diagnose the disease, as is reported in this case report.

Malignant transformation of fibrous dysplasia (FD) in long bones is rare (less than 1% of cases) and occurs in the form of osteosarcomas. They can occur on FD associated with multiple myxomas, which corresponds to Mazabraud syndrome. To our knowledge, only six cases of FD malignant transformation into osteosarcoma in the context of Mazabraud syndrome have been described. Here, we describe the first case of malignant transformation of femur FD into chondrosarcoma in a 51-year-old female patient. The patient had significant changes in the size and number of myxomas around the femur before the malignant transformation into chondrosarcoma. She underwent complete femur replacement with a favourable result after 4 years' follow-up. Close surveillance is strongly recommended in this syndrome, particularly when the number and size of myxomas increase., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

Background: We tried to compare the functional and psychosocial outcomes after various reconstruction methods according to tumor location following resection of osteosarcoma in distal femur., Methods: We retrospectively reviewed 51 patients who underwent limb-salvage surgery of osteosarcoma in distal femur in our institution, 30 males and 21 females with an average age of 21 years (range 13-51 years). We classified osteosarcoma in distal femur into 3 types, and organized affected limb reconstruction methods after wide resection. MSTS and QOL scores were used to analyze the functional and psychological outcomes., Results: After a mean follow-up of 43 months (12-225 months), there is no difference on functional results and QOL scores among three reconstruction groups (p > 0.05) and among three types groups (p > 0.05). No difference could be noticed on tumor-free survival and total survival among three reconstruction groups (p > 0.05) and three type groups (p > 0.05). In ≤2-year, better functional scores could be found in prosthesis group, rather than the other two inactivated-bone groups (p < 0.05)., Conclusions: Biological reconstruction with alcohol-inactivated autograft replantation could avoid prosthesis related complications and achieved comparable results with prosthesis following resection of osteosarcoma in distal femur. Different reconstruction options could be chosen according to tumor location, such as the distance to Insall line.

We report 2 cases of late recurrence of osteosarcoma after 6 and 7 years. One patient had pulmonary metastasis, and the other had soft tissue recurrence. Both patients underwent complete resection and chemotherapy. The first patient achieved complete remission and remained disease-free 47 months later and had no limitation in his daily life. The second patient had a re-recurrence and underwent further resection and chemotherapy. He remained disease-free 35 months later and could walk using a T-handled walking cane.

Background: Treatment of chondroblastoma in the femoral head is challenging owing to the particular location and its aggressive nature. There is little published information to guide the surgeon regarding the appropriate approach to treating a chondroblastoma in this location. We developed a modified trapdoor procedure to address this issue. The primary modification is that the window surface of the femoral head is covered by the ligamentum teres rather than cartilage as in the traditional procedure., Questions/purposes: We assessed (1) the clinical presentation of chondroblastoma of the femoral head and treatment results with the modified trapdoor procedure in terms of (2) the frequency of local recurrence, (3) complications, and (4) functional outcomes using the Musculoskeletal Tumor Society (MSTS) score., Methods: Between 1999 and 2010, we treated 14 patients for chondroblastoma of the femoral head. All patients received the modified trapdoor procedure. Of those, 13 were available for followup at a minimum of 36 months (mean, 66 months; range, 36-117 months) and one patient was lost to followup. There were nine males and four females, with a mean age of 18 years (range, 9-29 years). Clinical features were ascertained by chart and radiographic review, and recurrence, complications, and functional outcomes (MSTS score) were recorded from chart review. Patterns of bone destruction were evaluated using the Lodwick classification, which ranges from IA (geographic appearance with sclerotic rim) to III (permeative appearance)., Results: The symptoms at diagnosis were pain in nine patients and discomfort in four. The mean duration of symptom was 11 months (range, 1-36 months). The physis was open in two patients, closing in one, and closed in 10. The patterns of bone destruction were evaluated as Lodwick Class IA in six patients, Lodwick Class IB in five, and Lodwick Class IC in two. At latest followup, no local recurrence was observed. Two patients had postoperative complications. One had avascular necrosis of the femoral head and was treated with prosthesis replacement. The other had asymptomatic heterotopic ossification in the surgical field. The mean MSTS score was 29.6 (range, 28-30)., Conclusions: Based on this small series, we believe our modified trapdoor procedure is a safe, effective means of treating a chondroblastoma in the femoral head, but additional clinical evaluation with more patients is necessary to confirm our findings., Level of Evidence: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Context: Tumor-induced osteomalacia is curable if the tumors can be totally excised. However, when the tumors are present in locations that make surgery disproportionately risky, the need for less invasive strategies like radiofrequency ablation (RFA) is realized., Patients and Methods: We describe three patients with suspected tumor-induced osteomalacia who were treated in our department between 2006 and 2013 with tumors in surgically difficult locations and were subjected to single or multiple sessions of RFA. The response was documented in terms of symptomatic improvement, phosphorus normalization, and follow-up (99m)Technitium-labelled hydrazinonicotinyl-Tyr3-octreotide ((99m)Tc HYNIC TOC) scan., Results: Two of the three individuals, patient A (with a 1.5 × 1.2-cm lesion in the head of the right femur) and patient B (with a 1.3 × 1.2-cm lesion on the endosteal surface of the shaft of the left femur), achieved complete remission with single sessions of RFA. Three months after the procedure, (99m)Tc HYNIC TOC scans revealed the absence of uptake at the previous sites, corroborating with the clinical improvement and phosphorus normalization. Patient C had a large 5.6 × 6.5-cm complex lesion in the lower end of the left femur with irregular margins, loculations, and bone grafts placed in previous surgery. He failed to achieve remission after multiple sessions of RFA due to the complex nature of the lesion, although the tumor burden was reduced significantly as documented on serial (99m)Tc HYNIC TOC scans., Conclusions: Although surgery remains the treatment of choice, RFA could be an effective, less invasive, and safe modality of treatment in judiciously selected patients.

Pregnancy complicated by Non Hodgkin lymphoma (NHL) is rare, about 100 cases have been reported. We will describe the case of a multifocal bone lymphoma revealed by a left hyperalgetic and deficient cruralgia in a female in the second trimester of pregnancy.

Background: Coccidioidomycosis is an endemic fungal infection in the southwestern of United States. Most infections are asymptomatic or manifest with mild respiratory complaints. Rare cases may cause extrapulmonary or disseminated disease. We report two cases of knee involvement that presented as isolated lytic lesions of the patella mimicking neoplasms., Case Presentation: The first case, a 27 year-old immunocompetent male had progressive left anterior knee pain for four months. The second case was a 78 year-old male had left anterior knee pain for three months. Both of them had visited general physicians without conclusive diagnosis. A low attenuation lytic lesion in the patella was demonstrated on their image studies, and the initial radiologist's interpretation was suggestive of a primary bony neoplasm. The patients were referred for orthopaedic oncology consultation. The first case had a past episode of pulmonary coccioidomycosis 2 years prior, while the second case had no previous coccioidal infection history but lived in an endemic area, the central valley of California. Surgical biopsy was performed in both cases due to diagnostic uncertainty. Final pathologic examination revealed large thick walled spherules filled with endospores establishing the final diagnosis of extrapulmonary coccidioidomycosis., Conclusions: Though history and laboratory findings are supportive, definitive diagnosis still depends on growth in culture or endospores identified on histology. We suggest that orthopaedic surgeons and radiologists keep in mind that chronic fungal infections can mimic osseous neoplasm by imaging.

Background: When the primary tumor is unknown, the biopsy and the histopathological examination (associated with immunohistochemistry and molecular profiling) will identify the type and subtype of the tumor and, if possible, the site of origin. The classification in type and subtype will allow to assess the prognosis and to indicate the most appropriate therapeutic approach., Aim: Identification of the unknown primary tumor by biopsy and immunohistochemistry from the femoral bone metastasis, the clinical results and the survival rate after osteosynthesis ± cement stabilization of the lesion., Patients and Methods: 52 patients with femoral metastasis were included in a prospective study. The gender ratio was 30 women/22 men (average age - 64 years and six months; range: 33-82 years). Thirty-three patients had pathologic bone fracture, while 19 had osteolytic lesions without fracture. The mean follow-up for survivors was 34 months (range: 17-56 months). Surgical treatment consisted in hemiarthroplasty, osteosynthesis with DHS (Dynamic Hip Screw), proximal femoral nail, locked centromedullary nail or DCS (Dynamic Condylar Screw) ± cement. In 19 (36.54%) cases, the primary tumor was not known. In these cases, biopsy and histological examination with immunochemistry were performed. Twenty-three (52.27%) patients underwent chemotherapy. Clinical and radiological check-ups were performed every three months in the first year and every six months after that., Results: Pain was ameliorated in all cases. Deambulation was achieved in 45 out of 52 (86.54%) patients. Survival rate was 76.92% (40/52) at six months and 59.61% (31/52) at 12 months. At the end of the follow-up period, 18 (34.61%) patients were alive, 24 (46.15%) were deceased and 10 (19.23%) were lost to follow-up. After eliminating the patients lost to follow-up, the survival rate was 33.33% (9/27) in the pathologic fracture group and 60% (9/15) in the osteolytic lesion without fracture group., Conclusions: Most of the patients (86%) could be mobilized immediately after surgery. The expected survival rate one year after surgery is around 60%. The goals of osteosynthesis are the same, regardless the location of the lesion and the implant used: pain amelioration, appropriate stability for immediate full weight bearing, durability for patient's life expectancy. All extended osteolytic lesions must be reinforced at the time of the surgical procedure. The presence of a pathologic fracture is a negative prognosis factor for the medium term survival rate.

The purpose of this study was to assess whether the use of a joint-sparing technique such as curettage and grafting was successful in eradicating giant cell tumours of the proximal femur, or whether an alternative strategy was more appropriate. Between 1974 and 2012, 24 patients with a giant cell tumour of the proximal femur were treated primarily at our hospital. Treatment was either joint sparing or joint replacing. Joint-sparing treatment was undertaken in ten patients by curettage with or without adjunctive bone graft. Joint replacement was by total hip replacement in nine patients and endoprosthetic replacement in five. All 11 patients who presented with a pathological fracture were treated by replacement. Local recurrence occurred in five patients (21%): two were treated by hip replacement, three by curettage and none with an endoprosthesis. Of the ten patients treated initially by curettage, six had a successful outcome without local recurrence and required no further surgery. Three eventually needed a hip replacement for local recurrence and one an endoprosthetic replacement for mechanical failure. Thus 18 patients had the affected joint replaced and only six (25%) retained their native joint. Overall, 60% of patients without a pathological fracture who were treated with curettage had a successful outcome.

Although standard magnetic resonance angiography (MRA) has many advantages in the evaluation of peripheral artery disease, it has poorer spatial resolution compared with computed tomographic angiography and digital subtraction angiography. The MRA blood pool contrast agents have an extended half-life. By allowing longer imaging times, MRA enables the acquisition of high-resolution images as well as providing simultaneous visualization of both arteries and veins. Two cases of popliteal artery aneurysm are presented, in which MRA with a blood pool contrast agent provided critical clues to the correct diagnosis: a traumatic pseudoaneurysm of the popliteal artery caused by a femoral osteochondroma and a large fusiform popliteal aneurysm compressing the adjacent popliteal vein, causing venous congestion and leg pain., (Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

Pathological fracture is not uncommon in musculoskeletal oncological surgical practice. When complicated by infection, it is both limb- threatening and life-threatening problem. Pathological fractures require urgent investigation, not urgent treatment. We present the case of a 75-year-old man who presented with an infected sarcoma causing a pathological fracture of his right femur.

Objective: To study and evaluate the unusual findings in the natural history of parosteal osteosarcomas. Parosteal osteosarcomas are well-differentiated tumours of low grade malignancy, but may dedifferentiate into a more aggressive lesion., Material and Methods: We reviewed 7 parosteal osteosarcomas treated in La Paz Hospital between 2005 and 2009 (3 were dedifferentiated parosteal osteosarcomas). The authors analysed the clinical and radiological features, histology, treatment and outcomes in this patient group., Results: There were 5 men and 2 women, with a mean age of 32.14 years, range 24-47 years. Three of seven tumours (42.8%) were dedifferentiated osteosarcomas. All three patients received chemotherapy. One patient presented with pulmonary metastases. No patient died., Conclusions: It is important to understand that the biological phenomenon of dedifferentiation can occur in parosteal osteosarcomas. The prognosis, treatment and survival changes radically., (Copyright © 2011 SECOT. Published by Elsevier España, S.L. All rights reserved.)

We report a case of giant cell tumor of the left distal femur with simultaneous bilateral pulmonary metastases. Pathologic examination of the left lung nodule showed a metastatic giant cell tumor with a noncontinuous ossified rim, and the right lung nodules were totally hyalinized or ossified without residual giant cell tumor components. Hyalinization was a consistent finding in both the primary bone lesion and the pulmonary metastases. Because the patient did not receive chemotherapy or radiotherapy before her surgical procedure, we believe that these changes represent spontaneous tumor regression., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Different aetiologies including the femoroacetabular impingement (FAI) may cause a painful hip, especially in young pa - tients. Two general types of femoroacetabular impingement have been described, the pincer- and the cam type impingement. The latter is characterized by a femoral deformity, usually a bump on the head-and-neck junction that impinges on the acetabular rim. The authors describe the case of a 21-year-old male, bodybuilder, suffering from progressive hip pain with impairment of exercise tolerance, gait and other daily activities. Besides limitation of hip internal rotation physical examination was normal. He had a transitory response to non-steroid anti-inflammatory drugs. Initially performed MRI of the pelvis shows predominant inflammation of the hip joint. In external performed arthroscopy the biopsies of the capsule demonstrated chronic synovitis. In the follow up hip pain remains, however, diagnosis was still unclear. Re-evaluation of the formerly performed and a follow up MRI, and of an additional CT, the findings were compatible with an osteoid osteoma in the femoral cervico-cephalic transition causing itself a cam impingement and monarthritis. The adopted therapeutic strategy consisted on arthroscopic excision of the nidus and trimming of the femoral neck, with clinical recovery after surgical intervention.

Osteoid osteoma is a common benign bone tumor affecting the young adult with typical clinical and radiographic presentation in its most common locations. However, when arising in unusual intra-articular locations, diagnosis may appear confusing and lead to delayed management. We present the case of a 24-year-old man with intra-articular osteoid osteoma of the hip involving the posteroinferior quarter of the femoral head. This unusual location was at the origin of unexplained pain and delayed diagnosis made 18 months after the onset of symptoms since the initial magnetic resonance imaging (MRI) examination could not identify the lesion whereas it was detected on bone scintigraphy and thin slice CT imaging. Due to the complex location providing difficult access for radioguided techniques, an open surgical management was suggested and performed through a limited posterolateral approach with no hip dislocation, after identification of the circumflex pedicle. Following complete surgical excision of the tumor, the diagnosis could be confirmed after histopathologic analysis. No recurrence was observed., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Granulocytic sarcoma, chloroma or myeloblastoma are observed in 3% to 7% of acute myeloid leukaemia and represents localized tumour composed of collection of immature leukaemic cells. It appears most frequently in patients with M2, M4 and M5 subtypes of acute myeloid leukaemia, Case Outline: A 58-year-old female presented with pain and oedema of the right upper limb in November 2009. After two months the patinet had fracture dislocation and numerous osteolytic lesions of the right femur. Immunohistochemistry of tumour biopsy showed megakaryoblastic granulocytic sarcoma which was CD31++, F-XIII++, CD34-, FVIII+++, S100-, aktin-, EMA++, Bcl2++, CD43++, with positive proliferative marker measured with Ki-67 positivity in more of 50% of cells. Aspirate of bone marrow and immunophenotyping with flowcytometry revealed diagnosis of acute megakaryoblastic leukaemia. The course of the disease was rapid and the patient died before commencing chemotherapy, five months after first complaints., Conclusion: Granulocytic sarcoma is extramedullary localization of collection of leukaemia cells which can proceed, to arise concomitantly with leukaemia, or may be the only manifestation of the disease. The diagnosis can be established only with immunohystochemistry.

The authors describe the case of an 89-year-old patient with a one-month history of pain in the right knee and a pathological fracture of the distal femur. Excisional biopsy showed a classical osteogenic sarcoma. The patient died after six months of palliative surgical and oncological therapy; her primary disease generalised and progressed with numerous metastases. The authors discuss the possibilities of histological diagnostics of osteogenic sarcoma and its differential diagnosis. They draw attention to a possible occurrence of this type of sarcoma also in elderly patients, and not only in adolescents.

POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome is a rare multisystem paraneoplastic disorder. A 40-year-old male with a history of peripheral neuropathy and erectile dysfunction presented with a pathological fracture of the neck of the femur, found to be a solitary plasmacytoma. Additional unusual features included splenomegaly, hyperprolactinaemia and skin changes. The patient had a total hip replacement at a specialist orthopaedic hospital and is due to undergo radiotherapy to the femoral lesion and autologous stem cell transplantation.

Background: Malignant degeneration in association with orthopaedic implants is a known but rare complication. To our knowledge, no case of osseous malignant fibrous histiocytoma after anterior cruciate ligament reconstruction is reported in the literature., Case Presentation: We report a 29-year-old male Turkish patient who presented with severe pain in the operated knee joint 40 months after arthroscopic anterior cruciate ligament reconstruction. X-ray and MR imaging showed a large destructive tumor in the medial femoral condyle. Biopsy determined a malignant fibrous histiocytoma. After neoadjuvant chemotherapy, wide tumor resection and distal femur reconstruction with a silver-coated non-cemented tumor knee joint prosthesis was performed. Adjuvant chemotherapy was continued according to the EURAMOS 1 protocol., Conclusions: Though secondary malignant degeneration after orthopaedic implants or prostheses is not very likely, the attending physician should take this into consideration, especially if symptoms worsen severely over a short period of time.

We report a rare case of late recurrence of a giant cell tumour (GCT) of bone 16 years after curettage and cryosurgery treatment. A 46-year-old man presented with swelling and progressively worsening pain in the lateral aspect of his right distal femur. He had undergone 4 procedures elsewhere to manage a GCT of bone and its recurrence 16 to 23 years previously. He underwent en bloc resection with an adequate wide margin and reconstruction with prosthesis. At the one-year follow-up, there was no evidence of recurrence or metastasis.

Malignant lower-limb metaphyseal and diaphyseal bone tumours that have not yet invaded the epiphysis can usually be managed with limb-sparing surgery. Reconstructions using intercalary allografts, autoclaved autografts, extracorporeally irradiated autografts, vascularised autografts, and distraction osteogenesis have all achieved favourable results. In patients with metastatic disease and a short life expectancy, reconstruction with allografts or autografts should be carefully considered because a long recovery period is needed. An intercalary endoprosthesis provides immediate stability, a short recovery period and a low implant failure rate. Nonetheless, it may be expensive when there is inadequate space for stem insertion, necessitating a custom-made endoprosthesis. We present a 12-year-old boy with stage-III osteosarcoma of the metaphysis and diaphysis of the femur who underwent knee joint salvage and reconstruction with a retrograde, locked, intramedullary nail surrounded with methylmethacrylate. At the one-month follow-up, the patient could walk unassisted. At the 2-year follow-up, his Musculoskeletal Tumor Society score was 83%. The patient died from lung metastases at 31 months. He had not experienced any complications with the reconstruction.

Percutaneous osteoplasty (POP) as a technical extension of percutaneous vertebroplasty (PVP) has been used to treat malignant disease that affects the skeletal system. POP has demonstrated good outcome for pain relief and functional improvement. Few studies have reported on the efficiency of POP to treat malignancies located in the femoral head. We designed a pilot study with the use of POP to treat intractable pain caused by a femoral head metastatic tumor in a 43-year-old man. During the follow-up period, the patient experienced sustained pain relief and improvement of quality of life that persisted for more than three months.

Enchondromatoses include a heterogeneous group of congenital syndromes characterized by the presence of multiple enchondromas associated with musculo-skeletal malformations secondary to limb shortening, scoliosis, pathological fractures and pseudoarthrosis. The main complication of enchondromas is their malignant transformation to secondary chondrosarcomas, which may occur in up to 25% of cases. The multiple enchondromatosis syndromes have many clinical similarities and the differential diagnosis is therefore difficult to make. We present the clinical case of a 38-year-old patient with a diagnosis of multiple familial enchondromatosis who developed a pelvic chondrosarcoma that was treated with external hemipelvectomy. We reviewed the literature concerning the specific aspects of Mafucci, Oilier and multiple familial enchondromatosis syndromes.

The authors report a case of osteoid osteoma distal to a hip prosthesis in a 56-year-old patient. This rare association was difficult to diagnose; at first, the pain seemed to be of mechanical origin, suggesting a delayed painful reaction to the prosthesis. The results of bone scan as well as the CT scan ones helped orient the diagnosis. Excision biopsy, using bone trephining, completed by an iliac bone auto-graft resulted in a cure with no residual instability of the prosthesis above the tumor. Bone scan with radio-isotopes to localize the lesion was particularly helpful in this instance to secure the final diagnosis., (2009 Elsevier Masson SAS. All rights reserved.)

Osteoid osteoma is the third most common benign bone tumor. It usually affects the diaphysis of long bones, especially the femur or the tibia. This paper presents the case of a 22-year-old male patient, with pain in the left knee. The x-ray and ultrasound of the knee were normal. The three phase bone scintigraphy revealed a focal uptake in the mid shaft of the left femur, strongly suggestive of osteoid osteoma. This case shows the significant role of bone scintigraphy in the diagnosis of an osteoma osteoid with atipical presentation.

Osteosarcomas are a heterogeneous group of tumors with diverse anatomical, clinical, and progressional characteristics. Parosteal osteosarcoma, or juxtacortical osteosarcoma, is a rare form of osteosarcoma that develops at the bone surface, but has a better prognosis than other conventional osteosarcomas. We report the observation of a 22-year-old female patient whose initial presentation was an enormous tumefaction of the knee that had been progressing for 10 years. The biopsy concluded in PO of the lower third of the femur. Staging was negative. The tumor had reached an enormous size and required amputation of the left lower extremity. A custom external prosthesis was manufactured to get her back to walking. Eight years after surgery, no local recurrence or metastasis has been detected. Parosteal osteosarcoma is a rare form of osteosarcoma with very slow progression (in spite of the particularly dramatic presentation in our observation), with an excellent prognosis and very rare metastasis., (2009 Elsevier Masson SAS. All rights reserved.)

Numerous options exist for intercalary segmental reconstruction after bone tumor resection. We present the extension of a recently developed surgical two-stage technique that involves insertion of a cement spacer, induction of a membrane, and reconstruction of the defect with cancellous and cortical bone autograft in a 12-year-old child. The boy was referred to our center for treatment of a right femoral diaphyseal Ewing's sarcoma. The first stage involved resection of the tumor and reconstruction with a locked intramedullary nail and a polymethylmethacrylate cement spacer. Seven months after the initial procedure during which adjuvant chemotherapy was given, the second-stage procedure was performed. The cement was removed and cancellous and cortical bone autograft was grafted in the membrane created around the cement spacer. Touchdown weightbearing was allowed immediately, partial weightbearing was resumed 6 weeks after the operation, and full weightbearing was allowed 4 months later. Successive plain radiographs showed rapid integration of the autograft to the host bone with bone union and cortical reconstitution. The principle of the induced membrane reconstruction seems applicable to intercalary segmental reconstruction after bone tumor resection in children.

In this retrospective clinical study, 6 cases of osteosarcoma of the bone have been analyzed. Five patients were with parosteal osteosarcoma and one with periosteal osteosarcoma. The study was performed at the Clinic for Orthopaedic Surgery in Skopje, Macedonia, from 1995 to 2005. This tumor represents 1.5% of all primary bone tumors treated at the Clinic in the 11 year period. The age of the 6 patients (2 female and 4 male) ranged from 8 to 39 years (average 23.8). The history analysis of the patients showed misinterpreted diagnosis in 50% of the cases, with 83.3% rate of local recurrence, 33.3% of metastases and 33.3% of mortality. Follow-up varied from 11 months to 9 years (average 4.5). The clinical and histopathological findings (identical with those reviewed in the literature) confirmed occurrence of two biologically different types of parosteal osteosarcoma: predominant type is originally "benign" but has a definite malignant potential, causing metastases after long symptom-free interval. The other type is highly malignant from the beginning. More radical surgery is recommended for the latter category of tumors, followed by chemotherapy. Compartmental, radical "en bloc" resection, followed by regular review of the patients, is recommended for the former (Tab. 1, Fig. 3, Ref. 20). Full Text (Free, PDF) www.bmj.sk.

Myeloma or Kahler-Bozzolo disease represents around 1% of all malignant tumors and 10% of the haematological variety; it is a B-lymphocellular malignant neoplasia which originates from plasma cells that produce monoclonal immunoglobulin, infiltrating in and destroying the adjacent bone tissue. Myeloma may be distinguished at radiological imaging in four distinct types: single osseous lesions (solitary plasmacytoma), diffused skeletal effects (myelomatosis), diffused osteopenia and sclerosing myeloma. It is known that initial osteolysis may not be shown through radiographic examination or CT; the lysis only becomes evident when there is a bone loss of over 50%, usually in the presence of a > or = 0.5 cm focal lesion. We present here the clinical-radiological aspects of a solitary bone plasmacitoma (SBP) of the knee of a 35 year old male which was not evidenced at radiological examination or CT but was evident as a 3 cm focal alteration at MR. The lesion was confirmed by PET and the histological diagnosis was performed by a CT guided bioptic sample.