Parosteal Osteosarcoma: A Benign-Looking Tumour, Amenable to a Variety of Surgical Reconstruction.

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.

Capicua Transcriptional Repressor-Rearranged Undifferentiated Round Cell Sarcoma Metastatic to the Brain Treated with Surgery and Stereotactic Radiosurgery.

BACKGROUND: Ewing-like sarcoma with capicua transcriptional repressor (CIC) rearrangement is a unique class of undifferentiated round cell sarcomas characterized by CIC-double homeobox 4 gene fusion. Despite showing great histologic resemblance to Ewing sarcomas, they have proved to be a distinct pathological entity from the immunohistochemistry and genetic examinations and the response to treatment. We have presented a case of CIC-rearranged Ewing-like sarcoma with cerebral metastasis managed with operative resection and gamma knife radiosurgery. CASE DESCRIPTION: A 56-year-old woman had initially presented with an ulcerating lesion of the right fifth toe. The histological and immunohistochemical analysis revealed features consistent with CIC-rearranged Ewing-like sarcoma, which was confirmed with genetic analysis. Despite aggressive local control and a multidrug chemotherapy regimen, the patient developed multifocal metastases involving the lungs, femur, and cerebrum. The cerebral lesions were managed with surgery and gamma knife radiosurgery, with mixed results. CONCLUSION: CIC-rearranged Ewing-like sarcomas have recently been recognized as a distinct disease entity with a highly aggressive course. Treatment paradigms have yet to be defined to properly manage such an aggressive pathological process.

Plasmocitoma ovárico bilateral detectado por 18F-FDG PET/TC / Bilateral ovarian plasmacytoma detected on 18F-FDG PET/CT

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Rotational Tibio-Pelvic Constrained Hip Arthroplasty: A Surgical Technique: A Case Report.

CASE: A 25-year-old man presented with chronic bone and soft tissue infection of the right thigh following resection and radiation of epithelioid sarcoma. Multiple revisions and debridement procedures had failed to control the infection and left him unable to ambulate. We describe a modified Van Nes rotationplasty using a constrained, prosthetic hip between the tibia and pelvis following femur resection. With 18 months of follow-up, the patient was able to walk with a prosthetic device without evidence of recurrent infection. CONCLUSIONS: We report this rotationplasty as a potential approach to avoid hip disarticulation in cases requiring extensive debridement for incurable infection.

Osteosarcoma after the age of fifty: A clinicopathological study.

INTRODUCTION: Osteosarcoma, a primary malignant bone tumor, has a well-recognised double peak of incidence in early adolescence and after 50 years. This study investigates the clinical features and prognostic factors of patients older than 50 years with osteosarcoma. MATERIEL AND METHODS: From January 2000 to December 2012, in one bone tumor reference center, 32 patients aged more than 50 years at the diagnosis (mean age: 62.4 years (50-85), sex ratio: 13 males, 19 females) diagnosed with osteosarcoma were included. Patients younger than 50 years at diagnosis or with a non-histologically proved osteosarcoma were excluded. For each patient, we registered medical history, tumor location, systemic and local extension, treatment, and survival. RESULTS: 62% were located in the extremities and 28% in the axial skeleton. 6 were secondary sarcomas. Mean delay between first symptoms and biopsy was 7.4 months (range from 0 to 28 months). Ten patients had a systemic osteosarcoma with one or more pulmonary metastases. Six patients were treated with palliative care (18.8%). Eighteen patients received neodajuvant chemotherapy, sixteen of them received postoperative chemotherapy. Twenty-five patients had surgery. Postoperative complications were reported in eight cases (25%). Overall survival for all 31 patients was 25% at 5 years and 6.2% at 10 years. Survival without metastases was 15.6% at 5 years and nil at 10 years. Median survival time for the 22 localised osteosarcoma patients was 4 years (0.9-12.6) versus 1.2 years (0.3-12.3) for the 10 systemic osteosarcoma patients (p = 0.01). CONCLUSION: Metastases at diagnosis, age, axial location are worse prognostic for survival.

Simultaneous hyperthermia-chemotherapy effect by arterial injection of Fe(Salen) for femur tumor.

We previously identified a novel nanomagnetic particle, N,N'-bis(salicylidene)ethylenediamine iron [Fe(Salen)]. Fe(Salen) not only shows antitumor effects but also magnetic properties. We found that Fe(Salen) can be used for magnet-guided drug delivery and visualization of accumulated drug by magnetic resonance imaging (MRI) because of its magnetism. In addition, Fe(Salen) can generate heat by itself when exposed to an alternating current magnetic field (AMF), resulting in a hyperthermia effect. Herein, we partly elucidated the antitumor mechanism of Fe(Salen) and carried out an i.v. repeated dose toxicity study to decide the therapeutic amount. Furthermore, we evaluated the antitumor effect of selective intra-arterial injection or i.v. injection of Fe(Salen) by catheter and the hyperthermia effect of Fe(Salen) when exposed to AMF in vivo. We used a rabbit model grafted with VX2 cells (rabbit squamous cell carcinoma) on the right leg. Intra-arterial injection of Fe(Salen) showed a greater antitumor effect than did i.v. injection. The combination of Fe(Salen) intra-arterial injection and AMF exposure showed a greater antitumor effect than did either Fe(Salen) or methotrexate (MTX) without AMF exposure, suggesting that AMF exposure greatly enhanced the antitumor effect of Fe(Salen) by arterial injection by catheter. This is the first report that the effectiveness of Fe(Salen) was evaluated in the point of administration route; that is, selective intra-arterial injection by catheter. Taken together, these results indicate a new administration route; that is, selective arterial injection of Fe(Salen) by catheter, and the development of a new strategy of simultaneous hyperthermia-chemotherapy in the future.

Chemotherapy Curtails Bone Formation From Compliant Compression Fixation of Distal Femoral Endoprostheses.

BACKGROUND: Modulated compliant compressive forces may contribute to durable fixation of implant stems in patients with cancer who undergo endoprosthetic reconstruction after tumor resection. Chemotherapy effects on bone hypertrophy and osteointegration have rarely been studied, and no accepted radiologic method exists to evaluate compression-associated hypertrophy. QUESTIONS/PURPOSES: (1) What was the effect of chemotherapy on the newly formed bone geometry (area) at 1 year and the presumed osteointegration? (2) What clinical factors were associated with the degree of hypertrophy? (3) Did the amount of bone formation correlate with implant fixation durability? (4) Was the amount of new bone generation or chemotherapy administration correlated with Musculoskeletal Tumor Society (MSTS) score? METHODS: Between 1999 and 2013, we performed 245 distal femoral reconstructions for primary or revision oncologic indications. We evaluated 105 patients who received this implant. Ten were excluded because they lacked 2 years of followup and two were lost to followup, leaving 93 patients for review. All underwent distal femur reconstruction with the compliant compressive fixation prosthesis; 49 received postoperative chemotherapy and 44 did not. During this period, the implant was used for oncology patients < 60 years of age without metastases and with > 8 cm of intact, nonirradiated bone distal to the lesser trochanter and ≥ 2.5 mm of cortex. Our cohort included patients with painful loosening of cemented or uncemented stemmed femoral megaprostheses when revision with the compliant compressive device was feasible. Patients with high-grade sarcomas all received chemotherapy, per active Children's Oncology Group protocols, for their tumor diagnosis. At each imaging time point (3, 6, 9, 12, 18, 24 months), we measured the radiographic area of the bone under compression using National Institutes of Health open-access software, any shortening of the spindle-anchor plug segment distance as reflected by the exposed traction bar length, and prosthesis survivorship. Clinical and functional status and MSTS scores were recorded at each followup visit. Duration of prosthesis retention without aseptic loosening or mechanical failure was evaluated using Kaplan-Meier analysis, censoring patients at last followup. RESULTS: Chemotherapy was associated with the amount of overall bone formation in a time-dependent fashion. In the 12 months after surgery there was more bone formation in patients who did not receive postoperative chemotherapy than those who did (60.2 mm, confidence interval [CI] 49.3-71.1 versus 39.1, CI 33.3-44.9; p = 0.001). Chemotherapy was not associated with prosthesis survival. Ten-year implant survival was 85% with chemotherapy and 88% without chemotherapy (p = 0.74). With the number of patients we had, we did not identify any clinical factors that were associated with the amount (area) of hypertrophy. The hypertrophied area was not associated with the durability of implant fixation. MSTS scores were lower in patients treated with chemotherapy (25 versus 28; p = 0.023), but were not correlated with new bone formation. CONCLUSIONS: The relationships among chemotherapy, bone formation, and prosthetic survivorship are complex. Because bone formation is less in the first year when the patient is being treated with chemotherapy, it is not clear if the rehabilitation schedule should be different for those patients receiving chemotherapy compared with those who do not. The relationship between early bone formation and the timing of weightbearing rehabilitation should be evaluated in a multicenter study. LEVEL OF EVIDENCE: Level III, therapeutic study.

Functional recovery after surgical stabilization and postoperative radiotherapy due to metastases of long bones.

PURPOSE: To reinvestigate the functional recovery after combined treatment with surgery and postoperative irradiation of complete or impending pathologic fractures of long bones. METHODS: We retrospectively evaluated the results of external beam radiation therapy (EBRT) carried out after 68 orthopedic stabilization procedures (femur, n = 55, 80.8%; humerus, n = 13, 19.2%) for actual or impending pathological fracture of long bone in 61 patients with skeletal metastases. The mean normalized total dose was 34.7 ± 7.8 Gy. Endpoints were patient's functional status (FS; 1 = normal pain free status; 2 = normal use with pain; 3 = significantly limited used; 4 = nonfunctional status), a need for a secondary procedure to the same site and overall survival following surgery. RESULTS: Overall, 75% of patients achieved normal functional status (FS 1-2) within 12 weeks after surgery. Functional recovery in surviving patients reached 93%. Median survival was 17 months (95% confidence interval 13.7-20.2). Secondary surgical intervention at the same location was necessary in 3 patients (4.4%). On multivariate analysis, only general status (p = 0.011) and growing potential of primary tumor (p = 0.049) were associated with achieving normal functional status within 12 weeks after surgery and radiotherapy. The applied radiation schemes demonstrated a comparable impact on functional recovery. CONCLUSIONS: Our results confirm the effectiveness of stabilizing surgery and fractionated postoperative radiotherapy in terms of functional recovery, supporting prior results assessing postsurgical radiotherapy versus follow-up. The patient's general status is a strong prognostic factor for functional recovery. Rapidly growing tumors may hinder achievement of a normal functional status.

Ewing Sarcoma of the Femur.

A 26-year-old active woman with knee pain was seen by her primary care physician and diagnosed with patellofemoral pain (PFP). Radiographs of the knee were interpreted as noncontributory. Her knee symptoms continued, and 8 weeks later she was referred to a physiatrist, who confirmed the PFP diagnosis and referred her to physical therapy. Repeat radiographs revealed a destructive mass involving the diaphysis of the femur and surrounding soft tissue. Subsequent magnetic resonance imaging further characterized the mass as an aggressive bone lesion, and biopsy confirmed that it was Ewing sarcoma. J Orthop Sports Phys Ther 2018;48(7):594. doi:10.2519/jospt.2018.7835.

Not All Patients Undergoing Stabilization of Impending Pathologic Fractures for Renal Cell Carcinoma Metastases to the Femur Need Preoperative Embolization.

BACKGROUND: Preoperative transarterial embolization has been utilized in the surgical treatment of metastatic renal cell carcinoma of the femur to decrease perioperative blood loss. However, few studies have documented its efficacy in decreasing the proportion of patients receiving transfusions in the setting of prophylactic treatment of impending pathologic femur fractures. QUESTIONS/PURPOSES: In a population of patients with metastatic renal cell carcinoma of the femur who underwent prophylactic fixation, the purpose of this study was to quantify and compare the proportion of patients who received at least one transfused unit of blood between a group treated with preoperative embolization and a group without preoperative embolization. METHODS: A retrospective study was performed using a Medicare claims-based database. International Classification of Diseases, 9 Revision and Current Procedural Terminology codes were used to identify 1285 patients with metastatic renal cell carcinoma of the femur who underwent prophylactic fixation. The proportion of patients who received one or more blood transfusions was compared between 135 patients who underwent preoperative embolization and a group of 1150 concurrent control patients who did not undergo preoperative embolization. The control group was older than the embolization group, with 44% of these patients > 75 years old and 33% of the embolization group > 75 years. There was no difference in the female:male ratio between groups. Statistical comparisons of outcomes related to transfusion percentages were performed using Pearson chi square analysis with p < 0.05 considered significant. With the numbers available, we had 80% power to detect a difference in the percentage of patients transfused of 11% between the study groups at α = 0.05. RESULTS: No difference in transfusion percentage was observed between preoperative transarterial embolization (41 of 135 [30%]) and the control group (359 of 1150 [31%]; relative risk, 0.973; 95% confidence interval, 0.743-1.274; p = 0.84). The percentage of all patients who received a transfusion was 31% (400 of 1285). CONCLUSIONS: Preoperative embolization may not be mandatory in the prophylactic treatment of metastatic renal cell carcinoma of the femur, as demonstrated by the 69% of patients who received zero units of blood despite not receiving embolization. However, assessment of the efficacy of embolization in decreasing blood loss in the current study is limited as a result of biases associated with the database design of the study; the decision of whether to send a patient for embolization should be made on a case-by-case basis. The current study does not identify specific risk factors that should factor into this decision and underscores the need for further research in this regard. A plausible future research design to account for the low numbers and selection bias that limited the current study as well as the existing studies might be a multicenter, retrospective case-control study. LEVEL OF EVIDENCE: Level III, therapeutic study.

MRI Identification of the Osseous Extent of Pediatric Bone Sarcomas.

BACKGROUND: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone. QUESTIONS/PURPOSES: (1) Does the quantitative pathologic bony margin correlate with that measured on preoperative MRI? (2) Are T1- or T2-weighted MRIs most accurate in determining a margin? (3) Is there a difference in predicting tumor extent between MRI obtained before or after neoadjuvant chemotherapy and which is most accurate? METHODS: We retrospectively studied a population of 211 potentially eligible patients who were treated with limb salvage surgery between August 1999 and July 2015 by a single surgeon at a single institution for primary sarcoma of bone. Of 131 patients (62%) with disease involving the femur or tibia, 107 (51%) were classified with Ewing's sarcoma or osteosarcoma. Records were available for review in our online database for 79 eligible patients (37%). Twenty-six patients (12%) were excluded because of insufficient or unavailable clinical or pathology data and 17 patients (8%) were excluded as a result of inadequate or incomplete MR imaging, leaving 55 eligible participants (26%) in the final cohort. The length of the resected specimen was superimposed on preresection MRI sequences to compare the margin measured by MRI with the margin measured by histopathology. Arithmetic mean differences and Pearson r correlations were used to assess quantitative accuracy (size of the margin). RESULTS: All MR imaging types were positively associated with final histopathologic margin. T1-weighted MRI after neoadjuvant chemotherapy and final histopathologic margin had the strongest positive correlation of all MR imaging and time point comparisons (r = 0.846, p < 0.001). Mean differences existed between the normal marrow margin on T1-weighted MRI before neoadjuvant chemotherapy (t = 8.363; mean, 18.883 mm; 95% confidence interval [CI], 14.327-23.441; p < 0.001), T2-weighted MRI before neoadjuvant chemotherapy (t = 8.194; mean, 17.204 mm; 95% CI, 12.970-21.439; p < 0.001), T1-weighted after neoadjuvant chemotherapy (t = 10.808; mean, 22.178 mm; 95% CI, 18.042-26.313; p < 0.001), T2-weighted after neoadjuvant chemotherapy (t = 10.702; mean, 20.778 mm; 95% CI, 16.865-24.691; p < 0.001), and the final histopathologic margin. T1-weighted MRI after neoadjuvant chemotherapy compared with the final histopathologic margin had the smallest mean difference in MRI-measured versus histopathologic margin size (mean, 5.9 mm; SD = 4.5 mm). CONCLUSIONS: T1 MRI after neoadjuvant chemotherapy exhibited the strongest positive correlation and smallest mean difference compared with histopathologic margin. When planning surgical resections based on MRI obtained after neoadjuvant chemotherapy, for safety, one should account for a potential difference between the apparent margin of a tumor on an MRI and the actual pathologic margin of that tumor of up to 1 cm. LEVEL OF EVIDENCE: Level III, diagnostic study.

Reinforced cementoplasty using dedicated spindles in the management of unstable malignant lesions of the cervicotrochanteric region.

OBJECTIVES: In long bones, cementoplasty alone does not provide sufficient stability, which may cause secondary fractures. This study reviewed the safety and efficacy of reinforced cementoplasty (RC) (percutaneous internal fixation using dedicated spindles combined with cementoplasty) for unstable malignant lesions of the cervicotrochanteric region (CTR) of the proximal femur. METHODS: Eighteen consecutive patients (nine women [50%] and nine men [50%]; mean age 55.1 ± 16.2 years; range 22-85) underwent RC for 19 unstable lesions of the CTR (16/19 [84.2%] bone metastases, 3/19 [15.8%] multiple myeloma lesions). All the patients were considered unsuitable for surgery. Clinical outcome was judged with a mean follow-up of 8.8 ± 7.2 months (range 1-27). The primary endpoints were occurrence of secondary fractures during the follow-up period and local pain relief measured by a visual analogue scale (VAS). RESULTS: No secondary fracture occurred. Mean VAS improved from 5.9 ± 3.1 (range 0-10) to 2.3 ± 2.4 (range 0-7) at 1 month (p = 0.001) to 1.6 ± 1.7 (range 0-5) at final follow-up (p = 0.0002). One symptomatic cement pulmonary embolism was recorded. CONCLUSION: RC is an original minimally invasive technique providing pain relief and effective bone stability for unstable malignant lesions of the cervicotrochanteric region in patients unsuitable for open surgery. KEY POINTS: • Reinforced cementoplasty (RC) combines intralesional spindling with cementoplasty. • RC provides effective bone stability and pain relief. • RC is a suitable minimally invasive option for patients in poor general condition.

Effect of Unplanned Therapy on the Prognosis of Patients with Extremity Osteosarcoma.

Unplanned therapy for extremity osteosarcoma can result in erroneous surgical procedures and lack of neoadjuvant chemotherapy before the first operation. Our aim was to compare the prognosis between patients with extremity osteosarcoma who received unplanned therapy and those who received standard treatment. This was a retrospective review of patients with extremity osteosarcoma who received appropriate surgical treatment and neoadjuvant chemotherapy (n = 79) and those who received unplanned therapy (n = 24) between June 2000 and October 2014. Survival rate, local recurrence rate and metastasis rate were compared between the two groups. We found that patients who had unplanned therapy had a higher local recurrence rate (41.7% vs. 21.5%; P = 0.049) and a shorter mean time for recurrence (8.90 vs. 14.59 months; P = 0.018). There was no significant difference between groups in the 5-year survival rate (56.3% vs.67.8%; P = 0.356), metastasis rate (45.8% vs. 30.4%; P = 0.125) and mean time to metastasis (23.18 vs.18.24 months; P = 0.396). Our findings suggest that unplanned therapy for extremity osteosarcoma can result in failure of local control. The use of supplementary interventions after unplanned therapy, such as neoadjuvant chemotherapy and limb salvage surgery, may explain the similar survival and metastasis rates between patients receiving unplanned therapy and those receiving standard treatment.

Risk-Based Therapy for Localized Osteosarcoma.

BACKGROUND: The outcome of localized osteosarcoma has remained constant over the past 30 years. Histological response to preoperative chemotherapy is the best predictor of outcome. Strategies to alter treatment based on histological response have not resulted in increased survival. PROCEDURE: Patients with localized osteosarcoma received preoperative chemotherapy with cisplatin, doxorubicin, and methotrexate. Patients whose tumors had a good histological response (≥90% necrosis) continued with the same treatment postoperatively. Patients with poor histological response (<90% necrosis) received three courses of melphalan 100 mg/m(2) on day -4, cyclophosphamide 2,000 mg/m(2) on days -3, and -2 followed by stem cell infusion. RESULTS: Fifty-two patients were enrolled. Median age was 14 years, and 56% of patients were male. The femur was the most common site. Forty patients underwent limb salvage surgery and amputation was performed in six patients. Forty-eight percent of tumors showed good histological response. Forty patients were evaluable for outcome; 18 patients with poor histologic response received high-dose chemotherapy. The 5-year event-free survival (EFS) and overall survival (OS) for patients treated on the high-dose chemotherapy arm were 28% (95% confidence interval [CI], 10-49) and 48% (95% CI, 23-69), respectively. The 5-year EFS and OS for patients treated on the standard chemotherapy arm were 62% (95% CI, 36-80) and 74% (95% CI, 44-90), respectively. All patients who received high-dose chemotherapy developed grade 3 or higher hematological toxicity. There were no treatment-related deaths. CONCLUSIONS: Postoperative alkylator intensification with high-dose cyclophosphamide and melphalan in patients with localized osteosarcoma with poor histological response failed to improve survival.

Extracorporeal Irradiation in Malignant Bone Tumors.

Extracorporeal irradiation (ECI) is relatively a rare method used in the management of malignant bone tumors (MBT). It consists of en block removal of the tumor bearing bone segment, removal of the tumor from the bone, irradiation and re implantation back in the body.

Vaginal delivery after hemipelvectomy and pelvic radiotherapy for chondrosarcoma.

Chondosarcoma of the proximal femur is a rare malignant disorder in women of (pre-) childbearing age, for which a radical resection through a hemipelvectomy could be indicated. We describe a case of a 36-year-old primigravida with a hemipelvectomy (2004) who had a history of radiotherapy of the pelvic and uterine regions after an atypical cartilaginous tumour. After an uncomplicated pregnancy, she had a spontaneous rupture of the membranes at 41+2 weeks and an uncomplicated vaginal delivery with physiological development of the infant. On the basis of the literature review, vaginal delivery after hemipelvectomy should be advocated and discussed with the patient and involved care providers. Women with radiotherapy in the pelvic and uterine areas have an increased risk of stillbirth, placental attachment disorders, impaired fetal growth, fetal malposition and preterm labour, but no association with prolonged rupture of the membranes has been described.