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Objective: To study the imaging features of patients with chronic ulcers of the leg that were associated with malignancy.Design and Patients: All patients who on biopsy were proven to have malignancy--the majority of which were squamous cell carcinoma-were included in a prospective study. Ulcers limited to the foot were excluded but ulcers of the leg which extended into the foot were included. Amputation was performed in all but two patients, due to pain, bleeding or tissue necrosis.Results: The etiology was multifactorial. The mean duration of the ulcers was 36 years including venous ulcers, extensive scarring of the leg secondary to infection, injury or burns. One ulcer was secondary to a snake bite. The remainder, usually in the upper part of the leg, had repeated episodes of blunt trauma or knife wounds, which were also complicated by infections which failed to heal or, if they healed, regularly recurred. Although arterial insufficiency was not primary in any patient, most were of advanced age and it may have been an element in some patients. Despite infection, osteomyelitis was present in only one patient. The essential features were bone destruction, soft tissue mass and periosteal reaction. The bone destruction was visible on the radiographs in all but one case. The soft tissue masses varied in size but in general were very large. The periosteal reaction varied in type but most commonly was lamellated. The classic undulating solid periosteal reaction of venous stasis was only occasionally present. The periosteal reaction was nonspecific in the majority of cases and did not aid in the diagnosis or etiology. MRI and CT studies were performed in six patients. These were helpful in defining the extent of bone destruction and periosteal reaction but were not essential in management.Conclusion: Chronic ulcer present for decades that then undergoes malignant change is a disease of developing countries where patients only consult physicians when they have developed complications such as pain, bleeding or tissue necrosis. Chronic ulcers may require to be biopsied at regular intervals as malignant change in these ulcers is directly related to their duration. [ABSTRACT FROM AUTHOR]

A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro. [ABSTRACT FROM AUTHOR]

Reconstruction of the distal third of the tibia due to resection of a malignant tumor has some hindering factors, such as a thin subcutaneous layer, neurovascular bundles that cross compartments, prolonged operative duration, specific orthopedic material, and a trained multidisciplinary team. Allografting with material from tissue banks is part of this orthopaedic arsenal., Objective: To describe the protocol used at Instituto Nacional de Traumatologia e Ortopedia Jamil Haddad., Methods: Series of six cases subjected to resection with oncologic margins, allograft reconstruction, and use of a retrograde ankle nail as limb-salvage surgery. Three of the six patients were women, the lesions were on average 9.3 cm long, and the average operative duration was 3.25 hours., Results: The main short-term complication (≤ 30 days) was peroneal nerve palsy, while the main long-term complication (> 30 days) was surgical site infection (two cases). Consolidation of the two foci occurred in three patients, and two patients developed asymptomatic pseudoarthrosis of the proximal focus with consolidation of the distal focus., Conclusion: Despite the complications, the proposed surgery gives patients the chance to preserve their limb in the face of immediate radical surgery. Level of Evidence IV, Case Series ., Competing Interests: All authors declare no potential conflict of interest related to this article.

Although the relationship between hip arthroplasty and the development of sarcoma was first described in the literature about forty years ago, this association is extremely rare. In the present case report, we describe the association between orthopedic implants and soft tissue sarcoma in a 79-year-old man who underwent primary total hip arthroplasty (THA) for coxarthrosis 24 years ago. In the present case report, we describe the clinical evolution and the radiographic and histopathological findings of the lesion. In the intraoperative period of the second revision surgery, loosening of the acetabular and femoral components in association with extensive areas of necrosis and metallosis was evidenced. We performed debridement of the hip and right thigh region and removed the implants. Due to the extent of the lesion and to necrosis, it was not possible to perform a new joint reconstruction. The histopathological diagnosis of high-grade undifferentiated pleomorphic sarcoma associated with extensive areas of metallosis was confirmed in tissue adjacent to the implant. The patient developed pulmonary metastases and died 6 months after the diagnosis. Despite the rarity of this association, sarcomas should be considered in the differential diagnosis of aseptic loosening, especially in the presence of metallosis in the peri-implant tissue. To our knowledge, the 24-year latency period between primary THA and the establishment of a sarcoma diagnosis is one of the longest reported to date., Competing Interests: Conflito de Interesses Os autores declaram não haver conflito de interesses., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ( https://creativecommons.org/licenses/by/4.0/ ).)

Bone sarcomas, although rare, are associated with significant morbidity and mortality. The most frequent primary bone cancers include osteosarcoma, chondrosarcoma and Ewing sarcoma. The treatment approaches are heterogeneous and mainly chosen based on precise tumour staging. Unfortunately, clinical outcome has not changed significantly in over 30 years and tumour grade is still the best prognosticator of metastatic disease and survival. An option to improve this scenario is to identify molecular biomarkers in the early stage of the disease, or even before the disease onset. Blood-based liquid biopsies are a promising, non-invasive way to achieve this goal and there are an increasing number of studies which investigate their potential application in bone cancer diagnosis, prognosis and personalised therapy. This review summarises the interplay between clinical and molecular aspects of the three main bone sarcomas, alongside biomarker discovery and promising applications of liquid biopsy in each tumour context., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Although osteosarcoma is a rare disease, with a global incidence rate estimated at 5.0/million/year, it is the most frequent primary bone sarcoma in children and adolescents. In translational research, the patient-derived xenograft (PDX) model is considered an authentic in vivo model for several types of cancer, as tumorgrafts faithfully retain the biological characteristics of the primary tumors. Our goal was to investigate the association between PDX formation and clinical findings of osteosarcoma patients and the ability of the model to preserve in immunocompromized mice the characteristics of the parental tumor. A fresh sample of the patient tumor obtained from a representative biopsy or from surgical resection was implanted into nude mice. When tumor outgrowths reached ~1,500mm³, fresh PDX fragments were re-transplanted into new hosts. Engraftment in mice was obtained after a latency period of 19-225 days (median 92 days) in 40.54% of the implanted samples. We confirmed the histopathological fidelity between the patient tumor and their respective established PDXs, including the expression of biomarkers. PDX take rate was higher in surgical resection samples, in post-chemotherapy surgical samples and in samples from patients with metastatic disease at presentation. In conclusion, we have shown that the osteosarcoma PDX model reliably recapitulates the morphological aspects of the human disease after serial passage in mice. The observation that more aggressive forms of osteosarcoma, including those with metastatic disease at presentation, have a higher efficiency to generate PDXs provides a promising scenario to address several unanswered issues in clinical oncology.

Objective: The purpose of this study was to determine whether chemical-shift magnetic resonance imaging (MRI) could be useful in the diagnosis of osteoid osteoma when clinical and radiological tumor features are inconclusive., Materials and Methods: This retrospective study included 17 patients who underwent chemical-shift MRI for the evaluation of osteoid osteoma. For all patients, two musculoskeletal radiologists independently recorded signal intensities on in-phase and out-of-phase images in the nidus of the tumor, in abnormal-intensity bone marrow surrounding the lesion, and in normal-appearing bone marrow. For each region, relative signal intensity ratios were calculated by dividing out-of-phase by in-phase values. Relative ratios > 1 were considered indicative of neoplastic lesions. Statistical analysis was carried out to analyze the sample. Inter-observer and intra-observer agreement for each imaging method were assessed using intraclass correlation coefficients according to the Fleiss method and a value > 0.65 was considered to indicate substantial agreement., Results: The mean relative signal intensity ratios were 1.2 (range, 0.9-1.4) for the nidus and 0.35 (range, 0.11-0.66) for the surrounding tissue; these values differed significantly from the relative signal-intensity ratios for normal-appearing bone marrow ( p < 0.05)., Conclusion: Chemical-shift MRI is useful for the diagnosis and evaluation of osteoid osteoma.

Objective: The purpose of this study was to reproduce a mouse model of bone sarcomas for use in cancer research., Methods: A fresh sample of the tumor tissue was implanted subcutaneously into nude mice. When the patient-derived xenograft (PDX) reached a volume of 1500 mm 3 , it was harvested for re-implantation into additional mice. Histology was used to compare the morphological characteristics of different generations of sarcoma xenografts with the primary tumor., Results: Sixteen sarcoma tissue samples were engrafted into nude mice. Nine patients were diagnosed with osteosarcoma, two with chondrosarcoma, two with malignant peripheral nerve sheath tumor, one with synovial sarcoma, one with pleomorphic sarcoma, and one with Ewing's sarcoma. PDX tumors were generated in 11 of the 16 implanted specimens (69% success rate in P1). Six P1 tumors grew sufficiently for transfer into additional mice, producing the P2 generation, and three P2 tumors established the P3 generation., Conclusion: PDX tumors generated from bone sarcomas were successfully established in immunodeficient mice and reproduced the characteristics of the primary tumor with a high degree of fidelity. The preclinical PDX model described herein may represent an important tool for translational oncology research and for evaluating therapeutic strategies for bone sarcomas. Level of Evidence I; Experimental study., Competing Interests: All authors declare no potential conflict of interest related to this article.

Objective: To introduce the intralesional calcitonin and methylprednisolone percutaneous injection method, which results in the promotion of primary aneurysmal bone cyst (ABC) healing., Methods: A retrospective cohort study involving 76 patients diagnosed with ABC was performed between 2005 and 2014. Patients treated with calcitonin and methylprednisolone injection and who underwent more than 2 years of follow-up were considered eligible for the study (n=47). The Enneking staging and Capanna classification systems were used during the initial evaluation. Treatment response was assessed by Rastogi radiographic grading based on the degree of healing. X 2 and Wilcoxon signed-rank tests and odds ratio calculations were used in the statistical analysis with a 5% significance level., Results: The proximal tibia extremity was the most commonly affected site (17.0%). Thirty-three (70.3%) ABC cases were staged as B3 and 28 (59.7%) were classified as type II. The average number of injections performed was 2.8 per patient, with an average reduction of the initial lytic area of 83.7% (p-value=0.00001). Satisfactory results for 91.4% (n=43; p-value=0.00001) were obtained and 5 recurrences occurred. No side effects were observed., Conclusion: Intralesional calcitonin and methylprednisolone percutaneous injection is a minimally invasive, effective, and safe method for promoting primary ABC healing. Level of evidence IV, Type of study: case series. , Competing Interests: All authors declare no potential conflict of interest related to this article.

Osteosarcoma is the most common primary bone tumor in children and young adults. The median survival of osteosarcoma patients has not significantly improved since 1990, despite administration of different classes of chemotherapy agents, such as methotrexate, cisplatin and doxorubicin. Cancer stem cells (CSCs) are responsible for the resistance of osteosarcoma to chemotherapy and OCT4, SOX2 and SSEA4 have been used to identify CSCs in osteosarcoma. Here, we used low-passage patient-derived osteosarcoma cells and osteosarcoma cells directly isolated from patients before and after chemotherapy treatments to evaluate the effects of chemotherapy on stem cell markers expression. We demonstrate that primary osteosarcoma cells are resistant to methotrexate treatment and sensitive to cisplatin and doxorubicin in vitro. We also verified that cisplatin and doxorubicin reduce the expression of SOX2 and OCT4 in primary osteosarcoma cells whereas methotrexate does not alter SOX2 and OCT4 expression, however it increases SSEA4 expression in primary osteosarcoma cells. Finally, we found that, although the combination treatment cisplatin plus doxorubicin inhibited the in vivo growth of osteosarcoma cells in NOD-SCID gamma mice subcutaneously injected with SaOs2, the combination treatment cisplatin plus doxorubicin plus methotrexate did not inhibit the in vivo growth of these cells. These observations may provide an explanation for the poor response of osteosarcomas to chemotherapy and point to the need of reevaluating the therapeutic strategies for human osteosarcomas.

Objective: Giant cell tumors are benign bone neoplasms that are relatively rare in adults and their biological behavior is still unpredictable. The incidence of local recurrence has presented variation between 0% and 65% in studies conducted worldwide, but few data are available on this complication in the Brazilian population., Methods: Information on 155 patients with confirmed histological diagnoses of giant cell tumor who were treated in our institution's orthopedic oncology service between January 2000 and July 2014 was gathered. Demographic characteristics were evaluated and compared between patients who presented local recurrence during the clinical follow-up., Results: Local recurrence was observed in 26 patients (16.7%), of whom 22 were female (84.6%). The most common site of local recurrence was the distal femur (38.4%). Eleven patients presented early recurrence, while 15 cases were diagnosed after 15 months, representing 42.3% and 57.7%, respectively. Metastases were identified in five patients (3.2%)., Conclusion: Tumor-related factors did not show any increased incidence of local recurrence of giant cell tumors. Surgical treatment with an intralesional margin is a valid option for treating local recurrences and does not show any difference in disease-free survival in relation to other types of procedures. Clinical treatment is reserved for cases of unresectable tumors or when surgical treatment is impossible.

Background: Liposclerosing myxofibrous primary bone tumor is a rare benign bone lesion that was characterized by complex mixture of various histological elements., Methods: We have studied the radiological, clinical and pathological features of nine patients with this disorder. Pain and limping were the main symptoms., Results: Radiographic images typically showed a geographic lytic lesion with thick sclerotic margin, reflecting a pattern of slow growth. Histological sections revealed a polymorphic neoplasia characterized by predominant proliferation of stellate and fusiform cells aimed the myxoid matrix., Conclusions: These features suggest that the lesion may represent a variant of fibrous dysplasia with a high risk of malignant transformation.

Objective: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas., Methods: Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005., Results: Mean age at diagnosis was 33 years old, ranged from 10 to 70. The primary site of presentation was the upper extremity in twelve patients (48%). The size of the tumor was given in 19 cases, with the mean size of 5 cm, while they ranged from 1.5 to 15 cm. Surgery was made in 17 patients, with eleven amputation. Tumors margins were free in fifteen patients, positive in three and in seven were not studied. Six received any type of chemotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy. Local recurrence occurred in thirteen cases (52%). Nodal spread was diagnosed in nine (36%). Pulmonary metastases were diagnosed in seven patients (28%). Six patients underwent cancer treatment in its entirety at the National Institute of Cancer. At present twelve patients are alive without disease, two have disease and eleven patients have died., Conclusion: Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases. Occurs predominantly in young patients, mainly on the superior member extremities. Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins. These patients require carefully follow-up to evaluate local recurrence, nodal metastases, and pulmonary metastases.

Ewing's sarcoma is a round-cell tumor that arises most often in a medullary cavity. This neoplasm is uncommon in a subperiosteal location. We report a new case of a 12-year-old boy with a periosteal Ewing's sarcoma, located in the femur, who was treated by cortical segmental resection associated with chemotherapy. Two years after surgery the patient was free of disease. In reviewing the literature of 29 cases, it seems the prognosis is better in periosteal compared with intramedullary Ewing's sarcoma.