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Resumo Objetivo Avaliar o tempo de sobrevida, a taxa de falha e suas causas, e os resultados funcionais de endopróteses cimentadas, com corpo em polietileno, empregadas após ressecção de tumores ósseos primários do fêmur distal. Métodos Estudo retrospectivo, que incluiu 93 procedimentos primários e 77 de revisão, realizados entre 1987 e 2014. A sobrevida foi obtida pela análise de Kaplan Meyer, e os fatores de risco para falha do implante foram avaliados por meio do modelo de riscos proporcionais de Cox. As causas de falha da endoprótese foram classificadas segundo Henderson et al. em cinco tipos: falha de partes moles, soltura asséptica, fratura estrutural, infecção e recorrência do tumor. A avaliação funcional foi realizada por meio do sistema de classificação funcional da Musculoskeletal Tumor Society (MSTS) para sarcomas ósseos da extremidade inferior, versão brasileira (MSTS-BR). Resultados Osteossarcoma foi o diagnóstico mais comum; 64,5% dos pacientes tinham menos de 20 anos; e o seguimento médio foi de 124,3 meses. A taxa de falha do implante primário foi de 54,8%, e a sobrevida média foi 123 meses. A estimativa de sobrevida do implante primário foi de 63,6%, 43,5%, 24,1%, 14,5% em 5, 10, 15 e 20 anos, respectivamente. A causa de falha mais comum foi a do tipo 2 (37,3%). Idade ≤ 26 anos e lado direito foram fatores de risco para falha. A pontuação média no MSTS-BR foi de 20,7 (variação: 14 a 27). Conclusão Os resultados obtidos para a taxa de falha e o tempo de sobrevida do implante estão de acordo com os da literatura, de forma que o procedimento estudado é adequado e apresenta resultados funcionais satisfatórios, inclusive em longo prazo.

Background Cancer patients configure a risk group for complications or death by COVID‐19. For many of them, postponing or replacing their surgical treatments is not recommended. During this pandemic, surgeons must discuss the risks and benefits of treatment, and patients should sign a specific comprehensive Informed consent (IC). Objectives To report an IC and an algorithm developed for oncologic surgery during the COVID‐19 outbreak. Methods We developed an IC and a process flowchart containing a preoperative symptoms questionnaire and a PCR SARS‐CoV‐2 test and described all perioperative steps of this program. Results Patients with negative questionnaires and tests go to surgery, those with positive ones must wait 21 days and undergo a second test before surgery is scheduled. The IC focused both on risks and benefits inherent each surgery and on the risks of perioperative SARS‐CoV‐2 infections or related complications. Also, the IC discusses the possibility of sudden replacement of medical staff member(s) due to the pandemic; the possibility of unexpected complications demanding emergency procedures that cannot be specifically discussed in advance is addressed. Conclusions During the pandemic, specific tools must be developed to ensure safe experiences for surgical patients and prevent them from having misunderstandings concerning their care.

Background There are limited data on surgical complications for patients that have delayed surgery after severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection. We aimed to analyze the surgical outcomes of patients submitted to surgery after recovery from SARS‐CoV‐2 infection. Methods Asymptomatic patients that had surgery delayed after preoperative reverse‐transcription polymerase chain reaction (RT‐PCR) for SARS‐CoV‐2 were matched in a 1:2 ratio for age, type of surgery and American Society of Anesthesiologists to patients with negative RT‐PCR for SARS‐CoV‐2. Results About 1253 patients underwent surgical procedures and were subjected to screening for SARS‐CoV‐2. Forty‐nine cases with a delayed surgery were included in the coronavirus disease (COVID) recovery (COVID‐rec) group and were matched to 98 patients included in the COVID negative (COVID‐neg) group. Overall, 22 (15%) patients had 30‐days postoperative complications, but there was no statistically difference between groups –16.3% for COVID‐rec and 14.3% for COVID‐neg, respectively (odds ratio [OR] 1.17:95% confidence interval [CI] 0.45–3.0; p = .74). Moreover, we did not find difference regarding grades more than or equal to 3 complication rates – 8.2% for COVID‐rec and 6.1% for COVID‐neg (OR 1.36:95%CI 0.36‐5.0; p = .64). There were no pulmonary complications or SARS‐CoV‐2 related infection and no deaths within the 30‐days after surgery. Conclusions Our study suggests that patients with delayed elective surgeries due to asymptomatic preoperative positive SARS‐CoV‐2 test are not at higher risk of postoperative complications.

e23527 Background: Extremity soft-tissues sarcomas are a rare entity that represent 1% of solid tumors in adults. Surgery is the main treatment and limb sparing resection with adjuvant radiotherapy (RT) is associated with good local control. However, high rates of distant relapses motivated the evaluation of adjuvant systemic treatment protocols. We aim to compare survival rates between two cohorts of patients treated with two different preoperative protocols. Methods: An observational comparative cohort study were carried out. Two cohorts of patients with high grade, larger then 5.0 cm, and non-metastatic soft tissue sarcomas of extremities, treated with two distinct preoperative institutional protocols, in two different periods of time, were compared. Group 1 included patients treated in the period from January, 1995 to December, 2004, when patients with high risk extremities soft tissue sarcomas were submitted to preoperative radiation therapy (a total dose of 30 Gy) concomitant with doxorubicin (60 mg/m2); this protocol included adjuvant chemotherapy with doxorubicin and ifosfamide. Group 2 included patients treated in the period from April, 2005 to July, 2012, when our group changed the preoperative protocol to neoadjuvant chemotherapy without radiation, with four cycles doxorubicin (60 mg/m2 per cycle) and ifosfamide (9.000 mg/m2 per cycle), followed by surgery, and postoperative radiation. Data from the group 1 were retrospectively collected, and data from group 2 were from our prospective database. Results: Eighty-eight patients were included (37 in group 1 and 51 in group 2). There were no statistical differences between the groups with regards to age, gender, location and tumor size. Predominant histological subtypes were synovial sarcoma (27.3%), pleomorphic sarcoma (15.9%), leiomyosarcoma (11.4%) and liposarcoma (8%). Relapses occurred in 60.5% of patients, most in the lungs (48.1%). Amputation rates were 14.3% in the group 1 and 4.5% in the group 2. Five-year local recurrence free survival was 81.7% for the group 1 and 91.7% for the group 2. There was no difference in median overall survival when comparing group 1 and 2 (47 versus 78 months, respectively; IC 95 3.967 – 132.033; p = 0.49), but our data showed higher recurrence free survival in group 2 (19 versus 132 months; IC 95 5.9 – 60.09; p = 0.025, respectively). Among patients from the group 2, whom presented less than 10% of viable tumor cells in the surgical specimens (good responders), no local or distant recurrences have occurred. Conclusions: In our retrospective analysis, neoadjuvant chemotherapy was associated with greater recurrence free survival in patients with high-grade extremity soft tissue sarcomas as compared to neoadjuvant chemoradiation. For patients with high risk of distant relapse, neoadjuvant chemotherapy should be considered after a multidisciplinary discussion.

Resumo O angiossarcoma osseo (AO) e uma patologia rara de comportamento biologico variavel e com prognostico reservado. Pouco se conhece sobre o seu tratamento oncologico e sua etiologia ainda e desconhecida. Os autores apresentam um caso de lesao litica em femur proximal que se disseminou para outros ossos (tais como coluna e crânio), pulmao e sistema nervoso central. Foi instituido tratamento ortopedico, com vistas a uma melhoria da qualidade de vida e ao conforto do paciente. O diagnostico de AO maligno de alto grau foi confirmado pelo especime cirurgico. Apesar disso e do tratamento oncologico feito, o paciente apresentou uma evolucao rapida e agressiva com desfecho desfavoravel.

Children's Cancer Institute Rafael Accordi Foundation, Porto Alegre, RS, Brazil Background: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). Methods: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included. The treating physicians defined the modality of local control based on the recommendations of the coordinating center and the patient and tumor characteristics. Possible associations of local control modality with local failure (LF), disease-free survival (DFS), event-free survival (EFS), overall survival (OS), and clinical characteristics were analyzed. Results: Mean patient age was 12.8 years (range, 2 to 25 years) and median follow-up time was 4.5 years (range, 2. 3 to 6.7 years). Forty-seven patients underwent surgery, 13 received radiotherapy, and 13 received both. The 5-year EFS, OS, and DFS for all patients was 62.1%, 63.3%, and 73.1%, respectively. The 5-year cumulative incidence (CI) of LF was 7.6% for surgery, 11.1% for radiotherapy, and 0% for postoperative radiotherapy (PORT) (p = 0.61). The 5-year EFS was 71.7% for surgery, 30.8% for radiotherapy, and 64.1% for PORT (p = 0.009). Conclusions: There was a significant effect of local control modality on EFS and OS in the study. Surgery and PORT modalities yielded very close results. The group treated with radiotherapy alone had considerably worse outcomes. This may be confounded by greater risk factors in these patients. There was no significant effect of local control modality on the CI of LF and DFS. HCPA, Serv Orthoped & Traumatol, Rua Ramiro Barcelos 2350, BR-90035903 Porto Alegre, RS, Brazil Univ Fed Rio Grande do Sul, HCPA, Dept Pediat, Porto Alegre, RS, Brazil Pontificia Univ Catolica Rio Grande Sul PUCRS, Dept Pediat, Hosp Sao Lucas, Porto Alegre, RS, Brazil HCPA, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Fed Sao Paulo UNIFESP, Support Grp Children & Adolescents Canc GRAACC, Sao Paulo, SP, Brazil Hosp Canc Infantojuvenil, Fundacao Pio 12, Barretos, SP, Brazil Ctr Hosp Pereira Rossell, Montevideo, Uruguay Hosp AC Camargo Canc Ctr, Orthoped Serv, Sao Paulo, SP, Brazil Santa Casa Misericordia Porto Alegre, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Sao Paulo, Orthoped Trauma Inst, Hosp Clin Sao Paulo, Sch Med, Sao Paulo, SP, Brazil Santa Casa Misericordia Sao Paulo HSCSP, Dept Orthoped & Traumatol, Sao Paulo, SP, Brazil Pontificia Univ Catolica Rio Grande Sul PUCRS, Hosp Sao Lucas, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Estadual Paulista UNESP, Hosp Clin Botucatu, Sch Med, Botucatu, SP, Brazil Inst Canc Infantil, Porto Alegre, RS, Brazil Univ Fed Sao Paulo UNIFESP, Support Grp Children & Adolescents Canc GRAACC, Sao Paulo, SP, Brazil Children's Cancer Institute Rafael Accordi Foundation, Porto Alegre, RS, Brazil Web of Science

Schwannoma is a benign neural sheath tumor of the soft tissue, and its intraosseous presentation is very rare. It is estimated that intraosseous schwannomas represent 0.2% of all bone tumors. The tumor may affect any site of the skeleton, including the mandible, the sacrum, vertebral bodies, the ulna, the humerus, the femur, the tibia, the patella, the scapula, the ribs, and small bones of the hand. The involvement of the calcaneus has only been reported four times in the literature. The present study reports the case of a 49-year-old male with right hindfoot pain and a radiological finding of an osteolytic bone lesion in the calcaneus. The diagnosis was confirmed by histopathological study. The treatment of choice was an intralesional resection with adjuvant local control, and bone defect substitution with polymethylmethacrylate and fixation with two cannulated screws. The patient had a satisfactory postoperative evolution; after 1 year, he is asymptomatic, with good functional response and no evidence of disease. The present case report shows the clinical, radiological, and pathological features of a rare benign bone neoplasm. Moreover, intraosseous schwannoma should be included in the differential diagnosis of osteolytic calcaneal lesions.

Resumo O osteoma osteoide e um tumor osseo primario benigno que acomete mais o sexo masculino na segunda e terceira decadas da vida. Radiograficamente, caracteriza‐se por um nicho radiolucente cercado por osso esclerotico reativo, principalmente em ossos longos da extremidade inferior. Clinicamente, apresenta uma dor persistente de longa duracao, com pioria noturna e melhoria com salicilatos. Embora possa ser uma lesao autolimitada, com duracao media de tres anos, a resseccao da lesao e uma opcao de tratamento devido a intensidade da dor e intolerância ao uso prolongado de anti‐inflamatorios nao hormonais. Sua suspeita diagnostica baseia‐se principalmente na historia clinica e nos achados radiograficos, a confirmacao e feita pelo estudo anatomopatologico. O tratamento cirurgico classico e a excisao cirurgica completa do nicho, porem sao descritas desvantagens como a dificuldade para a localizacao intraoperatoria da lesao, risco de fratura durante o procedimento, tempo de internacao hospitalar para controle algico e resultado estetico desfavoravel. Relatamos uma serie de casos tratados com termoablacao por radiofrequencia guiada por tomografia computadorizada em nosso servico. Trata‐se de um metodo percutâneo seguro e eficaz que tem como objetivo a cura, minimiza o trauma e a morbidade do procedimento, quando comparado com o metodo convencional de resseccao em bloco.

Background: The localization in tumor tissue of various markers by immunohistochemistry can help to establish a diagnosis or predict prognosis. Nitric oxide is associated with tumors and has been studied indirectly by nitrotyrosine analysis and with use of the enzymes nitric oxide synthase (NOS)1, NOS2, and NOS3. Nitric oxide reacts with superoxide anions to yield peroxynitrite, which has toxic effects on genes. Peroxynitrite adds a nitro group to the benzene ring of tyrosine to form nitrotyrosine. The accumulation of nitrotyrosine, a stable product in cells, indicates the formation of peroxynitrite. Nitric oxide stimulates the production of cyclooxygenase-2 (COX-2), which has been associated with angiogenesis in tumors. Neovascularization influences tumor prognosis, as demonstrated by microvessel studies with use of CD34, an immunohistochemical endothelial cell marker. This study examines the expression of these markers in chondrosarcomas and their relation to histological grade and prognosis. Methods: Tissue microarrays composed of formalin-fixed tissue samples from 101 patients with chondrosarcoma were immunohistochemically stained to localize NOS1, NOS2, NOS3, COX-2, nitrotyrosine, and CD34. Five samples of normal cartilage were used as controls. Patient demographics, selected surgical variables, and tumor grade were tabulated, and the associations were analyzed. Analyses of local and overall survival rates were performed with use of the Kaplan-Meier method, and multivariable analyses were performed. Results: There was a significant association of nitrotyrosine, COX-2, and CD34 with histological grades (p = 0.022, p = 0.014, and p = 0.028, respectively), but not with overall prognosis (p = 0.064, p = 0.143, and p = 0.581, respectively). The presence of NOS2 was associated with a lower rate of local disease-free survival (p = 0.038), and positive expressions of NOS1 and NOS2 were associated with decreased overall survival rates (p = 0.007 and p < 0.001, respectively). On multivariable analysis, NOS2 expression demonstrated an independent prognostic impact on local disease-free survival; NOS1 and NOS2 expression was a dependent variable, and their isolated or combined expression was related to lower overall survival rates (p = 0.046 and p = 0.004) (hazard ratio, 3.17 [95% confidence interval, 1.0 to 9.8] and 5.58 [95% confidence interval, 1.7 to 18.0], respectively). Conclusions: Immunohistochemical markers may have an independent value in predicting the prognosis for patients with chondrosarcoma.

CASE We report the case of a 38-year-old woman who presented with a lytic bone lesion in the left scapula. A biopsy showed a tumor with the histologic appearance of thyroid tissue. She also was found to have a thyroid nodule and an enlarged ovary, both of which were excised. The thyroid nodule turned out to be a benign colloid nodule, and the ovary contained a monodermal teratoma composed of thyroid tissue (struma ovarii). The lesion in the scapula eventually was proven to be metastatic malignant struma ovarii. CONCLUSION Malignant struma ovarii with bone metastases is very rare, and its diagnosis is a challenge. There are some documented cases with bone metastases, mostly to the thoracic and lumbar spine. The optimal treatment of choice for the bone lesion is unknown because of the rarity of its presentation.

Primary osseous angiosarcoma is a rare entity with variable biological behavior and poor prognosis. Little is known about the oncologic treatment and its etiology is still unknown. This study presents a case of lytic lesion in the right femur with dissemination to other bones, such as the vertebral column and skull, and to the lungs and central nervous system. Orthopedic surgery was performed in order to improve quality of life. Surgical specimen confirmed the diagnosis of high-grade malignant osseous angiosarcoma. Despite oncologic and orthopedic treatment, the patient had rapid and aggressive progression with a poor outcome.

Simple Summary Desmoplastic small round cell tumor is a rare neoplasm with extremely aggressive behavior. Despite the multimodal treatment for newly diagnosed patients with chemotherapy, cytoreductive surgery and radiation, the cure rate is still low. For relapsed or progressive disease, there is limited data regarding second and third-line therapies. Novel agents have shown only modest activity. Recent molecular changes have been identified in this disease and this opens opportunities to be explored in future clinical trials. Abstract Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs. The treatment of advanced and recurrent disease with tyrosine kinase inhibitors, such as pazopanib, sunitinib, and mTOR inhibitors was evaluated by small trials. Recent studies using comprehensive molecular profiling of DSRCT identified potential therapeutic targets. In this review, we aim to describe the current studies conducted to better understand DSRCT biology and to explore the new therapeutic strategies under investigation in preclinical models and in early phase clinical trials.