Assessment of medical students' knowledge of primary limb sarcomas.

INTRODUCTION: Typically, oncology is not a structured part of the curriculum in Brazilian medical schools. Furthermore, sarcomas, which are uncommon tumors, are seldom covered in depth. A lack of comprehensive education on sarcomas might result in medical professionals being ill-equipped to care for patients with this condition. OBJECTIVES: To assess medical students' understanding and awareness of sarcomas and the specific principles related to these tumors. MATERIALS AND METHODS: A quantitative, cross-sectional study was conducted using a questionnaire, applied to medical students, focusing on the epidemiology, pathophysiology, and treatments of bone and soft tissue sarcomas. In all tests, the significance level adopted was 5%. The SPSS version 25.0 software was used. RESULTS: Of the 825 questionnaires distributed, 325 were returned. Educational sessions on sarcomas did not appear to significantly improve the student's knowledge. Only 29.5% of students identified the lack of pain as an indicator of potential malignancy in soft tissue sarcomas, while 73.8% correctly recognized pain as a symptom of bone sarcomas. Limb amputation as the optimal surgical method for patient recovery was incorrectly reported by 39.1% of the sample. CONCLUSION: A great part of the surveyed population does not have adequate knowledge about the basic concepts associated with limb sarcomas. The minority of them are satisfied with the knowledge gained during their medical education about these tumors. Inadequate medical academic training may initially lead to the wrong clinical management of patients with bone and soft tissue tumor lesions. An educational effort is needed to enhance oncology education for medical students, especially concerning sarcomas.

Prevalence and clinical implications of germline pathogenic variants in cancer predisposing genes in young patients across sarcoma subtypes.

BACKGROUND: Sarcomas are a rare and diverse group of cancers occurring mainly in young individuals for which an underlying germline genetic cause remains unclear in most cases. METHODS: Germline DNA from 177 children, adolescents and young adults with soft tissue or bone sarcomas was tested using multigene panels with 113 or 126 cancer predisposing genes (CPGs) to describe the prevalence of germline pathogenic/likely pathogenic variants (GPVs). Subsequent testing of a subset of tumours for loss of heterozygosity (LOH) evaluation was performed to investigate the clinical and molecular significance of these variants. RESULTS: GPVs were detected in 21.5% (38/177) of the patients (15.8% in children and 21.6% in adolescents and young adults), with dominant CPGs being altered in 15.2% overall. These variants were found in genes previously associated with the risk of developing sarcomas (TP53, RB1, NF1, EXT1/2) but also in genes where that risk is still emerging/limited (ERCC2, TSC2 and BRCA2) or unknown (PALB2, RAD50, FANCM and others). The detection rates of GPVs varied from 0% to 33% across sarcoma subtypes and GPV carriers were more likely to present more than one primary tumour than non-carriers (21.1%×6.5%; p=0.012). Loss of the wild-type allele was detected in 48% of tumours from GPV carriers, mostly in genes definitively associated with sarcoma risk. CONCLUSION: Our findings reveal that a high proportion of young patients with sarcomas presented a GPV in a CPG, underscoring the urgency of establishing appropriate genetic screening strategies for these individuals and their families.

[Epidemiology and Outcomes of Soft Tissue Sarcomas: Insights from a Decade of Head and Neck Surgery at Sótero del Río Hospital, Santiago, Chile]. / Sarcomas de partes blandas: Análisis de la experiencia de diez años del equipo de cirugía de cabeza y cuello en el Hospital Sótero del Río.

BACKGROUND: Soft tissue sarcomas (STS) are rare malignant tumors of mesenchymal origin. They are associated with genetic and environmental risk factors. Their clinical manifestations are nonspecific, requiring a high level of suspicion. The first-line treatment is surgical. Positive margins are the only independent predictor of local recurrence and worse survival rates. Strict follow-up is recommended due to its high recurrence rate. AIM: Analyze the casuistry of STS treated with curative intent by the head and neck surgery team at the Sótero del Río Hospital (HSR) at Santiago, Chile between 2013 and 2023. METHODS: A retrospective, descriptive study of patients with STS managed by the HSR head and neck surgery team. Clinical presentation, diagnostic and therapeutic tools, and oncological results are analyzed. RESULTS: 26 patients were included, 84.6% female, with an average age of 61.7 years. Only 30.8% presented identifiable risk factors. The most common histology was undifferentiated pleomorphic sarcoma (26.9%), and the predominant location was extremities (46.2%). 77.8% of head and neck sarcomas and 58.8% of trunk and extremity sarcomas occurred in advanced stages. Disease-free survival was 66.6%; 68.2% in patients with negative surgical margins, and 60.0% in positive margins at five years. CONCLUSIONS: STS is an infrequent pathology. This study corresponds to the first retrospective research on STS in Chile. We require establishing a specialized multidisciplinary team to optimize the management and follow-up of STS patients.

Subsequent malignant neoplasms in the pediatric age in retinoblastoma survivors in Argentina.

BACKGROUND: Retinoblastoma survivors in low- and middle-income countries are exposed to high-intensity treatments that potentially place them at higher risk of early subsequent malignant neoplasms (SMNs). METHODS: We followed 714 (403 [56.4%] nonhereditary and 311 [43.5%] hereditary) retinoblastoma survivors diagnosed from August 1987 to December 2016, up to the age of 16 years. We quantified risk of SMNs with cumulative incidence (CI) and standardized incidence ratios (SIR) analysis. Multivariate regression Cox model was used to determine the association of treatments and risk of SMNs. RESULTS: Median follow-up was of 9 years (range: 0.18-16.9) and 24 survivors (3.36%) developed 25 SMNs (n = 22 hereditary, n = 2 nonhereditary). SMNs included sarcomas (osteosarcomas, Ewing sarcomas, rhabdomyosarcomas; n = 12), leukemias (n = 5), and central nervous system tumors (CNS; n = 3). All cases of acute myeloid leukemia (AML) and most of Ewing sarcomas occurred within 5 years of retinoblastoma diagnosis. The type of SMN was the main indicator of mortality (five of five patients with leukemias, six of 12 with sarcomas, and zero of three with CNS tumors died). Compared to the general population, radiation increased the risk of Ewing sarcoma in hereditary survivors by 700-fold (95% CI = 252-2422.6) and chemotherapy increased the risk of AML by 140-fold (95% CI = 45.3-436). The CI of SMNs for hereditary survivors was 13.7% (95% CI = 8.4-22.1) at 15 years. CONCLUSION: Retinoblastoma survivors from Argentina are at higher risk of developing SMNs early in life compared to the general Argentinean population, especially those treated with radiation plus chemotherapy. AML and Ewing sarcoma presented within 5 years of retinoblastoma diagnosis are associated with chemotherapy and radiation exposure.

Overview of Adult Sarcoma Burden and Clinical Pathways in Brazil.

PURPOSE: Sarcomas are a heterogeneous group of cancers classified as rare cancers and are often poorly characterized. In Brazil, little is known about the adult sarcoma burden and patients' clinical pathways. MATERIALS AND METHODS: We analyzed data from the Brazilian Hospital-Based Cancer Registries System, which encompasses the entire country. The histologic criteria included sarcomas according to the International Classification of Diseases for Oncology, 3rd edition. All cases were histology-based. No central pathology review was performed. Patients < 18 years old were excluded. The variables were analyzed according to the center type, hospital patient volume (> 70 patients/year for 3 consecutive years), and geographical region. The results were based on valid data, and the missing values were reported. RESULTS: From 2000 to 2017, a total of 312 units and 49,878 cases were identified. Missing data proportion was stable. Soft tissue sarcomas were predominant, followed by bone sarcomas and gastrointestinal stromal tumors. The Southeast concentrated on the largest number of patients (51%), of high-complexity centers (CACONs; 52%), and of patients treated at CACONs (56.9%). In all regions, the majority of patients had localized disease at diagnosis. The proportion of patients starting their treatment within 60 days from diagnosis at CACON was 59.3% and 62.3% at others. Ten hospitals achieved the established threshold for high-volume center, of which seven were CACON. CONCLUSION: This article highlights the need for further research on the profile of patients with sarcoma in Brazil and the importance of providing them a more effective diagnostic and therapeutic approach. This initiative is critical not just for planning treatment strategies but also to allocate medical resources and to improve quality of care and sarcoma patients outcomes.

A demographic and clinical panorama of a sixteen-year cohort of soft tissue sarcoma patients in Brazil.

Little is known about soft tissue sarcomas (STS) in Brazil, once the federal statistics regarding estimates on incidence and mortality of the most common cancers that affect the Brazilian population currently do not include STS. This study aims to perform a broad evaluation and description of the epidemiological profile, access to treatment and main clinical outcomes of the Brazilian STS patient. A population-based cohort study of 66,825 patients who underwent procedures related to STS treatment registered in the Brazilian public health system (Sistema Único de Saúde, SUS) databases. Median age was 57 years, 30% of them older than 65 years and 50.7% of the cohort was female. The majority, 50,383 patients (75.4%), was diagnosed between 2008 and 2015. Most prevalent anatomic sites were upper and lower limbs (12.6%) and the registry of sarcomas without a specific location comprehended 29.7% of the cohort. The majority of patients resided in the Northeast (40.2% of the patients). Surgery was the first treatment modality in 77.7% of the cases. For survival analysis, only patients with stage and histological grade information were included. The 1-, 5- and 10-year survival rate of the patients was, respectively, 75.4% (95% CI = 74.1-76.7%), 43.4% (95% CI = 41.5-45.5%) and 18.6% (95% CI = 14.8-23.3%).

Extremities Soft Tissue Sarcomas, more Common and as Dangerous as Bone Sarcomas / Sarcomas de partes moles nos membros, mais comuns e tão graves quanto os sarcomas ósseos

Abstract Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissues arcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bones arcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.

Resumo Os sarcomas musculoesqueléticos são doenças raras que exigem atenção. Frequentemente, apresentam alto grau de malignidade ao diagnóstico e se subestimados podem evoluir de forma agressiva local e sistemicamente. Apresentam-se como sarcoma de partes moles e sarcomas ósseos, sendo os de partes moles quatro a cinco vezes mais comuns. A maioria dos sarcomas de partes moles ocorre nos membros. Os subtipos mais comuns nas crianças e adolescentes são o rabdomiossarcoma e o sarcoma sinovial, nos adultos o sarcoma pleomórfico indiferenciado, lipossarcoma, leiomiossarcoma, mixofibrossarcoma e sarcoma sinovial; todos de alto grau de malignidade histológica. Muitos sarcomas de partes moles são confundidos com tumores benignos de partes moles, 100 vezes mais comuns, por isso são ressecados sem o planejamento necessário, acarretando em amputação de um membro que poderia ter sido preservado. Como em todos os cânceres, o fator prognóstico mais importante é a doença metastática. Na sua vigência, a taxa de sobrevida global cai em torno de 20 a 30%. As taxas de sobrevida no geral são parecidas entre os sarcomas ósseos e de partes moles, portanto o sarcoma de partes moles, além de mais prevalente, mostra-se tão agressivo quanto os sarcomas ósseos, por isso merece muita atenção dos ortopedistas que frequentemente são a primeira linha de atendimento dos portadores destes tumores.

Oral radiation-induced sarcomas: Systematic review.

BACKGROUND: The aim of this study was to integrate the available data published on radiation-induced sarcoma of the oral cavity into an analysis of its clinical features, treatment modalities and prognostic factors. METHODS: An electronic search was undertaken in September 2019. The eligibility criteria included publications that had enough clinical and histological information to confirm the diagnosis. RESULTS: Forty-two publications with 122 radiation-induced sarcoma of the oral cavities (RISOCs) were included. The mean latency period was 114 months and mean radiation total dose was 62.5 Gy. The tumors were more prevalent in males between 50 and 60 years old and the mandible was the most affected site. Osteosarcoma was the most prevalent histological type and patients were mostly treated with radical surgery. CONCLUSIONS: RISOC showed a poor survival rate of 15.1% in 5-year follow-up. Gender and histological type were independently associated with survival.

The occurrence and pattern of head and neck sarcomas: a comprehensive cancer center experience.

PURPOSE: This study aimed to analyze the demographics, clinicopathological, treatment, and survival characteristics of head and neck sarcomas diagnosed in a reference center in the Brazilian Northeast. MATERIALS AND METHODS: This retrospective cohort study reviewed the clinical records of patients with head and neck sarcomas. Epidemiologic data consisted in clinical location, age, gender, histopathological diagnosis, clinical TNM staging and treatment. Outcome variables were local recurrence and survival. The statistical analyses were performed by a binary logistic regression analysis. The survival analysis was assessed through the Kaplan-Meier curve. RESULTS: Sixty-nine patients with head and neck sarcomas (male 39; female 30) were analyzed. The most common histologic subtypes were rhabdomyosarcoma, dermatofibrosarcoma, and pleomorphic sarcoma. The mean age of the patients at the time of diagnosis was 38.1 years old. A total of 31 patient died (sarcoma-related death) up to the end of the follow-up, with a mean follow-up rate of 1.63 years. A multivariate analysis revealed that anatomical site, treatment modality, histopathological diagnosis, and clinical stage of the disease were associated with specific survival, reaching statistical significance. CONCLUSION: This study demonstrates the impact of important clinical-pathological parameters on the overall prognosis of head and neck sarcomas.

The Brazilian TP53 mutation (R337H) and sarcomas.

Sarcomas represent less than 1% of all solid neoplasms in adults and over 20% in children. Their etiology is unclear, but genetic susceptibility plays an important role in this scenario. Sarcoma is central in Li-Fraumeni Syndrome (LFS), a familial predisposition cancer syndrome. In Brazil, the high prevalence of p.Arg337His mutations in the TP53 gene brings about a unique condition: a cluster of LFS. In the present work, we studied 502 sarcoma patients not selected by age or family history in an attempt to assess the impact of the so-called "Brazilian germline TP53 mutation" (p.Arg337His) on this tumor type. We found that 8% of patients are carriers, with leiomyosarcoma being the main histologic type of sarcoma, corresponding to 52.5% of the patients with the mutated TP53 gene. These findings emphasize the importance of genetic counseling and can better guide the management of sarcoma patients.

A multicenter study of oral sarcomas in Brazil.

OBJECTIVES: The aim of this study was to investigate the prevalence of oral sarcomas from geographic regions of Brazil. MATERIALS AND METHODS: A cross-sectional study was conducted on biopsies obtained from January 2007 to December 2016 at twelve Brazilian oral and maxillofacial pathology centres. Gender, age, evolution time, clinical aspects, tumour location, tumour size at diagnosis, radiographic aspects and histopathological diagnosis were evaluated. Data were analysed using descriptive statistical methods. RESULTS: From 176,537, a total of 200 (0.11%) oral sarcomas were reported, and the most prevalent were osteosarcomas (74 cases; 37%) and Kaposi's sarcomas (52 cases; 26%). Males were more affected than females at a mean age of 32.2 years old (range of 3-87 years). The most common symptoms were swelling¸ localised pain and bleeding at a mean evolution time of 5.14 months (range <1-156 months). The lesions were mostly observed in the mandible (90 cases; 45%), with a mean tumour size of 3.4 cm (range of 0.3-15 cm). Radiographically, the lesions presented a radiolucent aspect showing cortical bone destruction and ill-defined limits. CONCLUSIONS: Oral sarcomas are rare lesions with more than 50 described subtypes. Osteosarcomas and Kaposi's sarcomas were the main sarcomas of the oral cavity in Brazil.

[Primary malignant hepatic tumors other than hepatocarcinoma or cholangiocarcinoma. A series of cases]. / Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma.

BACKGROUND: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. AIM: To review a registry of liver biopsies performed to diagnose hepatic tumors. PATIENTS AND METHODS: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. RESULTS: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. CONCLUSIONS: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma / Primary malignant hepatic tumors other than hepatocarcinoma or cholangiocarcinoma: a series of cases

Background: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. Aim: To review a registry of liver biopsies performed to diagnose hepatic tumors. Patients and Methods: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. Results: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. Conclusions: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

Current situation of soft tissue sarcomas: Registry of a Latin American cancer institute. / Situación actual de los sarcomas de partes blandas: registro de un instituto oncológico de Latinoamérica.

INTRODUCTION: Soft tissue sarcomas (SFT) are a group of rare and heterogeneous neoplasms (representing less than 1% of cancer in adults and 15% in pediatric patients), for which there is no updated records in the Latin American population. This study aims to describe the current situation of patients treated at a cancer institute in Latin America. METHODS: We obtained records from 250 patients with a diagnosis of SFT, treated at the National Institute of Neoplastic Diseases of Peru (INEN) during the period 2009-2013, with a mean follow-up of 62 months. The following data were recorded: epidemiological, clinical, treatment and follow-up. The analysis of global survival was done with the Cox proportional hazards model. RESULTS: SFT showed a greater frequency in males (60.8%), with a peak incidence after 50 years of age (69.6%). Tumor location was predominantly in the lower extremities (64.4%), and the most frequent histologic subtypes were: undifferentiated pleomorphic sarcoma (34%) and liposarcomas (25.6%); clinical stage iii was the most frequent (30.8%). The 5-year overall survival rate was 63.9%, while the statistical analysis found a significant association between global survival and the variables: age (>50 years), tumor size (>5cm), depth (subfascial), histologic grade (G3), local and distant recurrence, showing shorter survival times in these groups. CONCLUSIONS: This study has clarified the epidemiology, treatment and prognosis, as well as the variables that have an impact on the survival of the Latin American patients with SFT studied.

Demographic and clinicopathologic distribution of head and neck malignant tumors in pediatric patients from a Brazilian population: A retrospective study.

BACKGROUND: The incidence of pediatric head and neck cancer (PHNC) is increasing worldwide, especially when compared with childhood cancer in general. However, there is still a lack of knowledge about the demographic profile of such patients across the globe. Therefore, the aim of this study was to describe demographic, topographic, and histopathological features of PHNC patients from a single Brazilian institution. METHODS: Medical records were retrospectively reviewed for all cancer cases diagnosed from 1986 to 2016 affecting patients aged 19 years and younger. The demographic variables (age, gender, race), topographic aspects of primary tumors, and histopathological diagnoses were collected and analyzed by descriptive statistics. RESULTS: Three hundred and sixty-seven (5.11%) head and neck malignant tumors were found among 7181 pediatric cancers diagnosed in this period. Mean age at diagnosis was 9.35 years with male (65.67%) predominance. Patients between the age group of 10-14 years presented the higher prevalence of malignant tumors. In terms of race, 73.02% of the patients were white and 9.54% were black. The main affected anatomic site was the neck and lymph nodes (41.42%), followed by nasopharynx (22.89%) and thyroid gland (6.54%). The most common cancer type was lymphoma (52.86%), followed by carcinoma (22.89%), and sarcoma (19.07%). Burkitt lymphoma, nodular sclerosis Hodgkin lymphoma, nasopharyngeal carcinoma, and rhabdomyosarcoma were the most common histopathological diagnoses (16.62%, 13.08%, 12.81%, and 12.81%, respectively). CONCLUSION: This study originally demonstrated that lymphomas may be more frequent than carcinomas and sarcomas in Brazilian PHNC patients.

Cáncer Bucomaxilofacial en Niños III: Tumores Malignos del Tejido Blando / Oral and Maxillofacial Cancer in Children III: Malignant Soft Tissue Tumors

RESUMEN: El grupo de neoplasias malignas de tejido blando de la región de cabeza y cuello en pacientes pediátricos está representado por carcinomas, sarcomas, melanomas y tumores de diferenciación incierta. La neoplasia más prevalente en la población pediátrica es el Rabdomiosarcoma, seguido por el carcinoma de células escamosas. Los rangos de presentación son muy amplios, siendo los grupos entre 2-6 años y 15-19 años los que presentan mayor incidencia. Se ha planteado que la etiología de estas neoplasias es incierta. El tratamiento de estas neoplasias es comúnmente de enfoque multimodal, combinando un procedimiento quirúrgico con quimioterapia y radioterapia. El pronóstico y sobrevida del paciente dependerán principalmente del momento en que se realice el diagnóstico de la lesión. Un diagnóstico y tratamiento temprano favorecen las posibilidades de sobrevida y el pronóstico del paciente. Este estudio corresponde a la 3ra parte de "Cáncer bucomaxilofacial en niños". Se hará referencia a los distintos tumores malignos del tejido blando en la población pediátrica en el territorio de cabeza y cuello, abarcando sus generalidades, etiología, epidemiología, tratamiento y pronóstico.

ABSTRACT: Head and neck malignant tumors in pediatric patients comprise carcinoma, sarcoma, melanoma and tumours of uncertain differentiation. Within the pediatric population, the most prevalent neoplasm is rhabdomyosarcoma, followed by squamous cell carcinoma. There is a wide range in the presentation, and it varies significantly with age groups of 2-6 and 15-19 year-olds who present the higher incidence rates. For this reason, it has been suggested that the etiology of head and neck neoplasms remains unclear. Treating these pathologies usually involves a multimodal approach that combines surgery, radiation and chemotherapy. Prognosis and survival rates depend mainly of the stage at the time of diagnosis. Early diagnosis and treatment can improve prognosis and survival rates. In this 3rd part of "Maxillofacial Cancer in Pediatric Patients", we studied a variety of malignant tumors in head and neck soft tissue from a paediatric sample. Specifically, we aim to analyze their etiology, epidemiology, treatment and prognosis.

Epidemiological Profile and Treatment Outcomes in Young Adults (19-29 Years of Age) Treated for Cancer in a Tertiary Hospital in São Paulo, Brazil.

BACKGROUND: Worldwide, the incidence of cancer in young adults (20-39 years) is increasing, and represents an important cause of mortality in this age group. A retrospective study was undertaken to provide information that may lead to improved treatment outcomes. METHODS: Epidemiological, clinicopathological, treatment, and survival information were retrieved from the electronic database registry of a tertiary referral hospital in São Paulo, Brazil for patients 19-29 years of age diagnosed with cancer between January 2007 and December 2012. RESULTS: There were 960 patients with a median age at diagnosis of 26 years; female patients comprised 59.2%. A previous diagnosis of malignancy was present in 2.3%; 0.4% had malignant tumors that were radiation-associated; regular alcohol use was present in 10.4%; 9% of patients reported tobacco use; a family history of cancer was present in 41.7%. Malignant tumors included carcinomas (45.7%), germ cell and trophoblastic neoplasms (12.3%), and lymphomas (12.1%). Median follow-up was 47.7 months (range: 0.62-100.9 months) during which time 111 patients (13.5%) died. Carcinomas (n = 43, 38.7%), soft tissue sarcomas (n = 18, 16.2%), and leukemias (n = 10, 9.0%) were the most common causes of death. CONCLUSIONS: This study has shown that carcinomas represent the most common malignancy in adolescents and young adults referred to a tertiary cancer center in Brazil and are the most common cause of mortality. Because clinical outcome may be affected by multiple factors in this patient population, further global studies are needed to characterize this population and improve clinical care.

Trends in Cancer Mortality Among Adolescents and Young Adults in Brazil.

PURPOSE: Adolescents and young adults (AYA) with cancer comprise an intermediate age group between pediatric and adult oncology, and have a spectrum of different types of cancers. Survival among this group has not improved as much as in younger children with cancer. The aim of this study was evaluate the trends in cancer mortality of AYA aged 15-29 years in Brazil. METHODS: Data were extracted from the Atlas of Cancer Mortality databases from 1979 to 2013. Age-specific mortality rates were calculated based on the deaths from each type of cancer and the period via a direct method using the proposed world population age groups. To identify significant changes in the trends, we performed joinpoint regression analysis. RESULTS: The mortality rates per million were 54 deaths in those aged 15-19 years, 61 deaths in those aged 20-24 years, and 88 deaths in those aged 25-29 years. Leukemias, lymphomas, and central nervous system (CNS) tumors occurred at high rates in all age groups. Rates of cervical cancer were highest in those aged 25-29 years. There were significant increases in mortality trends in the North and Northeast regions for all tumor groups, especially CNS tumors. A small decrease in the mortality rate from lymphomas was observed in the South and Southeast regions. CONCLUSION: Mortality in Brazilian AYA was slightly higher than in other studies conducted throughout the world. When separated by tumor type, Brazil presents a specific pattern, with high mortality from cervical cancer.

Leiomiossarcoma de Veia Cava Inferior: Relato de caso e revisão da literatura

"O leiomiossarcoma de veia cava inferior é um tumor raro, constituindo apenas 0,5% dos sarcomas de tecidos moles. À semelhança de outros sarcomas de origem vascular, estão associados a um mau prognóstico. O conhecimento do comportamento desses tumores é limitado devido a sua raridade e complexidade, não havendo ainda um tratamento padrão. Entretanto, a ressecção cirúrgica com margens negativas constitui a opção terapêutica e é a única estratégia capaz de proporcionar sobrevida a longo prazo. A raridade desse tumor foi o fator determinante para a realização do presente trabalho, que tem como objetivo revisar a literatura médica sobre os aspectos epidemiológicos, patológicos e clínicos, e relatar, através de análise descritiva, um caso de sarcoma de veia cava inferior operada no Instituto Nacional de Câncer José Gomes Alencar da Silva (INCA). Para isso, apresentamos o caso de uma paciente de 60 anos, do sexo feminino, matriculada pelo serviço de cirurgia abominopélvica com diagnóstico histológico pré-operatório de leiomiossarcoma de veia cava inferior. O tumor estava localizado abaixo da emergência dos vasos renais e foi tratado com ressecção cirúrgica com margens livres e radioterapia de intensidade modulada (IMRT) adjuvante no leito tumoral. A paciente se encontra em seguimento ambulatorial após dez meses de tratamento inicial sem evidências de recidiva ou progressão da doença. O relato de caso apresentado sugere que o tratamento cirúrgico radical é eficaz no controle desta patologia e a reconstrução com prótese vascular segura no que diz respeito ao risco de trombose, mesmo após o uso de radioterapia local, respeitando o regime de anticoagulação profilática"

"The leiomiosarcoma of inferior vena cava is a rare tumor, accounting for only 0,5% of soft tissue sarcomas. Like the other sarcomas of vascular origin, they are associated with a poor prognosis. The knowledge of the behavior of these tumors is limited due to its rarity and complexity, and there is no standard treatment. However, surgical resection with negative margins is the therapeutic option and is the only strategy capable of providing long-term survival. The rarity of this tumor was the determining factor for the present study, which aims to review the medical literature on epidemiological, pathological and clinical aspects and to report, through a descriptive analysis, a case of inferior vena cava sarcoma operated at the Nacional Cancer Institute José Gomes Alencar da Silva (INCA). For this, we present the case of a 60-year-old female patient enrolled in the abdominal-pelvic surgery service with a preoperative histological diagnosis of leiomyosarcoma of the inferior vena cava. The tumor was located below the emergence of the renal vessels and was treated with free margin surgical resection and adjuvant intensity modulated radiotherapy (IMRT) in the tumor bed. The patient is undergoing an outpatient follow-up after ten months of initial treatment with no evidence of relapse or disease progression. The case report suggests that the radical surgical treatment is effective in the control of this pathology and the vascular prosthesis reconstruction safe regard to the risk of thrombosis, even after the use of local radiotherapy, respecting the prophylactic anticoagulation regimen"