Ultra-rare sarcomas: a consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

Background Among sarcomas, which are rare cancers, many types are exceedingly rare; however, a definition of ultra-rare cancers has not been established. The problem of ultra-rare sarcomas is particularly relevant because they represent unique diseases, and their rarity poses major challenges for diagnosis, understanding disease biology, generating clinical evidence to support new drug development, and achieving formal authorization for novel therapies.Methods The Connective Tissue Oncology Society promoted a consensus effort in November 2019 to establish how to define ultra-rare sarcomas through expert consensus and epidemiologic data and to work out a comprehensive list of these diseases. The list of ultra-rare sarcomas was based on the 2020 World Health Organization classification, The incidence rates were estimated using the Information Network on Rare Cancers (RARECARENet) database and NETSARC (the French Sarcoma Network's clinical-pathologic registry). Incidence rates were further validated in collaboration with the Asian cancer registries of Japan, Korea, and Taiwan.Results It was agreed that the best criterion for a definition of ultra-rare sarcomas would be incidence. Ultra-rare sarcomas were defined as those with an incidence of approximately <= 1 per 1,000,000, to include those entities whose rarity renders them extremely difficult to conduct well powered, prospective clinical studies. On the basis of this threshold, a list of ultra-rare sarcomas was defined, which comprised 56 soft tissue sarcoma types and 21 bone sarcoma types.conclusions Altogether, the incidence of ultra-rare sarcomas accounts for roughly 20% of all soft tissue and bone sarcomas. This confirms that the challenges inherent in ultra-rare sarcomas affect large numbers of patients.Experimentele farmacotherapi

The Spiral Economy: a Socially Progressive Circular Economy Model?

The Circular Economy (CE) is a well-established sustainability framework within the industry and business contexts, with strong advocates such as the Ellen MacArthur Foundation. It is designed to be restorative and regenerative, and through a continuous cycle aims to keep products, components, and materials at their highest utility and value at all times. It focuses on preserving and enhancing natural capital, optimising resource yields, and minimising system risks by managing finite stocks and renewable flows. The CE principles align strongly with the Sustainable Development Goal (SDG) framework, which is a relevant lens for further understanding how industry can strategically address environmental and social issues. However, while the CE framework claims to account for the three inter-related and co-evolutionary sustainability dimensions of social, economic and environmental it is challenged for its overt emphasis on the latter two aspects with less done to achieve social value and benefits for those involved in creating and managing the restorative and regenerative cycles. This chapter aims to address this key gap by critically reviewing relevant literature and arguing for a more socially integrated and progressive CE. It develops and presents an original conceptual framework called the Spiral Economy (SE)

Metabólitos sanguíneos de vacas de corte suplementadas ou não com sais de cálcio de ácidos graxos durante o período pré e/ou pós-parto

Avaliou-se a concentração de metabólitos sanguíneos de vacas de corte mantidas em pastagem natural recebendo suplementação com sais de cálcio de ácidos graxos (SCAG) durante 45 dias antes do parto (PRE), suplementação com SCAG durante 45 dias antes do parto e 63 dias pós-parto (PREPOS), suplementação com SCAG durante 63 dias pós-parto (POS) e de vacas não suplementadas (PN). As coletas de sangue foram realizadas em média 64 dias antes do parto, e aos 21, 42 e 63 dias pós-parto. Não ocorreu interação significativa entre tratamentos e períodos. As concentrações plasmáticas de β-hidroxibutirato (βHB), triglicerídeos (TRIG), colesterol, glicose e ureia não foram afetadas significativamente pela suplementação de gordura protegida. A análise de regressão mostrou queda linear significativa da concentração de TRIG no sangue com o aumento da produção de leite (PL) para as vacas do tratamento PN (TRIG = 23,10 - 2,18*PL, R² = 0,31) e efeito quadrático para as vacas do PRE (TRIG = 6,54 - 1,75*PL + 0,30*PL², R² = 0,62). Nos animais dos tratamentos POS e PREPOS, não houve efeito da produção de leite sobre a concentração de TRIG, indicando que a suplementação durante o período de produção de leite auxilia na manutenção de um balanço energético positivo. As concentrações de colesterol plasmático aumentaram, e as de triglicerídeos e ureia diminuíram linearmente até o final do experimento

Características dos componentes não integrantes da carcaça de novilhos superjovens da raça Devon, terminados em diferentes sistemas de alimentação

O objetivo do estudo foi avaliar as características dos componentes não integrantes da carcaça de novilhos Devon terminados em confinamento (CONF), em pastagem de clima temperado (pastagem de azevém - Lolium multiflorum Lam - PTEM) ou em pastagem de clima tropical (associação de pastagem de milheto - Pennisetum americanum (L.) Leeke - e capim-papuã - Bracharia plantaginea - PTRO). Os novilhos, ao início da terminação, estavam com 320kg e 15 meses de idade. Os animais confinados foram alimentados com relação volumoso:concentrado de 60:40; o volumoso era constituído de silagem de milho, e o concentrado de farelo de trigo, milho e minerais. Os animais foram abatidos com pesos semelhantes de 388,3; 386,7 e 375,8kg no CONF, na PTEM e na PTRO, respectivamente. Os animais da PTRO apresentaram maior (P<0,10) rendimento de carcaça quente (RCQ) relativo a 100kg de peso corporal vazio (RCQPCV) do que os da PTEM, 64,6 versus 62,6%, e os do CONF apresentaram RCQPCV intermediário, 63,7%. Os pesos absolutos do fígado, 5,22; 4,43 e 3,87kg, do conjunto dos órgãos internos, 12,81; 11,37 e 10,83kg, do rúmen-retículo, 7,62; 6,54 e 6,06kg, da gordura do coração, 1,26; 0,65 e 0,30kg, e dos intestinos, 9,97; 7,13 e 7,49kg, foram mais altos (P<0,05) nos animais da PTEM, em relação aos do CONF e da PTRO, respectivamente. A mesma ordem de grandeza ocorreu com os pesos relativos desses órgãos. A PTRO e o CONF originaram animais com maior (P<0,05) peso de conteúdo gastrintestinal em relação à PTEM, respectivamente, 60,27; 55,32 e 41,21kg. O CONF proporcionou animais com pesos absolutos mais elevados (P<0,05) do omaso, 5,17kg, em relação aos da PTEM, 3,70kg, e este peso foi intermediário nos animais da PTRO, 4,77kg. A mesma ordem de grandeza ocorreu com os pesos relativos do omaso, 1,61; 1,12 e 1,54%

Performance of feedlot steers finished with different fat sources in diet

The objective of this study was to evaluate the effect of different fat sources in finishing diets on performance of feedlot steers. Twenty steers, with average age of 20 months and initial live weight of 260 ± 41.3 kg, were feedlot fed during 126 days. The animals received corn silage as roughage and the tested concentrates were: basic concentrate (BC); basic concentrate + whole rice bran and rice oil (WRB); basic concentrate + 3 % of fatty acids calcium salts (M3) and basic concentrate + 6 % of fatty acids calcium salts (M6). Each treatment had animals with the following genetic group: 1 pure Charolais (CH), 1 pure Nellore (NE), 2 11/16 CH 5/16 NE, 1 21/32 NE 11/ 32 CH. No significant differences were observed for crude protein, digestible energy, neutral and acid detergent fibers intakes. The animals that consumed 6 % of fatty acid calcium salts presented higher ether extract intake (0.77 kg/day), while those that consume 3 % or whole rice bran and oil showed intermediate value (0.51 kg/day) and the ones that consumed basic concentrated presented lower value (0.25 kg/day). Similar averages were observed for average daily weight gain (1.142, 1.199, 1.365 and 1.391 kg/day, for BC, WRB, M3 and M6, respectively) and final weight (421 kg).O objetivo deste estudo foi avaliar efeito do uso de diferentes fontes de gordura na dieta sobre o desempenho de novilhos confinados em fase de terminação. Foram utilizados 20 novilhos, com idade média inicial de 20 meses e peso vivo médio inicial de 260 ± 41,3 kg, que permaneceram con- finados por 126 dias. Os animais receberam silagem de milho como volumoso e os concentrados tes- tados foram: concentrado base (BC); concentra- do base + farelo de arroz integral e óleo de arroz (WRB); concentrado base + 3 % de sais de cálcio de ácidos graxos (M3) e concentrado base + 6 % de sais de cálcio de ácidos graxos (M6). Cada tratamento possuiu animais pertencendo aos seguintes grupos genéticos: 1 puro Charolês (CH), 1 puro Nelore (NE), 2 11/16 CH 5/16 NE, 1 21/ 32 NE 11/32 CH. Não foram observadas diferenças entre os consumos de proteína bruta, energia digestível, fibras em detergente neutro e ácido. Os animais que consumiram 6 % de sais de ácidos graxos apresentaram o maior consumo de extrato etéreo (0,77 kg/dia), enquanto que aqueles que consumiram a dieta M3 e WRB apresentaram valor intermediário de consumo (0,51 kg/dia) e os que consumiram BC mostraram o menor valor (0,25 kg/ dia). Similaridades de valores foram observadas para o ganho de peso médio diário (1,142; 1,199; 1,365 e 1,391 kg/dia, respectivamente para CB, WRB, M3 e M6) e peso final (421 kg)

Adequate Local Control in High-Risk Soft Tissue Sarcoma of the Extremity Treated with Surgery Alone at a Reference Centre : Should Radiotherapy Still be a Standard?

BACKGROUND: Established practice for the management of soft tissue sarcoma (STS) of the extremity and trunk wall combines perioperative radiotherapy (RT) with limb-preserving surgery. OBJECTIVE: The aim of this study was to explore whether high-quality surgery at high-volume centers may offer equivalent local control in selected cases, when RT needs to be avoided. METHODS: All consecutive adult cases of primary, high-risk STSs treated in a high-volume reference center over a 12-year timeframe were included, and, on retrospective analysis, were divided into two groups. Group A received RT with surgery, and Group B received surgery alone. The primary endpoint was local recurrence-free survival (LRFS). RESULTS: Overall, 390 patients were included (318 in Group A and 72 in Group B), with a median follow-up of 53 months. The main reasons for avoiding RT were patient choice and technical considerations (vascular bypass or flap reconstruction). No difference in R0 resection was seen between the groups (79% vs. 70%; p = 0.18), but Group A had more G3 tumors (80.5% vs. 68%; p = 0.021). No difference in 5-year LRFS was evident (84% vs. 81%; p = 0.16). CONCLUSIONS: LRFS did not differ between patients with high-risk STSs receiving perioperative RT and those treated with surgery alone. The study was retrospective and omission of RT was largely uncontrolled with inherent bias. Nonetheless, data suggest that in experienced centers, the omission of RT did not diminish local disease outcome. Future studies on a selective approach to RT administration are awaited