CLINICAL AND EPIDEMIOLOGIC EVALUATION OF DESMOID TUMORS IN A BRAZILIAN SARCOMA REFERENCE CENTER.

Introduction: Desmoid Tumors (DT) are rare neoplasms with higher incidence in younger women. Methods: Retrospective, single-center analysis of patients with DT. Variables were age, sex, biopsy, treatment and recurrence. The disease-free survival (DFS) was calculated with the Kaplan-Meier method. Results: 242 patients were evaluated, mean age was 34 years, 70.7% women, 44.4% originated in the trunk/abdomen and 54.5% had size > 5cm. Surgery was performed in 70.2%, 31% with negative margin and only 57% with previous biopsy. Recurrence rate was 38% and 1,2,5-year DFS was 75.3%, 64.2%, 57.8%, respectively. Size (p = 0.018) and tumor location in the dorsum (p = 0.001), extremities (p = 0.003) and pelvis (p = 0.003) were related to higher relapse rate. Conclusion: our data reinforces the need to gather data from real world practice and the importance of awareness of DT and medical education about DT behavior and best approach due to the high rates of surgery and elevated number of patients treated without biopsy. Level of Evidence III; Retrospective Comparative Study.

Introdução: Os tumores desmóides (TD) são neoplasias raras com maior incidência em mulheres jovens. Métodos: Trata-se de uma análise retrospectiva, em um único centro, de pacientes com TD. As variáveis foram idade, sexo, biópsia, tratamento e recorrência. A sobrevida livre de doença (SLD) foi calculada pelo método de Kaplan-Meier. Resultados: Foram avaliados 242 pacientes, com idade média de 34 anos, 70,7% mulheres, 44,4% com origem no tronco/abdômen e 54,5% com tamanho > 5 cm. A cirurgia foi realizada em 70,2%, 31% com margem negativa e apenas 57% com biópsia prévia. A taxa de recorrência foi de 38% e a SLD de 1, 2 e 5 anos foi de 75,3%, 64,2% e 57,8%, respectivamente. O tamanho (p = 0,018) e a localização do tumor no dorso (p = 0,001), nas extremidades (p = 0,003) e na pelve (p = 0,003) foram relacionados a uma maior taxa de recidiva. Conclusão: Nossos dados reforçam a necessidade de coletar dados da prática do cenário real e a importância da conscientização da TD e da educação médica sobre o comportamento da TD e a melhor abordagem, devido às altas taxas de cirurgia e ao elevado número de pacientes tratados sem biópsia. Nível de Evidência III; Estudo Comparativo Retrospectivo.

Claudin 18.2 as a New Biomarker in Gastric Cancer-What Should We Know?

Gastric cancer (GC) remains a formidable global health challenge, ranking among the top-five causes of cancer-related deaths worldwide. The majority of patients face advanced stages at diagnosis, with a mere 6% five-year survival rate. First-line treatment for metastatic GC typically involves a fluoropyrimidine and platinum agent combination; yet, predictive molecular markers have proven elusive. This review navigates the evolving landscape of GC biomarkers, with a specific focus on Claudin 18.2 (CLDN18.2) as an emerging and promising target. Recent phase III trials have unveiled the efficacy of Zolbetuximab, a CLDN18.2-targeting antibody, in combination with oxaliplatin-based chemotherapy for CLDN18.2-positive metastatic GC. As this novel therapeutic avenue unfolds, understanding the nuanced decision making regarding the selection of anti-CLDN18.2 therapies over other targeted agents in metastatic GC becomes crucial. This manuscript reviews the evolving role of CLDN18.2 as a biomarker in GC and explores the current status of CLDN18.2-targeting agents in clinical development. The aim is to provide concise insights into the potential of CLDN18.2 as a therapeutic target and guide future clinical decisions in the management of metastatic GC.

Targeting KRAS in Pancreatic Ductal Adenocarcinoma: The Long Road to Cure.

Pancreatic ductal adenocarcinoma (PDAC) remains an important cause of cancer-related mortality, and it is expected to play an even bigger part in cancer burden in the years to come. Despite concerted efforts from scientists and physicians, patients have experienced little improvement in survival over the past decades, possibly because of the non-specific nature of the tested treatment modalities. Recently, the discovery of potentially targetable molecular alterations has paved the way for the personalized treatment of PDAC. Indeed, the central piece in the molecular framework of PDAC is starting to be unveiled. KRAS mutations are seen in 90% of PDACs, and multiple studies have demonstrated their pivotal role in pancreatic carcinogenesis. Recent investigations have shed light on the differences in prognosis as well as therapeutic implications of the different KRAS mutations and disentangled the relationship between KRAS and effectors of downstream and parallel signaling pathways. Additionally, the recognition of other mechanisms involving KRAS-mediated pathogenesis, such as KRAS dosing and allelic imbalance, has contributed to broadening the current knowledge regarding this molecular alteration. Finally, KRAS G12C inhibitors have been recently tested in patients with pancreatic cancer with relative success, and inhibitors of KRAS harboring other mutations are under clinical development. These drugs currently represent a true hope for a meaningful leap forward in this dreadful disease.