RESUMEN
Hepatocellular carcinoma (HCC) is often diagnosed at a late stage and frequently recurs despite curative intervention, leading to poor survival outcomes. Frontline systemic therapies include combination immunotherapy regimens and tyrosine kinase inhibitors. We report a case of a 38-year-old woman with chronic hepatitis B and C coinfection-associated non-cirrhotic HCC, which recurred in the peritoneum after initial resection of her primary tumor. Disease progression occurred on both atezolizumab/bevacizumab and lenvatinib, and she was subsequently treated with gemcitabine and oxaliplatin (GEMOX) chemotherapy and exhibited a profound clinical response on imaging with normalization of alpha fetoprotein (AFP) after several months. Following extensive multidisciplinary discussion, she underwent cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) that removed all visible macroscopic tumor. Her pathology demonstrated a complete pathologic response. She received two additional months of postoperative chemotherapy, and then proceeded with close monitoring off therapy. To our knowledge, this is the first reported case of a complete pathologic response to GEMOX chemotherapy in the context of CRS/HIPEC for peritoneal metastases in HCC, after progression on standard immunotherapy and tyrosine kinase inhibitor treatments. In this report, we review the current systemic treatment landscape in HCC. We highlight potential consideration of cytotoxic chemotherapy, which is less frequently utilized in current practice, in selected patients with HCC, and discuss the role of CRS/HIPEC in the management of peritoneal metastases. Further investigation regarding predictors of response to systemic treatments is strongly needed. Multidisciplinary management may ultimately prolong survival in patients with advanced HCC.
RESUMEN
Slipping rib syndrome (SRS) is a disorder that occurs when one or more of the eighth through tenth ribs become abnormally mobile. SRS is a poorly understood condition leading to a significant delay in diagnosis and therapeutic management. History and a physical exam are usually sufficient for a diagnosis of SRS. The utility of dynamic ultrasounds has also been studied as a useful diagnostic tool. Multiple surgical techniques for SRS have been described within the literature. Cartilage rib excision (CRE) has been the most common technique utilized. However, the literature has shown a high rate of recurrence and associated risks with the procedure. More recently, minimally invasive rib fixation and costal cartilage excision with vertical rib plating have been shown as successful and safe alternative techniques. This may be an effective, alternative approach to CRE in adult and pediatric populations with SRS.
RESUMEN
Thoracic outlet syndrome (TOS) involves the compression of neurovascular structures in the thoracic outlet. TOS subtypes, including neurogenic (nTOS), venous (vTOS), and arterial (aTOS) are characterized by distinct clinical presentations and diagnostic considerations. This review explores the incidence, diagnostic challenges, and management of TOS with a focus on the innovative approach of Robotic First Rib Resection (R-FRR). Traditional management of TOS includes conservative measures and surgical interventions, with various open surgical approaches carrying risks of complications. R-FRR, a minimally invasive technique, offers advantages such as improved exposure, reduced injury risk to neurovascular structures, and shorter hospital stays. A comprehensive literature review was conducted to assess the outcomes of R-FRR for TOS. Data from 12 selected studies involving 397 patients with nTOS, vTOS, and aTOS were reviewed. The results indicate that R-FRR is associated with favorable intraoperative outcomes including minimal blood loss and low conversion rates to traditional approaches. Postoperatively, patients experienced decreased pain, improved function, and low complication rates. These findings support R-FRR as a safe and effective option for medically refractory TOS.
RESUMEN
OBJECTIVES: Though much information exists about the diagnosis, treatment, and epidemiology of myasthenia gravis (MG), a comprehensive data registry and biorepository is critical to better understand disease mechanisms, treatment outcomes, and the impact of treatment strategies. We aimed to design and implement the "Exploring Outcomes and Characteristics of Myasthenia Gravis (EXPLORE-MG) Registry" to address these knowledge gaps. METHODS: A web-based, non-interventional, longitudinal, observational disease and outcomes registry was developed; incorporating NIH recommended common data elements for the study of MG. Individuals diagnosed with MG based on prespecified criteria were eligible to participate. The registry was further strengthened by a complementary biorepository. An interim analysis was completed on registry data collected through data-lock in 2017. RESULTS: A total of 232 MG patients, followed at the Yale MG Clinic from 2011 to 2017, were enrolled, which included 2142 total visit entries. Of the 232 MG patients (mean age 60 years, range 17-99; female:male, 1.04:1), 165 were acetylcholine receptor antibody-positive, 20 were muscle-specific kinase antibody-positive, and 47 were seronegative. This cohort consisted of 64 patients with ocular disease, 168 patients with generalized disease, and 65 patients post-thymectomy, including 20 with thymoma-associated MG. CONCLUSIONS: Identification of key clinical features that may predict treatment responsiveness or provide insight into patient outcomes is essential to improve patient care. As current research focuses on the development of patient-tailored, targeted-treatment regimens, this registry can help provide important clinical and epidemiological data from a large contemporary patient cohort with long-term follow-up. REGISTRATION: ClinicalTrials.gov Identifier: NCT03792659.