RESUMEN
Background: This study aims to investigate the safety and efficacy of guideline-directed fluid resuscitation (GDFR) compared with conservative fluid management in end-stage renal disease (ESRD) patients with sepsis by evaluating 90-day mortality and intubation rate. Methods: Following PRISMA guidelines, a systematic review was conducted across multiple databases using specific keywords and controlled vocabulary. The search strategy, implemented until October 1, 2023, aimed to identify studies examining fluid resuscitation in ESRD patients with sepsis. The review process was streamlined using Covidence software. A fourth reviewer resolved discrepancies in study inclusion. A random-effects model with the generic Mantel-Haenszel method was preferred for integrating odds ratios (ORs). Sensitivity analysis and publication bias analysis were performed. Results: Of the 1274 identified studies, 10 were selected for inclusion, examining 1184 patients, 593 of whom received GDFR. Four studies were selected to investigate the intubation rate, including 304 patients. No significant mortality or intubation rate difference was spotted between both groups [OR = 1.23; confidence interval (CI) = 0.92-1.65; I2 = 0% and OR = 1.91; CI = 0.91-4.04]. In most studies, sensitivity analysis using the leave-one-out approach revealed higher mortality and intubation rates. The Egger test results indicated no statistically significant publication bias across the included studies. Conclusion: Our research contradicts the common assumption about the effectiveness of GDFR for sepsis patients with ESRD. It suggests that this approach, while not superior to the conservative strategy, may potentially be harmful.
RESUMEN
We describe a rare and remarkable transformation of an immature mediastinal teratoma into high-grade angiosarcoma in a 21-year-old male. Mediastinal teratomas, particularly immature ones, are exceedingly rare, representing a small fraction of germ cell tumors (GCTs). Our case describes the clinical journey of the patient, who initially presented with acute chest pain and was subsequently diagnosed with an immature teratoma following imaging studies and elevated tumor markers. Despite an initial positive response to cisplatin-based chemotherapy, surveillance imaging revealed liver masses, which a biopsy confirmed as angiosarcoma. This transformation underscores the aggressive nature of immature teratomas and the propensity for sarcomatous differentiation, particularly in the mediastinum. The case contributes valuable insight into the management and surveillance of mediastinal non-seminoma germ cell tumors (MNGCT), a subset of GCTs with limited literature. We believe this case is the first in the literature to describe a transformation from an immature teratoma in the mediastinum to a high-grade angiosarcoma.
RESUMEN
We present a case of an adult male who presented with pancytopenia accompanied by symptomatic anemia, necessitating chronic transfusions. He was diagnosed with systemic mastocytosis with an associated hematologic neoplasm. Following an inadequate response to midostaurin therapy, the patient was initiated on the newly approved avapritinib. The patient showed significant improvements in all three blood cell lines; however, he developed leg edema, blepharedema, and gum bleeding on this medication. This case underscores the intricacies of managing a patient with advanced systemic mastocytosis, the emerging role of highly selective KIT inhibition in its treatment, and the practical management of adverse medication effects.
RESUMEN
Dermatofibrosarcoma protuberans (DFSP) is a low- to intermediate-grade dermal soft tissue malignant tumor (sarcoma) with a high local recurrence rate but low metastatic potential. DFSP is characterized by uniform spindle cell fascicles arranged classically in a storiform pattern and by CD34 immunoreactivity. On gross examination, DFSP usually manifests as a white or yellow soft tissue mass with a smooth outer surface and poor circumscription. In this study, we report a case of DFSP with fibrosarcomatous transformation, a rare but well-known phenomenon encountered in DFSP that is correlated with an increased risk of adverse outcomes in patients with DFSP. A 45-year-old male presented with a progressively enlarging lump on his left shoulder, initially suspected of being a lipoma but diagnosed as a fibrosarcomatous transformation of DFSP. Surgical resection was performed, with the subsequent identification of metastatic sarcoma in pulmonary nodules. Robotic-assisted thoracoscopy excised the nodules, confirming metastatic sarcoma with aggressive behavior. Despite negative adjuvant treatment plans, the patient remains under surveillance with imaging, showing no recurrence in recent scans. Continued follow-up with medical and surgical oncology is planned. DFSP is a rare soft tissue sarcoma characterized by indolent growth and low metastatic potential, except in fibrosarcomatous transformation cases. Molecularly, DFSP is defined by a COL1A1-PDGFB fusion transcript that is targetable with imatinib therapy. Treatment involves wide surgical resection, with adjuvant radiation therapy in select cases. Radiation therapy may be employed in cases with close or positive margins, while conventional chemotherapy has limited utility. Multidisciplinary collaboration is crucial for optimal management. Overall, this case underscores the challenges in diagnosing and managing aggressive sarcomas like fibrosarcomatous DFSP, emphasizing the importance of vigilant surveillance and multidisciplinary collaboration in optimizing patient outcomes. Further research is needed to understand the mechanisms underlying fibrosarcomatous transformation and to explore novel therapeutic avenues for this challenging malignancy.