RESUMEN
Bing-Neel syndrome (BNS) is a rare complication of Waldenstrom macroglobulinemia (WM) defined by a lymphoplasmacytic infiltration of the central nervous system (CNS). Patients present with a range of neurologic symptoms of variable severity. Diagnosis requires a low threshold of suspicion and is considered in WM patients with unexplained neurologic symptoms. It can occur in the presence of quiescent serum markers of WM. Direct CNS tissue biopsy should be pursued if feasible and remains the gold standard for diagnosis. No standard of care treatment exists, but expert guidelines suggest intravenous chemotherapy in standard dose or high-dose regimens or use of oral ibrutinib. Consideration is also made for intravenous rituximab, intrathecal therapies, and autologous stem cell transplantation. Patient factors and tolerability should drive decisions regarding treatment choice in this arena, given a lack of data for standard frontline therapy.
RESUMEN
BACKGROUND: High-dose interleukin-2 (HD IL-2) is used in the treatment of metastatic renal cell carcinoma (mRCC) and has an overall response rate (ORR) of 12-20% and a complete response rate (CR) of 8% in unselected populations with predominantly clear cell type renal cell carcinoma. Nearly 10-15% of patients with renal cell carcinoma exhibit sarcomatoid differentiation, a feature which correlates with a median overall survival (OS) of 9 months and overall poor prognosis. We report a single institution experience with 21 patients with mRCC with sarcomatoid features post-nephrectomy who were treated with HD IL-2. METHODS: Twenty one patients with mRCC with sarcomatoid features post-nephrectomy who underwent therapy with HD IL-2 were identified at the University of Pittsburgh Medical Center from 2004 to 2016. Baseline patient characteristics, HD IL-2 cycles, time to progression, and subsequent therapies were evaluated. OS and progression-free survival (PFS) in the cohort were calculated using the Kaplan-Meier method. Disease characteristics were evaluated for significance using the Fischer's exact test and Wilcoxon rank sum test. RESULTS: Patients were predominantly Caucasian males with a median age of 54 years. A majority, 86% of these patients, had metastatic disease at time of initial presentation, primarily with lung and lymph node involvement. The ORR and CR with HD IL-2 was 10% and 5%, respectively. Initial localized disease presentation is the only variable that was significantly associated with response to HD IL-2 (p = 0.0158). Number of HD IL-2 doses did not correlate with response with a mean of 16.5 and 15.0 total doses in responders and non-responders, respectively (p = 0.53). Median PFS with HD IL-2 was 7.9 months (95% CI, 5.0-21.3). Median OS was 30.5 months (95% CI 13.3-57.66). Within the subset of patients who had progression on IL-2, median OS was 19.4 months (95% CI, 13.3-35.3). In patients who received second-line therapy, median PFS was 7.9 months (95% CI 2.4-10.2). CONCLUSION: In patients with mRCC with sarcomatoid features, use of HD IL-2 was associated with a modest ORR and a higher survival compared to historical controls (patients with mRCC and sarcomatoid features). Thus, HD IL-2 may have a role in treating selected patients with mRCC with sarcomatoid features.
Asunto(s)
Carcinoma de Células Renales/secundario , Interleucina-2/uso terapéutico , Neoplasias Renales/secundario , Sarcoma/patología , Ensayos Clínicos como Asunto , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Interleucina-2/farmacología , Estimación de Kaplan-Meier , Masculino , Persona de Mediana EdadRESUMEN
GOALS: To explore the utility of endoscopic ultrasound (EUS) in the evaluation of chronic upper abdominal pain (UAP) of undetermined etiology. BACKGROUND: Chronic UAP is a common problem with a challenging diagnosis and management. The role of EUS in the diagnosis of UAP may minimize additional testing; however, few studies describe the percentage of new diagnoses yielded in these patients. STUDY: We conducted a retrospective analysis by reviewing electronic medical records at Scott and White Memorial Hospital, Texas A&M Health Sciences Center for patients with abdominal pain for ≥ 12 months not explained by previous workup referred for EUS for chronic UAP from January 1, 1998 through October 1, 2007. Patients with previous EUS in past 12 months were excluded from the study. Patient demographic data and imaging performed 6 months before and 24 months after EUS were reviewed and results documented. RESULTS: EUS was successful at diagnosing a new clinical etiology of chronic UAP in 33 patients (8.89%) with previous workup that was unrevealing for a definitive diagnosis. The most frequent diagnoses included pancreaticobiliary tree abnormalities, chronic pancreatitis, and fatty liver disease. CONCLUSIONS: Our results support the fact that the majority of patients UAP with prior imaging will have no identifiable organic etiology found on EUS to explain their pain; however, we suggest that EUS be considered in patients with suspected pancreatic or biliary pathology.