Citations

BACKGROUND Malignant peripheral nerve sheath tumors (MPNSTs) are rare and highly malignant tumors of neural crest origin. No matter their anatomical location, they are difficult to treat and carry a poor prognosis. OBSERVATIONS This report highlights the complexities of the diagnosis and management of a patient with MPNST of the cauda equina. The tumor retained the expression of H3K27me3, which is expected in spinal MPNSTs but uncommon in most sporadic and neurofibromatosis type 1–driven MPNSTs. LESSONS The described diagnostic nuance should be taken into consideration in patients with suspected MPNST of the cauda equina, as these tumors require swift neurosurgical intervention in order to maintain neurological function. Additional treatment modalities include neoadjuvant and adjuvant chemotherapy and radiotherapy. Although improvements in local control and progression-free survival have been reported, the role of radiation therapy and chemotherapy has been underinvestigated due to the rarity of MPNSTs presenting in this region. https://thejns.org/doi/10.3171/CASE24723

Background. End-stage kidney disease patients with concomitant heart failure (HF) with reduced ejection fraction are often denied kidney transplantation. The aims of this study were to explore factors predictive of suitability for kidney transplant and to assess cardiovascular outcomes in patients with impaired left ventricular ejection fraction (LVEF) after transplant. Methods. We evaluated 109 consecutive adults with LVEF ≤40% at the time of initial kidney transplant evaluation between 2013 and 2018. Posttransplant cardiovascular outcomes were defined as nonfatal myocardial infarction (MI), admission for HF, cardiovascular death, and all-cause mortality. Results. A cardiologist participated in kidney transplant evaluation for 87% of patients and was present at 49% of transplant selection conferences. Twenty-four patients (22%) were denied by a cardiologist for kidney transplant‚ and 59 (54%) were denied by the selection committee, of whom 43 were because of cardiovascular risk. Forty-two (38%) patients were approved for kidney transplant. On univariate analysis, the variables associated with denial for kidney transplant included cardiologist denial, higher cardiac troponin T, prior coronary intervention, cardiovascular event, positive stress study, lower ejection fraction, and lower VO2 max (all P < 0.05). Cardiologist denial was the most significant predictor of denial for kidney transplant in different multivariate models. At a median follow-up of 15 mo, 5 (5%) suffered nonfatal MI, 13 (12%) were hospitalized for HF exacerbation, and 17 (16%) died. Only 22 patients, 52% of those approved, underwent kidney transplant. After kidney transplant, there was 1 death, 1 nonfatal MI, and 3 hospitalizations for HF. Median LVEF improved from 38% before listing to 55% posttransplant. Conclusions. Cardiologist denial was the primary predictor of rejection for kidney transplant. Despite careful selection, prevalence of cardiovascular events and mortality after kidney transplant was 23%. There is need for a structured multidisciplinary approach for patients with impaired LVEF.

Introduction: End stage renal disease (ESRD) patients with concomitant heart failure (HF) are often denied kidney transplantation (KTx). The aims of this study were to explore factors predictive of suitability for KTx and to assess cardiovascular (CV) outcomes in patients with impaired left ventricular ejection fraction (LVEF) presenting for KTx evaluation. Methods: We evaluated 109 consecutive adults with LVEF≤40% at the time of initial KTx evaluation between 2013 and 2018. Post-transplant CV outcomes were defined as non-fatal MI, admission for HF, CV death and all-cause mortality. Results: Mean age was 58.2 years (SD11.9), 78% were male, 58% had diabetes, 70% had history of CV events and 42% had ischemic cardiomyopathy. Mean LVEF was 31.5% (SD 6.47). Eighty patients had nuclear stress imaging; 10% were positive for reversible ischemia and 43% for prior infarct. Mean VO2max was 14.4(SD 5.71)ml/kg/min (31 patients). A cardiologist evaluated 93% of patients and was present at 58% of selection committee meetings. Twenty-four patients (22%) were denied by a cardiologist for KTx and 59 (54%) were denied by the selection committee, of whom 43 were due to CV risk. On univariate analysis, the variables associated with denial for KTx were: cardiologist denial, denial due to CV risk, Native American race (6% of cohort), higher NT-pro-BNP, prior MI, coronary intervention, positive stress study, anemia, lower EF and lower VO2max (all p<0.05). On multivariate analysis, cardiologist denial was the only significant predictor of denial for KTx (OR: 29.4, p=0.0007). At median follow-up of 15 months, 5 (5%) suffered non-fatal MI, 13 (12%) were hospitalized for HF exacerbation and 17 (16%) died. Only 22 (20%) underwent KTx. Post-KTx, there was one death, one non-fatal MI and 3 hospitalizations for HF. Mean LVEF improvement was 16% (SD12.9). Conclusions: Only 38% of ESRD patients with LVEF≤40% presenting for KTx evaluation were approved and of those, only 52% received KTx. Cardiologist approval was the primary predictor of suitability for KTx. Despite careful selection, prevalence of CV events and mortality after KTx was 23%. There is need for a consistent multidisciplinary approach during KTx evaluation, including cardiologist input, to improve CV outcomes.