Histiocytic sarcoma (HS) is a rare aggressive malignancy with a dismal prognosis and no agreed-upon standard treatment. Classically, the diagnosis of HS has been difficult to confirm and has relied ...on inaccurate, crude techniques. Therapy often involves intensive chemotherapeutic regimens, surgery, and/or radiotherapy, which are poorly tolerated with variable response rates. Patients often die of diffusely metastatic disease. Modern diagnostic techniques are helping to slowly uncover more uniquely customized therapeutic approaches in this enigmatic disease. We present a review of the current literature regarding HS diagnosis, treatment, and outcomes. Additionally, we describe the first reported case of HS transdifferentiated from follicular lymphoma that had a dramatic and durable response to rituximab/bendamustine alone as initial treatment. Unlike traditional chemotherapy regimens, this treatment was well tolerated and had a good toxicity profile. The combination of rituximab and bendamustine warrants further investigation in the treatment of HS, especially those originating from prior follicular lymphoma. Modern immunohistochemical and molecular profiling techniques are beginning to reveal heterogeneity among HS tumors and potentially therapeutic targets.
Endobronchial ultrasound-guided transbronchial needle aspiration is a commonly performed procedure in patients with mediastinal lymphadenopathy. Its role has been widely established in the diagnosis ...and the staging of lung cancer and other benign disease, but there is still debatable evidence regarding its role in the diagnosis, the subtyping, and the treatment of lymphoproliferative diseases. Consequently, there are no guidelines or statements to guide the choice of diagnostic modality in such cases. We review the available literature to evaluate the myth that endobronchial ultrasound-guided transbronchial needle aspiration does not provide sufficient diagnostic material for the accurate diagnosis and subtyping of mediastinal lymphoma.
Opinion statement
Meningiomas are the most frequently diagnosed primary brain tumor accounting for nearly one third of all primary brain and central nervous system tumors reported in the United ...States. According to the 2007 World Health Organization classification scheme, Grade I meningiomas are benign, Grade II defines atypical lesions, while Grade III meningiomas are anaplastic or frankly malignant tumors. Not surprisingly, Grade II and III meningiomas usually follow a more aggressive course and confer a worse prognosis. The diagnosis of meningioma is confirmed by pathologic examination and improvements in imaging help to better define when observation versus intervention is appropriate. Surgical resection, when possible, is the mainstay of treatment. When complete resection is not possible, stereotactic radiosurgery (SRS) can result in disease stabilization. Chemotherapy has yet to result in reproducible long-term disease free or overall survival benefits. Biologic agents remain under investigation. Hemangiopericytomas are rare dural based sarcomas. These tumors are known for their aggressiveness, high recurrence rates and their proclivity to metastasize to extracranial locations. Gross total resection when feasible remains the treatment of choice.
Abstract
Background:
Symptoms of psychological distress, including fear of cancer recurrence (FCR) and quality of life (QOL) deficits are common along the hematopoietic stem cell transplantation ...(HCT) survivorship trajectory. Identifying patterns over time could contribute to timely interventions.
Materials and Methods:
HCT recipients completed the Distress Thermometer (DT), the Center for Epidemiologic Studies-Depression scale (CES-D), the Fear of Relapse and Recurrence Scale, and the Functional Assessment of Cancer Therapy (FACT-BMT) at hospital admission, discharge, 3, 6, 12, and 24 months post-HCT. Demographic data and performance status (PS) were collected at baseline. Mean scores (standard deviation) and frequencies were calculated. We utilized a linear mixed model approach on the repeated measures data (outcome of FCR, with predictors of distress, depressive symptoms and QOL). A multivariate repeated measures regression was constructed to assess what variables were associated with FCR.
Results:
A total of 198 patients completed questionnaires at admission. A total of 144 patients were deceased or lost to follow-up at 2 years. Both CES-D (
P
= .006) and DT (
P
= .0019) scores changed significantly over time and were higher at hospital discharge. FCR did not change significantly (
P
= .28). QOL was most impaired at hospital discharge. FCR did not correlate with actual recurrence. A significant percentage of recipients were afraid of cancer recurrence; however, a much greater percentage did not feel that fear of recurrence got in the way of enjoying life. QOL (
P
< .0001) and PS (
P
= .014) were significant predictors of FCR. A substantial percentage of patients reported significant (>16) depressive symptoms and distress levels (≥4) during the 2-year study period.
Conclusions:
Depressive symptoms and distress were highest at discharge, whereas overall QOL was lowest. FCR was prominent; yet for the majority, it was not an impediment to enjoying life. A psychosocial intervention may be most useful if introduced at hospital discharge and initiated during the first 3 months following HCT when distress is high.
IntroductionAccumulation of excess intra-peritoneal fat is associated with left ventricular (LV) dysfunction in those not receiving treatment for cancer. We sought to determine if increases in ...intra-peritoneal fat were associated with declines in LV ejection fraction (LVEF) after receipt of potentially cardio-toxic chemotherapy.MethodsIn 61 patients (32 lymphoma, 23 breast cancer, 6 sarcoma) we obtained magnetic resonance imaging (MRI) assessments of abdominal fat (intra-peritoneal IP, retroperitoneal RP and subcutaneous SQ) at the level of the L2 vertebra, as well as LVEF prior to and 24 months after initiating cardio-toxic chemotherapy (70% doxorubicin, 1% trastuzumab, 5% taxol, 26% docetaxel, and 67% cyclophosphamide). Correlations between 24-month change in LVEF and baseline measures of body mass index (BMI), IP, RP & SQ fat were identified after accounting for baseline LVEF, height, gender, anthracycline use, and time.ResultsA total of 61 participants (age 53 ± 15 yrs; 32% men, 78% Caucasian) exhibited an average baseline BMI of 30 ± 6 kg/m, height of 170 ± 11cm, and a baseline LVEF of 62 ± 8%. After accounting for height, baseline LVEF, anthracycline use, gender and time between visits, baseline IP fat volume was associated with a LVEF decline (r=-0.27, p=0.047) while BMI, RP and SC fat were not (p= 0.09 to 0.41 for all). Those older than median age of 52 yrs. with high IP fat trended toward greater declines in LVEF (r=0.31, p=0.082) when compared to younger individuals (<52 yrs.) (r=0.24, p=0.346). Within the entire cohort, those with high IP/low SQ fat experienced marked declines in LVEF (p= 0.026).ConclusionsPre-chemotherapy intra-peritoneal fat (not necessarily reflected in an assessment of BMI) is associated with declines in LVEF 2 years after receipt of cardio-toxic chemotherapy. These findings suggest those with larger amounts of intra-peritoneal fat are at higher risk for developing left ventricular dysfunction upon receipt of cardio-toxic chemotherapy.
The Hematopoietic Cell Transplantation (HCT) – Specific Comorbidity Index (HCT-CI) measures the risk of transplant related mortality and predicts overall survival (OS) based on co-morbidities. In a ...prospective validation study of the HCT-CI by the Center for International Blood and Marrow Transplant Research (CIBMTR), HCT-CI ≥ 3 was associated with lower OS in both allogeneic (allo) and autologous (auto) HCT regardless of diagnoses, age, or conditioning. In contrast to the patients (pts) analyzed in the CIBMTR study, pts transplanted at Wake Forest more often have HCT ≥3.
We retrospectively analyzed data from pts transplanted from Sep 2014 to Sep 2016. 2-yr OS was observed for all pts. Results from 3 tools were used to compare predicted vs observed OS: HCT-CI, Disease Risk Index (DRI), and the CIBMTR survival calculator.
Over 2 yrs, 216 pts were transplanted – 132 auto, median age 62 (26-78) and 84 allo, median age 55.5 (16-74). 59% of pts had a HCT-CI ≥3. In both groups, predicted and observed OS were not statistically different for those with HCT-CI 0. In contrast, predicted and observed OS for HCT-CI ≥3 were significantly different (p<0.0001). Using the CIBMTR survival calculator, predicted and observed 1-yr OS for all allo pts in the dataset were no different; however, in the HCT-CI ≥3 subset, observed 1-yr OS was significantly better than predicted. Additionally, observed vs predicted 2-yr OS using the DRI was significantly different in every risk group. Regardless of the tool applied, we consistently had better observed OS than predicted OS for high risk pts. Of pts with HCT-CI ≥ 3, 80.4% had a pulmonary score of 2 or 3.
Compared to the dataset utilized by the CIBMTR for validation of the HCT-CI, scores ≥ 3, largely accounted for by poor pulmonary function, are over represented in our population. Further analysis will need to be done to determine if the regional prevalence of poor pulmonary function is contributing to a significant difference in the predicted vs observed OS in our allo pts.
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