Health-related quality of life (HRQoL), anxiety, and depression in patients with desmoid type fibromatosis.

INTRODUCTION: Data on the impact of desmoid type fibromatosis (DTF) on emotional distress and health-related quality of life (HRQoL) is sparse. METHODOLOGY: In this prospective cross-sectional study, patients with DTF and healthy controls were asked to fill the EORTC QLQ-C30, GAD-7, and PHQ-9 questionnaires. The objectives were to determine HRQoL, anxiety, and depression in patients with DTF. RESULTS: Two hundred four subjects (102 DTF patients and 102 healthy controls) were recruited. The median age of DTF patients at recruitment into the study was 31 years (IQR, 25-37 years). There was a female preponderance with a male:female ratio of 1:1.83. Appendicular skeleton and abdomen sites were most commonly involved in 59% and 22.5% respectively. About half (54%) of patients were currently on sorafenib and 41% were under active surveillance. The mean global health status in DTF patients was 65.58 ± 22.64, significantly lower than healthy controls. Similarly, DTF patients scored low on all functional scales except cognitive functioning. The symptom scale showed a significantly higher symptom burden of fatigue, pain, insomnia, and financial difficulties. Anxiety and depression was observed in 39.22% and 50% of DTF patients respectively. DTF patients had higher rates of mild, moderate, and severe anxiety and depression compared to healthy controls. CONCLUSION: DTF patients have significant symptom burden, poor functioning, and heightened anxiety and depression as compared to healthy controls. HRQoL, anxiety, and depression should be routinely used to assess symptom burden and treatment efficacy in DTF patients.

Autologous Hematopoietic Stem Cell Transplant: A Potential Option for Relapsed Pediatric Acute Myeloid Leukemia With Lack of Matched Sibling Donor and Resource Challenge.

Though allogeneic hematopoietic stem cell transplant (HSCT) is standard for relapsed pediatric acute myeloid leukemia, often it may not be accessible due to donor unavailability and resource limitations. We retrospectively analyzed outcomes of 26 children (mean age: 10.9±4.5 y) who underwent autologous HSCT in complete remission 2. Three-year event-free survival and overall survival were 50.1±10.7% and 63.3±9%, respectively; with 1 treatment-related death and 46% relapse rate. Notwithstanding the retrospective study design and somewhat favorable patient characteristics, the outcomes are comparable with those of other series and our own allogeneic HSCT data. Autologous HSCT is a potential alternative to allogeneic HSCT, especially in countries with poorly maintained donor registries.

Trabectedin in Advanced Sarcomas-Experience at a Tertiary Care Center and Review of Literature.

Background There is sparse literature on trabectedin in advanced soft-tissue sarcomas from developing world. It would be interesting to know about use and outcomes of trabectedin in Indian patients. Method In a retrospective study, consecutive patients treated with trabectedin from 2016 to 2019 were analyzed. Patients with L-sarcomas were treated at a dose of 1.5 mg/m 2 , while those with translocation-related sarcomas were treated at a dose of 1.2 mg/m 2 as a 24-hour infusion through peripherally inserted central catheter line. From July 2019, infusions were administered through an ambulatory elastomeric pump, while before that patients were admitted for 24 hours. We used SPSS version 23.0 for statistical calculation. Result A total of 20 patients received trabectedin with a total of 116 infusions. The median age was 46 years (range: 22-73 years). The male ( n = 11, 55%) and female patients were almost equal ( n = 9, 45%). Thirteen patients (65%) had Eastern Cooperative Oncology Group Performance Status 1. Majority of the patients had leiomyosarcoma ( n = 8, 40%); remaining comprised of liposarcoma (3, 15%), translocation-related sarcomas excluding myxoid liposarcoma ( n = 8, 40%) and others ( n = 1,5%). Most common site was extremity ( n = 11, 55%) followed by retroperitoneal ( n = 3, 15%), visceral ( n = 3, 15%), and others ( n = 3,15%). Median number of previous lines received was 2 (range: 0-4). Median number of trabectedin cycles received was 4 (range: 1-17). Best response assessed was stable disease ( n = 10, 50%), progressive disease ( n = 6, 30%), partial response ( n = 1, 5%), and not assessed in 3 patients. After a median follow-up of 19 months, median progression-free survival was 4 months. Conclusion In this heavily treated population (composed of L-sarcomas and translocation-related sarcomas) with many patients with poor performance status, the outcome with trabectedin is in synchrony with literature. However, the need of 24-hour admission might deter quality of life. Elastomeric pump seems to be a reasonable alternative to admission and can be a breakthrough in administering trabectedin, especially in developing countries.

Clinical approach to childhood mediastinal tumors and management.

Mediastinal tumours are not uncommon in paediatric population and often pose a diagnostic challenge. They include a variety of entities including developmental, inflammatory, infectious and neoplastic; most are malignant. These lesions can be classified based on imaging according to the specific compartment (anterior, middle and posterior), generating a focused differential diagnosis. This combined with a rational, clinically oriented approach based on patient's history, focused physical examination, age, gender, symptoms, signs, anatomic localization, imaging characteristics and laboratory investigations including tumor markers paves way to a presumptive diagnosis guiding additional and prudent investigations. For example, a suspicion of lymphoma should be kept in a child presenting with a neck mass and superior vena cava syndrome. Neuroblastoma should be suspected among children younger than 5 years old with a posterior mediastinal mass. Such a structured approach along with histopathology will lead to an exact diagnosis. Surgery remains the mainstay of treatment of most benign and malignant non-lymphoid tumours. For optimal management, a combined modality of treatment incorporating chemotherapy and radiotherapy is often required in malignant tumours and is associated with high survival rates in these patients. In the present article, we review the approach to evaluation of mediastinal masses in childhood from a clinical perspective.

Advances in soft-tissue sarcoma - There are no mistakes, only lessons to learn!

BACKGROUND: In this decade the treatment of advanced sarcoma has seen many highs and lows in terms of successful trials and failed trials. This is possible due to great collaborations, newer therapies and histology focused trials. METHODS: In ASCO 2019 many sarcoma trials were presented and we chose 3 challenging clinical trials that widen our perspective on soft tissue sarcoma. We have critically analyzed the data and have discussed the implications of these trials on current practice. First trial was ANNOUNCE trial which was done to confirm the efficacy of olaratumab after its dramatic success in advanced soft tissue sarcoma in a phase 2 trial. Another trial STRASS trial, which was unique because of being first successfully conducted randomized trial addressing preoperative radiotherapy in retroperitoneal soft tissue sarcoma. Third trial was phase 2 trial SARC 028 trial exploring the role of immunotherapy in pleomorphic undifferentiated sarcoma and liposarcoma subgroup. RESULT: ANNOUNCE trial failed to show OS benefit in olaratumab/doxorubicin arm as compared to doxorubicin/placebo arm. Based upon this FDA has revoked the approval of olaratumab leading to nihilism and disappointment amongst oncologists. In STRASS trial failed to meet the primary end point though there was a benefit in the liposarcoma subgroup in terms of abdominal recurrence free survival. There are several reasons that this trial might have failed. First, RPSs are not homogeneous population. RPSs might behave very differently as per the histopathology ranging from well differentiated LPS to leiomyosarcoma. Since the event rate in well-differentiated liposarcoma might happen late, the median follow-up of 43 months might not be sufficient. In SARC trial ORR in pleomorphic undifferentiated sarcoma (PUS) cohort was 9/40 (22.5%), while response rates in liposarcoma cohort were 4/39 (10.2%). There was poor correlation between the response and the tumor cells' PD-L1 positivity. Simultaneously, we must not take for granted the role of pembrolizumab in PUS as the previous study (PEMBROSARC) had also showed dismal outcomes with immunotherapy. CONCLUSION: In this paper we discuss the intricacies of these trials and how they affect the rapidly changing landscape in advanced soft tissue sarcoma.

Long lasting response of trabectedin in patient with gastric leiomyosarcoma with liver metastasis: an update to previous report.

Leiomyosarcoma of the stomach is an extremely rare malignancy for which treatment in advanced disease is hardly reported. Here, we report a case of a 48-year-old man with metastatic gastric Leiomyosarcoma who had previously received a combination of gemcitabine and docetaxel followed by pazopanib after detection of metastasis. The patient was started on trabectedin as per protocol and had disease control continuing for 17 cycles of trabectedin. His quality of life and absence of significant toxicities highlight the noncumulative nature of trabectedin and potential benefit in responding cases.

Sleep Duration, Insomnia Symptoms, and Emotion Regulation among Black Women.

INTRODUCTION: This study explored the associations between sleep duration and emotion regulation among urban black women (mean age=59 ± 7 yrs). METHOD: Eligible women (n=523) provided sociodemographic data during face-to-face interviews. We used the Comprehensive Assessment and Referral Examination Physical to measure health status; women also estimated their habitual sleep duration. We utilized a modified version of Weinberger's conceptual model of repression, the Index of Self-Regulation (ISE) to measure emotion regulation. ISE scores were derived by amalgamating the defensive subscale from the Social Desirability Scale and the anxiety subscale from the State-Trait Anxiety Inventory. RESULTS: The median habitual sleep duration was 7 hours; 20% of the women were short sleepers (<6 hours) and 6% were long sleepers (>8 hours). Short sleepers, rather than long sleepers, had a greater likelihood of reporting insomnia symptoms than those sleeping 6-8 hours [63.4% vs. 28.1%; Χ2 = 41.87, p<0.001]. In the first logistic regression model, the odds of being a short sleeper for low regulators were 3 times greater than for high regulators [OR = 3.22 95% CI: 2.05-5.06; p<0.0001]. In multivariate-adjusted analysis, OR was reduced to 2.06, but remained significant. In the second logistic model, the likelihood of being a long sleeper among low regulators were 37% greater than for high regulators, but results were not significant [OR=1.37, 95% CI: 0.62-3.01; NS]. DISCUSSION: Short and long sleep duration are associated with reduced ability for emotion regulation. Women sleeping 6-8 hrs might be more adept at regulating emotions in their daily lives. Insomnia symptoms might mediate associations between emotion regulations and sleep durations.

Immune response profiles after caterpillar exposure: a case report.

RATIONALE: The role of the immune response to caterpillar exposure is not well described. This case study is the first to report a patient who presented with an allergic reaction after exposure to the larvae of the sycamore tussock moth, Halysidota harrisii Walsh, 1864. METHODS: Blood was collected from an allergic asthmatic adult (m/42 y/o) at 2 hrs - 2 wks after contact urticaria with associated dyspnea after exposure to the larvae of the sycamore tussock moth, Halysidota harrisii Walsh, 1864. Distributions of blood lymphocytes (CD4(+), CD8(+), CD8(+)CD60(+), CD19(+), CD23(+), CD16/56(+), CD25, CD45RA(+), CD45RO(+)), monocytes (CD1d(+)), levels of serum immunoglobulins (IgM, IgG, IgA, IgE), and cytokines (IFN-γ, IL-4, TNF-α) were studied (flow cytometry, nephelometry, UniCAP Total IgE Fluoroenzymeimmunoassay, cytokine ELISA, clinical toxicology). RESULTS: Numbers of CD4(+) T cells, CD25(+) cells, CD19(+) B cells, and CD1d(+) monocytes decreased (22, 27, 33, 20%, respectively) one week post reaction, CD45RA(+) naïve T cells decreased at 36 hours (21%),while CD8(+)CD60(+) T cells and CD23(+) cells decreased 48 hrs (33, 74%, respectively) post reaction. In contrast, numbers of CD16/56(+) NK precursor cells increased (60%) 12 hrs, then decreased (65%) 48 hrs post reaction; other lymphocyte subsets were unaffected. Serum IgM, IgG and IgA were within normal range; however, serum IgE demonstrated a bimodal elevation at 2 hrs (15%) and one week post reaction. Levels of IFN-γ, IL-4, and TNF-α were not detected in serum pre-exposure (<1.0-4.0 pg/mL). However, high levels of IFN-γ (187-319 pg/mL) and TNF-α (549-749 pg/mL) were detected in serum 24-36 hrs and 3.5-24 hrs post reaction, respectively. In contrast, levels of IL-4 were undetected (<1.0 pg/mL) in serum at all time points. CONCLUSIONS: Exposure to the larvae of the sycamore tussock moth, Halysidota harrisii Walsh, 1864 may result in increased cytokine levels and blood CD16/56(+) NK precursor cells.