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Introduction: Haematological malignancies contribute to a significant number (8.2%) among cancer patientsin India. Bursa-Acute Lymphoblastic Leukaemia (B-ALL), ThymusAcute Lymphoblastic Leukaemia (T-ALL) and Acute Myeloid Leukaemia (AML) are the three main types of leukaemias distinguished based on flow cytometry. Expression of Cluster of Differentiation (CD) markers of a lineage distinct to the blast population is termed as aberrant expression (expression of B/T cell markers in AML or myeloid markers in ALL). Role of aberrant marker expression in leukaemias remain an enigma till date. Aberrant expression of antigens may be associated with adverse outcomes. Aim: To study the expression of aberrant markers and their association with the remission status postinduction therapy in ALL and AML. Materials and Methods: A retrospective cross-sectional study done accessing the medical records of Acute Leukaemia patients admitted from 1st January 2019 to 31st December 2019 at Kidwai Memorial Institute of Oncology, Bengaluru. A total of 144 cases were included of which 86 cases were of AML and 58 cases were ALL. ALL was further divided into B-ALL and T-ALL with 40 and 18 cases respectively, 18 cases of T-ALL and 86 cases of AML were included. Demographic and clinicohaematological parameters were recorded. All quantitative variables were described as Mean {Standard deviation(SD)} and all qualitative variables were depicted as number (proportion). Statistical significance assessed by Chi-square and Fischer-Exact test using Statistical Package for the Social Sciences (SPSS) version 22.0. Results: Majority of patients belonged to 16-25 years age group with a male preponderance of 58.3%. Aberrant marker expression was associated with the remission status with a p-value of 0.23 and 0.185 in ALL and AML patients respectively and was statistically not significant. While the Chi-square test when applied to the total cases (both ALL and AML combined) the p-value was 0.03 and statistically significant. Conclusion: Aberrant marker expression might predict poor response to induction therapy in acute leukaemias. However, larger studies are needed to confirm these results

Background: Neuroendocrine carcinoma (NEC) is a rare tumor arising from the diffuse neuroendocrine system. Most of these present in the advanced stage and palliative chemotherapy remains the only option. The prognosis remains poor with the standard chemotherapy regimen of platinum and etoposide (EP) providing modest survival benefit. Methods: The study was done for 3 years at a tertiary cancer center in South India. Patients with a diagnosis of metastatic NEC were analyzed for clinical and pathological characteristics. The treatment outcomes and prognostic factors were evaluated using appropriate statistical test. Results: A total of 114 patients of metastatic NEC satisfied the inclusion criteria and were analyzed. Gastrointestinal including hepatobiliary tract (33%) was the most common site of primary disease followed by lung (26%), genitourinary (15%), head and neck (14%), and unknown primary (9%). On analysis of pattern of metastasis, liver (65%) was the most common site followed by bone (54%) and lung (42%). The median overall survival was 11 months with a statistically significant difference between pulmonary and extrapulmonary disease (8 vs. 13 months; P = 0.003). Ki67% value was strongly associated with prognosis (hazard ratio 0.517, 95% confidence interval; 0.318–0.840, P = 0.008) whereas age, sex, and lactate dehydrogenase level did not show any relation with survival. Conclusion: The outcome of advanced NEC with standard chemotherapy remains poor. Larger studies with other therapeutic and novel agents are warranted to improve the treatment outcomes.

Background: T-cell prolymphocytic leukemia (T-PLL) is a rare lymphoid malignancy with dismal prognosis. Most patients have increased lymphocyte count (>1,00,000/dL) and widespread disease at presentation. Despite high response rate seen with alemtuzumab, the disease relapse is inevitable. Materials and Methods: This was a retrospective observational study done at a tertiary cancer center in South India. All patients diagnosed with T-PLL from August 2010 to July 2015 were studied for the clinical characteristics, pathological findings and treatment outcomes. Results: Seven patients were diagnosed as T-PLL over a period of 5 years. The median age at diagnosis was 51 years. In the present series, 6 patients (86%) had splenomegaly and 3 had hepatomegaly (43%). Generalized lymphadenopathy was seen in 4 (57%) patients at presentation. Skin lesions were seen in 5 (71%) patients, whereas pleural effusion was seen in only one patient (14%). All had elevated total leukocyte count, with more than 1, 00,000/dL in 4 patients. The median survival was 5 months with different chemotherapy (CT) regimens (5 patients treated with CT and 2 received best supportive care). Conclusion:T-PLL is a rare disease with no definite treatment guidelines. At present, the best outcomes are achieved if treatment with alemtuzumab is followed by stem cell transplant, but the disease invariably relapses. Countries where affordability remains a big challenge, the best approach needs to be defined beyond the monoclonal antibodies and transplant.

Soft tissue sarcomas (STS) are a diverse group of rare solid tumors of mesenchymal cell origin with distinct clinical and pathological features. They account for less than 1% of all adult malignancies and 15% of pediatric neoplasms. They include over hundreds of different histological subtypes. Many of these subtypes can occur at any age and are not confined to a specific site. Each subtype displays variable clinical behavior. Low incidence, variable presentation, behavior, and long-term outcomes further make it challenging to treat. There are multiple ongoing trials that focus on the anatomic site and histologic subtype to tailor the treatment. Further rarity of each histotype is a major barrier to recruit patients to randomized controlled trials. A multidisciplinary approach is mandatory in all cases of soft tissue sarcomas. The purpose of this review is to thoroughly understand the existing literature on history, incidence, epidemiology, etiology, histology, pathogenesis, diagnostic modalities, prognosis, management, and post treatment surveillance of STS. Uterine sarcomas, gastrointestinal stromal tumors (GIST), and pediatric sarcomas are not included here. It briefly highlights various molecular aberrations, changes in staging as per the American Joint Committee on Cancer (AJCC) 8, drugs that are used off-label in specific subtypes of sarcoma along with the recent advances. The classification of STS is undergoing continuous evolution. A wide variety of subtypes can only be diagnosed accurately with sophisticated molecular diagnostic tests and with the involvement of expert geneticists and pathologists to interpret it. There is no clarity on tailoring the treatment of STS to date. There is always a question on how best we can incorporate chemotherapy and radiotherapy along with surgery as a part of multimodality treatment. The heterogeneity of STS has hindered the development of robust, evidence-based treatment strategies, and our therapeutic approach is neither histology-specific nor widely standardized. Increased knowledge about sarcoma biology could help to discover new and more effective treatment strategies and help overcome the therapeutic challenge imposed by this deadly disease. Continued collaboration among various sarcoma centers globally will be of prime importance to optimize STS management. This will allow studies to be both sufficiently large and reasonably focused to generate evidence that is clinically meaningful in specific STS patient populations. [ABSTRACT FROM AUTHOR]

This multicenter, double-blind, randomized study compared the efficacy, pharmacokinetics (PKs)/pharmacodynamics (PDs), safety and immunogenicity profile of RTXM83 vs. reference rituximab (R-rituximab), both with CHOP, as first-line treatment of diffuse large B-cell lymphoma (DLBCL). A total of 272 patients <65 years of age, with good prognosis (136 per arm) were randomized (1:1) to receive six cycles of either RTXM83 or R-rituximab. The primary efficacy endpoint was achieved (overall response rate of 83.6% for RTXM83 and 82.9% for R-rituximab) with a difference 0.7% between arms (95%CI: [–8.77% to 10.17%]) fulfilling the predefined non-inferiority margin (–13%). Similar number of patients reported at least one adverse event (AE) (131 per arm) or one serious AE (47 with RTXM83 and 45 with R-rituximab). Anti-drug antibody development was comparable between the arms. PK/PD secondary endpoint results support similarity between the compounds. RTXM83 exhibits non-inferior efficacy and similar safety/immunogenicity to R-rituximab, being an accessible alternative for the treatment of patients with previously untreated DLBCL. [ABSTRACT FROM AUTHOR]

BACKGROUND: Thymic epithelial tumors (TET) are the most common tumors of the anterior mediastinum. Patients with advanced/metastatic disease are usually treated with palliative chemotherapy (CT). Unfortunately, even though various palliative CT regimens have been used for long time, there is a real scarcity of published Indian data regarding the experience of palliative CT in metastatic TET (mTET). MATERIALS AND METHODS: This is a retrospective analysis of mTET patients treated between January 2010 and September 2017. Patients who received at least three cycles of first-line palliative CT were included for analysis of response rates, toxicity, and survival and prognostic factors. RESULTS: Of the 49 mTET patients, 27 (55.1%) were males. The median age at diagnosis was 52 years (range: 25-65). Eighteen patients (36.7%) had Masaoka Stage IVa disease, and the rest of the patients had IVb disease. The most common site of metastasis was pleuropericardium (n = 18), followed by lungs (n = 16) and lymph nodes (n = 9). The median progression-free survival and overall survival (OS) were 11.2 months (95% confidence interval [CI], 8.7-13.6) and 20.2 months (95% CI, 17.1-22.8), respectively, for the whole cohort (n = 49). The median OS of patients with Stage IVa disease was significantly better than that of the patients with Stage IVb disease (log-rank P = 0.000). Moreover, the "responders" to first-line CT had a significantly better median OS than the "nonresponders" (log-rank P = 0.000). Various first-line palliative CT regimens were well tolerated in our patients. CONCLUSION: Adriamycin Cisplatin Vincristine Cyclophosphamide (ADOC), Cyclophosphamide Adriamycin Cisplatin, and paclitaxel + carboplatin all are viable first-line palliative CT options for mTET and showed a comparable survival in Indian patients. The present study suggested that "responders" to first-line CT and those with Stage IVa disease might have a better survival than "nonresponders" and those with Stage IVb disease, respectively. [ABSTRACT FROM AUTHOR]