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Rhabdoid tumor commonly occurs in the kidney and has an aggressive clinical course with high mortality. Central nervous system is the most common extrarenal site. Extrarenal extracranial rhabdoid tumor (EERT) is rare. EERT usually presents in childhood, and presentation in adulthood is extremely rare. This tumor is often difficult to diagnose, and there is no established standard of care due to the paucity of cases. We herein report a case of extrarenal rhabdoid tumor of the pelvis in a young adult and discuss the presentation and possible treatment options of this rare tumor. [ABSTRACT FROM AUTHOR]

Introduction: The treatment of primary CNS lymphoma(PCNSL) comprises high dose Methotrexate(HDMTX) based chemotherapy followed by whole brain radiotherapy(WBRT). The major drawback of this treatment approach is long term neurotoxicity that may lead to poor quality of life, dementia and death. We intended to assess the feasibility of response adapted WBRT after HDMTX based chemotherapy in patients with PCNSL in the Indian setting. Materials and Methods: We screened 32 patients and enrolled 22 eligible patients with PCNSL (age 18-80 years, ECOG PS 0-3, HIV seronegative, biopsy proven PCNSL, no significant end-organ dysfunction) attending our institute from 2015 to 2017 in a prospective phase II trial. The patients underwent 5 two-weekly cycles of MPV induction chemotherapy with Methotrexate 3.5g/ m2 IV D1 with Leucovorin rescue for 3 days, Vincristine 1.4mg/ m2(capped at 2mg) IV D1, Procarbazine 100mg/m2 P.O. D1-7 in odd number cycles. Rituximab 375mg/m2 IV D1 q2weeks was added in 14 patients as per patient-preference and affordability. Patients with complete response(CR) to induction chemotherapy were given reduced dose WBRT 23.4Gy/13fractions/2.5 weeks while those with partial response(PR), stable or progressive disease(SD or PD) were given standard dose WBRT 45Gy/25fractions/5 weeks. Thereafter 2 cycles of consolidation chemotherapy with Cytarabine 3g/m2/day, IV D1 and D2 were given 1 month apart. The primary endpoints of the study were assessment of response rate and progression free survival (PFS). The secondary endpoints of the study were assessment of overall survival (OS), toxicity profile of treatment, molecular subtype of lymphoma, EBV status (by immunohistochemistry for EBV LMP-1) and serial changes in quality of life (EORTC-QLQ-C30 and BN 20 module) and neuropsychological parameters. Results: The median age at diagnosis was 51.5 years (range: 31-67 years) and the male: female ratio was 13:9. The ECOG PS was 3, 2 and 1 in 13(59.09%), 5(22.73%) and 4(18.18%) patients respectively. Out of 19 patients who completed HDMTX based induction chemotherapy, 10 (52.63%) patients achieved CR, 8 (42.11%) patients had PR and 1 patient had PD. Two patients on RMPV regimen died due to chemotherapy related toxicities. Induction chemotherapy was otherwise well tolerated with severe (grade3/4) toxicities being mostly haematological- anaemia in 1 patient, neutropenia in 8(36.36%) patients and thrombocytopenia in 1 patient. Nine patients received reduced dose and 9 received standard dose WBRT. In our study, WBRT was excellently tolerated with no reported grade 3/4 toxicity. Consolidation chemotherapy with high dose Cytarabine was given in 15(68.18%) patients. Grade3/4 neutropenia was observed in 3(20%) patients during consolidation chemotherapy. After a median follow-up period of 11.25 months (mean 12.41 months), 4 patients had disease progression and 8 patients had died, the causes being disease progression in 2, chemotherapy related toxicity in 2 and non-cancer related in 3 patients. The estimated median OS was 19 months. The median PFS had not been reached. The actuarial rates of PFS were 94.1% and 50.2%, disease free survival were 86.4% and 61.4% and OS were 68.2% and 48.5%, respectively at 1 and 2 years. Three patients in reduced dose WBRT arm had recurrence and 2 of them died of progressive disease, whereas there was no recurrence or disease related death in standard dose WBRT arm. On univariate analysis of OS, use of RT (p value<0.0001), use of consolidation Ara-C (p value 0.026) and negative CSF cytology (p value 0.0076) led to significantly improved outcome. On multivariate analysis of OS, only CSF cytology retained prognostic significance with p value of 0.021 and hazard ratio (HR) of 6.71. On univariate analysis of PFS, age?50 years and use of standard dose WBRT (45Gy) led to significantly improved outcome (p value 0.03 and 0.02 respectively). The overall response rates to induction chemotherapy with and without Rituximab were not significantly different (90.9% versus 100%; p value 0.409). There was no significant difference in treatment outcome according to the molecular subtype of PCNSL (germinal centre and activated B cell DLBCL in 3 and 13 patients respectively). In our study immunohistochemistry for EBV LMP-1 was negative in all assessed specimens (N=19). Serial neuropsychological assessments revealed marked improvement in general cognition and other domains (e.g. verbal fluency and motor speed) after induction chemotherapy, which persisted for 6 months after completion of primary treatment and then stabilised. The mean EORTC Global Health Status/Qol score declined from 58.3 at baseline to 41.67 after induction chemotherapy and then increased to 66.67 at 6 and 12 months after completion of treatment (p value 0.748). . Conclusion: In patients with newly diagnosed PCNSL, reduced dose WBRT after complete response to HDMTX based chemotherapy may lead to suboptimal clinical outcome due to higher risk of recurrence, progression and early death. Addition of Rituximab to MPV regimen, does not enhance the response rate or significantly impact the survival outcome but increases the acute toxicity. Whole brain radiotherapy in both reduced and standard dose, does not appear to have short term neuropsychological and quality of life detriment. However longer follow up is required to make definitive conclusions. [ABSTRACT FROM AUTHOR]

Background: Liquid biopsy is emerging as a non-invasive tool, providing a personalized snapshot of a primary and metastatic tumour. It aids in detecting early metastasis, recurrence or resistance to the disease. We aimed to assess the role of circulating tumour cells (CTCs) as a predictive biomarker in recurrent/metastatic head and neck cancer (head and neck squamous cell carcinoma (HNSCC)). Methodology: Thirty-five patients receiving palliative chemotherapy underwent blood sampling [2 mL in Ethylenediaminetetraacetic acid (EDTA) vial] at baseline and at 3 months intervals. The CTCs were isolated and evaluated using anti-epithelial cell adhesion molecule antibody-based enrichment using the OncoDiscover platform. Results: CTCs isolated from 80% of patients (n = 28) showed the sensitivity of cell detection at the baseline and 3 months intervals. The median CTC count was 1/1.5 mL of blood and the concordance with clinic-radiological outcomes was 51.4%. The median CTC count (1 (range:0-4) to 0 (range:0-1)) declined at 3 months in responders, while the non-responders had an increase in levels (0 (range :0-2) to 1 (range :0-3)). Although CTCs positively correlated with progression-free survival (PFS) and overall survival (OS), the association of CTCs did not show a significant difference with these parameters (PFS: 6 months versus 4 months; hazard ratio: 0.68; 95% confidence interval (CI): 0.29-1.58, p = 0.323; OS: 10 months versus 8 months; hazard ratio: 0.54; 95% (CI):0.18-1.57 p = 0.216) between CTC positive and CTC negative patients at 3 months. Conclusion: This study highlights the utility of CTC as a disease progression-monitoring tool in recurrent HNSCC patients. Our findings suggest the potential clinical utility of CTC and the need for exploration in upfront settings of the disease as well (NCT: CTRL/2020/02/023378). [ABSTRACT FROM AUTHOR]

A letter to the editor is presented concerning the concurrent use of phenytoin and radiotherapy in a patient with central nervous system (CNS)-metastatic breast cancer.

The study aimed to assess the patterns of care and treatment outcomes in patients with sinonasal malignancy (SNM), treated at our institute from January 2018- March 2023. Patients with biopsy proven SNM were included in this retrospective analysis. Progression free survival (PFS) was defined as the time interval from the date of diagnosis to the date of disease progression (locally, regionally, or distant). Overall survival (OS) was defined from the date of diagnosis to the last follow-up or death. Univariate Cox regression analysis was done to evaluate the relationship between treatment factors and survival outcomes. Time to event data was evaluated using Kaplan. -Meier method. A total of 93 patients with diagnosed SNM between 2018-2023 were included in the study. The median age at diagnosis was 42.4 years (IQR= 23.3- 55.8). 81.7% of the patients were male. Maxillary sinus was the most common site involved (57.1%) followed by nasal cavity (38.9%) and ethmoid sinus (12%). The most common histological types were carcinoma (50.5%) followed by sarcoma (19.4%), esthesioneuroblastoma (7.5%), and lymphoma (7.5%). Among carcinoma, squamous cell carcinoma (61.6%) was the most common subtype followed by adenoid cystic carcinoma. The mean tumor size was 5.6 centimetres (range 1.6- 12.2). 20.4% of patients had regional lymph nodal involvement and 5.4% of patients had metastatic disease at presentation. Intent of treatment was curative in 76.3% of patients. Primary surgical treatment was done in 45.2% patients, of which 45% of patients received adjuvant radiotherapy and 37.5% of patients received adjuvant chemoradiation. Radical chemoradiotherapy was administered in 28.8% of patients. All patients received conformal IMRT/VMAT radiotherapy to a dose of 60Gy -66Gy in 30-33 fractions. Median follow-up was 27.9 months (IQR: 8.8-46.9). In the curative treatment group, the median OS was 35.6 months (IQR: 16.2- 54.3) and the median PFS was 29.5 months (IQR: 12.8- 51.7). In univariate analysis, patients who did not complete the planned treatment were associated with decreased OS (p-value= 0.03; HR= 5.57; 95%CI= 1.21- 25.57), advanced-stage disease was associated with inferior PFS (p- value= 0.026; HR= 9.81; 95%CI=1.29- 74.44). In multivariate analysis, the advanced stage was associated with worse PFS (p-value= 0.03; HR= 10.18; 95%CI=1.32- 78.4). The disease progressed locally in 20.5%, regionally in 5.4%, and distant metastasis in 9.7% of patients' post-radical treatment. Lung was the most common site of metastasis. Out of 23.7% of patients who received palliative treatment, 40.9% of patients received both palliative radiotherapy and chemotherapy, 31.8% received palliative radiotherapy alone and 0.4% received palliative chemotherapy alone. The median OS was 13.5 months (IQR: 6.4- 19.7) and the median PFS was 5.3 months (IQR: 1.6- 12.8) in the palliative group. Sinonasal tumors are a complex and diverse group of cancers. The advanced stage of presentation, histologic diversity, and anatomical proximity to critical neurovascular structures make the treatment of SNM challenging. The median OS was 35.6 months and the median PFS was 29.5 months, with inferior PFS in advanced-stage disease. Early diagnosis and multi-disciplinary approach are imperative in the management of SNM. [ABSTRACT FROM AUTHOR]

Background & objectives: Survival of patients with multiple myeloma (MM) has improved in the past two decades following use of novel agents and autologous stem cell transplantation. To determine predictors of long-term outcome, data of MM patients who underwent autologous stem cell transplantation (ASCT) at a tertiary care centre in north India were retrospectively analyzed. Methods: Between 1995 and 2016, 349 MM patients underwent ASCT. Patients' median age was 52 yr, ranging from 29 to 68 yr, 68.2 per cent were males. Thirty three per cent patients had international staging system (ISS) Stage III and 68.5 per cent had received novel agents-based induction. High-dose melphalan (200 mg/m2) was used for conditioning; patients with renal insufficiency (estimated glomerular filtration rate <40 ml/min) received melphalan 140-150 mg/m2. Results: Post-transplant, 317 of 349 (90.8%) patients responded; complete [complete response (CR)] −213 (61%)], very good partial response (VGPR) −62 (17.8%) and PR in 42 (12%)]. Induction with novel agents, pre-transplant chemosensitive disease, transplant in first remission and serum albumin (≥3.5 g/dl) were predictors of significant response. At a median follow up of 73 months, median overall survival (OS) was 90 months [95% confidence interval (CI) 70.8-109.2], and progression-free survival (PFS) was 41 months (95% CI 33.0-49.0). On multivariate analysis, achievement of CR post-transplant, transplant in first remission, ISS Stages I and II (vs. III), absence of extramedullary disease and serum albumin ≥3.5 g/dl were predictors of prolonged OS. For PFS, achievement of post-transplant CR and transplant in first remission were predictors of superior outcome. Interpretation & conclusions: Treatment with novel agents, achievement of complete remission post-transplant, ISS Stages I and II, absence of extramedullary disease and transplant in first remission were predictors of long-term survival for patients with MM. [ABSTRACT FROM AUTHOR]

Introduction: Children ≤5 years of age with Ewing's sarcoma (ES) possibly have a distinct disease biology, data on which are scarce. We evaluated clinical features, outcomes, and prognostic factors of ES among children with age ≤5 years., Methods: Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups., Results: Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, p = .018) and EFS (HR = 2.47, p = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, p = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (p < .001) and lesser baseline metastases (p = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, p = .29) or localized cohort (HR = 1.77, p = .09)., Conclusions: Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders., (© 2024 Wiley Periodicals LLC.)

Background: Head and neck squamous cell carcinoma (HNSCC) is a huge burden in India with the majority of patients presenting in advanced unresectable stages. Innovative, low-cost but efficacious regimens that can be easily administered in the outpatient setting are the need of the hour. We envisaged assessing whether a readily available triplet therapy of erlotinib + methotrexate + 5-fluorouracil (EMF) is efficacious in terms of extending life and maintaining the quality of life in such patients., Patients and Methods: This was a single-arm, phase II, investigator-initiated interventional study. Thirty-five platinum-resistant/refractory patients of HNSCC were treated with a combination of erlotinib 150 mg, methotrexate 40 mg/m2 (d1, d8) and 5-fluorouracil 500 mg/m2 (d1, d8) every 28 days till progression or unacceptable toxicities. The primary endpoint was overall response rates (ORRs) at 3 months; additional endpoints were disease control rate (DCR) at 3 months, overall survival (OS) and progression-free survival (PFS), safety and patient-reported quality of life., Results: The ORR and DCR at 3 months were 45.7% and 68.5%, respectively. The PFS was 5 months (95% confidence interval (95% CI): 3.9-6 months) and the OS was 9 months (95% CI: 7.4-10.5 months). The 3- and 6-month PFS rates were 86% ± 6% and 45% ± 9%, respectively, while the OS rates at 3 and 6 months were 91% ± 5% and 68% ± 8%, respectively. Rash, mucositis and fatigue were common adverse events occurring in 23 (65%), 14 (40%) and 9 (25.7%), respectively. The most common grade 3 events seen were rash in 5 (14.2%) and diarrhoea in 2 (5.7%). Clinically significant improvement from baseline was seen in many domains of Quality of Life Core Questionnaire and Quality of Life Head and Neck Module., Conclusions: The triplet regimen of EMF is a feasible and safe therapeutic option in patients with platinum-resistant/refractory HNSCC. It has demonstrated favourable response rates and improvement in quality of life; however, a randomised phase III study would add more robust value (NCT: CTRI/2020/02/023378)., Competing Interests: The authors have no conflicts of interest., (© the authors; licensee ecancermedicalscience.)

Introduction: Prognostic scores in Ewing sarcoma including baseline clinical and laboratory characteristics are necessary for pre-treatment risk stratification. In this study, we formulated and validated a prognostic model for baseline risk categorization in Ewing sarcoma., Materials and Methods: A retrospective single-institutional study was conducted on Ewing sarcoma patients treated uniformly between January 2003 and December 2018. Baseline clinical/pathological characteristics and survival outcomes were noted from medical records. The cohort was randomised into a derivation and validation cohort. A prognostic score was formulated by including independent prognostic factors from the derivation cohort by multivariable analysis. The prognostic model was validated in the validation cohort along with estimation of its predictive ability., Results: A total of 860 patients were included with 40.3% having baseline metastases. Tumor diameter >5 cm (HR 2.04; P<0.001; score 2), baseline metastases (HR 2.33; P<0.001, score 2), and total leucocyte count >11000/mm 3 (HR 1.44; P=0.015; score 1) were independent predictors of overall survival in derivation cohort and included for prognostic score calculation. Patients were categorized into low (score 0), intermediate (score 1-3) and high-risk (score 4-5) groups. Harrell's c-indexes of the model were 0.625, 0.622 and 0.624 in the derivation, validation and whole cohort respectively. The timed AUC of ROC of the prognostic score-group for 5-year survival was 0.72, 0.71 and 0.73 in the derivation, validation and whole cohort respectively., Conclusions: We have formulated and validated a prognostic score for Ewing sarcoma incorporating baseline clinical and laboratory parameters, with fair predictive ability for risk stratification and facilitating risk-adapted personalized therapy., Competing Interests: None., (AJTR Copyright © 2022.)

Aim: Primary pineal melanoma is a rare tumor. We herein review the histogenesis, pathology, radiology and therapeutic options of this rare tumor., Material and Methods: We conducted a PUBMED search using a combination of keywords such as "primary pineal melanoma", "CNS melanoma", and "pineal tumor" and identified 16 cases of primary pineal melanoma. Clinical features, pathologic characteristics and treatment details of these patients were noted from respective case reports. We also describe a case of a 45-year-old Indian woman with primary pineal melanoma treated with a combination of surgery and post-op radiation., Results: The median age at presentation is 50 years. Median duration of symptoms is 6 weeks. Common symptoms at presentation include headache (58.8%), personality changes (41.2%), gait disturbance (35.3%) and Parinaud's syndrome (29.4%). Surgery, radiotherapy and chemotherapy have been used in 29.4%, 47.1% and 23.5% of patients respectively. Median overall survival is 56 weeks. Leptomeningeal dissemination and ventricular ependymal spread were noted in 70.6% and 35.3% patients, respectively., Conclusion: Combined modality treatment comprising maximal safe surgery and post-operative radiation should be preferred in patients with localized pineal melanoma without leptomeningeal dissemination. Taking a cue from other subsites of melanoma, chemotherapy can perhaps be deferred until recurrence.