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The diagnosis of hereditary or familial breast cancers influences the locoregional approach to breast cancer, with most patients undergoing mastectomy to avoid or minimize the use of adjuvant radiation therapy. We evaluated the current literature about known high- and moderate-penetrance genes and studied their impact on local control, toxicities, and contralateral breast cancers after adjuvant radiation therapy. The aim is to encourage the safe use of adjuvant radiation therapy when indicated in concordance with the updated guidelines.

Introduction The standard of care in locally advanced NSCLC is concurrent chemo- radiation. This audit aims to document different treatment approaches offered in real world scenario and the resultant outcomes. Methodology Institute medical records during 2014–2018 were retrieved. Demographic and clinical data, different treatment offered with associated outcomes and the side effects were analysed. Various prognostic factors, disease‐free survival (DFS), and overall survival (OS) were evaluated. Results 396 patients were eligible for the analysis of which 278 patients were treated with curative intent. The elderly population constituted 53% and 85.3% were male. Among allpatients, 86.7% were NSCLC and 13.3% were SCLC and 25.1% of them presented in locally advanced stage. The proportion of squamous cell and adenocarcinoma are 55.8% and 31.3% respectively. Although 71.6% of curative patients were given neoadjuvant therapy, only 53.2% could complete the scheduled number of cycles. Only 31.6% could actually receive chemo- radiotherapy. Surgical excision was done in 19.4% of patients. The compliance to concurrent and sequential chemo- radiation were 52.1% and 70.7% respectively. The median overall survival of the curative intent cohort is 17.5 months (9.3- 30.7 IQR) and the progression free survival is 9 months (4.4- 16.5 IQR). The median OS of the palliative intent cohort is 9.1 months (3.1- 15.7 IQR) and the PFS is 6.5 months (4.2- 12.7 IQR). Although, definitive chemo- radiation had lower median PFS, it provided significantly superior long-term survival outcome when compared to surgery with adjunct therapy. Median PFS, median OS, OS at 1.5 and 2- years were not significantly different. No significant differences in survival outcomes between concurrent versus sequential chemo- radiation approaches. Sequential CTRT, surgery with adjunct therapy had significantly better median PFS. Smoking, reception of intended treatment, and definitive chemo- radiation were significant prognostic factors in multivariate analysis. Conclusion Compared to global figures the outcome in the present study observed poorer outcome in terms of median PFS associated with neoadjuvant chemotherapy and concurrent chemo- radiation. Surgery may not lead to better outcomes in locally advanced NSCLC patients and the standard of care still remains definitive chemo- radiation.

IDH wild-type (wt) grade 2/3 astrocytomas are a heterogenous group of tumors with disparate clinical and molecular profiles. cIMPACT-NOW recommendations incorporated in the new 2021 World Health Organization (WHO) Classification of Central Nervous System (CNS) Tumors urge minimal molecular criteria to identify a subset that has an aggressive clinical course similar to IDH -wt glioblastomas (GBMs). This paper describes the use of a panel of molecular markers to reclassify IDH -wt grade 2/3 diffuse astrocytic gliomas (DAGs) and study median overall survival concerning for to IDH -wt GBMs in the Indian cohort. IDH -wt astrocytic gliomas (grades 2, 3, and 4) confirmed by IDHR132H immunohistochemistry and IDH1/2 gene sequencing, 1p/19q non-codeleted with no H3F3A mutations were included. TERT promoter mutation by Sanger sequencing, epidermal growth factor receptor amplification, and whole chromosome 7 gain and chromosome 10 loss by fluorescence in situ hybridization was assessed and findings correlated with clinical and demographic profiles. The molecular profile of 53 IDH -wt DAGs (grade 2: 31, grade 3: 22) was analyzed. Eleven cases (grade 2: 8, grade 3: 3) (20.75%) were reclassified as IDH -wt GBMs, WHO grade 4 ( TERT promoter mutation in 17%, epidermal growth factor receptor amplification in 5.5%, and whole chromosome 7 gain and chromosome 10 loss in 2%). Molecular GBMs were predominantly frontal (54.5%) with a mean age of 36 years and median overall survival equivalent to IDH -wt GBMs (18 vs. 19 mo; P =0.235). Among grade 2/3 DAGs not harboring these alterations, significantly better survival was observed for grade 2 versus grade 3 DAGs (25 vs. 16 mo; P =0.002). Through the incorporation of a panel of molecular markers, a subset of IDH -wt grade 2 DAGs can be stratified into molecular grade 4 tumors with prognostic and therapeutic implications. However, IDH -wt grade 3 DAGs behave like GBMs irrespective of molecular profile.

Background Laryngeal sarcoma is rare. We performed a systematic review and individual patient analysis to evaluate the patterns of care, prognostic factors, and role of radiotherapy in laryngeal soft tissue sarcoma. Methods A systematic search on PubMed and Google scholar was done. An individual patient data analysis was done. Results Of the 300 cases of laryngeal sarcoma, 80% underwent surgery. 44% underwent larynx preservation surgery and 25% received radiotherapy with surgery. Median progression free survival (PFS) was 48 months and overall survival (OS) of 224 months for the entire cohort. Patients with large primary, cartilage invasion, and positive margins had numerically worse PFS. Cartilage invasion and primary tumor size >3 cm were the most common risk factors for adjuvant radiation therapy. Patients receiving radiotherapy were not associated with better survival. Conclusion Laryngeal sarcoma associated with a good survival. Larynx preservation surgery is feasible in nearly half patients. Adjuvant radiotherapy may be warranted in patients poor prognostic factors.

Radiation therapy is considered the standard treatment for early glottic cancers; and recent trials have evaluated the role of hypofractionation with mixed results. We conducted this systematic review and meta-analysis to assess the impact of hypofractionation in early glottic cancers. We performed a comprehensive search of the PubMed, Embase and Google scholar to look for studies which have evaluated the role of hypofractionation in early glottic cancers. Only prospective trials were included in the present analysis. RevMan software (Cochrane Collaboration’s Information Management System) was used for the meta-analysis. The analysis included a total of five studies and 1153 patients. Hypofractionation was found to significantly improve local control rates with an Odds ratio of 0.55 [95% CI 0.13–0.85]. The voice preservation rates with hypofractionation ranged from 86 to 94%. No significant improvement in overall survival was noted with the used of hypofractionation [hazard ratio 1.09, 95% CI 0.69–1.71]. There was an increased incidence of grade 2 or higher acute mucositis toxicity with use of hypofractionation [Odds ratio 1.54, 95% CI 1.12–2.11]. The incidence of acute skin toxicity was not increased with use of hypofractionation. Late toxicity was very low and not increased with use of hypofractionation. Moderate hypofractionation as compared to conventional fractionation, in laryngeal cancer, is associated with significantly improved local control without impact on overall survival. The use of hypofractionation is associated with an increased incidence of acute mucositis though incidence of long-term toxicity was not significantly increased. Hence, moderate-dose hypo-fractionation should be considered as the new standard of care in early laryngeal cancer.

Rosette forming glioneural tumors (RGNT) are a rare type of low-grade brain tumor included in 2007 in WHO classification. Given the benign nature of the disease, a complete surgical excision has been considered optimum. However, a handful of cases have reported the locally aggressive nature of RGNT. In addition, radiation may also be considered for a tumor located in areas where surgical excision is difficult. We present a similar case, where surgical risk was weighed against resection and we treated the patient with conformal radiation.

Background: Pancreatic cancer is a devastating disease with a 5-year survival rate of 5–10%. Radiation is commonly used in neoadjuvant and adjuvant settings to improve local control. Studies have shown that circulating lymphocyte count depletion after radiation has been associated with poor tumor control and inferior overall survival (OS) outcomes. Method: To better understand the impact of radiation-associated lymphopenia in pancreatic cancer, the authors undertook this systematic review and meta-analysis of clinical studies that have reported radiation-related lymphopenia in pancreatic cancer. Results: A systematic methodology search of PubMed, Embase and the Cochrane Library resulted in 2969 abstracts. Nine studies fulfilled the inclusion criteria. Six studies reported on outcomes in patients undergoing definitive chemoradiation and three studies comparing outcomes in stereotactic body radiotherapy versus definitive chemoradiation. The patients with severe lymphopenia were at increased risk of death with a pooled hazard ratio of 2.33 (95% CI: 1.79, 3.03; I2: 36%; p 2: 95%; p 2: 99%; p

Aim: Glioblastoma (GBM) is one of the most aggressive neoplasms of the central nervous system with dismal survival. In recent years, different variants of GBM have been described in the literature. GBM with areas of neuroectodermal differentiation (GBM-PNET) is a relatively new entity in GBM. Presence of the neuroectodermal component increases the propensity of systemic dissemination as with other intracranial primitive neuroectodermal tumors (PNET). The optimal treatment for these patients remains a controversy, with authors reporting local radiotherapy to craniospinal irradiation and chemotherapy. We intend to analyze the pattern of care for GBM with neuroectodermal component. Materials and Methods: We retrieved data of four patients with GBM-PNET treated in our institute; data were also retrieved from published series to derive treatment and outcome results. Results: In this series, we report the outcome of a series of four patients of GBM-PNET treated with adjuvant radiotherapy and temozolomide. All but one patient underwent gross total resection of the tumor. Adjuvant hypofractionated radiation with concurrent and adjuvant temozolomide was used in all cases. The median follow-up was 12.9 months in the present series. One patient experienced local recurrence 18 months after the treatment. A review of published literature on GBM-PNET was done; studies with details of patient outcome were used for an independent analysis. Twenty-three patients were identified, and the pooled analysis revealed a median progression free and overall survival of 10 and 25, months respectively. Extent of surgery, local radiation vs. craniospinal irradiation, and age at presentation had no impact on the survival. Conclusion: GBM PNET is a new entity with only few cases reported so far. Clinical behavior and treatment outcome of these tumors are not different from conventional GBM. However, these patients are at higher risk of CSF dissemination. Hence, an individualized treatment approach is best suited.

Introduction Myoepithelial carcinoma (MEC) is an extremely rare low grade salivary gland neoplasm [1–4]. A surgical resection is considered as corner stone of therapy. Role of adjuvant therapy is not clear. Methodology We performed systematic review and individual patient data analysis of 691 patients to look into the impact of adjuvant therapy and different prognostic variable for MEC. Results Data of 691 individual patients were retrieved from 340 publications. Median age of presentation was 56 years (Range: 0–103 years) with a trend of increasing incidence for increase in age. Major salivary glands (36.4%) were the commonest sub-site followed by minor salivary glands, skin and soft tissue, and breast. Median PFS and OS of entire cohort was 48 months (95% CI: 30–65 months) and 167 months (95% CI: 82–251 months). In univariate analysis A R0 resection was associated with significantly better PFS and OS. Median PFS and OS were significantly worse for patients with tumour size >5 cm compared to smaller tumours and for patients with a mitotic index >10/10 high power field (hpf) compared to lower mitotic index. Adjuvant radiation was found to reduce loco-regional recurrence. Adjuvant radiation and chemotherapy both were associated with negative impact on survival in univariate analysis. This negative impact on survival was lost in multivariate analysis. Conclusion MEC appears to be a low grade malignancy with good survival outcome. A R0 resection should be the standard of care. Adjuvant radiation should be considered for patients with adverse risk features to improve loco-regional disease control.

Objectives Xanthoastrocytoma (XA) is a low-grade glial tumor seen in young adults and there is lack of robust data on treatment of this rare tumor. In this systematic review and individual patient's data analysis, we aimed to look into the demography, pattern of care, survival outcomes, and prognostic factors in patients with both Grade II and III XA. Methods A comprehensive search was conducted with the Medical Subject Heading terms: “Xanthoastrocytoma; Pleomorphic Xanthoastrocytoma; Anaplastic Xanthoastrocytoma; Xanthoastrocytoma AND treatment; and Anaplastic Xanthoastrocytoma AND survival” to find all possible publications. Results A total of 325 individual patients from a total of 138 publications pertaining to XA were retrieved. Median age of the entire cohort was 19 years. About 56.1% of the patients underwent a gross total resection (GTR) and 31.4% underwent a subtotal resection. Nearly, 76.6% of the patients had a Grade II tumor and adjuvant radiation was delivered in 27.4% of the patients. Estimated 2- and 5-year progression-free survival (PFS) were 68.5 and 51.2%, respectively. Age, grade, and extent of surgery were significant factors affecting PFS. Estimated 2- and 5-year overall survival (OS) was 88.8 and 78%, respectively. The median OS for Grade II and Grade III tumors were 209 and 49 months, respectively. Age and extent of surgery were significant factors affecting OS. Conclusion XA is a disease of young adults with favorable prognosis. Younger patients (

Malignant tumors of the trachea (MTT) account for 0.01-0.4% of all cancer cases. The rarity of the tumor along with different histologies makes it is a great challenge on how to optimally treat tracheal tumors and most of the available data is from small retrospective data series. We performed a systematic review and individual patient data analysis to evaluate the patterns of care and survival outcomes in patient with MTT.A comprehensive search in Pub Med and Google scholar was done to find all possible publications related to malignant tumors of the trachea. The data on patient demograpphy, treatment, survival and recurrence pattern of individual patient was collected from the published data and was entered in a predesigned proforma. Progression free survival [PFS] and overall survival [OS] was calculated from the date of diagnosis to the date of documented progression and death respectively. Kaplan- Meier method was used for survival analysis and uni-variate analysis was performed using log rank test. SPSS v16 was used for all statistical analysis.733 patients were included in this analysis. The most common histology was adenoid cystic carcinoma (ACC) followed by squamous cell carcinoma (SCC). The gender ratio was 4.43: 1[male: female] in patients with SCC while it was 0.85:1[male: female] in ACC. Smoking and age50 years were associated with worse OS. The estimated median overall survival for entire cohort was 96 months. Survival was significantly better in patients with ACC than in patients with SCC [165 vs. 14 months, p 0.001]. The use of definitive surgery was associated with a significantly better survival of 180 months when compared to 48 months with radiation as local therapy, [p 0.001]. The radiation dose used also affected survival in patients with SCC with a better median OS of 24 months in patients who recieved more than 60 Gy vs 6 months in whom the dose was less than 60 Gy although not statistically significant (p = 0.011).ACC and SCC are the most common MTT. ACC has better prognosis compared to SCC. Surgery seems to provide better outcomes than radiation for ACC and sarcoma. Role of definitive radiotherapy versus surgery in SCC needs to be further studied.

Background The impact of radiation-related lymphopenia on clinical outcomes has been reported in various solid malignancies such as high grade gliomas, head and neck cancers, thoracic malignancies and gastro-intestinal malignancies but its impact is not clearly known in the context of common genito-urinary (GU) malignancies. Methodology To better understand the effect of radiation-associated lymphopenia in prostate and bladder cancer, we undertook this systematic review of clinical studies that have studied radiation-related lymphopenia in GU malignancies. A systematic methodology search of PubMed, Embase, and Cochrane library resulted in 2125 abstracts. Ten studies fulfilled the inclusion criteria which included any prospective, retrospective study or cohort study of prostate, urinary bladder, kidney, ureter, urethra, penile cancer in humans, and radiation should be part of treatment and intent has to be in definitive or adjuvant settings. Finally the study should have data on radiation-related lymphopenia. Results Four studies reported on the cancer-specific outcomes related to the lymphopenia. The incidence of low lymphocyte counts were documented in all the studies. Three studies analyzed the factors associated with the Lymphocyte depletion. Pooled incidence of severe lymphopenia was 29.25% and mild to moderate lymphopenia was 60.75%. Bone marrow volume receiving 40 Gy was associated with the incidence of lymphopenia. Conclusion One-third of the patients suffer from severe lymphopenia after radiation in prostate and bladder cancer. There are no clear data to support the correlation between severe lymphopenia and disease outcomes. Bone marrow dosimetry can affect the incidence and severity of lymphopenia. There is need of prospective datasets to identify the impact of radiation-related lymphopenia in GU malignancies focusing on long-term side effects, recurrence rates, and overall survival.

Background. Tumours of the eyelid are a rare subgroup of neoplasms with varied histology and inherent differences in clinical behaviour. Surgery is the standard of care, and adjuvant radiation therapy (RT) is given in the presence of features suggesting a high risk of local recurrence. The treatment of lymph nodes in the neck is debatable. We reviewed the utility of RT for lymph nodes in the neck in patients with malignant tumours of the eyelid. Methods. We reviewed medical records of all patients with tumours of the eyelid treated at our centre from July 2006 to December 2014 for their demographic, clinical profile, treatment details and outcome. Results. The records of 37 patients were included for analysis, of these 34 underwent surgery and 21 received adjuvant RT. Their median age was 60 (range 30–85) years. Sebaceous cell carcinoma was the most common (50.4%). The median disease-free survival (DFS) was 35 months (95% CI 17.9–52.0). The 1- and 3-year DFS were 82.7% and 45%, respectively. Univariate analysis showed a superior outcome with early stage (T1) tumours (p=0.01), RT dose of ≥60 Gy and those underwent lymph node dissection (p=0.03). The presence of high-risk factors including close or positive margin had an inferior outcome with a trend towards statistical significance (p=0.06). Conclusion. We found a favourable outcome with early T stage, RT dose of ≥60 Gy and lymph node dissection. High-risk histopathological features including close margins and positive lymph nodes merit adjuvant RT including regional lymph nodes.

Aim: Salivary duct carcinoma (SDC) is a rare and aggressive malignancy. The optimal treatment protocols are debated. Methodology: A systematic search and individual patient data analysis of published cases of SDC was performed. SPSS v21 was used for statistical analysis. Results: Data of 857 patients available. Median overall survival (OS) and progression-free survival (PFS) of the entire cohort 42 months and 24 months. Nodal involvement, males, primary size >5 cm, androgen receptor (AR) negativity significantly worse OS. Patients with surgery had a favorable median PFS (p = 0.000) and OS (p = 0.077). Patients with adjuvant radiation had better PFS (30 vs 18 months; p = 0.077). Conclusion: SDC has modest survival. Surgery and adjuvant radiation should be advocated for all patients. AR expression appears prognostic for survival.

Gliomatosis cerebri (GC) is an extremely rare form of infiltrating glioma which involves multiple lobes of the brain. The disease can affect any age groups and is known to have very poor outcomes in terms of survival. Often, we see a discordance between clinical and radiological findings, and the majority of the times it is clinically silent whereas radiologically they appear very extensive. The most common variant described has been the astrocytic type and various mixed phenotypes have also been defined. Due to the rare incidence of the disease, GC has not seen an established evidence-based management options and consensus has not been defined. Surgery is usually not an option considering the infiltrating and extensive nature of the tumor. The role of radiation therapy and chemotherapy has not been well established and the literature consists of conflicting reports on the evidence for their use. Due to rare incidence and the discordance in the treatment options, design of a clinical trial is very difficult to conclusively establish evidence-based guidelines. This chapter aims at providing the highlights of the tumor entity with a focus on the molecular pathology, radiological findings, and treatment options available. In conclusion, GC presents a unique dilemma for the treating oncologist due to the distinct pathology and molecular overlap with other diffuse gliomas.

Choroid plexus tumors (CPT) are a rare disease entity arising from choroid plexus epithelium. They are characterized by papillary and intraventricular growth. There are various grades of the tumor and choroid plexus carcinoma (CPC) is the most aggressive variant. The tumors are most prevalent in the pediatric age group and the adult variants are extremely rare. In children, the CPC occurs typically in the first 5 years of life and they represent around 1–4% of all pediatric brain tumors. Surgery in the form of gross total resection remains the mainstay of treatment, however, the role of adjuvant therapy in the form of radiotherapy or chemotherapy is not well defined due to lack of consensus. The rare incidence and the discordance in the treatment options, it becomes difficult to design clinical trials that can establish the role of adjuvant therapy. In this chapter, we aim to highlight the basic principles associated with the tumor entity with a focus on the molecular pathology, radiological findings, and treatment options available.

Rare/Uncommon brain tumors like ganglioglioma, neurocytoma, plasmacytoma, rosette forming glioneural tumors, etc. are difficult to manage due to absence of good evidence-based guidelines. The role of complete resection, adjuvant radiotherapy as well as recurrence patterns are not well described. This chapter will summarize salient features of such tumors along with evidence-based management guidelines.

Radiation induced brain injury can be acute, early delayed, and late effects. The acute and early delayed effects are usually reversible while radiation necrosis may be irreversible. The chapter will summarize the symptoms, evaluation, and treatment of radiation induced brain injury.

Primary CNS lymphoma is a rare CNS tumor. It occurs mainly in elderly and has a 5-year survival of 35%. High-dose methotrexate-based chemotherapy forms the backbone of treatment. This is followed by consolidation whole-brain radiotherapy or autologous stem cell transplant. No standard treatment exists for recurrent disease.

Tumors of the pineal region are uncommon and deep-seated and constitute around 10% of intracranial neoplasm found in children. These tumors are about ten times more common in children than adults; however, the overall incidence is very rare. The tumors are diverse in cell origin and germ cell tumors are the most common variant found in the pineal region. They present with features related to obstructed hydrocephalus and signs and symptoms suggestive of brainstem compression. The tumors have been classified into various types based on various biomarkers and have been explained in detail in this chapter. Due to the rare prevalence of the disease, the treatment options are not well established and there exists controversy regarding the management options available. The tumors in the pineal region range from the benign to the malignant ones and this chapter aims at presenting the overview of the various types of these tumors with a discussion on the management options.