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   Table of Contents - Current issue
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July-September 2019
Volume 8 | Issue 3
Page Nos. 137-202

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ORIGINAL ARTICLES: BREAST CANCER  

Use of animation video in surgical decision-making for treatment of early breast cancer in Indian women p. 137
Sapana Bothra, Sabaretnam Mayilvaganan, Prabhaker Mishra, Anjali Mishra, Amit Agarwal, Gaurav Agarwal
DOI:10.4103/sajc.sajc_179_18  
Introduction: Surgical decision-making in early breast cancer is difficult for the patient and also for the treating clinician, especially when the patient is not completely aware of the available options. Adjuncts such as animation video with case scenarios can be helpful in this regard. We used an animation video to help in decision-making and evaluated the effect of such adjunct in Indian women with early breast cancer. Materials and Methods: An animation video of running time of 4 min and 11 s was shown to forty patients with early breast cancer, who filled in a patient satisfaction multimedia questionnaire at the end of the animation. Results: Seventeen (42.5%) patients underwent breast-conserving surgery (BCS) while the rest 23 (57.5%) patients underwent the mastectomy. All forty patients were satisfied with the animation video. The mean score of the utility of the video to improve understanding of the disorder, better organization of treatment, stimulated interest in the relations, and saved unnecessary discussion was 88.50, 88.50,88.3, and 90.3, respectively. Age and literacy status did not significantly affect the scores. Discussion: All the patients found the video useful and most patients made the decision on the first attempt. Patients' perspective about BCS is influenced by the fear of recurrence, fear of multiple surgeries, complications, and also the counseling provided by the surgeon. Conclusion: In this situation, such animation videos provide an unbiased view on the operative procedure and help in decision-making.
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Quadriplegia secondary to abiraterone-induced severe hypokalemia p. 139
Jignesh B Rajvanshi, Manin D Chaudhary, Priyanka D Malekar, Nitin K Singhal, Shirish S Alurkar
DOI:10.4103/sajc.sajc_343_18  
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ORIGINAL ARTICLES: BREAST CANCER Top

Correlation of digital mammography and digital breast tomosynthesis features of self-detected breast cancers with human epidermal growth factor receptor type 2/neu status p. 140
Chaitra Sonthineni, Namita Mohindra, Vinita Agrawal, Zafar Neyaz, Neeraj Jain, Sabaretnam Mayilvagnan, Gaurav Agarwal
DOI:10.4103/sajc.sajc_300_18  
Context: Breast cancer is a heterogeneous disease with several histological subtypes. Its prognosis and management are influenced by human epidermal growth factor receptor type 2 (HER2/neu) expression. Varying grades of HER2/neu overexpression are likely to have different morphological features. Digital breast tomosynthesis (DBT) enhances lesion visibility and hence that it may reveal features closer to histomorphological findings. Aims: The aim of this study is to correlate digital mammography (DM) and DBT findings of self-detected tumors with HER2/neu status, to determine whether differences in imaging features can help predict the degrees of HER2/neu overexpression. Settings and Design: Prospective study conducted in a tertiary care hospital. Methods: For 100 consecutive patients with self-detected lumps, DM and DBT data were reviewed by two radiologists who were blinded to histopathology. Of these, 63 patients with histologically proven breast cancer were recruited and their DM and DBT findings compared and correlated with HER2neu status (scores 0–3+). Statistical Analysis: Pearson's Chi-squared test and Fisher's exact test were used (SPSS version 22.0, IBM). Results: Morphology of lesions at both DM and DBT varied with HER2/neu status (P = 0.04 and 0.015, respectively). HER2-0 tumors mostly presented as masses without microcalcifications (88.8%), while most of HER2-3+ tumors as masses or asymmetries with microcalcifications (61.9%). The presence or absence of calcifications varied significantly with HER2/neu status. Breast imaging-reporting and data system (BI-RADS) scoring varied significantly (P < 0.001) with higher HER2 signal, more frequently associated with BI-RADS 5 score. Conclusion: DM and DBT features vary with the intensity of HER2 immunostaining. Higher BI-RADS scores, microcalcifications, and spiculated margins are frequently associated with HER2/neu 3+ lesions.
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ORIGINAL ARTICLE: EPIDEMIOLOGY OF CANCER AND CANCER SCREENING Top

Perspectives of primary healthcare providers on implementing cancer screening services in tribal block of Maharashtra, India p. 145
Anushree D Patil, Neha R Salvi, Begum Shahina, A Sharmila Pimple, A Gauravi Mishra, L Sanjay Chauhan, Beena Nitin Joshi
DOI:10.4103/sajc.sajc_290_18  
Background: Breast, cervical, and oral cancers contribute to majority of cancer deaths among women in India. However, there is poor implementation of screening programs at primary health care (PHC). There is a need to understand the perspectives of healthcare providers at PHC level for feasibility of implementation of a cost-effective cancer screening program, particularly in the rural and tribal areas that are under served by cancer services. Materials and Methods: A continuous medical education (CME) program on “Prevention and early detection of common cancers” was held for all Medical Officers of Palghar District, Maharashtra. A self-administered questionnaire was used to assess the knowledge, attitude, practices, perspectives regarding common cancers, screening methods, and human papilloma virus (HPV) vaccination. A pre- and post-assessment was carried out before the commencement and on completion of the CME among 76 participants. Results: Knowledge about etiology of common cancers was high; however, awareness of risk factors was low. There were knowledge gaps about HPV vaccination. There was overall improvement about the available screening methods and knowledge of HPV vaccine and dosages after the CME (pretest 65% to posttest 95%). Providers had no experience in performing cervical cancer screening on a routine basis. While the majority of the providers (97%) indicated that screening for cancer was essential and feasible at PHC level; however, training (52%) and resources (53%) would be needed. Conclusion: Healthcare providers though from the underserved tribal areas, were optimistic to implement screening for common cancers and were willing to take training for the same. This emphasizes the need for educating and training the healthcare providers with simple techniques for effective implementation of cancer screening programs in underserved areas.
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Severe hypoglycemia with trastuzumab: An unseen adverse event p. 149
BP Amrith, Parveen Jain, Krushna A Chaudhari, Mansi Sharma, Ullas Batra
DOI:10.4103/sajc.sajc_400_18  
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ORIGINAL ARTICLE: GENITOURINARY CANCERS Top

Prostate cancer survival estimates: An application with piecewise hazard function derivation p. 150
Atanu Bhattacharjee, Atul Budukh, Rajesh Dikshit
DOI:10.4103/sajc.sajc_245_18  
Background: The hazard function is defined as time-dependent. However, it is an overlooked area of research about the estimation of hazard function within the frame of time. The possible explanation could be carried by estimating function through the changes of time points. It is expected that it will provide us the overall idea of survival trend. This work is dedicated to propose a method to work with piecewise hazard rate. It is a data-driven method and provides us the estimates of hazard function with different time points. Methods: The proposed method is explored with prostate cancer patients, registered in the Surveillance, Epidemiology, and End Results Program and having aged at diagnosis with range 40–80 years and above. A total of 610,814 patients are included in this study. The piecewise hazard rate is formulated to serve the objective. The measurement of piecewise hazard rate is compared with Wald-type test statistics, and corresponding R function is provided. The duration of follow-ups is split into different intervals to obtain the piecewise hazard rate estimates. Results: The maximum duration of follow-up observed in this study is 40 years. The piecewise hazard rate changes at different intervals of follow-ups are observed almost same except few later intervals in the follow-up. The likelihood of hazard in earlier aged patients observed lower in comparison to older patients. The hazard rates in different grades of prostate cancer also observed separately. Conclusion: The application of piecewise hazard helps to generate statistical inference in a deeper manner. This analysis will provide us the better understanding of a requirement of effective treatment toward prolonged survival benefit for different aged patients.
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An exceptional response to nivolumab in relapsed and refractory malignant mesothelioma p. 159
Vikas T Talreja, Vanita Noronha, Amit Joshi, Vijay Patil, Abhishek Mahajan, Kumar Prabhash
DOI:10.4103/sajc.sajc_18_19  
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ORIGINAL ARTICLES: GI CANCERS Top

Adjuvant chemotherapy in stage II–III operated colon cancer patients from a nontrial cohort in a low colon cancer prevalence country with predominant use of modified CAPOX p. 160
Anant Ramaswamy, Rushabh Kothari, Ashwin Desouza, Tarachand Gupta, Sandeep Bairwa, Akhil Kapoor, Amit Kumar, Pradeep Ventrapati, Mukta Ramadwar, Sarika Mandavkar, Nita Chavan, Avanish Saklani, Vikas Ostwal
DOI:10.4103/sajc.sajc_176_18  
Background: Data regarding the practice of adjuvant chemotherapy, specifically with modified CAPOX, and survival outcomes in operated colon cancer patients from a nontrial cohort in a lower-middle income and low prevalence nation like India is scarce. Materials and Methods: Patients who underwent upfront curative resection for colon cancer from January 2013 to December 2016 were analyzed for baseline variables and outcomes. Results: A total of 491 patients underwent curative resection in the predefined time period. The median age of the patients was 53 years (range: 17–87). Patients with Stage I, Stage II, and Stage III disease comprised 7.9%, 44.8%, and 45.4% of the entire cohort, respectively. Patients with Stage I cancer were observed. Adjuvant chemotherapy was planned for 384 patients (78.2%), with the doublet regimens (capecitabine-oxaliplatin, or 5-fluorouracil-oxaliplatin) being used commonly (77.6%). Common toxicities were Hand-foot syndrome (Grade 2/3 - 21.4%) and peripheral neuropathy (Grade 2/3 - 20.1%). About 85% of patients receiving monotherapy (capecitabine or 5 fluorouracil) and 81.2% of patients receiving doublet chemotherapy (mCAPOX or modified FOLFOX-7) completed their planned adjuvant treatment. With a median follow-up of 22 months, estimated 3 years event-free survival was 86%, and overall survival (OS) was 93.6%. Stage, younger age (<50 years), underlying cardiovascular abnormalities, need for dose reductions and noncompletion of planned chemotherapy predicted for inferior estimated 3-year OS on multivariate analysis. Conclusions: Adjuvant chemotherapy especially with modified CAPOX appears well tolerated in the Indian population and early survival outcomes appear to be comparable to published literature.
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Calculation of a clinical predictive factors identifying peritoneal disease on a staging laparoscopy in gastric cancers p. 166
Caleb Harris, Vikas Ostwal, Dilip Harindran Vallathol, Rohit Dusane, Sarika Mandavkar, Shraddha Patkar, Anant Ramaswamy, Shailesh V Shrikhande
DOI:10.4103/sajc.sajc_182_18  
Introduction: Staging laparoscopy (SL) is the current standard staging workup for loco-advanced gastric cancers (GCs). Materials and Methods: We analyzed the data of all patients with loco-regionally advanced, nonmetastatic GCs, who underwent SL for the evaluation of peritoneal carcinomatosis (PC). Results: Between December 2013 and October 2016, 363 patients underwent SL, of which 75 (20.7%) were found to have PC on SL. Age ≤40 years, CA 19-9 > upper limit of normal, and low serum albumin levels (≤3.5 g/dl) correlated significantly with the presence of PC on SL. There was a statistically significant difference in the median overall survival between patients with radiologically detected PC and SL detected PC (8.67 months vs. 15.3 months;P < 0.0001).Conclusion: SL upstaged disease status in 20.7% of patients. Clinical factors, identified in this study, need further validation in larger prospective cohorts before being used in clinical practice. Patients with radiologically detected PC have lower survival as compared to those with PC on SL.
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ORIGINAL ARTICLE: GYNAECOLOGICAL CANCER Top

Clinicopathological profile of adnexal masses presenting to a tertiary-care hospital in Bhutan p. 168
Rojna Rai, Pema Choden Bhutia, Ugyen Tshomo
DOI:10.4103/sajc.sajc_303_18  
Context: Adnexal masses of ovarian origin are of growing concern due to high fatality associated with ovarian malignancy because they are diagnosed at advanced stage due to vague symptoms and absence of recommended screening tests. Aims: The aim was to study the prevalence of histopathologic types of adnexal masses in different age groups and to analyze the accuracy of preoperative evaluation in diagnosing ovarian malignancy. Settings and Design: This was a cross-sectional study carried out in the Department of Obstetrics and Gynecology of a tertiary care hospital in Bhutan with gynecologic-oncology services, from January to December 2017. Subjects and Methods: Women presenting with adnexal mass were evaluated and those meeting criteria were enrolled. They were evaluated preoperatively with complete history, examination, ultrasound, and tumor markers. Risk of malignancy index (RMI) was calculated for all patients. Following surgery, histopathology results were compared with preoperative evaluation. Statistical Analysis Used: Chi-square test, t-test, Cohen's Kappa, and receiver operating characteristic curve analysis were used for statistical analysis. Results: Of 165 patients evaluated, 127 fulfilling criteria were enrolled. Adnexal masses of ovarian origin were most common (n = 102, 80.3%), of which 12.7% were malignant. Epithelial ovarian malignancy was the most common malignant ovarian tumor, serous cystadenocarcinoma being the most common. Malignancy was significantly more in older, postmenopausal women with high RMI. Seven out of 11 women with high RMI were diagnosed in Stage 3 or 4. RMI score at cutoff of 200 was 54.6% sensitive and 85.7% specific. Conclusions: Adnexal mass of ovarian origin was the most common. Malignancy was significantly more in older, postmenopausal women with high RMI. RMI showed moderate correlation in diagnosing epithelial ovarian malignancies.
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LETTERS TO THE EDITOR Top

Immunotherapy-induced acute pulmonary thromboembolism: A case report Highly accessed article p. 172
Waseem Abbas, Gaurav Dixit, Ranga Raju Rao, Vineet Govinda Gupta, Swati Popli
DOI:10.4103/sajc.sajc_25_19  
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ORIGINAL ARTICLES: HEAD AND NECK CANCER Top

Palliative chemotherapy in carcinoma nasopharynx p. 173
Vijay M Patil, Amit Joshi, Vanita Noronha, Vikas Talreja, Vijai Simha, Sachin Dhumal, Bhavesh Bandekar, Arun Chandrasekharan, Kumar Prabhash
DOI:10.4103/sajc.sajc_230_18  
Introduction: Nasopharyngeal carcinoma is a rare malignancy. We conducted an audit of systemic therapies received in palliative setting in carcinoma nasopharynx and studied their outcomes. Methods: Patients who underwent first-line palliative systemic chemotherapy between January 2014 and April 2017 for carcinoma nasopharynx at the department of medical oncology at authors' institute were selected for this analysis. Toxicities, responses, progression-free survival (PFS), and overall survival (OS) were analyzed. In addition, a Quality-Adjusted Time without Symptoms or Toxicity analysis with threshold utility analysis was performed. Results: Fifty-one patients were included in this analysis. The indication of palliative chemotherapy was locoregionally recurrent disease in 25 (49.0%) patients and metastatic disease in 26 (51.0%) patients. The overall response rate was 62.0% (n = 33). The median PFS was 225 days (95% confidence interval [CI]: 164–274 days) and median OS was 513 days (95% CI: 286–931 days). The restricted mean TOX state duration was 2.6 days (95% CI: 0.3–4.9), restricted mean TWiST duration was 219.2 days (95% CI: 184.0–254.4), and restricted mean REL duration was 74.3 days (95% CI: 38.1–110.4). Conclusion: Systemic cytotoxic therapy in nasopharyngeal cancers is associated with high response rates and clinically meaningful PFS; with low duration of time spent in adverse events.
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A prospective randomized study comparing concurrent chemoradiation with weekly and 3 weekly cisplatin in locally advanced oropharyngeal carcinoma p. 178
R Nanda, Aradhana Katke, N Suneetha, B Thejaswini, Tanvir Pasha, KP Jagannath, GV Giri, K Govind Babu
DOI:10.4103/sajc.sajc_270_18  
Introduction: The chemotherapy schedules with cytotoxic dose or weekly regimes are still challenging, weighing the benefits versus toxicities. This prospective randomized study is an attempt to assess the efficacy of two schedules of cisplatin in management of locally advanced HNSCC. Objectives: The objectives of this study was to evaluate tolerance, tumour response and toxicities of concurrent chemoradiation with cisplatin in weekly and three weekly regimes. Methods: Locally advanced oropharyngeal squamous cell carcinoma patients fit for concurrent chemoradiation with cisplatin 40 mg/m2 (weekly) and 100 mg/m2 (3 weekly) were randomized to Arm A and B concurrently with radiotherapy of 70Gy/35frs/7 weeks. Statistical Analysis: Chi-square/ Fisher Exact test has been used to find the significance of study parameters on categorical scale between the groups. The statistical software SPSS 15.0 was used. Results: Between December 2010 and January 2013, 60 patients were enrolled. The median cycles of cisplatin in Arm-A was 5 and 2 in Arm-B. The complete response of 80.9% vs 75% and partial response of 14.3% vs 12.5% was observed in both arms respectively. There was no statistical difference in acute radiation and hematological toxicities between the two groups. With median follow up of 28 months, the 2 and 5 years overall survival was 55% and 58%; 41.6% and 32.3% in arms A and B respectively. Conclusion: In our study of locally advanced oropharyngeal carcinoma treated with radical radiotherapy comparing concurrent chemotherapy with cisplatin weekly vs 3 weekly had no significant difference in overall response, complete response and acute toxicities.
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ORIGINAL ARTICLES: HEMATOLYMPHOID MALIGNANCIES Top

Langerhans cell histiocytosis: An enigmatic disease p. 183
Anubha Jain, Sushil Kumar, Priyanka Aggarwal, Mohan Kumar, Vineeta Gupta
DOI:10.4103/sajc.sajc_211_18  
Background: Langerhans cell histiocytosis (LCH) is a poorly understood disease with heterogeneous clinical presentation ranging from unifocal bony involvement to disseminated disease with life-threatening complications. Materials and Methods: The clinical profile, laboratory findings, treatment, and long-term outcome were retrieved from maintained medical records from January 2006 to January 2016 and were retrospectively analyzed. The extent of the disease was classified as per the LCH-III trial of “The Histiocyte Society.” The assessment and categorization of treatment response followed LCH III trial definitions. Results: A total of 28 children with LCH were diagnosed. The age ranged between 5 months and 9 years, with a mean of 3½ years. The M: F ratio was 3:1. Single system, unifocal and multifocal bone diseases were seen in nine (32.1%) and two (7.1%) cases, respectively. Disseminated disease without risk organ involvement was seen in six (21.1%), whereas disseminated disease with risk organ involvement was seen in 11 (39.3%) cases. The most common presentation was bony involvement (19 [67.8%]), out of which 16 (88.8%) had skull involvement. During follow-up, 17 (60.7%) were in complete remission though five (17.8%) of them relapsed, but achieved second remission. Two (7.1%) were lost to follow-up. Six (21.4%) had progressive disease of which four expired and two abandoned treatment. Two (10.7%) refused the initiation of treatment. Conclusion: A better understanding of the disease, early suspicion, and diagnosis can improve the outcome of patients with LCH.
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LETTERS TO THE EDITOR Top

Neoadjuvant chemotherapy in technically unresectable adenocarcinoma of parotid p. 185
Sachin Dhumal, Arun Chandrasekharan, Vijay Patil, Vanita Noronha, Amit Joshi, Ameya Puranik, Venkatesh Rangarajan, Kumar Prabhash
DOI:10.4103/sajc.sajc_87_19  
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ORIGINAL ARTICLES: HEMATOLYMPHOID MALIGNANCIES Top

Mutation profile in Indian primary myelofibrosis patients and its clinical implications p. 186
Vinod R Patil, S Chandrakala, Shruti Mantri, Rajesh Patil, Nilesh Wasekar, Farah Jijina
DOI:10.4103/sajc.sajc_276_18  
Background: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by abnormal proliferation of megakaryocytes, bone marrow fibrosis, and extramedullary hematopoiesis. We did mutation profile of 50 patients of PMF and tried to correlate it with initial clinical presentation of these patients. Materials and Methods: All new and follow up patients who were diagnosed as PMF based on WHO 2016 definition of PMF were included. Mutation profile of these patients including JAK2 V617F, JAK2 exon 12, CALR and MPL mutations was done and all clinical, demographic and laboratory details were recorded. Results: Total 50 patients were enrolled out of which 29 were males and 21 were females. Out of these patients, 32 (64%) were JAK2 positive, 13 (26%) were CALR positive, 1 (2%) were MPL positive and 4 (8%) were triple negative. As compared to JAK2+ve patients and triple negative group, CALR positive patients were younger, had lower total leucocyte count, larger spleen size, lower dynamic international prognostic scoring system (DIPSS) score and higher grade of fibrosis of marrow. Conclusion: This study depicts that incidence of JAK2 and CALR mutations in Indian PMF patients is fairly similar to that in rest of the world. CALR positive patients have better clinical parameters at presentation and have better prognosis as compared to JAK2 positive patients.
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ORIGINAL ARTICLES: LUNG CANCERS Top

Patterns of brain metastasis in anaplastic lymphoma kinase - rearranged and epidermal growth factor receptor-mutated lung cancer patients in magnetic resonance imaging p. 189
Ullas Batra, Vivek Mahawar, Ankush Jajodia, Avinash Razdan, Himanshu Mahanthi, Venkata Pradeep Babu Koyyala
DOI:10.4103/sajc.sajc_98_18  
Introduction: The optimal management of neuroparenchymal lesions in cases of lung cancer is exigent as this frequent yet notorious complication negatively impacts the morbidity and mortality index. Aims: This study is aimed at recognizing various patterns of neuroparenchymal metastasis in patients of lung cancer with epidermal growth factor receptor (EGFR)- and anaplastic lymphoma kinase (ALK)-positive mutations. Material and Methods: The radiological findings of the neuroparenchymal lesions were analyzed and the statistical data were charted. We identified two groups of patients with neuroparenchymal lesions among a cohort of 340 patients having EGFR-positive (68) and ALK-positive (24) mutations (total: 24 + 68 = 92). Results: We observed that among the ALK group, leptomeningeal spread was less compared to EGFR group (2/24 as opposed to 18/68). Morphological heterogeneity and central necrosis in the parenchymal lesion which were associated with unfavorable outcomes were predominant in ALK group (8/24) as opposed to EGFR group (2/68). Ancillary findings but pertinent to survival and morbidity such as presence of perilesional edema, hemorrhage, and hydrocephalus on magnetic resonance imaging were also analyzed. The mutation-specific differential imaging spectrum could be attributed to biological differences between these cancers.
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Application of international association for the study of lung cancer/American Thoracic Society/European Respiratory Society criteria for the diagnosis of lung carcinomas on small biopsies: A tertiary care center experience p. 191
KV Vinu Balraam, Divya Shelly, Prabha S Mishra, KS Sampath, Reena Bharadwaj
DOI:10.4103/sajc.sajc_163_18  
Background: The new IASLC/ATS/ERS classification provides standardized terminology for lung cancer diagnosis in small biopsies and cytology specimens. Objectives: The aim was to study the feasibility of the guidelines using one marker for adenocarcinoma (ADC) and one for squamous cell carcinoma (SQCC) in non-small cell lung carcinomas (NSCLCs). Subject and Methods: In this study, we reviewed all the formalin-fixed paraffin-embedded tissue blocks diagnosed as lung carcinoma between July 2016 and December 2017. Cases were labeled as SCLC, ADC, SQCC, NSCLC favor ADC, NSCLC favor SQCC, NSCLC-not otherwise specified (NOS), and NSCLC-NOS possible adeno-SQCC (ADSQCC) as per IASLC/ATS/ERS 2011 guidelines. A three-step approach incorporating morphology, immunohistochemistry (IHC), and molecular analysis was used. Results: One hundred and nine cases were included. Six of the 109 cases were SCLC and 1 case was of large-cell neuroendocrine type. Of the remaining 102, 51 were diagnosed based on their classical histomorphology into SQCC (8) and ADC (43). Remaining 51 cases required IHC/special stains for categorization. The panel comprised anti-CK7, anti-thyroid transcription factor-1 (TTF-1), and anti-p63. Twenty-nine were positive for anti-TTF-1 and thus labeled as NSCLC favor ADC. Fifteen were labeled as NSCLC favor SQCC as they were highlighted by anti-p63. Four cases showed reaction to both the antibodies in different sets of tumor cells and thus were classified as NSCLC-NOS, possible ADSQCC. Remaining 3 cases did not show reaction to any of the antibodies and hence were labeled NSCLC-NOS. Conclusion: The need for every laboratory to use minimal tissue for ancillary tests to diagnose lung carcinoma on small biopsies is reemphasized. Tissue from small biopsies needs to be preserved not only for the diagnosis but also for molecular testing and evaluation of markers of resistance to therapy, in this era of personalized medicine.
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ORIGINAL ARTICLE: PEDIATRIC AND ADOLESCENT CANCERS Top

Retinoblastoma: A retrospective analysis of 141 patients from 1983 to 2013 at a tertiary care hospital in Nagpur, India p. 195
Rohit S Kabre, Krishna M Kamble
DOI:10.4103/sajc.sajc_314_18  
Purpose: There is scarcity of data regarding clinical presentation and outcome of retinoblastoma patients in India. Objectives: The objective of this study was to assess the clinical profile of retinoblastoma patients in a tertiary care hospital in India from 1983 to 2013. Subjects and Methods: A retrospective analysis of clinical records of 141 patients with retinoblastoma registered from 1983 to 2013 at Government Medical College, Nagpur, India, was conducted. Demographics, clinical features, modes of treatment, and outcome of the patients were assessed. Results: Majority of patients (81 [57.45%]) presented in the age group of 1–3 years and were males. One hundred and fourteen patients (80.85%) had unilateral disease, while rare presentations of trilateral/quadrilateral retinoblastoma were also noted. Proptosis was seen in 81 (57.45%) patients as presenting symptom. Eighty-nine patients (63.12%) had locally invasive disease-involving sites. Forty-four patients (31.19%) developed distant metastasis. Surgical management and external beam radiotherapy were followed in majority of patients. Trend of increased usage of chemotherapy was seen from the mid-1990s. One hundred and twelve patients (79.43%) died with the disease. Conclusions: Data from this study show late diagnosis, leading to poor outcome for patients with advanced retinoblastoma, which is in accordance with data from other developing countries. Even though management of patients changed in accordance with changing standard of care over the decade, mortality remained high.
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LETTERS TO THE EDITOR Top

Pilomatrixoma arising from maxillary sinus – First of its kind p. 197
TR Sruthi, Subrat K Behera, Smruti Swain, Monalisa Patsani
DOI:10.4103/sajc.sajc_54_19  
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ORIGINAL ARTICLE: SUPPORTIVE CARE AND OTHERS Top

Clinical and epidemiological profile of neuroendocrine tumors: An experience from a regional cancer center from Western India p. 198
Rahul Suhas Kulkarni, Asha S Anand, Sonia K Parikh, Harsha P Panchal, Apurva A Patel, Dhruv P Mehta, Priyanka Patel
DOI:10.4103/sajc.sajc_364_18  
Background: Most of the data on neuroendocrine tumors (NETs) are from the Western literature. Indian studies regarding clinicopathological characteristics and treatment outcomes are lacking. Methods: This is a prospective observational study of all new patients with NETs (except small-cell lung cancer) registered at our tertiary care cancer institute from November 2014 to November 2016. A total of 97 new patients were registered, of which 20 were lost to follow-up before starting any planned treatment. Epidemiological and clinicopathological features of all these 97 patients were studied, and the remaining 77 patients were analyzed for treatment response and survival analysis. Results: The median age at diagnosis was 49 years (20–74 years) with male preponderance (M: F = 1.85:1). The most common primary site of origin was pancreas (34/97 = 35%), followed by unknown primary origin (19%), small intestine (9%), and pulmonary (6%). Of 97 patients, 91 (93.8%) presented with nonfunctional symptoms, 3 (3.1%) had purely functional symptoms, and 3 (3.1%) presented with both functional and nonfunctional symptoms. The most common presenting symptom was abdominal pain (59.7%), followed by jaundice (9.3%), whereas watery diarrhea (83.3%) and flushing (66.7%) were the most common functional symptoms. Sixty-six percent (64/97) of cases were metastatic at presentation. A strong correlation was noted between the primary site of origin and metastatic presentation (P = 0.016). Chemotherapy was the most common primary therapy (40.2%), followed by surgery (28.6%), watchful waiting (15.6%), and somatostatin analogs (11.7%). The median event-free survival was highest for patients undergoing surgery (10 months). Conclusions: The clinicopathological profile of NETs in the Indian population differs from Western countries. Majority of patients present with metastatic disease, thus representing a need for creating awareness among patients and medical fraternity and formulating Indian guidelines for optimized treatment.
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