Epithelioid angiosarcoma of the pericardium: Case report of the challenges in the management of a rare malignancy with poor prognosis.

ABSTRACT: A primary epithelioid angiosarcoma of the pericardium is a rare soft tissue malignancy. This report describes a young adult woman who presented with progressive dyspnea and pericardial effusion. She was found to have pericardial mass on imaging along with extensive myocardial infiltration. The biopsy of the mass revealed epithelioid angiosarcoma, which was CD34 and CD31-immuno-positive. Due to unresectable disease, she was given a trial of immunotherapy followed by conventional chemotherapy. She showed partial response on interim assessment, but progressed soon after the completion of six cycles and succumbed to her rapidly progressive illness within nine months of diagnosis. This report discusses the diagnostic and therapeutic challenges faced while managing this disease of aggressive tumor biology.

Real world study of safety and efficacy of lorlatinib as second line and beyond in ALK-rearranged advanced non-small cell lung cancer patients in India - a multicentre chart review study (ROSELAND).

Background: Lorlatinib, an anaplastic lymphoma kinase (ALK)-inhibitor, is approved as frontline as well as subsequent line of therapy in ALK-rearranged advanced non-small cell lung cancer (NSCLC). There is limited literature about safety and efficacy of lorlatinib in Indian patients. Materials and methods: This was a retrospective multicentre study on patients with ALK-rearranged advanced NSCLC received lorlatinib as second line and beyond between May 2017 and December 2021. ALK was tested either by immunohistochemistry or fluorescent in-situ hybridisation. Clinicopathologic features, treatment details, toxicity and outcomes were analysed. Results: A total of 38 patients were enrolled with a median age of 54 years (range: 30-72) and male: female ratio of 20:18. Fifteen (44%) patients had brain metastases at baseline. Lorlatinib use was - second line in 11 (29%), third line in 21 (55%) and fourth line in 4 (11%) of patients, respectively. The best radiologic response to lorlatinib was - complete response in 9 (24%), partial response in 17 (46%), stable disease in 9 (24%) and progressive disease in 2 (5%) of patients, respectively. After a median follow-up of 76.6 months (95% CI: 68.9-100), the median progression-free survival (PFS) of lorlatinib was not reached (95% CI: 24.3-not reached) and median overall survival (OS) of the whole cohort was 93.1 months (95% CI: 62-not reached). Both median PFS (p = 0.48) and median OS (p = 0.74) was similar between second line and later line use of lorlatinib. Thirty-three (87%) patients experienced treatment-related toxicity and six (16%) patients required dose modification. Conclusion: Lorlatinib was highly efficacious in terms of overall response rate, median PFS and median OS in this small real-world cohort of advanced ALK+ve NSCLC with a manageable safety profile.

Solid papillary carcinoma of the breast.

Solid Papillary Carcinoma (SPC) of the breast is a rare tumor with an incidence of less than 1%, mainly affecting elderly females. It is morphologically characterized by well-defined nodules with low-grade nuclear features associated with fibrovascular cores and shows neuroendocrine differentiation. SPC can be in-situ or invasive but has a favorable prognosis. It is a morphological mimicker of some pre-malignant conditions leading to its frequent misdiagnosis. An appropriate immunohistochemical (IHC) panel workup helps in distinguishing this tumor from its various morphological mimics. In this report, we present one such case of SPC with a small focus of invasion, reviewing the literature.

Real-World Breast Cancer Patient Follow-Up Practices by Medical Oncologists in India-A Survey Report.

Amol PatelBackground In India, breast cancer patients' post-treatment follow-up practices are not known. We did this survey to understand how the breast cancer patients are followed-up and tried to explore the challenges associated with it. Methods We conducted a survey-based study among Indian oncologists. Seven questions were framed pertaining to follow-up practices. Answers were provided in the form of multiple options. Google forms platform was used. Survey was circulated through social media apps and through mail. We sought suggestions and opinions to address the challenges from participants. Results A total of 158 medical oncologists responded to this survey. 10% were not aware that only history and clinical examination are the scientific recommendations for follow-up. Ninety percent of the medical oncologists felt clinical breast examination as an uncomfortable practice for patients and physicians and 39% ordered a chest X-ray and an ultrasound abdomen. Annual mammogram was ordered by 83%, and blood investigations were recommended by 14% routinely. The majority (49.6%) felt that the absence of a female attendant, physician and patient factors were responsible for nonadherence to clinical breast examination. The DEXA scan was recommended by 84 (53%) medical oncologists regularly for patients on aromatase inhibitors, while 23 (14%) did not recommend it. Conclusion There is a disparity between scientific recommendations and real-world follow-up practices. A large number of medical oncologists relied on chest X-ray and ultrasound abdomen. There is an unmet need to address this issue.

A clinicoepidemiological and management profile of metastatic carcinoma gallbladder in the northeast part of Indian patients in a tertiary care center.

Introduction: Metastatic gallbladder cancer (GBC) is a highly fatal malignancy and it is difficult to treat the advanced stage of GBC. In India, northern and northeastern states are the worst affected by this disease. We, hereby, report the clinicoepidemiological and management profile of 242 patients of metastatic carcinoma of GB. Materials and Methods: In this study, a total of 242 cases of metastatic GBC (detected either on the first presentation or during follow-up) were managed at the Department of Medical Oncology tertiary care oncology center in the northeast part of India from May 2018 to September 2019. On presentation, all patients were subjected to detailed history and clinical examination, followed by requisite investigations and were treated as per the existent guidelines. Results: One-hundred and forty-two patients were female, while 100 patients were male out of 242 patients. Female patients with metastatic GBC presented with the mean age of 54, while for males, 51.4 years. The most common presentation was pain abdomen (81.8%), while the second most common was anorexia (77.2%), followed by weight loss (62.8%) and mass per abdomen (60.7%). The most common site of metastasis recorded in our study was the liver (79.7%), followed by nonregional abdominal lymph node (69.4%) and ascites (64.4%). Out of the 242 patients, 24 patients had presented in poor Eastern Cooperative Oncology Group Performance Status (≥3) hence were deemed unfit for any oncological interventions. About 136 (56.1%) patients had presented with features of obstructive jaundice, however only 108 patients were subjected to biliary drainage procedure. After the biliary drainage procedures, only one-third (38 out of 136; 35.1%) of patients were finally able to receive chemotherapy. Conclusion: In India, unfortunately, many patients present very late during the course of their illness. There is a need for the development of effective chemotherapy or targeted therapy and also there is an unmet need for patients' education. There has been an increase in the incidence of this malignancy, especially in the Northeast part of India; hence, it is the need of the hour to study various epidemiological and causative factors of the disease. Furthermore, the development of therapies for the effective management of this malignancy is really required.

Cancer in the Adolescent and Young Adults (AYA) and Children: A Comprehensive Analysis of the Epidemiology and Psychosocial Morbidity in the Indian Population.

Bhupesh GuleriaAims Adolescent and young adults (AYAs), children with cancer, and their guardians have unique psychosocial morbidities adversely effecting quality of life (QOL). This is measurable using patented tools. We analyzed epidemiological and clinicopathological patterns of solid organ cancers in this subgroup. We also assessed psychosocial morbidity and changes in QOL faced by them. Methods All patients aged 2 to 39 years, newly diagnosed with cancer from April 2017 to March 2019 were included. Clinical history, diagnosis, staging, treatment, outcomes, and follow-up were recorded. The National Comprehensive Cancer Network (NCCN) distress thermometer and European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ C-30) were used to assess psychosocial morbidity of AYAs, children ≥ 12 years, and parents of children < 12 years. Pediatric Quality of Life Inventory (Peds QL) version 3.0 was used for children < 12 years. Data was analyzed using descriptive statistics. Results A total of 571 patients (512 AYAs, 59 children) were enrolled. Median age was 30 years with male predominance (58.1%). Most cases (98.6%) were absent from school or work. Carcinoma breast was the most common in females (29.3%) and non-Hodgkin lymphoma in males (12.6%). 91.06% had overall NCCN distress score ≥ 4. Also, 73.81 and 79.49% had "quite a bit" or "very much" responses on functional and symptom scales, respectively, in EORTC QLQ C-30 questionnaire. Peds QL version 3.0 revealed total score ranging from 276 to 523 for each patient. Conclusion AYAs and children with cancer are extremely vulnerable to psychological stress and morbidity. Use of well-established tools help in assessing their mental status and timely psychiatric referral can be initiated.

Correlation of programmed death-ligand 1 expression with gene expression and clinicopathological parameters in Indian patients with non-small cell lung cancer.

OBJECTIVES: The aim of this study is to evaluate the incidence of programmed cell death-ligand 1 (PD-L1) expression in non-small cell lung cancer (NSCLC) cases and its correlation with gene mutation and clinicopathological parameters. METHODS: Samples from NSCLCs patients were studied for PD-L1 expression through immunohistochemistry (IHC) using Rabbit anti-human PDL-1/CD274 Monoclonal Antibody. Genetic mutations were studied using IHC/fluorescence in situ hybridization (FISH) methods (for anaplastic lymphoma kinase [ALK]) or polymerase chain reaction/gene sequencing analysis (for epidermal growth factor receptor [EGFR]). Pearson's correlation coefficient (r) was used for correlation analysis. PD-L1 expression was analyzed for association with clinicopathological features. RESULTS: Of the 101 NSCLC cases, PD-L1 expression was observed in 33.66% (34/101) cases; tumor proportion score of <50%: 67.65% (23/34) and ≥50%: 32.35% (11/34) cases. PD-L1 positivity was seen in; males: 35.5%, females: 28%, smokers: 37.7%, cases with brain metastasis: 20%, cases with pleural effusion: 20.8%, and histopathological evaluation (well-differentiated: 21.42%, moderately-differentiated: 13.79%, poorly-differentiated: 36.11%, and adenosquamous disease: 40.9%). Genetic mutation studies revealed PD-L1 positivity in 18.1% cases with EGFR mutation, 50% of ALK-IHC positive cases, and 33.3% ALK-FISH positive cases. No or very weak correlation (r < 0.3) in PD-L1 expression with gene mutations or clinicopathological parameters was observed. CONCLUSIONS: The study demonstrated PD-L1 expression in ~ 1/3rd cases of NSCLC patients. No or very weak correlation was observed for PD-L1 expression with genetic mutations and other parameters studied. The presence of gene mutations in PD-L1 expressed samples suggests further investigation on PD-L1 inhibitors in such patients for decisive treatments.

Genomic Landscape and Targeted Treatment of Gallbladder Cancer: Results of a First Ongoing Prospective Study.

Background Prognosis of gallbladder cancer (GBC) has not changed in the past 20 years. Comprehensive genomic profiling (CGP) carries potential to determine the actionability for multiple targets, including ERBB2 , ERBB3 , MET , ROSI , FGFR , and PIK3 . This study evaluates the role of CGP and targeted therapies. Methods This is a multicenter, prospective, single-arm study. All consecutive patients of unresectable and/or metastatic GBC of age ≥18 years were enrolled. Hybrid capture-based CGP was performed by Foundation Medicine CDx. All patients received first-line chemotherapy with gemcitabine-cisplatin regimen. Patients with ERBB2/3 amplification received trastuzumab with capecitabine or nab-paclitaxel, and patients with MET amplification were treated with crizotinib. For ERBB2/3 mutations, lapatinib plus capecitabine regimen was used. Results Fifty patients were studied with a median age of 56 years (range 26-83) and a male-to-female ratio of 1:1.6. ERBB2 and ERBB3 amplification was seen in 9 (18%) and 2 (4%) patients, respectively. Four patients with ERBB2 amplification received trastuzumab and/or lapatinib, showed partial response, and maintained response beyond 12 weeks. One patient had mixed response, whereas two patients progressed on trastuzumab and lapatinib. Three patients with ERBB3 mutations showed response to lapatinib-capecitabine. One patient with MET amplification responded to crizotinib for 4 weeks. PIK3 mutations were present in 14% of cases and were independent of ERBB aberrations. Conclusion GBC is enriched in 28% of patients with ERBB2 and ERBB3 amplifications and/or mutations. Responses are seen with lapatinib in concurrent ERBB2 mutation and amplification. ERBB3 mutation showed response to lapatinib. MET and PIK3 are new findings in GBC, which may be targeted.

Solid papillary carcinoma of the breast

Solid Papillary Carcinoma (SPC) of the breast is a rare tumor with an incidence of less than 1%, mainly affecting elderly females. It is morphologically characterized by well-defined nodules with low-grade nuclear features associated with fibrovascular cores and shows neuroendocrine differentiation. SPC can be in-situ or invasive but has a favorable prognosis. It is a morphological mimicker of some pre-malignant conditions leading to its frequent misdiagnosis. An appropriate immunohistochemical (IHC) panel workup helps in distinguishing this tumor from its various morphological mimics. In this report, we present one such case of SPC with a small focus of invasion, reviewing the literature.