Ewing's sarcoma in adolescents and adults - 10-year experience from a tertiary cancer center in India.

BACKGROUND: Ewing's sarcoma (EWS) is an aggressive small round cell tumor, affecting bone and soft tissues and is mostly seen in childhood and second decade of life. EWS accounts for 10-12% of bone tumors in more than 15 years age group and is even rarer after 40 years of age. MATERIALS AND METHODS: This retrospective analysis was conducted among patients aged more than 15 years with histologically proven EWS. RESULTS: Among 240 cases of EWS treated at our center during 2001-2010, 130 (54%) were more than 15 years of age. The median age was 20 years with a male: female ratio of 2.4:1. Ninety percent had skeletal EWS, 10% had extra skeletal EWS, and 37% patients were metastatic at presentation. Eighty-two received curative treatment with chemotherapy (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide (VAC/IE)) along with local treatment, radiotherapy (RT) in 61, surgery alone in seven, and RT plus surgery in 14. Two- and 5-year overall survival (OS) was 43.3% and 25.5%, respectively, for the entire series. The OS for the non-metastatic group was 63.2% at 2 years and 36.5% at 5 years, and the progression free survival was 53.7% at 2 years and 37.8% at 5 years. High lactate dehydrogenase was found to be a significant poor prognostic factor (P = 0.001). Median OS for localized central EWS was 49.2 months and that for peripheral EWS was 24 months. Patients more than 20 years of age with non-metastatic disease had better OS compared to those with 15-20 years of age. CONCLUSION: Treatment of EWS requires a multidisciplinary approach with radical surgery and/or radiation to control local disease and multiagent chemotherapy to control systemic disease. Long-term follow-up is essential because of disease relapse and treatment-related complications.

Metastasis from renal cell carcinoma-unusual presentations.

ABSTRACT: Renal cell carcinoma is one of the most lethal tumors of the urologic system and has high metastatic potential. The usual sites of metastasis are lung, bone, liver adrenal, lymph node, and brain. Thumb and oral cavity metastasis are unusual sites of metastasis. This case report describes two cases reported with unusual thumb and oral cavity metastasis. Patients with acral and oral metastasis are often misdiagnosed as benign inflammatory disease and treatment initiation is often delayed. This case report helps in drawing the attention that such lesions can be metastatic also.

Clinical Profile and Survival Outcome of Endometrial Cancer with p53 Mutation.

Clinicopathologic classification of endometrial cancer imperfectly reflects the tumor biology. Pathologic categorization - especially in high-grade tumors - results in an imprecise estimation of the risk of disease, recurrence, and death. Molecular subtyping is emerging as the standard of care in diagnosis and treatment of endometrial cancers. Molecular markers are important prognostic factors in tumor dissemination and early recurrence of endometrial cancers. TP53 mutation is an important prognostic factor for both serous and endometrioid cancers. The study aims to compare the clinical profile and overall survival of endometrial cancers with and without p53 mutation. Sixty-three patients who underwent surgical staging for carcinoma endometrium were included in the study.TP53 mutation status was determined based on p53 expression by immunohistochemistry (IHC) as a p53 wild or p53 mutant type. Data were analyzed for the clinical profile, p53 mutation status on IHC, histological pattern, tumor grade, stage of the disease, lymph node spread, recurrence pattern, treatment received, 2-year disease-free survival, and overall survival. Recurrence was noted in 12.7% patients after 2-year follow-up, of which 75% patients had p53 mutation. Significant association was seen between p53 expression and high-grade tumors, stage, cervical involvement, and adnexal involvement. The 2-year overall survival of the p53 wild type was 97.2% and the p53 mutant type was 91.7%. The 2-year disease-free survival for the p53 wild type was 94.3% and the disease-free survival of the p53 mutant variety was 83.5%. The 2-year disease-free survival for endometrioid carcinoma with p53 wild type was 100% and p53 mutant variety was 86.2% (p value 0.033). About 15.9% (10) patients were reassigned to the high-risk group needing chemotherapy and radiation according to the ESGO ESTRO 2021 consensus classification, due to their p53 mutation status. IHC to assess somatic p53 mutation may be done in endometrial biopsies irrespective of their histology. This may help to identify that the aggressive tumors thereby help in tailoring surgery, planning adjuvant treatment, and follow-up.

Uterine Sarcoma: Retrospective Study From A Single institute.

Abstract: Uterine sarcomas are heterogeneous group of tumours comprising 1% of gynaecological malignancies. There is lack of concences on optimal treatment of uterine sarcomas. This is because of lack of randomised controlled trials due to rarity of these tumours. Surgical management without spill remains the standard primary treatment. Most of the times uterine sarcomas are diagnosed postoperatively from histopathology report of either myomectomy or hysterectomy. This retrospective study analysed the clinico pathological characteristics, prognostic factors, treatment details and survival outcome of different types of uterine sarcomas. Materials and methods: This is a retrospective analysis of 59 patients of uterine sarcomas. All patients underwent surgery. Adjuvant chemotherapy or radiation treatment were given according to histopathological report and FIGO stage. Patients were followed up every three months for first two years and then every six months. Disease free survival (DFS) and Overall survival (OS) were calculated. Statistical analysis: The data were summarized using descriptive statistics as mean, percent and range. Survival probabilities were estimated using Kaplan-Meier method and the significance of difference between the survival curves were calculate using log-rank test. Results: Uterine sarcomas are rare and aggressive tumours of uterus. Majority of these tumours present in early stage. Surgery remains the main treatment modality. Role of adjuvant radiation treatment remains controversial. Tumour stage is the most important prognostic factor.

A rare complication of Yellow Phosphorous poisoning.

Yellow phosphorous is one of the most common toxins reported to cause fulminant hepatic failure. Yellow phosphorous is a component of rodenticides and fireworks, and is freely available over the counter. The mortality rate as high as 27% from fulminant hepatic failure has been reported following ingestion of yellow phosphorous.1 Multisystem toxicity affecting gastrointestinal, cardiac, renal and central nervous systems occurs.2 There is a paucity of literature on its haematological toxicity. In this case report, we highlight the clinically significant myelosuppression observed in a patient resulting from yellow phosphorous; a small literature review has also been done on this rare complication.

Tumor Marker Decline in Predicting Treatment Outcome among Poor-Risk Testicular Germ Cell Tumors-A Tertiary Cancer Center Data.

Lakshmi Haridas K.Introduction Testicular germ cell tumors are rare in India. Despite the advances in chemotherapy, poor-risk testicular nonseminomatous germ cell tumors (NSGCT) remain as a clinical challenge. Various prognostic factors have been described in this rare disease. The Indian data in this regard is scarce. Our study is the first attempt to assess the tumor marker decline with respect to treatment outcome in poor-risk NSGCT in Indian patients. Materials and Methods This retrospective study was done among newly diagnosed poor-risk NSGCT, treated at genitourinary clinic, at our tertiary cancer center during the period 2017 to 2019. The prognostic significance of tumor marker decline in them was correlated with 2-year progression-free survival (PFS) and 2-year overall survival (OS). Statistical Methods The association between two variables were assessed using chi-squared/Fischer's exact test. The PFS and OS were estimated using Kaplan-Meier method and the significance difference between survival curves was tested using log rank test. The risk for survival was estimated using cox regression analysis. A p -value of <0.05 was considered as significant. Results Out of 11 eligible patients, four (36%) had favorable tumor marker decline and seven (64%) had unfavorable decline. The 2-year PFS among favorable and unfavorable decline group were 66.7 and 42.9%, respectively ( p -0.358), and the 2-year OS was 66.7 and 71.4%, respectively ( p -0.974). Teratoma was not found to be a significant factor in our study. Tumors with only beta human chorionic gonadotropin (ßHCG) elevation were observed to have good outcome. Postchemotherapy unresectable residual disease showed a significant trend toward inferior survival, the 2-year PFS was 38 versus 100% ( p -0.188) and the 2-year OS was 62.5 versus 100% ( p -0.334) in patients with and without unresectable residual disease, respectively. Conclusion Majority of our poor-risk NSGCT patients had unfavorable tumor marker decline and progressive events. However, the survival difference was not significant, given the small sample size. Tumors with only ßHCG elevation were observed to have good outcome. Postchemotherapy unresectable residual disease showed a significant trend toward inferior survival.

Prognostic Factors for Survival in Patients with Carcinoma Endometrium.

Francis. V. JamesObjective The study aimed to see the clinical outcome and to identify prognostic factors for survival in patients with carcinoma endometrium. Methods Patients registered at Regional Cancer Centre, Thiruvananthapuram, Kerala, India, with carcinoma endometrium from January 2009 to December 2013 were identified from hospital registry. Data regarding patient demographics, tumor characteristics, treatment schedules, and follow-up were collected using a structured proforma. Survival estimates were generated using the Kaplan-Meier method. Univariate analysis was done using chi-square and Fisher's exact tests. Multivariate analysis using the Cox regression model was performed to determine the impact of prognostic factors on outcome. The statistical analysis was done using SPSS software version 11. Results The median follow-up of the 686 patients was 95 months (range 3-178 months).There were 432 stage 1 (63%), 100 stage II (14.6%), 108 stage III (15.7%), and 46 stage IV patients (6.7%). The 5-year overall survival was 89.2%. Prognostic factors for survival on univariate analysis were age 60 years or older, nonendometrioid histology, high-grade tumor, cervical stromal involvement, para-aortic node involvement, negative progesterone receptor expression, deep myometrial invasion advanced stage, surgery versus no surgery, serosal involvement, and ovarian and fallopian tube involvement. However, on multivariate analysis, age over 60 years, higher histological grade, advanced stage, and deep myometrial and parametrial invasion were associated with significantly poorer survival. Conclusion We found that age over 60 years at presentation, higher grade, advanced stage, deep myometrial invasion, and parametrial invasion were associated with poorer survival.

Erratum to: Prognostic Factors for Survival in Patients with Carcinoma Endometrium.

[This corrects the article DOI: 10.1055/s-0041-1735563.].

Successful Treatment of Late Isolated Bone Metastasis in a Patient with Bilateral Retinoblastoma Using an Unconventional Method.

Though survival in bilateral retinoblastoma (RB) has improved due to advancement in diagnostics and treatment modalities, children require long-term follow-ups for recurrence and second malignancies. We report a case of bilateral RB in a 7-month-old baby who was treated with chemotherapy, transpupillary thermotherapy, and periocular carboplatin for both eyes following which there was complete regression of tumour. Six and a half years after treatment, the child presented with metastatic recurrence of tumour in the left ulna. He was treated successfully with chemotherapy, extracorporeal radiation and reimplantation therapy. A less aggressive treatment approach for isolated bone relapse may be considered in selected cases.

Prognostic factors and outcomes of nonseminomatous germ cell tumours of testis-experience from a tertiary cancer centre in India.

Germ cell tumour of the testis is the most common cancer in young men in the western world. India has the lowest incidence globally, and hence Indian data are sparse. We report the outcomes of patients with nonseminomatous germ cell tumours of testis treated at a tertiary cancer centre in South India over a period of 10 years. Patients with a histopathological diagnosis of nonseminomatous germ cell tumours of the testis from 1 January 2006 to 31 December 2016 were included in the study. Patient demographics, tumour characteristics and treatment details were retrieved from case records. Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival (OS). Cox regression model was used to analyse the prognostic factors. One hundred and nineteen patients with nonseminomatous germ cell tumours of the testis were included in the study. The median follow-up was 81 months. The estimated 4-year OS and progression-free survival were 87.1% and 84.5%, respectively. The four-year OS for good, intermediate and poor-risk groups was 93.6%, 87.5% and 52.6%, respectively. The PFS at 4 years was 91.4%, 87.8% and 47.4% for good, intermediate and poor-risk groups, respectively. The presence of nonpulmonary visceral metastasis and biochemical response after chemotherapy were significant predictors for OS and PFS in multivariate cox proportional hazards regression. The survival figures are comparable to the rest of the world except in the poor prognostic risk group. The inferior survival noticed in this group of patients may be due to the lack of good salvage procedures. High-dose chemotherapy with stem-cell support may be considered more often for this group of patients.

Educational Status, Cancer Stage, and Survival in South India: A Population-Based Study.

PURPOSE: Lower socioeconomic status is associated with more advanced cancer at the time of cancer diagnosis. It is unknown whether this leads to inferior survival in low- and middle-income countries. Here, we explore the association between educational level and survival in South India. METHODS: The Trivandrum Cancer Registry (3.3 million population) was used to identify all cases of breast and cervical cancer (women) and oral cavity (OC) and lung cancer (men) diagnosed during 2012-2014. Educational level was classified as illiterate/primary school, middle school, and secondary school and above. Survival was measured from date of diagnosis using the Kaplan-Meier method. Cox proportional hazards regression modeling was used to describe the associations among education, stage of cancer at diagnosis, and survival. RESULTS: The study population included 3,640 patients with breast (n = 1,727), cervical (n = 425), OC (n = 702), and lung (n = 786) cancer. Educational level was 27%, 23%, and 32% for illiterate/primary, middle, and secondary school and above, respectively. The 5-year survival rate for breast cancer was 59%, 68%, and 73% (P = .001); for cervical cancer, 51%, 52%, and 60% (P = .146); and for OC cancer, 42%, 35%, and 48% (P = .012) for illiterate/primary, middle school, and secondary school and above, respectively. The survival gradient across social groups was substantially attenuated when stage was added to the multivariable model. There was no observed difference in survival across educational groups for lung cancer (2%, 4%, and 3%; P = .224). CONCLUSION: Data from this population-based study in South India demonstrate that patients from a lower educational background have inferior survival and that this is at least partially explained by having more advanced disease at the time of diagnosis. Public health efforts are needed to facilitate timely diagnosis and reduce disparities in cancer outcomes.

Ultrasensitive melanoma biomarker detection using a microchip SERS immunoassay with anisotropic Au-Ag alloy nanoboxes.

The detection of circulating biomarkers in liquid biopsies has the potential to provide a non-invasive route for earlier cancer diagnosis and treatment management. Melanoma chondroitin sulfate proteoglycan (MCSP) is a membrane protein characteristic for melanoma cell migration and tissue invasion with its soluble form (sMCSP) serving as a potential promising diagnostic surrogate. However, at the initial disease stage, the detection of sMCSP is challenging because of its low abundance and the required high specificity to analyze sMCSP in complex bodily fluids. Herein, we report a highly sensitive and high-throughput microchip that enables Surface Enhanced Raman Spectroscopy (SERS) immunoassay for parallel detection of up to 28 samples. Key to assay speed and sensitivity is the stimulation of an alternating current-induced nanofluidic mixing that improves target-sensor collision and displacement of non-specific molecules. Anisotropic Au-Ag alloy nanoboxes (NB's) with strong plasmonic hot spots provide single SERS particle sensitivity that enables ultrasensitive sMCSP detection of as low as 0.79 pM (200 pg ml-1). As a proof of concept study, we investigate the assay performance in simulated melanoma patient samples.

Successful Percutaneous Endovascular Repair in a Very Large, Symptomatic, Non-ruptured Abdominal Aortic Aneurysm with Severe Neck Angulation.

Percutaneous endovascular aneurysm repair (PEVAR) is a minimally invasive treatment option for patients with abdominal aortic aneurysms (AAA). PEVAR allows for the lower incidence of vascular access site complications and decreased procedure time, yet the utility of this technique depends on the anatomical characteristics of the aneurysm. A detailed evaluation of the access site vessels and aneurysm neck anatomy are critical for standard patient and device selection. An 84-year-old male presented to our institution with the sudden onset of abdominal pain and confusion. Subsequent imaging demonstrated the presence of a 9.5 cm fusiform, infrarenal abdominal aortic aneurysm with a greater than 60-degree neck angulation and bilateral common iliac aneurysms. The patient underwent percutaneous endovascular aneurysm repair (PEVAR), and a type IB endoleak seen at the end of the case was treated successfully. At the one-year follow-up, the patient remained asymptomatic with the AAA stable in size.  This case represents the largest reported symptomatic unruptured AAA repaired with a completely percutaneous technique to date. Building up the stent-graft from the bifurcation with a unibody modular device may allow for support to address the severe angulation of a very hostile neck. PEVAR is a viable option in patients with symptomatic AAA and can be performed despite severe aneurysm neck angulation.

Clinicopathological features and outcomes of adrenocortical carcinoma: A single institution experience.

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with aggressive behavior. Most of our knowledge about this rare tumor is based on retrospective case series. This study aimed at analyzing the clinicopathological features and outcomes of patients treated at a tertiary cancer center in India. PATIENTS AND METHODS: We retrospectively reviewed the data of patients with ACC registered from January 2006 to December 2015. RESULTS: Thirty-seven patients were included in the study, 20 males and 17 females. Median age was 49 (18-78) years. Hormonal overproduction was noticed in 27% of patients. Median tumor size was 10 cm (2-22). Seventeen patients had metastatic disease and 20 patients were localised at diagnosis. Median follow-up was 22 months and median overall survival (OS) was 23.46 months. OS at 2 years and 5 years was 46.1% and 21%, respectively. The median disease-free survival (DFS) was 20 months. DFS at 2 years and 5 years was 45% and 24%, respectively. Age, sex, tumor size, hormonal overproduction, tumor laterality, and stage of the disease did not influence survival. However, advanced stage was associated with higher risk for recurrence. (P = 0.03). CONCLUSION: ACC is a rare endocrine malignancy with very poor survival rates. Rate of recurrence is high even after complete surgery. Systemic treatment options are limited. Newer agents are needed to improve outcome.

Sociodemographic Factors and Stage of Cancer at Diagnosis: A Population-Based Study in South India.

PURPOSE: Lower socioeconomic status is associated with inferior cancer survival in high-income countries, but whether this applies to low- and middle-income countries is not well described. Here, we use a population-based cancer registry to explore the association between educational level and stage of cancer at diagnosis in South India. METHODS: We used the Trivandrum District population-based cancer registry to identify all cases of breast and cervical cancer (women) and oral cavity (OC) and lung cancer (men) who were diagnosed from 2012 to 2014. Educational status-classified as illiterate/primary school, middle school, or secondary school or higher-was the primary exposure of interest. Primary outcome was the proportion of patients with advanced stage disease at diagnosis defined as stage III and IV (breast, cervix, or OC) or regional/metastatic (lung). RESULTS: The study population included 4,547 patients with breast (n = 2,283), cervix (n = 481), OC (n = 797), and lung (n = 986) cancer. Educational status was 22%, 19%, and 26% for illiterate/primary, middle, and secondary school or higher, respectively. Educational status was missing for 33% of patients. The proportion of all patients with advanced stage disease was 37% (breast), 39% (cervix), 67% (OC), and 88% (lung). Patients with illiterate/primary school educational status were considerably more likely to have advanced breast cancer (50% v 39% v 36%; P < .001), cervix cancer (46% v 43% v 24%; P = .002), and OC cancer (77% v 76% v 59%; P < .001) compared with patients with higher educational levels. The proportion of patients with advanced lung cancer did not vary across educational levels (89% v 84% v 88%; P = .350). CONCLUSION: A substantial proportion of patients in South India have advanced cancer at the time of diagnosis. This is particularly true among those with the lowest levels of education. Future health awareness and preventive interventions must target less-educated communities to reduce delays in seeking medical care for cancer.

Histoplasmosis, heart failure, hemolysis and haemophagocytic lymphohistiocytosis.

Histoplasmosis is an endemic mycosis with global distribution, primarily reported in immunocompromised individuals. A 29-year old immunocompetent male presented with fever, hepatosplenomegaly and pancytopenia. His peripheral blood showed features suggestive of intravascular hemolysis and echocardiography showed features suggestive of pulmonary arterial hypertension. Bone marrow showed yeast with morphology suggestive of Histoplasma capsulatum. Further investigations revealed hyperferritinemia, hypofibrinogenemia and increased triglycerides. With a diagnosis of progressive disseminated histoplasmosis with secondary Haemophagocytic lymphohistiocytosis, he was successfully treated with amphotericin B followed by itraconazole. We report this case to highlight the atypical and rare manifestations of histoplasmosis.

Safety and Efficacy of Ultrasound-Guided Cryoablation for Benign Breast Fibroepithelial Lesions.

OBJECTIVE: The purpose of this study is to evaluate the safety and efficacy of cryoablation for benign breast fibroepithelial lesions (FELs) that otherwise warrant surgical excision, including symptomatic fibroadenomas, growing fibroadenomas, and cellular FELs. METHODS: All patients from 2016-2018 who had ultrasound-guided cryoablation of a symptomatic and/or growing fibroadenoma or cellular fibroepithelial lesion were reviewed. The electronic medical record was reviewed for any procedure-related complications and reduction in mass volume by ultrasound and was recorded to assess for efficacy. Patients were surveyed using a seven-point Likert scale to assess satisfaction with the procedure and a ten-point visual analog pain scale to assess level of discomfort. RESULTS: Twenty-four women (average age 37.1 years, range 19-57 years) with 26 FELs were treated with no adverse events and 100% technical success. Thirteen cellular FELs and 13 symptomatic and/or growing fibroadenomas were ablated. Twenty-two patients had an average imaging follow-up of 11.7 months (range 5-23 months), and 18 patients completed the follow-up survey. There was 92% (22/24) clinical success, defined as a greater-than-50% reduction in mass volume. Average reduction in mass volume was 86% overall. Eighty-five percent (17/20) would recommend the procedure to others and reported pain during the procedure to be minimal (average 2.4 out of 10) with overall satisfaction rating of 6.2 out of 7. Twelve symptomatic masses demonstrated a significant reduction in pain after cryoablation (P = 0.01). CONCLUSION: Cryoablation is a safe and efficacious minimally invasive nonsurgical alternative for the treatment of benign breast FELs.