Endometrial cancer risk factors, treatment, and survival outcomes as per the European Society for Medical Oncology (ESMO) - European Society of Gynaecological Oncology (ESGO) - European Society for Radiotherapy and Oncology (ESTRO) risk groups and International Federation of Gynecology and Obstetrics (FIGO) staging: An experience from developing world.

Introduction: There is limited data on endometrial cancer from developing countries. The risk groups as defined by the ESMO-ESGO-ESTRO and their recommendations for adjuvant treatment have redefined the management protocols. In this retrospective analysis, the outcomes are assessed in the light of the new risk groups and FIGO staging. Material Methods: One hundred and two patients of endometrial cancer reporting to the Department of Radiation Oncology from 2015 to 2019 were analysed retrospectively. Patients were stratified as per the ESMO-ESGO-ESTRO risk groups and FIGO staging. Patients were analysed for demographic profile, histopathology details, FIGO stage, treatment modalities received as per the ESMO-ESGO-ESTRO risk groups and the outcomes in terms of disease free survival and overall survival. Results: A total of 102 patients were analysed. The mean age at presentation was 57.7 years. Seventy four percent (74.41%) were stage I patients, 14.7 % were stage II, 8.8% were stage III and remaining 2% were stage IV. The mean disease free survival for the patients in FIGO stage I, II, III and IV were found to be 63.5 (59.9 - 67) months, 60.5 (54.2 - 66.9) months, 30.9 (21.5 - 40.2) months and 15.4 (7.8 - 23.0) months respectively. The 5-year overall survival of patients in Stage I was 90.3%. The 3-year mortality of Stage III patients was 58.3%. While there was no mortality observed among Stage II patients, none of the Stage IV patient survived beyond 20 months. The 5-year disease-free survival for patients in Low Risk (LR) group, Intermediate Risk (IR) group and High Risk (HR) group was found to be 91.3%, 90% and 87% respectively. None of the patient in High Intermediate Risk (HIR) group experienced progression of disease and 33.3% patients in advanced group were disease free at 2 years follow-up. The multivariate analysis showed that lymph node involvement is significantly associated with disease-free (p=0.03) and overall survival (p=0.04). Conclusion: Even in the developing world, majority of patients present in early stage with survival outcomes comparable to the West. FIGO stage and lymph node involvement continue to be the most important prognostic markers for disease outcomes.

Metastatic Ectopic Thymoma of the Neck without Mediastinal Involvement.

Introduction: Thymoma is a rare malignancy with usual location in the antero-superior mediastinum. Occurrence of an extra-mediastinal thymic malignancy in the neck with lung metastasis and without involvement of the mediastinum is an extremely rare condition. Staging systems and treatment guidelines are defined for mediastinal thymomas but not for ectopically located thymomas. Case Report: 38-year-old female presented with the chief complaint of a progressive neck swelling, located predominantly in the right lateral neck, extending to the midline. Computed Tomography showed a heterogenous peripherally enhancing mass with likely origin from the thyroid gland. The mass measured 12 x 6 x 3.5 cm in size and extended from the hyoid bone superiorly to the suprasternal location inferiorly. Additionally, there were multiple, variable sized subpleural nodules scattered in both lungs, suggestive of lung metastases. Histopathology and immunohistochemistry findings from neck mass confirmed the diagnosis of Thymoma Type A. Conclusions: Thymoma is a rare tumor that typically does not show aggressive behaviour. Extra-mediastinal neck thymoma with bilateral lung metastasis is an extremely rare presentation. Thymoma presenting as neck swelling without mediastinal extension on radiology, poses a diagnostic dilemma. Histopathology with immunohistochemistry helps to confirm the final diagnosis. Surgery is the mainstay for the management of localized tumors with adjuvant treatment reserved for incompletely resected tumors or advanced stage. Systemic metastasis are rare in this indolent tumor and chemotherapy regimens are investigational. Clinical presentation, prognostic factors, staging and management guidelines are still not well defined for this rare tumor with atypical location.

Health-related quality of life among breast cancer patients in India.

PURPOSE: This study evaluated the health-related quality of life (HRQoL) among breast cancer patients during various phases of treatment and with different treatment modalities, which helps in monitoring treatment outcomes, assessing the well-being of patients, and conducting health technology assessments. METHODS: A total of 534 interviews were conducted among the patients of breast cancer recruited at different stages of disease and with different treatment modalities. HRQoL was determined using EuroQoL five dimensions questionnaire with five levels (EQ-5D-5L), EuroQoL Visual Analogue Scale (EQ VAS), and the EORTC QLQ-BR23 instrument. The utility values were determined based on the Indian EQ-5D-5L value set. The socio-demographic and clinico-therapeutic determinants of HRQoL were evaluated using multiple linear regression. RESULTS: The mean utility value of breast cancer patients was 0.602 (SD = 0.311) and mean EQ VAS score was 75 (SD = 12.3). The mean utility value at diagnosis was 0.628, whereas utility value was 0.55, 0.595, and 0.64 for post-surgery, post-chemotherapy, and post-radiotherapy treatment groups, respectively. The most frequently reported problem was pain/discomfort (in 84.3% patients), followed by anxiety/depression (83.5%). On EORTC QLQ-BR23, the maximum symptom scale scores for systemic therapy side effects were reported in the post chemotherapy group. The body image score and future perspective score were better in patients undergoing breast conservative surgery (BCS) compared to patients undergoing modified radical mastectomy. Age, education, and employment status of the patient, type of treatment modality, and use of taxanes are the determinants of HRQoL in breast cancer patients. CONCLUSION: Clinical interventions should focus upon management of pain and anxiety. BCS should be offered to all eligible patients as it is associated with better HRQoL. Addressing the factors that independently affect the HRQoL will help in improving the treatment compliance and outcomes.

Epidemiological profile of retinoblastoma in North India: Implications for primary care and family physicians.

BACKGROUND: Retinoblastoma is the most common primary intraocular malignancy among children. Despite being curable in early stages, majority of the cases in India present in late stages, when outcomes are very poor. OBJECTIVES: The aim of this study was to assess the epidemiological profile, clinical characteristics, and treatment practices among retinoblastoma patients in north India. MATERIALS AND METHODS: Data on all patients with retinoblastoma, over a 10-year-time period from 2009 to 2018, who were treated in a tertiary care hospital in north India, were assessed. Data were analyzed to describe the demographic characteristics, clinical features in terms of stage at presentation, and management practices in terms of diagnostic investigations and treatment. The statistical significance for difference in percentages was assessed using Fischer's exact test at a 5% significance level. RESULTS: A total of 25 retinoblastoma patients were enlisted, of whom one was excluded as it was adult onset retinoblastoma. The median age at presentation was 3 years, with a male to female ratio of 1:1.4. Bilateral presentation was seen in 16.6% cases. Majority (66.6%) of the patients underwent magnetic resonance imaging of brain and orbit as a part of the diagnostic workup. Intraocular disease was seen in 58.3% patients, whereas 41.6% patients had extraocular disease. Local therapy with vision preservation could be used only in 8.3% patients, whereas 87.5% patients were referred for enucleation. Chemotherapy with combination of vincristine, etoposide, and carboplatin was used extensively both, in neoadjuvant setting (83.3%) and in the adjuvant setting. CONCLUSION: Despite availability of treatment for eye preservation, its utility is limited due to the advanced stage at presentation. Awareness about the disease and its symptoms for early diagnosis, especially with the Mid-Level Health Provider at Health and Wellness Centers, is likely to improve early reporting and treatment and meeting the Vision 2020 goals.

Evaluation of Angiogenesis in Multiple Myeloma by VEGF Immunoexpression and Microvessel Density.

Background Multiple myeloma (MM) is a plasma cell disorder characterized by monoclonal proliferation of plasma cells in bone marrow. Plasmablastic MM is a morphologic subset of MM, containing ≥2% plasmablasts of all plasma cells. Methods The study included 30 consecutively diagnosed patients of MM (6 plasmablastic, 24 nonplasmablastic) over a span of 2 years. Angiogenesis in MM was assessed by analysis of vascular endothelial growth factor (VEGF) immunoexpression by plasma cells and microvessel density (MVD) using anti-CD34 antibody. CD34 and VEGF immunohistochemical staining was performed in all the 30 cases. Angiogenesis was studied in relation to plasmablastic morphology and clinical profile to determine if any correlation exists between these. Results The mean VEGF expression of 80.83 ± 7.36 in plasmablastic myeloma cases was significantly higher compared with a mean VEGF of 53.54 ±17.09 in nonplasmablastic cases. Most of the cases (66.6%) of plasmablastic myeloma exhibited strong (3+) VEGF expression. The difference in mean VEGF expression between plasmablastic and nonplasmablastic cases was found to be statistically significant ( p = 0.001). The mean MVD in plasmablastic cases was 44.8 ± 3.69, while in the nonplasmablastic category, the mean MVD was 23.7 ± 5.14, difference being statistically significant ( p < 0.05). Also, a positive correlation was found between VEGF expression and MVD. Conclusion A moderate/strong VEGF intensity and higher MVD were found in cases of plasmablastic MM, suggesting that a more aggressive histological disease may be associated with increased production of VEGF. This finding might be helpful to identify a subset of patients with adverse prognosis and to provide antiangiogenic therapy to improve their survival. However, studies comprising larger number of patients are required to bring out a statistical significance to further substantiate these findings.

Limb Salvage Using Liquid Nitrogen-Treated Tumour-Bearing Autograft: A Single Institutional Experience of 10 Patients.

BACKGROUND: Many reconstruction methods have evolved to offer limb salvage surgery (LSS) to patients with musculoskeletal sarcomas. It can be achieved using endoprosthesis or biological reconstruction methods like allograft or autograft or a combination of both. In carefully selected patients, resected bone can be recycled and reimplanted after sterilisation using methods like irradiation, autoclaving, pasteurisation or liquid nitrogen. METHODS: From 2010 to 2016, 10 patients with primary musculoskeletal sarcoma underwent limb salvage surgery (LSS) by wide resection of the tumour and reconstruction using recycled autograft treated with liquid nitrogen. Intercalary resection was carried out in six patients and intra-articular in four. The resected bone was dipped in liquid nitrogen for 25 min, thawed at room temperature for 15 min followed by dipping in vancomycin-mixed saline for 10 min. The recycled bone was re-implanted into its original site and stabilised with internal fixation. RESULTS: At a mean follow-up period of 39.6 months (range 6-97 months), all patients had a good function (mean functional score of 80%) with no evidence of local recurrence in the re-implanted bone or otherwise. Union was achieved at 15 of the 16 osteotomy sites with a mean union time of 5.2 months (range 4-7 months) without any additional surgical interventions. In none of the patient, augmentation with vascularised/non-vascularised fibula was done. No complication like fracture of the autograft, implant failure or deep/superficial infection was reported in any patient. CONCLUSION: Recycled tumour-bearing autograft after treatment with liquid nitrogen is an anatomical, cost-effective, relatively simpler and reliable technique for reconstruction of bone defect after resection in selective primary musculoskeletal sarcoma patients.

Ovarian Small Cell Carcinoma of Hypercalcemic Type in an Adolescent Girl.

OBJECTIVE: Small cell carcinoma of ovary, hypercalcemic type is a rare malignancy with a dismal prognosis. The diagnosis is often confused with many other tumors. CASE REPORT: We describe a rare case of ovarian small cell carcinoma of hypercalcemic type in an adolescent. She presented with abdominal pain, awareness of mass and vomiting. She underwent exploratory laparotomy and right ovarian excision. The detailed histopathological examination including immunohistochemistry was suggestive of ovarian small cell carcinoma of hypercalcemic type. She had progressive disease on chemotherapy and ultimately died within 2 years of diagnosis. Due to rarity of this neoplasm and its aggressive nature, the optimal treatment regimen has not been established. CONCLUSION: We report this case because of its rare occurrence leading to clinical and diagnostic challenges and need to explore effective treatment options to improve survival in these patients.

Extraosseous Ewing's Sarcoma of the Parapharyngeal Space - A Rare Entity - with Review of Literature.

INTRODUCTION: Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parapharyngeal space is even rarer. To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature. CASE REPORT: We report a rare case of EES of the parapharyngeal space in an 8-year-old girl. She presented with complaints of earache, difficulty in breathing and swallowing and bleeding from the mouth. Investigations revealed a large parapharyngeal mass causing narrowing of the nasopharyngeal and oropharyngeal airway with skeletal and lung metastasis. Biopsy from the parapharyngeal mass was suggestive of malignant small round cell tumor. The patient was treated with chemotherapy and radiotherapy, but developed brain metastasis and succumbed to disease approximately 1 year after diagnosis. Herein, we describe the characteristic clinicopathological features and treatment with a comprehensive review of the literature. CONCLUSION: EES in this unusual location behaves aggressively, with a high rate of recurrence and distant metastasis. Aggressive multimodal treatment comprising of multi-agent chemotherapy, surgical resection if feasible, and radiotherapy should be considered.

Polymorphous Low Grade Adenocarcinoma of the Parotid in a Teenager.

INTRODUCTION: Polymorphous low grade adenocarcinoma (PLGA) is a rare salivary gland neoplasm with an indolent course. It occurs primarily in the minor salivary glands but can rarely occur in the major salivary glands. It usually occurs in the fifth to seventh decades of life with female preponderance. CASE REPORT: A 16-year-old male presented with recurrent painless swelling in the right preauricular region and with a history of surgical intervention at the same site in the past. His histopathology report was suggestive of pleomorphic adenoma. The swelling recurred after one year of excision and a superficial parotidectomy was performed. The detailed histopathological examination was suggestive of Polymorphous low grade adenocarcinoma. In view of close margins, the patient was given adjuvant radiotherapy. Thirty-three months post-surgery, he is alive and disease-free. We describe a rare case of PLGA of the parotid gland in a teenager with its clinical characteristics, histopathological features, and treatment. CONCLUSION: The occurrence of PLGA in the parotid gland is rare with only a few cases reported in literature. The diagnosis of PLGA is challenging due to morphological diversity.

Cutaneous Metastasis: A Study of 138 Cases Diagnosed by Fine-Needle Aspiration Cytology.

BACKGROUND: Cutaneous metastases can occur in a wide variety of internal malignancies and may be the first sign of a clinically silent visceral cancer. STUDY DESIGN: A retrospective analysis was made of 138 cases diagnosed with cutaneous and subcutaneous metastasis on fine-needle aspiration cytology (FNAC). Primary tumors of the skin/subcutis were excluded. RESULTS: Of 138 cases, the primary was known in 101 cases and unknown in 37 cases. The age of the patients ranged from 5 to 86 years, and 76 (55.1%) were male and 62 (44.9%) were female. Clinically, the most common lesion was a single nodule (n = 77, 55.8%). The chest wall was the predominant site (n = 53, 38.4%). In males and females, the most common primary sites were the lung (n = 16) and breast (n = 24), respectively. On cytology, the most common diagnosis was metastatic adenocarcinoma (n = 41, 29.7%). Of 37 cases with an unknown primary, FNAC helped to locate the primary site in 17 (45.9%) cases, while in 20 cases it remained undiagnosed. CONCLUSIONS: FNAC is a rapid and safe technique that can be used as a first line of investigation for confirmation of metastatic lesions of the skin. Critical evaluation of cytomorphological features along with relevant clinical details could help in the localization of an unknown primary site in some cases.

Apropos of a case of cutaneous metastasis from laryngeal cancer with review of literature.

Cutaneous metastasis from laryngeal carcinoma is a rare occurrence. A 55-year-old male patient with supraglottic cancer was treated with concurrent chemoradiation. Eighteen months later, he presented with ulceroproliferative growth on dorsum of the right hand. Biopsy revealed metastatic squamous cell carcinoma. Further investigations revealed underlying bone destruction with lung metastasis. In view of poor general condition and widespread dissemination of disease, palliative radiotherapy was delivered to the hand of the patient. He achieved satisfactory palliation in form of pain relief, control of bleeding, and discharge. The present report serves to emphasize the importance of properly diagnosing metastatic spread to unusual sites. Such metastasis is rare and is associated with a poor prognosis. Treatment is usually aimed at providing pain relief in these patients with limited life expectancy. Hence, we present a case of extensive cutaneous metastasis from laryngeal carcinoma with review of the literature.

Role of scintimammography in assessing the response of neoadjuvant chemotherapy in locally advanced breast cancer.

Locally advanced breast cancer (LABC) is a common cancer in the developing countries. Neoadjuvant chemotherapy (NACT) is a very important step in the treatment of such tumors and hence that the disease can be down staged and made amenable for surgery. All the tumors do not respond to the therapy equally. Hence, it becomes very important to predict the response of chemotherapy in such cases. This study evaluated the role of scintimammography in assessing the response to NACT in 23 patients with LABC. Histologically proven 23 patients of LABC were recruited in this study. Prechemotherapy tumor size was measured clinically in all patients and technitium (Tc)-99m sestamibi test was performed before NACT for each patient. Early (10 min) and delayed (2 h) image of the breast were acquired in anterior and lateral views after Tc-99m sestamibi intravenous injections and wash out rate (WOR) was computed. After 3-4 cycles of chemotherapy, surgery in the form of modified radical mastectomy was performed in 20 out of 23 patients (3 patients lost to follow-up) with pathologic evaluation of the residual tumor size. The pretherapy Tc-99m sestamibi WOR ranged from 8.3% to 68% with mean ± SD of 34.5% ±16.5%. The prechemotherapy Tc-99m sestamibi study predicted chemoresistance (WOR >45%) in 6 out of 20 patients and no chemoresistance (WOR <45%) in 14 out of 20 patients. When the WOR cut-off was set at >45%, the predictivity of the test was indicated by sensitivity of 91.7%, specificity of 62.5%, positive predictive value of 78.6%, and negative predictive value of 82.3% with a likelihood ratio of 0.1. Tc-99m sestamibi WOR is a reliable test for predicting tumor response to NACT. WOR >45% is highly predictive of chemoresistance with likelihood ratio of 0.1 than WOR <45% being predictive of chemoresponsiveness.

Ovarian granulosa cell tumor: clinical features, treatment, outcome, and prognostic factors.

BACKGROUND: Granulosa cell tumors are rare neoplasms characterized by long natural history and favorable prognosis. AIMS: The objective of this study was to determine the clinical presentation, treatment, outcome, and prognostic factors for patients of granulosa cell tumors. MATERIALS AND METHODS: A retrospective analysis of 26 patients of granulosa cell tumor of ovary from 2002 to 2011 was carried out. The records of all patients were analyzed to determine clinical presentation, treatment, survival, and prognostic factors. RESULTS: The median age of the patients was 50 years (range, 17-71 years). Abdominal pain was the most common presenting symptom. The median follow-up was 71.4 months (range, 21.6-149.9 months). The estimated 5 and 10 year overall survival (OS) was 84.6 and 72.5%, respectively. Event-free survival (EFS) was 76.5 and 52.9% at 5 and 10 years, respectively. Advanced stage was significant independent poor prognostic indicator for both OS and EFS. CONCLUSION: Majority of the patients with granulosa cell tumors of the ovary present in early stage. Surgery is the primary treatment modality for granulosa cell tumors. Advanced stage and presence of residual disease were associated with inferior survival, but only prospective studies can ascertain their definite role.

Paraneoplastic erythrocytosis in a malignant ovarian steroid cell tumour.

Neoplastic polycythemia is a condition in which high hematocrit levels result from a neoplastic lesion in the body. It is a rare condition and occur because of excess erythropoietin or androgen production by the neoplastic cells. Steroid cell tumours of the ovary though notorious for producing endocrine changes in the body have rarely been associated with polycythemia. We present the case of a post menopausal lady who developed polycythemia and features of virilisation. Further investigations revealed that she had steroid cell tumour of the left ovary. Her signs and symptoms resolved after surgical removal of the tumour.

Tetany by transfusion of small volume of blood: a rare case report.

A requisition for two units of packed red blood cells was received for a 54 year female, known case of genitourinary carcinoma. After transfusion of approximately 15-20 ml of blood, a call was received from resident in charge of radiotherapy ward stating that patient had clenching of hands along with circumoral tingling and paresthesias in her limbs. Her investigations showed decreased serum potassium and calcium levels but serum magnesium levels were not available. Multiple electrolyte disturbances probably precipitated tetany even by small volume of blood transfusion. We therefore recommend careful monitoring of electrolytes, including magnesium, before starting blood transfusion.

Conventional radiotherapy with concurrent weekly Cisplatin in locally advanced head and neck cancers of squamous cell origin - a single institution experience.

BACKGROUND: Platinum based concurrent chemo-radiation is the de-facto standard of care in the non-surgical management of locally-advanced head and neck cancer of squamous origin. Three-weekly single agent cisplatin at 100 mg/m2 concurrent with radical radiotherapy has demonstrated consistent improvement in loco-regional control and survival. This improvement is however at the cost of considerable hematologic toxicity and poor overall compliance. The routine use of this regime is improbable in developing countries with limited resources. We therefore aimed to determine the safety and efficacy of an alternative regime of weekly cisplatin and concurrent radiotherapy in such patients. MATERIALS AND METHODS: January-05 and April-12, 188 patients of locally-advanced head and neck cancer of squamous origin were treated with concurrent weekly-cisplatin at 35 mg/m2 and conventional radiotherapy 60-66Gy/30-33 fractions/5 days per week. RESULTS: Overall, 95% patients received planned doses of RT while 74% completed within the stipulated overall treatment time of <50 days. Eighty-two percent received at-least 5 weekly cycles. Grade-III/IV mucositis was seen in 58%/9% respectively, which resulted in mean weight loss of 9.2% from a pre-treatment mean of 54.5 kg. Grade-III hematologic toxicity-0.5%; grade II nephrotoxicity-2.5% and grade III emesis-3% were also seen. Grade-III/IV subcutaneous toxicity-10%/1% and grade-III/IV xerostomia-10%/0% were observed. Complete responses at the primary site, regional nodes and overall disease were seen in 86%, 89% and 83% patients respectively. The median and 5-years disease-free survival were 26 months and 39.4% respectively, while the median and overall survival were 27 months and 41.8% respectively. CONCLUSIONS: Weekly-cisplatin at 35 mg /m2 when delivered concurrently with conventional radical RT (at-least 66y/33 fractions) in locally-advanced head and neck cancer is well tolerated with minimal hematologic and neprologic toxicity and can be routinely delivered on an out-patient basis. It is an effective alternative to the standard 3-weekly cisplatin especially in the context of developing countries.

Long term survival in non-small-cell lung carcinoma with synchronous brain metastasis.

Brain metastasis is common in patients with non-small-cell lung carcinoma (NSCLC) and is associated with poor prognosis with a median survival rate below six months, even if treated with palliative radiotherapy and/or chemotherapy. Here, we report a case of NSCLC with synchronous brain metastasis treated with whole brain radiotherapy, systemic chemotherapy and oral gefitinib with survival of 62 months. We found that adequate treatment combining radiotherapy, chemotherapy and targeted therapy in a patient with non-small-cell lung cancer with brain metastasis can improve survival. This study shows how to integrate various treatment modalities in a patient with non small cell lung cancer with synchronous brain metastasis at presentation.