Sequential small cell transformation and T790M mutation in an epidermal growth factor-mutant lung adenocarcinoma: A rare occurrence with significant management implications.

BACKGROUND: Epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) resistance may be acquired via genotypic and/or phenotypic transformations. Herein, we report an extremely uncommon case with sequential small cell transformation and EGFR T790M mutation, in an elderly female with EGFR exon 21 L858R-mutant lung adenocarcinoma, following treatment with a first-generation EGFR-TKI. CASE: A 67-year-old female never-smoker presented with a cough and dyspnoea of 2 months' duration. Computerised tomography revealed a 39 mm lesion in the upper lobe of the right lung with pleural effusion. Pleural fluid cytology revealed metastatic lung adenocarcinoma, and EGFR testing revealed exon 21 L858R mutation. She was started on gefitinib. After a progression-free survival of 31 months, she presented with disease progression and multiple extra-thoracic metastases. Fine needle aspiration cytology of a chest wall lesion revealed metastatic small cell carcinoma. EGFR testing on this aspirate revealed persistent L858R mutation only. In view of small cell transformation, chemotherapy (etoposide and carboplatin) was administered. After 4 months, ascitic fluid cytology revealed metastatic adenocarcinoma with persistent L858R mutation and an acquired T790M mutation (both detected on liquid biopsy as well) indicating amplification of the adenocarcinoma clone and regression of the small cell carcinoma clone. She was then initiated on osimertinib. CONCLUSIONS: The index case highlights the significance of serial EGFR genotyping along with repeated tissue and/or blood sampling in the prompt detection of genetic and phenotypic resistance mechanisms to EGFR-TKIs. Furthermore, it lends evidence in support of the upfront treatment approaches targeting the heterogeneity of acquired EGFR-TKI resistance mechanisms.

Primary angiosarcoma of the seminal vesicle.

Angiosarcomas are rare but highly aggressive malignancies originating from lymphatic or vascular endothelial cells and may arise from any site in the body. Angiosarcomas of the genitourinary tract, especially of seminal vesicle origin, are extremely rare with only five reported cases. Surgery forms the mainstay of therapy in localised disease while adjuvant therapies are still being refined. We present the case of a 40-year old gentleman who presented with lower urinary tract symptoms and, on evaluation, was found to have a localised angiosarcoma originating in right seminal vesicle and offered laparoscopic resection, adjuvant paclitaxel (12 weekly cycles) and adjuvant radiation therapy (66 gray in 30 fractions). He developed a peritoneal nodular recurrence after 6 months of radiotherapy that was successfully salvaged with excision and metronomic chemotherapy, which he is currently receiving. Localised angiosarcomas need multimodality management despite small size. Attempts should be made for surgical salvage of limited recurrences whenever feasible.

Correlation of higher levels of soluble TNF-R1 with a shorter survival, independent of age, in recurrent glioblastoma.

The circulating levels of soluble tumor necrosis factor receptor-1 (sTNF-R1) and sTNF-R2 are altered in numerous diseases, including several types of cancer. Correlations with the risk of progression in some cancers, as well as systemic manifestations of the disease and therapeutic side-effects, have been described. However, there is very little information on the levels of these soluble receptors in glioblastoma (GBM). Here, we report on an exploratory retrospective study of the levels of sTNF-Rs in the vascular circulation of patients with GBM. Banked samples were obtained from 112 GBM patients (66 untreated, newly-diagnosed patients and 46 with recurrent disease) from two institutions. The levels of sTNF-R1 in the plasma were significantly lower in patients with newly-diagnosed or recurrent GBM than apparently healthy individuals and correlated with the intensity of expression of TNF-R1 on the tumor-associated endothelial cells (ECs) in the corresponding biopsies. Elevated levels of sTNF-R1 in patients with recurrent, but not newly-diagnosed GBM, were significantly associated with a shorter survival, independent of age (p = 0.02) or steroid medication. In contrast, the levels of circulating sTNF-R2 were significantly higher in recurrent GBM than healthy individuals and there was no significant correlation with expression of TNF-R2 on the tumor-associated ECs or survival time. The results indicate that larger, prospective studies are warranted to determine the predictive value of the levels of sTNF-R1 in patients with recurrent GBM and the factors that regulate the levels of sTNF-Rs in the circulation in GBM patients.

Primitive neuroectodermal tumor of the uterine cervix diagnosed during pregnancy: a rare case with review of literature.

Primitive neuroectodermal tumors of the cervix are very rare. A 28-year-old pregnant woman presented with a cervical mass. The tumor was staged as IB2. The biopsy from tumor was suggestive of malignant small round cell tumor. She then underwent termination of pregnancy followed by radical hysterectomy. Based on morphologic and immunohistochemical profile, a diagnosis of peripheral primitive neuroectodermal tumor of the cervix was made. The patient received adjuvant chemotherapy and radiotherapy. The patient is alive and disease-free 33 months post-surgery. The present case highlights the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix. Pregnancy should not be a barrier to early detection and treatment of this potentially aggressive tumor. The optimal treatment methods have not yet been established because of the rarity of the tumor.

Palliative approach in advanced pelvic osteosarcoma: a single centre experience of a rare disease.

BACKGROUND: Pelvic osteosarcoma is a rare and aggressive malignant neoplasm with poor outcomes. It represents only 5% of all osteosarcomas. The authors present our institute's experience in management and outcomes of five successive patients of pelvic osteosarcoma. MATERIALS AND METHODS: We retrospectively reviewed five patients of pelvis osteosarcoma treated in our institute from September 2008 to December 2010. Clinical characteristics and treatment (CCT) modality in form of surgery and chemotherapy were noted. Statistical analysis was done with regards to progression-free survival (PFS) using Kaplan-Meier survival analysis. RESULTS: The median age of the patients was 16.0 years. The median duration of symptoms was 9 months. One patient had lung metastases at presentation. All patients received systemic chemotherapy. One patient underwent surgery in the form of limb sparing approach. Three patients had partial response to treatment, one had complete response, and one had progressive disease. Median duration of PFS was 7 months only. CONCLUSIONS: Pelvic osteosarcomas are rare neoplasms with aggressive growth patterns. Survival results are poor in view of advanced stage of presentation and difficult surgical approaches. The combined modality approach is needed to improve the results.

Small cell carcinoma esophagus-A tertiary cancer center experience of a rare variant.

BACKGROUND: Esophageal cancer is quite prevalent worldwide and usually carries a poor prognosis. Histologically, although squamous cell carcinoma and adenocarcinoma predominate, small cell carcinoma (SmCC) cases have been reported. Overall, there is a paucity of literature regarding this variant. In this article, we aim to highlight this uncommon entity of carcinoma esophagus and share our experience of SmCC patients seen over a decade at our institute. METHOD: Records of patients with SmCC histology from 2010 to 2020 were assessed. Patients' demographic characteristics, clinical characteristics, treatment received, and outcomes were taken into consideration. Results were analyzed statistically using SPSS version 22. RESULTS: Fourteen patients (nine males and five females) with a median age of 57 years (range: 35 - 72 years) were analyzed. The majority of the patients 10/14 (71.4%) received palliative radiotherapy of either 30Gy/10 fractions or 35Gy/15 fractions. Only 1/14 (7.14%) patients received neoadjuvant chemotherapy and concurrent chemoradiation (CCRT). Overall, partial response was noted in all 11 patients (78.6%) who received treatment. The average median survival was 5 months (range: 1-11 months). CONCLUSION: Although the small sample size of the study prevents us from drawing a firm conclusion, we propose national and international collaborative prospective studies for framing definitive oncologic management strategies for this rare histological variant of esophageal cancer.

Role of external radiation in benign tumors: A clinical outcome and safety audit of 7 years from a tertiary care center in India.

PURPOSE: Radiotherapy (RT) is a well-established modality for treating malignancies, but its role in treatment of benign lesions has not been well explored. Herein, we present a retrospective analysis of a 7-year data regarding the benefit and the safety profile of RT for treating benign tumors in our institute. MATERIALS AND METHODS: Data of 23 patients who received RT for benign tumors from January 2015 to April 2022 were retrieved, and a retrospective analysis was conducted. All the pertinent demographic data, treatment and follow-up data were retrieved. The most common presentations were nasopharyngeal angiofibroma, vertebral hemangioma, paraganglioma, and others. The most common sites of occurrence were head and neck (43%) and paravertebral region (22%). Volumetric modulated arc therapy was the most commonly employed RT technique (39%), followed by three-dimensional conformal RT (34%) and two-dimensional conventional radiotherapy (26%). The median RT dose delivered was 36 Gy (range: 20-54 Gy). RESULTS: The median follow-up duration was 53 months (range: 3-120 months). Nine (39%) patients had progressive disease with a median time to progression of 8 months (range: 1-30 months). The median disease-free survival (DFS) was 70 months, while the 1-, 3-, and 5-year DFS rates were 97%, 88%, and 62%, respectively. Four patients (17%) died, all due to disease progression. The 1-, 3-, and 5-year overall survival rates was 97%, 85%, and 50%, respectively. The rate of radiation-induced cancer (RIC) was 0% as none of the patients had developed RIC secondary to radiation. CONCLUSION: RT is a safe and an effective option to manage benign tumors either in an adjuvant setting or in inoperable patients requiring definitive treatment, as well as in a setting to alleviate symptoms, providing excellent survival benefits. However, further prospective studies with much higher sample size are required to establish the absolute benefit and to estimate the risk of RIC, which will further direct for a better utilization of RT in treating benign tumors.

Malignant pleural mesothelioma: The disdained member of thoracic oncology!

Pleural mesothelioma is a very aggressive malignancy that arises from the pleural mesothelial cell lining and is linked strongly to prior asbestos exposure. The ban on asbestos has helped to lower the incidence, but in developing countries like India, it is expected to rise. It has an extended latency period usually progressing over decades and presents with nonspecific symptoms. It has a median survival ranging between 10-22 months. The diagnosis of malignant pleural mesothelioma is challenging and is done using computed tomography (CT), magnetic resonance imaging, or positron emission tomography-CT, with the last two predicting the resectability of the tumor better than CT alone. A pleural biopsy along with an array of immunohistochemical markers, such as p16, BRCA1 associated protein 1, and claudin-4, are required for a definitive diagnosis. Several genetic alterations have prognostic significance as well. The current histological subtype identification is indispensable for decision making because of the new therapeutic avenues being explored. The combination of nivolumab and ipilimumab-based immunotherapy outperformed platinum and pemetrexed-based chemotherapy in terms of survival benefit and improved quality of life especially for non-epithelioid subtypes. However, the latter continues to be a robust treatment option for patients with the epithelioid subtype. Surgery is recommended for resectable cases with radiotherapy being indicated in neoadjuvant, adjuvant, and palliative settings along with systemic treatment. This review article provides an overview of epidemiology, etiology, clinical manifestations, diagnostic approaches (including immunohistochemical and genetic markers), staging, and multidisciplinary approaches to current treatment for malignant pleural mesothelioma using surgery, chemotherapy, immunotherapy, and radiotherapy. It also sheds light on some recent studies (EMPHACIS, CALGB30901, Checkmate-743, etc.) that have led to significant developments in recent years with clinically meaningful results.

Sensitivity Response Analysis of Optical Surface Monitoring Systems Using the Fitzpatrick Scale: A Phantom Study.

Purpose: Optical surface monitoring systems (OSMSs) have gained substantial attention in modern radiation therapy, specifically in the context of surface guided radiation therapy, which offers real-time patient surface monitoring, ensuring accurate and effective radiation therapy treatments. The aim of this article is to evaluate the OSMS camera sensitivity toward different skin tones, categorized according to the Fitzpatrick scale, a universal classification of human skin tones, using a phantom. Methods and Materials: This study used Catalyst and Sentinel OSMSs (C-RAD). The Alderson RANDO female pelvis phantom, located at the isocenter in computed tomography simulation and treatment rooms, served as an experimental subject. Eighteen skin tone-matching cotton cloths, selected on the basis of Von Luschan chromatic and Fitzpatrick scales, were wrapped around the phantom for sensitivity evaluation. Camera sensitivity was optimized by adjusting threshold/gain (100%-600%) and integration time during individual scans in both rooms. Temporal response analysis spanned 2 months, with 16 measurements for each OSMS taken in varying light conditions. Results: The OSMSs successfully detected the surface of cloth-covered phantoms with varying mean (SD) integration times: 550 (34) to 950 (43) µs for the Sentinel system and 2300 (71) to 12,000 (400) µs for the Catalyst system. The sensitivity parameters differed for each skin tone, with lighter skin requiring shorter integration times and gain/threshold values. Darker skin tones necessitated higher parameters for optimal surface images. The reliability of the systems declined with excessive parameters, leading to noise and compromised accuracy in patient positioning. Conclusions: Optimized sensitivity parameters tailored to individual skin tones are crucial for effective real-time patient surface monitoring in radiation therapy, as variations in skin color can affect the accuracy of measurements. The precision of skin color measurements in OSMSs relies on carefully adjusting camera sensitivity parameters. However, careful consideration is essential, as larger values are required for darker skin tones, compromising reliability. This suggests the need for exploring alternative image guidance methods for patients with darker skin tones.

Periprosthetic metastases in carcinoma of unknown primary: A rare association.

ABSTRACT: Septic or aseptic loosening may cause bone loss around artificial prosthesis leading to prosthesis failure. This occurrence due to metastatic infiltration of bone or surrounding soft tissues is rare but has been occasionally reported. We report a case of an elderly lady presenting with swelling and pain at the site of previous hemiarthroplasty performed for traumatic injury. On evaluation, she was found to have a lytic femur lesion with a large soft-tissue component around the prosthetic joint. Biopsy suggested a metastatic carcinoma of renal origin, but screening of kidneys did not reveal any primary lesion. She had additional skeletal metastatic lesions but no other primary site was detected either. She was given palliative radiotherapy and systemic therapy (sunitinib) based on the histologic diagnosis of renal cell origin but did not tolerate it. Thereafter, she is continuing on zoledronate every 4 weeks and best supportive management since 4 months from diagnosis.

Simultaneous Integrated Boost (SIB) Versus Sequential Boost in Anal Cancer Patients: A Single-Center Experience.

PURPOSE: Concurrent chemoradiation is the standard of care for the treatment of anal cancer. Radiation can be delivered by sequential or simultaneous integrated boost (SIB) approach. The present study was conducted to compare the treatment outcomes and toxicity profile of patients with anal cancer treated with sequential boost and SIB approach. METHODS: A single-institution retrospective analysis of patients with squamous cell carcinoma of the anal canal treated between 2019 and 2022 with radical chemoradiation was performed. The sequential boost schedule consisted of 45 Gy in 25 fractions (1.8 Gy daily) to the gross tumor, nodes, and elective nodal volume, followed by a 9 Gy in five fractions boost to the gross disease. Patients receiving SIB were treated as per RTOG 0529 protocol. In both the groups, patients were treated with volumetric modulated arc therapy (VMAT). The two groups were compared in terms of overall survival (OS), colostomy-free survival (CFS), relapse-free survival (RFS), and acute toxicity profile. p-values < 0.05 were considered statistically significant. RESULTS: The patient and disease characteristics in both treatment arms were comparable. The only difference was a significantly longer overall treatment time of ≥ 50 days in the sequential arm (77.8% vs 43.8%, p = 0.04). The median follow-up was 18 months. The 2-year CFS was 80% in sequential vs 87.5% at 2 years for the SIB arm, 2-year OS 83.3% vs 58.6%, and 2-year RFS was 38.9% vs 41.7%, respectively. A total of 14 (77.8%) in sequential and 8 (50%) in the SIB arm had disease relapse. On univariate analysis, the involved pelvic lymph node significantly affected OS (HR 10.45, p = 0.03) while inguinal lymph node involvement adversely affected RFS (HR 6.16, p = 0.02). The most common acute toxicity was radiation-induced dermatitis, 15 (83.4%; 5 grade II, 10 grade III) in sequential vs 7 (43.8%; 3 each grade II and III) in the SIB group followed by hematological (61.1% vs 68.75%). However, the incidence of overall acute toxicities was significantly less in the SIB arm (p = 0.006). CONCLUSION: Our study showed that concurrent chemoradiation with the SIB-VMAT approach is well tolerated in patients of anal carcinoma and resulted in lesser treatment interruptions and comparable outcomes as compared to the sequential approach. Our results warrant further evaluation in a prospective study.

Cholangiocarcinoma: Recent Advances in Molecular Pathobiology and Therapeutic Approaches.

Cholangiocarcinomas (CCA) pose a complex challenge in oncology due to diverse etiologies, necessitating tailored therapeutic approaches. This review discusses the risk factors, molecular pathology, and current therapeutic options for CCA and explores the emerging strategies encompassing targeted therapies, immunotherapy, novel compounds from natural sources, and modulation of gut microbiota. CCA are driven by an intricate landscape of genetic mutations, epigenetic dysregulation, and post-transcriptional modification, which differs based on geography (e.g., for liver fluke versus non-liver fluke-driven CCA) and exposure to environmental carcinogens (e.g., exposure to aristolochic acid). Liquid biopsy, including circulating cell-free DNA, is a potential diagnostic tool for CCA, which warrants further investigations. Currently, surgical resection is the primary curative treatment for CCA despite the technical challenges. Adjuvant chemotherapy, including cisplatin and gemcitabine, is standard for advanced, unresectable, or recurrent CCA. Second-line therapy options, such as FOLFOX (oxaliplatin and 5-FU), and the significance of radiation therapy in adjuvant, neoadjuvant, and palliative settings are also discussed. This review underscores the need for personalized therapies and demonstrates the shift towards precision medicine in CCA treatment. The development of targeted therapies, including FDA-approved drugs inhibiting FGFR2 gene fusions and IDH1 mutations, is of major research focus. Investigations into immune checkpoint inhibitors have also revealed potential clinical benefits, although improvements in survival remain elusive, especially across patient demographics. Novel compounds from natural sources exhibit anti-CCA activity, while microbiota dysbiosis emerges as a potential contributor to CCA progression, necessitating further exploration of their direct impact and mechanisms through in-depth research and clinical studies. In the future, extensive translational research efforts are imperative to bridge existing gaps and optimize therapeutic strategies to improve therapeutic outcomes for this complex malignancy.

Survival and failure outcomes in locally advanced esthesioneuroblastoma: a single centre experience of 15 patients.

Esthesioneuroblastoma (ENB) constitutes 3 % of all malignant intranasal tumors. As the tumor is very rare, the number of patients of ENB treated in individual departments is small. We present our institute's experience in combined modality management of 15 successive patients of ENB treated from 2006 to 2010. Clinical characteristics and treatment modality in form of surgery, radiotherapy and chemotherapy were noted. Kadish stage C was the most common stage (12 patients) followed by stage B (3 patients). Fourteen patients underwent primary surgery, of which nine had total excision and five had subtotal excision. One patient was treated with combination of chemotherapy (CT) and radiotherapy (RT). Median RT dose delivered was 54 Gy. Twelve patients received CT with cisplatin and etoposide. Overall, eight patients had complete response, five had partial response, while one had static disease and progressive disease each. Two patients had distant metastases. Four-year loco-regional control (LRC) was 25 % and 4-year overall survival (OS) was 45 %. Most common presentation in our series was locally advanced tumors. Most of these patients require adjuvant RT, which helps in significant LRC. Systemic CT benefits in inoperable, advanced and high risk tumors. Risk-adapted and multimodality approach is the need of hour to achieve good control rates while minimizing treatment related toxicity.

Paratesticular rhabdomyosarcoma in young adults: A tertiary care institute experience.

INTRODUCTION: Paratesticular rhabdomyosarcoma (RMS) is a rare tumor arising from the mesenchymal tissues of the spermatic cord, epididymis, testis and testicular tunics. It represents only 7% of all patients entered in the Intergroup Rhabdomyosarcoma Study (IRS) and 17% of all malignant intrascrotal tumors in children less than 15 years old. We present our experience in combined modality management of 10 successive patients of paratesticular RMS. MATERIAL AND METHODS: We retrospectively reviewed 10 patients of paratesticular RMS treated in our institute from July 2004 to December 2010. Clinical characteristics and treatment modality in form of surgery and chemotherapy (CCT) were noted. Statistical analysis was done with regards to progression-free survival (PFS) and overall survival (OS) using Kaplan-Meier survival analysis. RESULTS: The median age of the patients was 16.5 years. The median duration of symptoms was 5 months. Five patients had retroperitoneal lymphadenopathy (RPLAP) while three had lung metastases and one had orbital metastases. All patients underwent high inguinal orchidectomy followed by systemic chemotherapy (CCT). Retroperitoneal node dissection was not a required staging procedure. Four patients had partial response to treatment while six had complete response. Mean duration of PFS was 48 months and mean OS was 56 months. CONCLUSIONS: Paratesticular RMS are rare neoplasms with aggressive growth patterns. Cure rates have dramatically improved and 60% of patients in our series had complete response. This success is due to development of multimodality and risk adapted treatment approaches.

Primary retroperitoneal squamous cell carcinoma: a literature review.

PURPOSE: Retroperitoneal squamous cell carcinoma is an extremely rare histological variant of retroperitoneal tumors. The exact etiology and origin of the same is still unknown. To date, only a few case reports have been published in the literature. Due to rarity, standard treatment protocol is not available. The article aims to review the literature and treatment options available for this uncommon entity, based on available data. METHODS: We searched the databases like PubMed, PubMed Central, Scopus, and Google Scholar with the keywords "Squamous cell carcinoma" and "retroperitoneal tumors". Peer-reviewed and recent articles were screened. RESULTS: Seven relevant articles comprising 14 cases were found. Due to the small number of reports, tabulation of treatment details and outcome was done. Like the sarcoma variant, these tumors also present with a large mass in the abdomen and abdominal discomfort. Association with human papillomavirus appears to be the most common factor that gives rise to squamous histology. CONCLUSION: Retroperitoneal SCC is an uncommonly diagnosed entity. Although no specific treatment guidelines exist for this uncommon malignancy; surgery followed by adjuvant or definitive radiotherapy with concurrent chemotherapy (in inoperable cases) seems a feasible option. Multicentre trials should be conducted for establishing definitive treatment strategies for this ailment.

Malignant Peripheral Nerve Sheath Tumor Arising from Small Bowel Mesentery: an Extremely Rare Case with Review of Literature.

PURPOSE: Malignant peripheral nerve sheath tumor (MPNST) of small bowel mesentery is a rare tumor. We report a rare case of MPNST of small bowel mesentery in a patient without neurofibromatosis (NF). METHODS: A 50-year-old male, with no features suggestive of NF1, presented to us with complaints of pain abdomen. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a mass in the infrarenal region. On laparotomy, mass was seen to be arising from the mesentery of the jejunum. En-bloc resection of the tumor was done, and histopathological examination was suggestive of malignant peripheral nerve sheath tumor of the small bowel mesentery. RESULT: Patient received adjuvant external beam radiotherapy to a dose of 50.4 Gy to the tumor bed. The patient was planned for chemotherapy but absconded and later came with recurrence. The patient finally succumbed to disease. CONCLUSION: Surgery is the mainstay of treatment. Adjuvant treatment should be based on histopathological report.

Delineating and sparing the ileal conduit in adjuvant radiotherapy for bladder cancer with modulated radiotherapy.

Purpose: We undertook a prospective planning study to describe the delineation of ileal conduit (IC) loop on radiotherapy planning computed tomography (RTP CT) scan as an organ at risk (OAR) and its sparing using volumetric modulated arc therapy (VMAT) during adjuvant irradiation of bladder malignancies. Materials and Methods: Fifteen patients with bladder malignancy needing adjuvant radiotherapy postoperatively and having normal renal function underwent delayed phase RTP CT from June 2020 to March 2021, with certain modifications (Foley's catheter through stoma, additional delayed scans). We identified the course of ureters, external stoma, IC, and uretero-ileal (right and left) anastomotic sites. VMAT plans were generated. Results: A step-by-step description is given. Genitourinary OARs include kidneys, ureters, uretero-ileal anastomoses, and IC. The contrast on delayed scan opacifies ureters and IC. IC can be seen three-dimensionally as a structure with two fixed ends (blind proximal end anterior to the right sacroiliac joint and the open distal end over the right anterior abdominal wall in parasagittal location) and a 15-20 cm hanging intraabdominal loop that lies adjacent to the right iliac vessels. For prescription doses (PD) of 50.4 gray and 54 gray, respectively, VMAT plan achieved IC dose maximum to less than PD and V50 lower than 10 cc. Stoma sparing traditionally used as a surrogate for IC sparing is insufficient due to the variable intraabdominal location of IC loop. Conclusions: Delineation of IC as an OAR is feasible with slight modifications in the RTP protocols. VMAT (or other forms of intensity modulated radiation therapy) can help IC sparing and should be considered when it lies in close proximity to target volumes and the risk of additional morbidity is considerable.

Treatment outcomes of adjuvant radiotherapy in adrenocortical carcinoma - A 13-years experience from a tertiary care centre.

Purpose: Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm, usually diagnosed in advanced stage. Role and efficacy of adjuvant radiotherapy has not been well defined. The objective of this study is to describe the various clinical characteristics and prognostic factors affecting the survival of ACC along with the role radiotherapy on overall survival and relapse free survival. Methods: A retrospective analysis of 30 patients registered between 2007 and 2019 was carried out. The medical records containing clinical and treatment details were analysed. Data was analysed using SPSS 25.0. Survival curves were computed using Kaplan-Meier method. Univariate and multivariate analyses were performed to analyze the prognostic factors affecting the outcome. A p value of less than 0.05 was considered to be statistically significant. Results: The median age of patients was 37.5 years (range, 5-72 years). 20 patients were females. Twenty-six patients had advanced stage (III/IV) disease while only four patients presented in early stage. Twenty-six patients underwent total adrenalectomy. Eighty three percent patients received adjuvant radiation therapy. The median follow up was 35.5 months (range, 7 monthss-132months). The estimated three- and 5-years overall survival (OS) was 67.2% and 23.3%, respectively. Capsular invasion and positive margins were the independent prognostic factors influencing both OS and relapse free survival (RFS). Out of 25 patients who received adjuvant radiation, only three patients had local relapse. Conclusion: ACC is a rare and aggressive neoplasm with majority of patients presenting in advanced stage. Surgical resection with negative margins remains the mainstay of treatment. Capsular invasion and positive margins are independent prognostic factors for survival. Adjuvant radiation reduces the risk of local relapse and is well tolerated. Radiation can be used effectively in adjuvant and palliative settings in ACC.

Sarcomas of uterine cervix: clinicopathological features, treatment, and outcome.

OBJECTIVE: Sarcomas constitute less than 1% of all cervical malignancies. The objective of this study was to determine the presentation, pathological findings, treatment, and outcome of patients with cervical sarcoma. METHODS AND MATERIALS: A retrospective analysis of 8 cases of cervical sarcoma diagnosed over a 4-year period from 2006 to 2009 was carried out. The medical records of all patients were reviewed. All pathologic specimens were reviewed by a single pathologist. RESULTS: Of 1804 patients with cervical malignancies, 8 cervical sarcomas were identified. All patients presented with vaginal bleeding and discharge. The lesions were clinically staged as IB2 (3), II B (1) and IIIB (4). Three patients had leiomyosarcoma, 4 patients had a diagnosis of undifferentiated endocervical sarcoma, and one had embryonal rhabdomyosarcoma. Of the 8 patients, 3 absconded after diagnosis. Primary surgery was done in 3 patients of which 2 patients received adjuvant radiotherapy and chemotherapy and one patient absconded after surgery. Primary radiotherapy was given in 2 patients. Three of 8 patients treated with combined modality treatment remain alive and disease free at the last follow-up. CONCLUSIONS: Cervical sarcomas are rare neoplasms and represent a spectrum on histopathology. Most patients present with vaginal bleeding and a bulky cervical mass at the time of diagnosis. The optimal management of these tumors is uncertain owing to its rarity; however, combined modality treatment can result in prolonged survival and cure.

Pediatric astroblastoma: a rare case with a review of the literature.

Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. We present a case of an 11-year-old girl who presented with headache and blurring of vision for 2 months. A well-demarcated mass was found in the right frontoparietal lobe on a brain MRI. The patient was treated with total tumor resection followed by postoperative radiotherapy. Histologically, the features were suggestive of high-grade astroblastoma. The patient is alive and disease free 23 months after surgery. The characteristic radiological and histopathological features and treatment of this case are described with a literature review.