Synovial sarcoma: a rare neoplasm of paranasal sinus.

Synovial Sarcoma (SS) is a rare soft-tissue malignant tumour. Its presentation in the head and neck region is uncommon. Because of the complex anatomy of the head and neck region, surgery with clear margins is not achievable. In such cases, a multi-modality approach is required as there is no established standard of care. In this report, we share the case of a girl who presented with nasal obstruction. Imaging revealed a mass involving the left nasal cavity, paranasal sinuses without intracranial extension. It was diagnosed as synovial sarcoma. She underwent surgical excision and adjuvant radiation therapy (RT) to the tumour bed, followed by an incomplete course of chemotherapy. Later on, she developed systemic disease. Considering the rarity of this case and lack of guidelines for standard treatment, we report on this case to share our experience with management and treatment outcome.

Postoperative radiotherapy for high-grade primary cardiac spindle cell sarcoma: a rare entity.

A patient in her early 20s presented with blood-stained sputum and shortness of breath. Initially, she was treated for pneumonia. Later, upon exacerbation of symptoms, further investigations were done which exhibited a left atrial mass causing compression of contralateral atrium. She underwent surgical resection of the mass, which was initially mistaken as myxoma. However, histopathological correlation revealed spindle cell sarcoma with focal myogenic differentiation. This case report highlights the role of Radiation Therapy in adjuvant setting with promising impact on improving local control after R2 resection. Cardiac spindle cell sarcoma, being one of the rarest cardiac tumours reported to date, warrants establishment of a Rare Tumour Multidisciplinary Team for management of such malignancies.

Compliance and clinical efficacy of vaginal dilator after radiotherapy for cervical and endometrial malignancies.

Objective: To investigate the compliance and clinical efficacy of vaginal dilators (VDs) as an educational intervention in patients receiving pelvic radiation therapy (RT) for endometrial and cervical malignancies. Material and methods: This is a single institution, retrospective chart review. Patients undergoing pelvic RT for endometrial or cervical cancer at our center were educated about the use of a VD starting 1 month after completion of RT. The patients were assessed after 3 months of prescribing VD. The demographic details and physical examination findings were extracted from medical records. Results: We identified 54 female patients at our institution during the 6-month duration. The median mean age of patients was 54 ± 9.9 years. Twenty-four (44.4%) had endometrial cancers and 30 (55.6%) were diagnosed with cervical cancers. All patients received external beam RT, 38 (70.4%) received a dose of 45 Gy, and 16 (29.6%) patients received 50.4 Gy. Brachytherapy was also received by all patients, 28 (51.9%) received 5 Gy × 2 fractions, 4 (7.4%) received 7 Gy × 3 fractions and 22 (40.7%) received 8 Gy × 3 fractions. The compliance with VD use was 36 (66.6%) patients. Twenty-two (40.7%) used 2-3 times a week, 8 (14.8%) used <2 times per week and 6 (11.9%) used only once a month, and 18 (33.3%) did not use the VD post-treatment. Per vaginal (PV) examination findings of the patient's vagina with normal mucosa were evaluated in 32 (59.3%) and adhesions were found in 20 (37.0%) and 2 (3.7%) were unable to examine due to dense adhesions. During examination 12 (22.2%) had bleeding PV, however, the majority of the patients, 42 (77.8%) experienced no bleeding PV. Out of the 36 patients who used a VD, it was found to be efficacious in 29 (80.6%) of patients. Upon stratification of efficacy with a frequency of VD, 72.4% (n = 21) efficacy was seen in patients using frequent VD as prescribed 2-3 times per week. Conclusion: The compliance and efficacy of VD use after radiation to pelvic in cervical and endometrial cancers at 3 months follow-up were found to be 66.6% and 80.6%, respectively. This shows that VD therapy is an effective interventional tool and patients should receive specialist education about vaginal stenosis as toxicity at the outset of treatment.

Solitary rib metastasis from primary nasopharyngeal carcinoma: A rare clinical presentation.

We report a case of a middle age male who presented to our tertiary care university hospital with the complaints of nasal obstruction and decrease hearing. The CT scan of head and neck exhibited a mass in nasopharynx and enlarged bilateral cervical lymph nodes. Biopsy from nasopharynx confirmed the lesion as poorly differentiated non-keratinizing squamous cell carcinoma and staged as cT2N2M0. He received neoadjuvant chemotherapy. Subsequently, he underwent chemo radiation therapy. He represented with left chest wall pain. Imaging confirmed isolated lesion on left sided 6th rib. Rib lesion was resected followed by radiation therapy to surgical bed and systemic treatment. The patient remained disease free for 4.5 years. Later, his disease relapsed, and he died of systemic disease progression. To the best of the author's knowledge, only few cases have been reported with isolated rib metastasis from nasopharyngeal carcinoma and this is the first case in which metastasectomy was considered.

Acute dermatitis in adult female patients receiving hypofractionated radiotherapy for breast cancer: experience from a low- and middle-income country.

Radiotherapy (RT) is an important component of treatment in the management of breast cancer patients. The radiation treatment paradigm has been shifted towards hypofractionated RT. This study aims to determine the severity of acute dermatitis in patients receiving hypofractionated RT for breast cancer at a tertiary care university hospital in Pakistan. Patients with biopsy-proven invasive breast carcinoma or DCIS who were referred for radical radiotherapy after discussion in the breast tumour board were retrospectively reviewed. Physical assessment of the patients for evaluation of the severity of radiation dermatitis will be carried out in the first week, last week and on the first follow-up after 1 month of completion of RT, according to the Radiation Therapy Oncology Group/European Organisation For Research And Treatment Of Cancer (RTOG/EORTC) criteria. We identified 92 female patients in 6 months at Aga Khan University Hospital, with a mean age of 53.1 years. Most of the treated patients had clinical stage 3 (64%) cancer, while others were stage 2 (42%), stage 1 (2%) and stage 0 (2%). The surgeries performed were mastectomy in 59 patients and breast-conserving surgery in 33 patients. Histology was Intra Ductal Carcinoma (IDC) (95%), DCIS (3%) and Invasive Lobular Carcinoma (ILC) (2%). Most of the patients received chemotherapy (96%). Radiotherapy dose was 4256 cGy in 16 fractions, followed by a boost of 10 Gy. The radiation techniques used were intensity-modulated radiotherapy (47.8%) and three-dimensional conformal radiotherapy (52.2%). Most of the patients experienced no toxicity (59%), while grade I toxicity was observed in 29% of the patients and grade II toxicity was observed in 11%. Only 1% of the patients experienced grade III skin toxicity. Hypofractionated radiation therapy is beneficial because of the shorter overall treatment time which reduces the socio-economic burden, not only for patients but also for radiotherapeutic institutions. However, extended follow-up is to be reported for long-term toxicity and other consequences.

Total skin electron beam therapy (TSEBT) in management of mycosis fungoides: A case series.

A retrospective review was conducted In Aga Khan University from January 2013-December 2020 for patients who received Total Skin Electron Beam Therapy (TSEBT) on the management of Mycosis Fungoides (MF). The clinical response rate and disease-free interval (DFI) was evaluated for three patients. All of the patients received a 3600 cGy dose of radiation. Clinical complete remission (cCR) was observed in two out of three patients, while one patient showed clinical partial response. Following the cCR to TSEBT, the DFI of the first patient with stage 1B lasted 30 months, while the second patient with stage IIB remained disease free for five months. TSEBT is an effective treatment for MF disease. It can thus be expected that patients with limited disease in the early stage can acquire a cCR with a longer DFI. The clinical outcomes of our study show concordance with the literature. The patients who had extensive (T3) skin involvement and had refractory diseases showed lower DFI with radiation.

Vagal Paraganglioma: A Rare Finding in a 31-Year-Old Male.

Vagal paraganglioma is a rare finding that develops from paraganglionic tissue found around the vagus nerve; it has a prevalence of 0.012% of all tumors. It is the third most common paraganglioma of the head and neck but still accounts for less than 5% of these tumors, and it has a well-established female prevalence. It is a difficult tumor to identify early based on its symptoms alone and only a thorough investigation can help solidify its diagnosis. In this report, we discuss a presentation of this phenomenon that is not only unique in its manifestation but also a very difficult diagnosis due to its deceptive presentation and multiple extensions. These masses need a good surgical regime to be removed properly and postoperative complications are very frequent in most of these cases.

Cannabis, a Miracle Drug with Polyvalent Therapeutic Utility: Preclinical and Clinical-Based Evidence.

Cannabis sativa L. is an annual herbaceous dioecious plant which was first cultivated by agricultural human societies in Asia. Over the period of time, various parts of the plant like leaf, flower, and seed were used for recreational as well as therapeutic purposes. The main chemical components of Cannabis sativa are termed as cannabinoids, among them the key psychoactive constituent is Δ-9-tetrahydrocannabinol and cannabidiol (CBD) as active nonpsychotic constituent. Upon doing extensive literature review, it was found that cannabis has been widely studied for a number of disorders. Very recently, a pure CBD formulation, named Epidiolex, got a green flag from both United States Food and Drug Administration and Drug Enforcement Administration for 2 rare types of epilepsies. This laid a milestone in medical cannabis research. This review intends to give a basic and extensive assessment, from past till present, of the ethnological, plant, chemical, pharmacological, and legal aspects of C. sativa. Further, this review contemplates the evidence the studies obtained of cannabis components on Alzheimer's, Parkinson's, amyotrophic lateral sclerosis, multiple sclerosis, emesis, epilepsy, chronic pain, and cancer as a cytotoxic agent as well as a palliative therapy. The assessment in this study was done by reviewing in extensive details from studies on historical importance, ethnopharmacological aspects, and legal grounds of C. sativa from extensive literature available on the scientific databases, with a vision for elevating further pharmaceutical research to investigate its total potential as a therapeutic agent.

Central nervous system metastases: a rare presentation of nasopharyngeal carcinoma.

We report a case of a male patient. He presented with nasal obstruction and epistaxis. The MRI of the brain showed a mass in the nasopharynx and enlarged cervical lymph nodes. Besides this, there was an extra-axial, dural-based lesion in brain and subcentimetre nodules in both lungs. He received induction chemotherapy followed by chemoradiation therapy to the primary site and dural-based metastatic deposit. He re-presented with bilateral lower limb weakness. The MRI of the spine showed metastatic deposits within the thoracic cord parenchyma and meningeal deposits at the lumbar region. Palliative radiation was used to treat spinal cord and meningeal metastases. He died a few months later because of systemic disease progression. Considering the rarity of this presentation in nasopharyngeal carcinoma and lack of clear guidelines for standard treatment, we report this case to share our experience of management.

Utility of post mastectomy radiotherapy among patients with T1/ T2 N1 disease: A retrospective cohort study.

BACKGROUND: Pakistan has the highest incidence of breast cancer among Asian Countries but there is insufficient representation of local data addressing breast cancer treatment and outcome. We sought to determine the role of post-mastectomy radiotherapy (PMRT) in T1- T2 breast cancer with 1-3 positive axillary lymph nodes. METHODS: Data was reviewed retrospectively of total 755 patients out of which 291 received PMRT and 464 did not from two large breast cancer centres. RESULTS: With a median follow up of 78 months, 4 (4.5%) patients developed loco regional recurrence (LRR) in the PMRT group while a substantial number 74 (24.4%) recurred in the non PMRT group (p = 0.000). Loco regional free survival rate (LRFS) and overall survival rate (OS) was significantly better for PMRT patients than non-PMRT patients (P = <0.000). Multivariate analysis identified young age, lymphovascular invasion, extra capsular extension, triple negative and ER/PR negative were independent prognostic factors affecting loco regional free survival (LRFS). CONCLUSION: Disease recurrence is a substantial issue in 1-3 node group despite early stage, PMRT has an instrumental effect in improving LRFS and OS.

Impact of Bariatric Surgery in Reducing Macrovascular Complications in Severely Obese T2DM Patients.

BACKGROUND: Evidence from real-world studies have suggested a reduced rate of macrovascular complications following bariatric surgery. We undertook this meta-analysis to investigate the impact of bariatric surgery on macrovascular disease outcomes in severely obese type 2 diabetes mellitus (T2DM) patients. METHODS: An extensive literature search was performed in PubMed from inception until March 2020. All cohort studies assessing the association between bariatric surgery and macrovascular complications in severely obese T2DM patients were included. Two independent reviewers screened the articles, extracted data, and assessed the quality using the Newcastle-Ottawa Scale. The primary outcome was to assess the impact of bariatric surgery and the risk of macrovascular complications. Statistical analysis was performed using Review Manager 5.3. RESULTS: This meta-analysis comprised of five studies including 49,211 participants (75% female), of which 14,434 underwent bariatric surgery and 34,777 underwent usual care. Participants who underwent bariatric surgery had a significantly lower risk of macrovascular complications as compared to those with non-surgical interventions (RR: 0.50 [95% CI: 0.35-0.73], p = 0.0003). In the subgroup analysis, based on the geographical regions, studies conducted in the USA showed a higher reduction (RR: 0.41 [95% CI: 0.32-0.53], p < 0.00001) in macrovascular complications as compared to other parts of the world. The risk of all-cause mortality was also significantly lower in patients with bariatric surgery (RR 0.39 [95% CI: 0.30-0.50], p < 0.00001). CONCLUSION: Bariatric surgery was associated with a 50% reduction in macrovascular complications along with 61% reduction in risk of all-cause mortality in morbidly obese T2DM patients.

A multidisciplinary approach to triage patients with breast disease during the COVID-19 pandemic: Experience from a tertiary care center in the developing world.

BACKGROUND: The COVID-19 pandemic has created a need to prioritize care because of limitation of resources. Owing to the heterogeneity and high prevalence of breast cancers, the need to prioritize care in this vulnerable population is essential. While various medical societies have published recommendations to manage breast disease during the COVID-19 pandemic, most are focused on the Western world and do not necessarily address the challenges of a resource-limited setting. AIM: In this article, we describe our institutional approach for prioritizing care for patients presenting with breast disease. METHODS AND RESULTS: The breast disease management guidelines were developed and approved with the expertise of the Multidisciplinary Breast Program Leadership Committee (BPLC) of the Aga Khan University, Karachi, Pakistan. These guidelines were inspired, adapted, and modified keeping in view the needs of our resource-limited healthcare system. These recommendations are also congruent with the ethical guidelines developed by the Center of Biomedical Ethics and Culture (CBEC) at the Sindh Institute of Urology and Transplantation (SIUT), Karachi. Our institutional recommendations outline a framework to triage patients based on the urgency of care, scheduling conflicts, and tumor board recommendations, optimizing healthcare workers' schedules, operating room reallocation, and protocols. We also describe the "Virtual Blended Clinics", a resource-friendly means of conducting virtual clinics and a comprehensive plan for transitioning back into the post-COVID routine. CONCLUSION: Our institutional experience may be considered as a guide during the COVID-19 pandemic, particularly for triaging care in a resource-limited setting; however, these are not meant to be universally applicable, and individual cases must be tailored based on physicians' clinical judgment to provide the best quality care.

Demonstration of a fast and easy sample-to-answer protocol for tuberculosis screening in point-of-care settings: A proof of concept study.

We sought to develop a smooth and low cost sample preparation and DNA extraction protocol, streamlined with a ready-to-use qPCR in a portable instrument to overcome some of the existing hurdles. Several solutions were evaluated as to their ability to liquefy a mucin-based matrix. Each liquefied matrix, supplemented with either Mycobacterium tuberculosis (MTB) H37Rv strain DNA or intact cells, was aliquoted onto a filter paper embedded with solubilizing agents, and was subsequently dried up. Most of the nucleic acids, including genomic DNA from the bacilli and the host, binds to the filter paper. Next, several protocols were evaluated to elute the DNA from the paper, using qPCR to detect the insertion sequence IS6110, a M. tuberculosis complex genomic marker. The limit of detection (LOD) of the best protocol was then evaluated using parallel seeding and colony counting. The protocol was also evaluated using seventeen sputum samples, previously characterized by the GeneXpert or culture. Two instruments (the ABI7500 Standard and the Q3-Plus system) and two reagents storage formats (frozen or ready-to-use) were evaluated. Solutions containing guanidine isothiocyanate exerted the best liquefying effect on the mucin-based matrix extracted from one 6-mm punches, followed by a brief incubation at 95°C. The resulting DNA contained impurities, but a simple 1:10 dilution elicited the detection of MTB and human genomic targets. The described protocol presented an apparent LOD of 02 CFU/mL of MTB. Challenging the protocol with previously characterized samples showed substantial agreement with GeneXpert MTB/RIF results (sensitivity of 90%, agreement of 88.9%, kappa coefficient of 0.77), and moderate agreement with culture results (sensitivity of 100%, agreement of 78.9%, kappa coefficient of 0.58). This work presents a sensitive proof-of-concept protocol for sputum liquefaction and decontamination followed by a simple DNA extraction procedure, in which the extraction steps are streamlined with a ready-to-use qPCR in a portable instrument that can be employed in low infrastructure settings.

Acute Toxicity and Local Response using Three Fractions of High Dose Rate (HDR) Brachytherapy for Curative Treatment of Carcinoma Cervix.

To determine the acute vaginal mucosal toxicity and clinical response of cervical cancer after definitive treatment with external beam radiotherapy (EBRT) and three fractions each of 8 Fray (Gy) high dose rate intracavitary brachytherapy (HDR-BT). STUDY DESIGN: Descriptive study. PLACE AND DURATION: Radiation Oncology Section, Department of Oncology, The Aga Khan University Hospital, Karachi, Pakistan from January 2008 till December 2015. METHODOLOGY: Protocol was formulated for carcinoma cervix to complete treatment in 7 weeks. Patients were treated with chemotherapy and pelvic EBRT to a total dose of 45 Gy/25 fractions, followed by three intracavitary HDR brachytherapy fractions of 8 Gy each. Vaginal toxicity and local clinical response was assessed at the end of treatment, at 4 and 8 weeks. RESULTS: A total of 57 patients were treated with HDR brachytherapy and 49 patients were evaluated for assessment of toxicity and response. According to FIGO staging system, two had stage IB2, one had IIA, thirty-six had IIB, seven had IIIB, one had IVA disease and two had IVB with para aortic nodes. Concurrent gemcitabine and cisplatin were given to 26 (46%); whereas, 28 (49%) received concurrent cisplatin alone. Grade III acute vaginal mucosal toxicity was seen in 52 and Grade IV acute vaginal mucosal toxicity was observed in 08 patients. At completion of treatment, 40 patients had complete clinical response, at 4 weeks follow-up, complete regression of disease was found in 3 more and at 8 weeks none had clinical residual disease. CONCLUSION: This regimen of HDR brachytherapy treatments is feasible, efficacious, and well-tolerated for carcinoma cervix in a setup with cost constraints. Long term toxicity and disease control remains to be reported with longer follow-up. Key Words: Carcinoma cervix, High dose rate brachytherapy, Acute toxicity, Local response, External beam radiation therapy, Intracavitary brachytherapy.

Combinations of alkaline hydrogen peroxide and lithium chloride/N,N-dimethylacetamide pretreatments of corn stalk for improved biomethanation.

The purpose of this study was to improve methane generation from corn stalk (CS) through alkaline hydrogen peroxide and lithium chloride/N,N-dimethylacetamide (AHP-LiCl/DMAc) pretreatment. Changes in the structures of treated and untreated CSs were investigated, and biomass components, including cellulose, hemicellulose and lignin, were analysed. Our findings revealed that AHP-LiCl/DMAc pretreatment improved accumulative methane yield by forceful delignification and effectively destroyed the structure of cellulose. The AHP-LiCl/DMAc-treated group had a maximum methane yield of 318.6 ± 17.85 mL/g volatile solid, which was 40.08% and 10.10% higher than the maximum methane yields of the untreated and AHP-treated group, respectively. This result showed enhanced cellulose dissolution by the ionic solvent of LiCl/DMAc and improved enzymatic saccharification in fermentative bacteria without structural modifications. The AHP-LiCl/DMAc treated group had higher glucose level, acetate followed by biomethanation process. Furthermore, the decrease in crystallinity indexes for AHP-LiCl/DMAc treated group was reported. Overall, this investigation proved a promising pretreatment approach for enhancing the degradation of CS into reducing sugars and improving methane generation.

Association of autoimmunity and cancer: An emphasis on proteolytic enzymes.

Cancer and autoimmune diseases are the two devastating conditions that together constitute a leading health problem worldwide. The rising burden of these disorders in the developing world demands a multifaceted approach to address the challenges it poses. Understanding the root causes and specific molecular mechanisms by which the progression of the diseases takes place is need of the hour. A strong inflammatory background and common developmental pathways, such as activation of immune cells, proliferation, increased cell survival and migration which are controlled by growth factors and inflammatory cytokines have been considered as the critical culprits in the progression and complications of these disorders. Enzymes are the potential immune modulators which regulate various inflammatory events and can break the circulating immune complexes via macrophages production. In the current manuscript, we have uncovered the possible role of proteolytic enzymes in the pathogenesis and progression of cancer and autoimmune diseases. In the light of the available scientific literature, we advocate in-depth comprehensive studies which will shed light towards the role of proteolytic enzymes in the modulation of inflammatory responses in cancer and autoimmune diseases together.

Growth stage and molybdenum treatment affect cadmium accumulation, antioxidant defence and chlorophyll contents in Cannabis sativa plant.

Cadmium (Cd) uptake and accumulation in plant tissues is affected by physiological stage of a plant and presence of mineral nutrients in soil. We investigate the effect of micronutrient Mo (0.5, 1.0 and 2.0 ppm) on biomass, Cd accumulation, photosynthetic pigments and endogenous phenolics and soluble proline in Cannabis sativa plant grown in 25 and 50 ppm Cd polluted soil. Molybdenum was applied as seed soaking and soil addition treatments. The plants were harvested in two stages i.e. vegetative (6 weeks) and reproductive stages (12 weeks). It was found that seed soaking treatment of 1.0 ppm Mo most significantly increased biomass, Cd accumulation (1.76 ±â€¯0.19 mg Cd/DBM) and phenolics (104.5 ±â€¯4.46 ppm) concentration in the plant tissues. Molybdenum treatments highly increased Cd bio-concentration at reproductive stage as compared to vegetative stage in plants grown in 50 ppm Cd polluted soil. Translocation of Cd from roots into leaves was significantly increased by Mo treatments at reproductive stage as compared to vegetative stage. Strong inter-correlations existed between total phenolics, Cd accumulation, dry biomass and chlorophyll contents of the plant.