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1.
Int J Cancer ; 154(8): 1413-1422, 2024 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-38088458

RESUMEN

The study aims to investigate the patient-reported cognitive deficits and objective neuropsychological functions in younger adult (YA) sarcoma patients (16-40 years of age). Ninety patients and 30 age-matched healthy controls from a single tertiary healthcare hospital, were recruited into four groups: Pre-chemotherapy (Pre Cx), During chemotherapy (During Cx), Post-chemotherapy (Post Cx) and Controls. Neurocognitive functions were assessed subjectively using FACT-Cog v3 questionnaire; objectively using ACE-III and neuropsychological tests (NPT). FACT-Cog scores of During Cx (P = .041) and Post Cx (P = .008) groups were significantly lower than Pre Cx group. ACE-III scores of During Cx (P = .048) and Post Cx (P = .043) groups were lower as compared to Pre Cx group. In addition, reaction times and accuracies of the NPT (Flanker's, Sternberg's and Emotional Stroop tests) were worse (P < .05) in During Cx and Post Cx groups as compared to either Pre Cx or control groups. In the Post Cx group, the dose of chemotherapy showed significant negative correlation with the Sternberg reaction time (P = .040) as well as the scores of language (P = .047), and attention (P = .044) domains of ACE-III. Observations demonstrate that cancer/chemotherapy-related neurocognitive deficits fail to improve even after cessation of treatment, and high dosage of chemotherapy used, could be an underlying factor. This emphasizes the need for developing 'model of care' in these patients for monitoring the side effects, and possible titration in the therapeutic regimen for sarcoma in YA.


Asunto(s)
Trastornos del Conocimiento , Disfunción Cognitiva , Sarcoma , Adulto , Humanos , Atención Terciaria de Salud , Disfunción Cognitiva/inducido químicamente , Trastornos del Conocimiento/inducido químicamente , Trastornos del Conocimiento/psicología , Sarcoma/tratamiento farmacológico , Medición de Resultados Informados por el Paciente , Cognición
2.
Eur J Nucl Med Mol Imaging ; 51(1): 233-244, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37642703

RESUMEN

PURPOSE: In the context of radioiodine-resistant follicular-cell derived thyroid cancers (RAI-R-FCTC), [18F]F-FDG PET/CT serves as a widely used and valuable diagnostic imaging method. However, there is growing interest in utilizing molecular imaging probes that target cancer-associated fibroblasts (CAFs) as an alternative approach. This study sought to compare the diagnostic capabilities of [68Ga]Ga-DOTA.SA.FAPi and [18F]F-FDG PET/CT in patients with RAI-R-FCTC. METHODS: In this retrospective study, a total of 117 patients with RAI-R-FCTC were included. The study population consisted of 68 females and 49 males, with a mean age of 53.2 ± 11.7 years. The aim of the study was to perform a comprehensive qualitative and quantitative assessment of [68Ga]Ga-DOTA.SA.FAPi and [18F]F-FDG PET/CT scans in RAI-R-FCTC patients. The qualitative assessment involved comparing patient-based and lesion-based visual interpretations of both scans, while the quantitative assessment included analyzing standardized uptake values corrected for lean body mass (SULpeak and SULavg). The findings obtained from the scans were validated by correlating them with morphological findings from diagnostic computed tomography and/or histopathological examination. RESULTS: Among the 117 RAI-R-FCTC patients, 60 had unilateral local disease, and 9 had bilateral lesions with complete concordance in the detection rate on both PET scans. [68Ga]Ga-DOTA.SA.FAPi had a higher detection rate for lymph nodes (95.4% vs 86.6%, p<0.0001), liver metastases (100% vs. 81.3%, p<0.0001), and brain metastases (100% vs. 39%, p<0.0001) compared to [18F]F-FDG. The detection rates for pleural and bone metastases were similar between the two radiotracers. For lung metastases, [68Ga]Ga-DOTA.SA.FAPi showed a detection rate of 81.7%, whereas [18F]F-FDG had a detection rate of 64.6%. Remarkably, [68Ga]Ga-DOTA.SA.FAPi was able to detect a bowel metastasis that was missed on [18F]F-FDG scan. The median standardized uptake values (SUL) were generally comparable between the two radiotracers, except for brain metastases (SULpeak [68Ga]Ga-DOTA.SA.FAPi vs. [18F]F-FDG: 13.9 vs. 6.7, p-0.0001) and muscle metastases (SULpeak [68Ga]Ga-DOTA.SA.FAPi vs. [18F]F-FDG: 9.56 vs. 5.62, p-0.0085), where [68Ga]Ga-DOTA.SA.FAPi exhibited higher uptake. CONCLUSION: The study results demonstrate the superior performance of [68Ga]Ga-DOTA.SA.FAPi compared to [18F]F-FDG PET/CT in detecting lymph nodal, liver, bowel, and brain metastases in patients with RAI-R-FCTC. These findings highlight the potential of [68Ga]Ga-DOTA.SA.FAPi as a theranostic tool that can complement the benefits of [18F]F-FDG PET/CT in the imaging of RAI-R-FCTC.


Asunto(s)
Neoplasias Encefálicas , Quinolinas , Neoplasias de la Tiroides , Femenino , Masculino , Humanos , Adulto , Persona de Mediana Edad , Fluorodesoxiglucosa F18 , Radioisótopos de Galio , Radioisótopos de Yodo , Tomografía Computarizada por Tomografía de Emisión de Positrones , Estudios Retrospectivos , Tomografía de Emisión de Positrones , Neoplasias de la Tiroides/diagnóstico por imagen
3.
BMC Cancer ; 23(1): 639, 2023 Jul 08.
Artículo en Inglés | MEDLINE | ID: mdl-37422615

RESUMEN

Synovial sarcoma (SS) is one of the commonest non-rhabdomyosarcoma soft tissue sarcoma with limited treatment options in the relapsed and advanced settings. The combination of gemcitabine and docetaxel has demonstrated its role predominantly in leiomyosarcoma and pleomorphic sarcomas but has not been prospectively studied in SS. This trial assesses the efficacy, tolerability and quality of life (QoL) with this regimen in metastatic/unresectable locally advanced relapsed SS.Patients and methods This was a single-arm, two-stage, phase II, investigator-initiated interventional study among patients with metastatic or unresectable locally advanced SS who had progressed after at least one line of chemotherapy. Gemcitabine 900 mg/m2 on days 1 and 8 and docetaxel 75 mg/m2 on day 8 were administered intravenously every 21 days. The primary endpoint was 3-month progression-free rate (PFR); overall survival (OS), progression-free survival (PFS), overall response rate (ORR), safety and quality of life (QoL) constituted the secondary endpoints.Results Twenty-two patients were enrolled between March 2020 and September 2021 and the study had to be closed early due to slow accrual. The study population comprised of 18 (81.8%) patients with metastatic disease and 4 (18.2%) patients with locally advanced, unresectable disease. The most common primary sites of disease were extremity in 15 (68%) and the median number of lines of prior therapies received was 1 (range 1-4). 3-month PFR was 45.4% (95% CI 24.8-66.1) and ORR was 4.5%. Median progression-free survival (PFS) was 3 months (95% CI 2.3-3.6) and median OS was 14 months (95% CI 8.9-19.0). 7 (31.8%) patients experienced grade 3 or worse toxicities, including anemia (18%), neutropenia (9%) and mucositis (9%). QoL analysis demonstrated significant decline in certain functional and symptom scales, while financial and global health scales remained stable.Conclusion This is the first prospective study on the combination of gemcitabine and docetaxel performed specifically in patients with advanced, relapsed SS. Although the accrual of patients could not be completed as planned, the therapy did produce clinically meaningful outcomes and met its primary endpoint of 3-month PFR. This result, along with the manageable toxicity profile and stable global health status on QoL analysis, should encourage further studies.Trial registration This trial was prospectively registered under the Clinical Trials Registry of India on 26/02/2020 (Registration number: CTRI/2020/02/023612).


Asunto(s)
Neutropenia , Sarcoma Sinovial , Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Docetaxel/uso terapéutico , Gemcitabina , Calidad de Vida , Sarcoma Sinovial/tratamiento farmacológico , Estudios Prospectivos , Desoxicitidina , Recurrencia Local de Neoplasia/tratamiento farmacológico , Sarcoma/patología , Neoplasias de los Tejidos Blandos/patología , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Resultado del Tratamiento
4.
Future Oncol ; 19(31): 2135-2145, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37860850

RESUMEN

Soft tissue sarcomas (STS) are rare heterogenous tumors derived from mesenchymal tissue. While surgery represents the primary treatment modality, the high recurrence rates following surgery alone necessitate consideration for systemic therapy in high-risk sarcomas. Despite multiple trials and meta-analyses over the last 3 decades, the role of chemotherapy remains controversial. It is crucial to accurately identify patients with high-risk diseases who may benefit the most from adjuvant and/or neoadjuvant chemotherapy. There is renewed interest in the potential to improve outcomes in localized resectable STSs with the addition of targeted and immunotherapeutic strategies. The review presented here is a summary of current evidence on systemic therapy in resectable localized STSs of the trunk and extremities to facilitate clinician decision-making.


Asunto(s)
Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Quimioterapia Adyuvante , Sarcoma/tratamiento farmacológico , Sarcoma/patología , Terapia Combinada , Terapia Neoadyuvante , Neoplasias de los Tejidos Blandos/tratamiento farmacológico
5.
Support Care Cancer ; 30(12): 10089-10098, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36350382

RESUMEN

INTRODUCTION: Data on the impact of desmoid type fibromatosis (DTF) on emotional distress and health-related quality of life (HRQoL) is sparse. METHODOLOGY: In this prospective cross-sectional study, patients with DTF and healthy controls were asked to fill the EORTC QLQ-C30, GAD-7, and PHQ-9 questionnaires. The objectives were to determine HRQoL, anxiety, and depression in patients with DTF. RESULTS: Two hundred four subjects (102 DTF patients and 102 healthy controls) were recruited. The median age of DTF patients at recruitment into the study was 31 years (IQR, 25-37 years). There was a female preponderance with a male:female ratio of 1:1.83. Appendicular skeleton and abdomen sites were most commonly involved in 59% and 22.5% respectively. About half (54%) of patients were currently on sorafenib and 41% were under active surveillance. The mean global health status in DTF patients was 65.58 ± 22.64, significantly lower than healthy controls. Similarly, DTF patients scored low on all functional scales except cognitive functioning. The symptom scale showed a significantly higher symptom burden of fatigue, pain, insomnia, and financial difficulties. Anxiety and depression was observed in 39.22% and 50% of DTF patients respectively. DTF patients had higher rates of mild, moderate, and severe anxiety and depression compared to healthy controls. CONCLUSION: DTF patients have significant symptom burden, poor functioning, and heightened anxiety and depression as compared to healthy controls. HRQoL, anxiety, and depression should be routinely used to assess symptom burden and treatment efficacy in DTF patients.


Asunto(s)
Fibromatosis Agresiva , Calidad de Vida , Humanos , Masculino , Femenino , Adulto , Calidad de Vida/psicología , Depresión/epidemiología , Depresión/etiología , Depresión/psicología , Estudios Transversales , Fibromatosis Agresiva/psicología , Estudios Prospectivos , Ansiedad/epidemiología , Ansiedad/etiología , Ansiedad/psicología , Encuestas y Cuestionarios
6.
Indian J Med Res ; 155(5&6): 546-553, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36348601

RESUMEN

Background & objectives: High mortality has been observed in the cancer population affected with COVID-19 during this pandemic. We undertook this study to determine the characteristics and outcomes of cancer patients with COVID-19 and assessed the factors predicting outcome. Methods: Patients of all age groups with a proven history of malignancy and a recent diagnosis of SARS-CoV-2 infection based on nasal/nasopharyngeal reverse transcriptase (RT)-PCR tests were included. Demographic, clinical and laboratory variables were compared between survivors and non-survivors groups, with respect to observed mortality. Results: Between May 11 and August 10, 2020, 134 patients were included from the three centres and observed mortality was 17.1 per cent. The median age was 53 yr (interquartile range 39-61 yr) and thirty four patients (25%) were asymptomatic. Solid tumours accounted for 69.1 per cent and breast cancer was the most common tumour type (20%). One hundred and five patients (70.5%) had received chemotherapy within the past four weeks and 25 patients (19.3%) had neutropenia at presentation. On multivariate analysis, age [odds ratio (OR) 7.99 (95% confidence interval [CI] 1.18-54.00); P=0.033], haemoglobin [OR 6.28 (95% CI 1.07-37.04); P=0.042] neutrophil-lymphocyte ratio [OR 12.02 (95% CI 2.08-69.51); P=0.005] and baseline serum albumin [OR 18.52 (95% CI 2.80-122.27); P=0.002], were associated with higher mortality. Recent chemotherapy, haematological tumours type and baseline neutropenia did not affect the outcome. Interpretation & conclusions: Higher mortality in moderate and severe infections was associated with baseline organ dysfunction and elderly age. Significant proportion of patients were asymptomatic and might remain undetected.


Asunto(s)
COVID-19 , Neoplasias , Neutropenia , Humanos , Anciano , Persona de Mediana Edad , Estudios Retrospectivos , SARS-CoV-2 , India/epidemiología , Neoplasias/complicaciones , Neoplasias/epidemiología , Neutropenia/complicaciones
7.
Future Oncol ; 17(7): 825-835, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33533642

RESUMEN

Extraskeletal osteosarcoma is a very infrequently diagnosed soft-tissue sarcoma subtype which has identical histological features to bone osteosarcoma. However, its demographics, presentation, radiology and treatment strategy differ from those of osteosarcoma. Its diagnosis can be at times challenging due to radiological and pathological mimics which have more common incidence. A multimodality approach is essential for optimizing the outcomes in extraskeletal osteosarcoma. Although there are certain caveats on inclusion of adjuvant therapies (radiotherapy and chemotherapy), in all cases surgical resection with wide local margins is considered the gold standard for adequate local control. The outcome in advanced disease remains dismal and there is a huge unmet need for prospective studies addressing the optimal treatment strategy. In this article, we review the evidence available for the management of extraskeletal osteosarcoma.


Asunto(s)
Osteosarcoma , Humanos , Osteosarcoma/diagnóstico , Osteosarcoma/epidemiología , Osteosarcoma/patología , Osteosarcoma/terapia
8.
Monaldi Arch Chest Dis ; 91(3)2021 Mar 31.
Artículo en Inglés | MEDLINE | ID: mdl-33794589

RESUMEN

Inflammatory myofibroblastic tumors (IMT) are rare soft tissue tumors of intermediate malignant potential occurring usually in children and adolescents. Treatment options for advanced diseases are limited. A 35-year-old lady presented to us with fever, cough and decreased appetite. On evaluation, she was diagnosed with left lung IMT. She underwent surgery and developed recurrence with pleural nodules after two years. Immunohistochemistry showed positivity for ALK (diffuse). Since recent evidence suggested that crizotinib is effective in advanced IMT with 50% response rates, she was treated with crizotinib 250mg BD with which she had a complete radiological response at three months. She has completed one year of treatment thus far and continues to be in complete remission. Treatment with ALK inhibitors like crizotinib has brought about a paradigm shift in the management of advanced ALK-positive IMT's with excellent clinical responses which are durable in a majority of cases.


Asunto(s)
Neoplasias Pulmonares , Adolescente , Adulto , Quinasa de Linfoma Anaplásico/genética , Niño , Crizotinib/uso terapéutico , Femenino , Humanos , Pulmón , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico
9.
Ultrastruct Pathol ; 44(2): 249-254, 2020 Mar 03.
Artículo en Inglés | MEDLINE | ID: mdl-32164472

RESUMEN

Mucosal malignant melanoma of the head and neck (HN) is a rare and aggressive neoplasm which constitutes only 1% of all melanomas. Neuroendocrine differentiation is an extremely unusual phenomenon in mucosal melanomas, of which five cases have been reported. We report a rare case of a 63-year-old female who developed sinonasal amelanotic melanoma with immunohistochemical expression of neuroendocrine markers, presenting a diagnostic dilemma. Ultrastructural evidence of melanosomes and neurosecretory granules aided in arriving at the diagnosis. Aberrant immunoexpression of neuroendocrine markers, particularly in an amelanotic melanoma, has critical diagnostic implications, as various malignancies with undifferentiated histomorphology occur at this site, many of which stain positively with neuroendocrine markers. We discuss the differential diagnoses and recommend a high index of suspicion so as not to miss the diagnosis of mucosal melanoma at this location.


Asunto(s)
Melanoma Amelanótico/diagnóstico , Melanoma Amelanótico/ultraestructura , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/ultraestructura , Biomarcadores de Tumor/análisis , Carcinoma Neuroendocrino/patología , Femenino , Humanos , Melanoma Amelanótico/patología , Microscopía Electrónica de Transmisión , Persona de Mediana Edad , Neoplasias de los Senos Paranasales/patología
11.
Future Oncol ; 11(10): 1463-70, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25963424

RESUMEN

Buparlisib (formerly BKM 120), an oral 2,6-dimorpholino pyrimidine derivative is a potent pan-PI3K inhibitor causing inhibition of PI3K downstream signaling including downregulation of p-Akt and p-S6R and apoptosis of cancer cells. Buparlisib is rapidly absorbed, has more than 90% bioavailability, good blood-brain barrier penetration and half-life of 40 h. Phase I trials have shown good disease control rate with tolerable toxicity profile at the recommended doses of 100 mg. The most common adverse events noted with buparlisib are rash, hyperglycemia, derangement of liver functions and psychiatric events. Several clinical trials with buparlisib alone or in combination with chemotherapy and targeted therapies are underway. Buparlisib has not yet been approved for regular use. Further randomized trials are required before buparlisib is approved for treatment of breast cancer.


Asunto(s)
Aminopiridinas/uso terapéutico , Antineoplásicos/uso terapéutico , Neoplasias de la Mama/tratamiento farmacológico , Morfolinas/uso terapéutico , Aminopiridinas/química , Aminopiridinas/farmacología , Animales , Antineoplásicos/química , Antineoplásicos/farmacología , Ensayos Clínicos Fase I como Asunto , Ensayos Clínicos Fase II como Asunto , Evaluación Preclínica de Medicamentos , Femenino , Humanos , Morfolinas/química , Morfolinas/farmacología , Fosfatidilinositol 3-Quinasas/metabolismo , Inhibidores de las Quinasa Fosfoinosítidos-3 , Inhibidores de Proteínas Quinasas , Proteínas Proto-Oncogénicas c-akt/metabolismo , Transducción de Señal/efectos de los fármacos , Serina-Treonina Quinasas TOR/metabolismo , Resultado del Tratamiento
12.
Future Oncol ; 11(8): 1191-200, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25832876

RESUMEN

BACKGROUND: Gemcitabine-platinum (Gem-P) is the current standard for patients with advanced gall bladder cancer. MATERIALS & METHODS: This is retrospective analysis of a prospectively maintained database of 210 patients with advanced gall bladder cancer treated with Gem-P between January 2012 and September 2013. RESULTS: Median age was 53 years, 65.2% females. In total,158 patients had metastatic and 52 had locoregional disease. Median number of cycles was 5 (1-12). At a median follow-up of 10 months, median overall survival/progression-free survival was 10/5 months, respectively. On multivariate analysis, patients who underwent prior surgery for primary and locoregional disease had a significantly better progression-free survival and those with locoregional disease had a significantly better overall survival. A total of 45.7% received second-line chemotherapy. CONCLUSION: Use of Gem-P in Indian patients leads to slightly worse outcomes suggesting an aggressive biology.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias de la Vesícula Biliar/tratamiento farmacológico , Neoplasias de la Vesícula Biliar/patología , Cuidados Paliativos , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Camptotecina/administración & dosificación , Camptotecina/análogos & derivados , Cisplatino/administración & dosificación , Desoxicitidina/administración & dosificación , Desoxicitidina/análogos & derivados , Supervivencia sin Enfermedad , Femenino , Fluorouracilo/administración & dosificación , Fluorouracilo/análogos & derivados , Neoplasias de la Vesícula Biliar/cirugía , Humanos , India , Mosquiteros Tratados con Insecticida , Masculino , Persona de Mediana Edad , Compuestos Organoplatinos/administración & dosificación , Oxaliplatino , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Gemcitabina
13.
Int J Surg Pathol ; : 10668969241246462, 2024 Apr 22.
Artículo en Inglés | MEDLINE | ID: mdl-38646679

RESUMEN

The clinicopathological spectrum of undifferentiated round cell sarcomas of bone and soft tissues is expanding after the 5th edition of the WHO classification. A 23-year-old male patient presented with a lump in his left thigh of 3 months' duration. Radiological examination revealed a well-defined, solid-cystic lobulated, soft tissue lesion in the proximal medial region of his left thigh, measuring 7.7 cm in the largest dimension. The referring diagnosis was an epithelioid sarcoma. Histopathological review of the tumor sections revealed a cellular tumor composed of malignant epithelioid to focally "rhabdoid-like" cells in a variable hyalinized and myxoid stroma with geographic areas of necrosis. In addition, there were areas reminiscent of hemangiopericytomatous vasculature. By immunohistochemistry, the tumor cells were diffusely positive for CD34, focally and distinctly for pan keratin (AE1/AE3). INI1/SMARCB1 and SMARCA4 (BRG1) were diffusely positive (normal). Next-generation sequencing with a wide sarcoma panel revealed EWSR1exon8::ZBT44exon4 fusion. The present example constitutes the first malignant epithelioid tumor with a hemangiopericytomatous growth pattern, exhibiting this rare fusion. The differential diagnoses of this tumor and their corresponding immunohistochemical profile are discussed. This example highlights the value of NGS in unraveling rare fusions and in differentiating these tumors from their several mimics.

14.
J Cancer Res Ther ; 20(3): 1097-1099, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-39023625

RESUMEN

ABSTRACT: The lung is the most common site of metastases in the case of phyllodes tumor of the breast followed by bone. However, pneumothorax as a presenting complaint in a patient of bilateral cavitating lung metastases from malignant phyllodes tumor of the breast has never been reported to our knowledge. We herein report a case of a 34-year-old female presenting with sudden onset of chest pain in already existing lung metastases who on imaging showed the development of bilateral pneumothorax. We should, therefore, be on the lookout for the potential development of spontaneous pneumothorax in such cases.


Asunto(s)
Neoplasias de la Mama , Neoplasias Pulmonares , Tumor Filoide , Neumotórax , Humanos , Femenino , Tumor Filoide/secundario , Tumor Filoide/patología , Tumor Filoide/diagnóstico , Tumor Filoide/cirugía , Tumor Filoide/complicaciones , Neumotórax/etiología , Neumotórax/diagnóstico , Adulto , Neoplasias de la Mama/patología , Neoplasias de la Mama/complicaciones , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/diagnóstico , Tomografía Computarizada por Rayos X
15.
Artículo en Inglés | MEDLINE | ID: mdl-38975722

RESUMEN

ABSTRACT: Sclerosing epithelioid fibrosarcoma (SEF) and low-grade fibromyxoid sarcoma (LGFMS) constitute a morphological continuum with certain overlapping histopathological features and MUC 4 immunopositivity. SEF is characterized by EWSR1 gene rearrangement, is relatively more aggressive and shows a limited response to chemotherapy. Lately, a subset of tumors with morphological features of SEF, but lacking MUC4 immunostaining and EWSR1 gene rearrangement have been observed. We report the first case of YAP1∷KMT2A-positive sarcoma from the Indian subcontinent along with a review of similar tumors reported previously. A case of 31-year-old male presented with a 3 cm painful lump in the right suboccipital region. Histopathological examination of the excised specimen revealed a cellular tumor composed of atypical spindle and epithelioid cells, exhibiting moderate nuclear pleomorphism, arranged in cords, embedded in a dense collagenous/hyalinized stroma. By immunohistochemistry, the tumor cells were diffusely positive for cyclin D1 and negative for MUC4, desmin, myogenin, ß-catenin, STAT6, myoD1, SMA, and S100P. By fluorescence in-situ hybridization, EWSR1 gene rearrangement was negative. Next-generation sequencing (NGS) revealed YAP1exon5∷KMT2Aexon4 fusion. Given a positive resection margin, he underwent a revision surgery involving wide local excision of the lesion including the outer table of the occipital bone followed by image-guided radiation therapy. Over 2 years of his follow-up, the patient is alive with no evidence of recurrence. Thus, YAP1∷KMT2A positive sarcomas have distinct molecular and overlapping histopathological features with SEF, with relatively less aggressive disease course. Documentation of additional similar tumors with long-term follow-up is required.

16.
Ecancermedicalscience ; 18: 1715, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39021554

RESUMEN

Background: Rare diseases are associated with unique challenges encountered in diagnosis, treatment and conduct of clinical research. Desmoid tumour (DT) is one such ultra-rare malignancy about which awareness among medical professionals remains limited. We developed a questionnaire to assess knowledge, attitude and practice (KAP) among medical professionals on DT. Methods: E-Delphi method was used for the assessment of KAP for DT amongst clinical experts (experience of >/= 3 years in DT). 22 open-ended statements were developed by the core research group using current consensus guidelines. In round 1, experts provided subjective feedback which was incorporated into a 35-item questionnaire. Round 2 entailed experts giving feedback as a 5-point Likert scale classified into agreement (median score >/=4), neutral (median score 3) and disagreement (median score <3). Feedback from Round 2 was incorporated and questions with neutral consensus were modified. Questions in Round 3 achieved consensus if >/= 75% participants agreed. Results: 11 (64.7%) of 17 contacted experts responded in Round 1 including 6 (54.4%) who gave additional inputs and 5 (45.6%) who agreed to all statements. In round 2, 8 out of 11 experts responded to the 35-item questionnaire on knowledge (n = 16), attitude (n = 8) and practice (n = 11). 32 questions obtained agreement and 3 (8.5%) had neutral consensus. These were modified for round 3, in which consensus on 2 (66.6%) was attained. The final questionnaire comprises 34 items with 15, 8 and 11 questions on in the sections of knowledge, attitude and practice (KAP), respectively.

17.
Ecancermedicalscience ; 18: 1657, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38425760

RESUMEN

Purpose: We aimed to explore the health-related quality of life (HRQoL), psychological issues and concerns among sarcoma survivors in India and assess their satisfaction with nursing care. Methods: This study employed a sequential mixed-methods design, enrolling 100 sarcoma survivors from July to December 2021, with data collected using standardised questionnaires for HRQoL, depression, anxiety, stress, cognitive impairment and self-structured satisfaction with nursing care. Qualitative data were gathered through focused group discussions. Results: The mean global health score among sarcoma survivors was 79.48 ± 16.26. A significant number of survivors had symptoms of mild-to-moderate depression (30%), severe anxiety (12%), stress (16%) and mild cognitive impairment (5%). Significant mean rank differences were observed between anxiety and financial difficulty (p < 0.05), emotional functioning (p < 0.001), cognitive functioning (p < 0.001), pain (<0.05), insomnia (p < 0.001), fatigue (p < 0.001), anorexia (p < 0.05) and nausea/vomiting (p < 0.001). Educational qualification had a significant association with depression and anxiety while family history of cancer emerged as a significant factor associated with anxiety and stress among survivors. Qualitative analysis revealed themes related to body image, societal discrimination, socio-economic impact, marriage concerns and fertility issues. Survivor satisfaction with nursing care was good. Conclusion: A substantial number of sarcoma survivors had an average HRQoL and experienced depression, anxiety and stress. Our study emphasizes the importance of holistic survivorship care, involving nurses in post-treatment support, and addressing societal discrimination and psychosocial concerns to enhance their quality of life. Implications for cancer survivors: Our study calls for a holistic approach to sarcoma survivor care and emphasizes the importance of personalised survivorship care plans led by nurses to address the diverse needs of sarcoma survivors in India. Such plans should encompass strategies for managing depression, anxiety and stress, along with addressing body image concerns and social support.

18.
Clin Genitourin Cancer ; 22(3): 102073, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38626661

RESUMEN

INTRODUCTION: Hand foot skin reaction (HFSR) is a common dose-limiting adverse effect of multi kinase inhibitors (MKI) whose mechanism is not fully understood, and the prophylaxis is inadequate. OBJECTIVE: In this pilot study, a double-blind, randomized placebo-controlled trial was conducted to evaluate the effect of topical urea in secondary prevention of sunitinib-induced HFSR in renal cell cancer patients. METHODS: Out of 55 screened patients, 14 were randomized to receive topical urea or placebo for four weeks. The association of HFSR with drug levels of sunitinib and its metabolite (n-desethyl sunitinib), genetic polymorphism of VEGFR2 gene, quality of life (QOL) and biochemical markers was also assessed. RESULTS: The results showed that urea-based cream was not superior to placebo (P = .075). There was no change in the QOL in both the groups. Single nucleotide polymorphism was checked for two nucleotides rs1870377 and rs2305948 located in VEGFR2 gene on chromosome 4. SNP (variant T > A) at rs1870377 was associated with appearance of new HFSR as compared to the wild type, although the association was not statistically significant (OR 0.714). There was no statistically significant difference between mean plasma levels of sunitinib and N-desethyl sunitinib in urea arm as compared to placebo arm as compared to placebo. The best fit population pharmacokinetic model for sunitinib was one compartment model with first order absorption and linear elimination. The median (IQR) of population parameters calculated from the population pharmacokinetics model for Ka, V and Cl was 0.22 (0.21-0.24) h-1, 4.4 (4.09-4.47) L, 0.049 (0.042-0.12) L/hr, respectively. CONCLUSION: The study suggested that the urea-based cream was not superior to placebo in decreasing the appearance of new HFSR in renal cancer patients receiving 4:2 regimen of sunitinib.


Asunto(s)
Carcinoma de Células Renales , Síndrome Mano-Pie , Neoplasias Renales , Sunitinib , Urea , Receptor 2 de Factores de Crecimiento Endotelial Vascular , Humanos , Sunitinib/administración & dosificación , Sunitinib/farmacocinética , Sunitinib/efectos adversos , Método Doble Ciego , Carcinoma de Células Renales/tratamiento farmacológico , Masculino , Femenino , Persona de Mediana Edad , Urea/análogos & derivados , Urea/farmacocinética , Urea/administración & dosificación , Neoplasias Renales/tratamiento farmacológico , Síndrome Mano-Pie/etiología , Síndrome Mano-Pie/prevención & control , Receptor 2 de Factores de Crecimiento Endotelial Vascular/genética , Proyectos Piloto , Anciano , Polimorfismo de Nucleótido Simple , Antineoplásicos/efectos adversos , Antineoplásicos/administración & dosificación , Calidad de Vida , Resultado del Tratamiento , Administración Tópica , Adulto , Indoles/administración & dosificación , Indoles/farmacocinética , Indoles/efectos adversos
19.
Indian J Radiol Imaging ; 34(3): 390-404, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38912250

RESUMEN

Background Primary lung sarcoma (PLS) differs in management protocols and prognosis from the more common primary lung carcinoma (PLC). It becomes imperative to raise a high index of suspicion on radiological and pathological features. Purpose The aim of this study is to highlight the variable imaging appearances of PLS compared with PLC, which impacts radiologic - pathologic correlation. Materials and Methods A retrospective observational study of 68 patients with biopsy-proven lung tumors who underwent baseline imaging at our tertiary care cancer hospital was conducted between January 2018 and March 2022. The patient details and imaging parameters of the mass on contrast-enhanced computed tomography (CECT) were recorded and analyzed for patients with PLS and compared with PLC. Follow-up imaging was available in 9/12 PLS and 52/56 PLC patients. Results Among 12 patients with PLS, 5 patients had synovial sarcoma on histopathology. PLS was seen in patients with a mean age of 40.8 years; the mass showed a mean size of 13.2 cm, lower lobe (75%), parahilar (75%), hilar involvement (41.7%), oval shape (41.7%), circumscribed (25%) or lobulated (75%) margins, lower mean postcontrast attenuation of 57.3 HU, fissural extension (50%), calcification (50%), and no organ metastasis other than to the lung. PLC (56 patients) was seen in the elderly with a mean age of 54.8 years; the mass showed a mean size of 5.7 cm, irregular shape (83.9%), spiculated margins (73.2%), higher mean postcontrast attenuation (77.3 HU), chest wall infiltration (30.4%), and distant metastasis (58.9%) at baseline imaging. A statistically significant difference ( p < 0.05) was seen between sarcoma and carcinoma in the mean age, size, site, shape, margins, postcontrast attenuation, presence of calcifications, fissural extension, and distant metastasis. Conclusion The distinct imaging features of sarcoma help in differentiating it from carcinoma. This can also be used to corroborate with histopathology to achieve concordance and guide clinicians on further approach.

20.
Artículo en Inglés | MEDLINE | ID: mdl-38778607

RESUMEN

OBJECTIVE: The current study was structured to evaluate the neuroprotective properties of andrographolide in the context of aluminum chloride (AlCl3)-induced neurotoxicity, along with its concurrent impact on spatial memory impairment in Wistar rats. The present investigation elucidated the biochemical and neurobehavioral outcomes of andrographolide treatment in rats, emphasizing the areas of the brain associated with memory, i.e., the cortex and the hippocampus. MATERIALS AND METHODS: Prolonged dosing of AlCl3 (7 mg/kg) intraperitoneally for 10 days exhibited a substantial enhancement in the values of oxidative stress markers associated with a reduction in the concentrations of antioxidant enzymes within the brain. The selection of andrographolide doses (1, 2, and 3 mg/kg) was grounded in precedent safety and toxicity investigations, with subsequent oral administration. The evaluation of behavioral parameters, specifically spatial memory, was conducted through the utilization of the Radial Eight Arm Maze (RAM) test. On the concluding day of the experiment, the assessment encompassed biochemical parameter analysis and histological scrutiny of the brain tissue. RESULTS: The oral dosing of andrographolide at 1, 2, and 3 mg/kg, in conjunction with AlCl3, effectively mitigated the behavioral deficits induced by aluminum exposure. Notably, a significant suppression of NFκB was uncovered in the rats treated with andrographolide. Furthermore, histopathological examinations of the cortex and hippocampus of rat brains provided corroborative evidence, demonstrating that andrographolide substantially alleviated the toxic impact of AlCl3, thereby maintaining the typical histoarchitectural arrangement of these regions. CONCLUSION: These findings collectively suggest that andrographolide holds the potential to counteract memory impairment instigated by aluminum toxicity, accomplished through the modulation of NFκB activity and the amelioration of the adverse consequences of AlCl3 exposure.

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