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1.
Indian J Radiol Imaging ; 31(3): 560-565, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34790298

RESUMO

Background Presence of extramural venous invasion (EMVI) is a poor prognostic factor for rectal cancer as per literature. However, India-specific data are lacking. Aim The aim of the study is to determine the prognostic significance of EMVI in locally advanced rectal cancer on baseline MRI. Materials and Methods We retrospectively reviewed 117 MRIs of operable non-metastatic locally advanced rectal cancers in a tertiary cancer institute. Three dedicated oncoradiologists determined presence or absence of EMVI, and its length and thickness, in consensus. These patients were treated as per standard institutional protocols and followed up for a median period of 37 months (range: 2-71 months). Kaplan-Meier curves (95% CI) were used to determine disease-free survival (DFS), distant-metastases free survival (DMFS), and overall survival (OS). Univariate analysis was performed by comparing groups with log-rank test. Results EMVI positive cases were 34/114 (29%). More EMVI-positive cases developed distant metastasis compared with EMVI-negative cases (14/34-41% vs. 22/83-26%). The difference, however, was not statistically significant ( p = 0.146). After excluding signet-ring cell cancers ( n = 14), EMVI showed significant correlation with DMFS ( p = 0.046), but not with DFS or OS. The median thickness and length of EMVI was 6 and 14 mm, respectively in patients who developed distant metastasis, as compared with 5 and 11 mm in those who did not, although this difference was not statistically significant. Conclusion EMVI is a predictor of distant metastasis in locally advanced non-metastatic, non-signet ring cell rectal cancers. EMVI can be considered another high-risk feature to predict distant metastasis.

2.
Indian J Urol ; 37(4): 357-360, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34759530

RESUMO

Spindle cell tumors of the prostate are very uncommon and the majority involve the prostate secondarily from adjacent organs. Gastrointestinal stromal tumors (GISTs) are specific C-kit (CD 117) expressing mesenchymal tumors occurring in the gastrointestinal tract, commonly in the stomach and intestine; however, it is seldom seen involving the prostate. Although primary prostatic GISTs have been described, majority of them are secondary involvement from rectal GIST. The patient usually presents with urinary tract symptoms or prostate enlargement simulating a prostatic neoplasm. GIST as a differential diagnosis for prostatic mass is never thought of. We present a series of five cases of GIST arising from/involving the prostate mimicking a primary prostatic malignancy and the challenges associated with them for diagnosis and treatment.

3.
Radiother Oncol ; 164: 216-222, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34597737

RESUMO

BACKGROUND & PURPOSE: To evaluate the efficacy and toxicity of dose-escalated image guided-intensity modulated radiation therapy (IG-IMRT) in osteosarcoma (OGS), chondrosarcoma (CS) and chordoma (CH) of head and neck (H&N) and pelvis. METHODS AND MATERIALS: In this prospective non-randomized study, 65 patients of H&N or pelvic OGS (24), CS (7) and CH (34) mandating definitive or post-operative radiotherapy from May 2013 to December 2018 were included. Radiotherapy doses in definitive setting were 72.0 Gy for CH and 70.2 Gy for OGS and CS; while in post-operative setting it was 66.6 Gy and 64.8 Gy respectively (at 1.8 Gy per fraction). RESULTS: Planned doses of radiotherapy could be completed in 61 (93.8%) patients; with grade III or higher acute and late toxicities of 3% and 0% respectively. With a median follow-up of 52 (range 6-92) months, the five-year actuarial local control (LC) rates were 66% in OGS, 38.1% in CS and 75.9% in CH; while cause-specific survival (CSS) rates were 54.7%, 64.3% and 92.2% respectively. There was no statistically significant difference in outcomes for patients receiving definitive and post-operative radiotherapy. Locally controlled disease at first follow-up after radiotherapy was associated with improved CSS and OS in CS (p = 0.014) and CH (p < 0.001). Radiotherapy resulted in significant and sustained improvement in Musculoskeletal tumour society (MSTS) score and reduction in pain score. Salvage re-irradiation was feasible in local progression after radiotherapy, with good outcomes and tolerability. CONCLUSION: Dose-escalated IG-IMRT results in good LC & functional improvement with minimal toxicity in OGS, CS and CH.

4.
JBJS Case Connect ; 11(3)2021 09 24.
Artigo em Inglês | MEDLINE | ID: mdl-34559732

RESUMO

CASE: A 36-year-old man presented with a subacute onset left upper limb weakness. Further investigation revealed a myoepithelial carcinoma arising from the C3-C5 vertebrae. He underwent 2-stage surgery with tumor excision and postoperative radiotherapy. An improvement in power was noted, and no recurrence was observed at the 1-year follow-up. CONCLUSION: Myoepithelial tumors are rare at skeletal locations and require a high degree of suspicion. Immunohistochemistry plays a vital role in establishing the diagnosis. A complete resection is paramount for a favorable outcome.

5.
Indian J Cancer ; 2021 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-34380840

RESUMO

Background: A comprehensive histopathology report of colorectal carcinoma surgery is important in cancer staging and planning adjuvant treatment. Our aim was to review histopathology reports of operated specimens of colorectal carcinoma in our institution between 2013 and 2015 to assess different histological parameters, including lymph node yield, and to evaluate compliance to minimum data sets. Methods: After approval by the institutional review board (IRB), we analyzed 1230 histopathology reports of colorectal carcinoma between 2013 and 2015. Various gross and microscopic findings (along with age, sex) were noted, for example, specimen type, tumor site, resection margins including circumferential resection margin (CRM), lymphovascular invasion, perineural invasion, pTNM stage, lymph node yield, etc. Results: Out of 1230 patients, 826 (67.15%) were men and 404 (32.85%) were women. The overall mean age was 52 (range: 18 - 90) years. There were 787 surgeries for rectal cancers. All reports commented on the type of specimen, tumor size (mean = 4.38 cm), proximal, and distal margins. Lymphovascular invasion (LVI) and the pT stage were mentioned in 98.06% and 99.84%, respectively. The overall mean lymph node yield was 18.38 (median = 15, range = 0-130 lymph nodes). A statistically significant difference in lymph node yield was detected between rectal and colonic cancer patients (14.79 and 27.26); post neoadjuvant therapy (NACT) cases, and NACT naive cases (13.51 and 25.11); and high tumor stage and low tumor stage disease (20.60 and 15.22). Not commenting on extramural vascular emboli, tumor budding, and CRM in non-rectal cancer cases were the lacunae. Conclusion: Our compliance with minimal data sets is satisfactory. The overall mean lymph node yield was 18.38 (median = 15). Extramural vascular emboli, tumor budding need to be captured.

6.
Abdom Radiol (NY) ; 46(12): 5536-5549, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34427742

RESUMO

PURPOSE: Signet Ring Rectal Cancer (SRRC) of rectum is rare high-grade subtype with poor prognosis and characteristic histopathology. We evaluated its imaging appearance and correlated its outcomes. MATERIALS AND METHODS: We conducted a retrospective review of the rectal MRIs of 97 patients with rectal SRRC, evaluating tumor morphology, T2 signal, length, location, pattern of tumor growth, nodal status and location, EMVI (extramural vascular invasion), site of metastases, and response to chemotherapy. The tumor signal on T2W images was categorized into intermediate, T2 hyperintense, and fluid/mucin bright. Imaging findings were correlated with risk of metastatic/ recurrent disease, disease-free survival, and overall survival. RESULTS: The median age of patients of SRRC in our study was 35 years and more frequently found in male patients. The common imaging features of SRRC were T2-hyperintense signal (63%), infiltrative growth pattern (76%), positive MR CRM (Circumferential Resection Margin on MRI) (84%), presence of EMVI (51%), and advanced T and N stage (97% and 84%, respectively). Peritoneum and nodes were the most common sites of metastases. Raised serum CEA (Carcino-embryonic Antigen) levels, positive MR CRM status, extramesorectal adenopathy, and advanced N stage had statistically significant predictive value for recurrence or metastases. Elevated serum CEA levels (p = 0.019) and intermediate T2 signal (p = 0.012) demonstrated significant independent association with poor overall survival, while advanced N stage (p = 0.033) demonstrated significant independent association with worse disease-free survival in multivariate analysis. CONCLUSION: SRRC affected young patients and demonstrated T2-hyperintense signal and subepithelial spread in an infiltrative pattern. Elevated CEA levels and T2-intermediate signal intensity are independent predictors for worse overall survival and advanced nodal stage is independent prognostic factor of poor disease-free survival. MRI rectum can pinpoint the pathology given the distinct MRI morphology and age of presentation.


Assuntos
Neoplasias Retais , Reto , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Invasividade Neoplásica/patologia , Recidiva Local de Neoplasia/diagnóstico por imagem , Estadiamento de Neoplasias , Prognóstico , Neoplasias Retais/diagnóstico por imagem , Neoplasias Retais/patologia , Reto/patologia , Estudos Retrospectivos
7.
Int J Cancer ; 149(7): 1495-1511, 2021 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-34146401

RESUMO

Lipocalin 2 is a siderophore-binding protein that regulates iron homeostasis. Lipocalin 2 expression is elevated in multiple tumor types; however, the mechanisms that drive tumor progression upon Lipocalin 2 expression remain unclear. When Lipocalin 2 is over-expressed, it leads to resistance to 5-fluorouracil in colon cancer cell lines in vitro and in vivo by inhibiting ferroptosis. Lipocalin 2 inhibits ferroptosis by decreasing intracellular iron levels and stimulating the expression of glutathione peroxidase4 and a component of the cysteine glutamate antiporter, xCT. The increase in xCT levels is dependent on increased levels of ETS1 in Lipocalin 2 over-expressing cells. Inhibiting Lipocalin 2 function with a monoclonal antibody leads to a decrease in chemo-resistance and transformation in vitro, and a decrease in tumor progression and chemo-resistance in xenograft mouse models. Lipocalin 2 and xCT levels exhibit a positive correlation in human tumor samples suggesting that the pathway we have identified in cell lines is operative in human tumor samples. These results indicate that Lipocalin 2 is a potential therapeutic target and that the monoclonal antibody described in our study can serve as the basis for a potential therapeutic in patients who do not respond to chemotherapy.


Assuntos
Biomarcadores Tumorais/metabolismo , Neoplasias Colorretais/tratamento farmacológico , Resistencia a Medicamentos Antineoplásicos , Fluoruracila/farmacologia , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Lipocalina-2/metabolismo , Animais , Antimetabólitos Antineoplásicos/farmacologia , Apoptose , Biomarcadores Tumorais/genética , Movimento Celular , Proliferação de Células , Neoplasias Colorretais/metabolismo , Neoplasias Colorretais/patologia , Humanos , Lipocalina-2/genética , Camundongos , Camundongos Nus , Prognóstico , Espécies Reativas de Oxigênio/metabolismo , Células Tumorais Cultivadas , Ensaios Antitumorais Modelo de Xenoenxerto
8.
J Pathol Inform ; 12: 3, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34012707

RESUMO

Background: The COVID-19 pandemic accelerated the widespread adoption of digital pathology (DP) for primary diagnosis in surgical pathology. This paradigm shift is likely to influence how we function routinely in the postpandemic era. We present learnings from early adoption of DP for a live digital sign-out from home in a risk-mitigated environment. Materials and Methods: We aimed to validate DP for remote reporting from home in a real-time environment and evaluate the parameters influencing the efficiency of a digital workflow. Eighteen pathologists prospectively validated DP for remote use on 567 biopsy cases including 616 individual parts from 7 subspecialties over a duration from March 21, 2020, to June 30, 2020. The slides were digitized using Roche Ventana DP200 whole-slide scanner and reported from respective homes in a risk-mitigated environment. Results: Following re-review of glass slides, there was no major discordance and 1.2% (n = 7/567) minor discordance. The deferral rate was 4.5%. All pathologists reported from their respective homes from laptops with an average network speed of 20 megabits per second. Conclusion: We successfully validated and adopted a digital workflow for remote reporting with available resources and were able to provide our patients, an undisrupted access to subspecialty expertise during these unprecedented times.

9.
Abdom Radiol (NY) ; 46(2): 498-505, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32813028

RESUMO

AIM: Assessing metastatic mesorectal nodal involvement is a challenge in rectal cancer, especially in the post chemoradiation setting. We aim to assess the accuracy of MRI for nodal restaging and the validity of SAR criteria (≥ 5 mm size being metastatic). MATERIALS AND METHODS: This was an IRB-approved retrospective study of 166 patients with locally advanced rectal cancers, operated after neoadjuvant treatment. Two dedicated oncoradiologists reviewed the 166 post-chemoradiation presurgical MRIs in consensus. Nodal size and morphology (shape, margins, and signal intensity) were noted. The most accurate cut-off for size for predicting positive pN status was determined using the Youden index. RESULTS: MRI understaged 30/166 (18%) and overstaged 40/166 (24%) patients using the SAR criteria. The most accurate cut-off for node size was 5.5 mm, with a sensitivity of 75%, specificity of 60.2%, PPV of 40.7%, NPV of 86.9% (95% CI:78-92.5%), accuracy of 64.2%, and area under the curve (AUC) 0.657 (95% CI-0.524-0.79). Morphological characteristics were not significant to determine involvement, with positive nodes including 42% of round and 31% of oval nodes, 40% of heterogeneous and 45% of homogeneous nodes, and 31% irregularly marginated and 46% nodes with regular margins being positive on pathology. MRI was accurate in predicting pathology for mucinous nodes in 9/29 (31%) cases. Seven cases which were yN2 on MRI and yN0 on pathology demonstrated mucinous changes on MRI and had acellular mucin on histopathology. CONCLUSIONS: MRI has good negative predictive value, poor positive predictive value and moderate accuracy in nodal restaging. The cut-off of 5.5 mm demonstrated in our study is close to the SAR cut-off of 5 mm in the post-treatment setting. MRI accuracy is lower in patients with mucinous nodes.


Assuntos
Neoplasias Retais , Quimiorradioterapia , Humanos , Imageamento por Ressonância Magnética , Estadiamento de Neoplasias , Neoplasias Retais/diagnóstico por imagem , Neoplasias Retais/patologia , Estudos Retrospectivos , Sensibilidade e Especificidade
10.
J Pediatr Urol ; 17(1): 69.e1-69.e8, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33087302

RESUMO

BACKGROUND: Studies describing intravascular involvement in Wilms tumor have focused on illustrating individual institutional experience and the elements of surgical management. Thrombus characteristics like extent, patterns of regression, and correlation with the surgical findings, intraluminal adhesion, and viable tumor in the thrombus, and patency of the inferior vena cava (IVC) have not been systematically described. OBJECTIVES: The aim of this study is to evaluate these thrombus characteristics and explore their impact on the overall outcomes. METHODS: All patients with histologically confirmed Wilms tumors with intravascular thrombus diagnosed in the pediatric oncology unit of Tata Memorial Hospital registered from 2006 to 2019 were included. Data regarding clinical, radiological, and surgical particulars were retrieved from the prospectively maintained institutional database. Specific data for the thrombus included: distal extent before and after neoadjuvant chemotherapy, correlation of extent with the surgical findings, completeness of thrombectomy, the presence of a viable tumor in the thrombus, and the patency of the IVC. Survival analysis was performed utilizing the Kaplan-Meier method on SPSS software version 25. RESULTS: The study included 43 (9.9%) of the 432 patients with Wilms tumor having intravascular extension. Retrohepatic IVC (33.3%) followed by atrioventricular (26%) formed the frequent levels of thrombus with maximum regression occurring after chemotherapy in the latter (Summary figure). The overall concordance rate between computed tomography (CT) scan and surgical findings for the presence of thrombus was 86% and 4 patients had the thrombus limited to a lower level than the preoperative scan. At a median follow-up of 5-years, the 5-year event-free and overall survival was 81% and 82.2% respectively. Atrioventricular thrombus (p = 0.003) and postoperative patency of IVC (p = 0.02) were significantly associated with inferior survival, while the extent of regression, thrombus fracture, and viability was not significant. DISCUSSION: The findings of this study bring forth the characteristics of intravascular tumor thrombus affecting the outcomes which can be validated in future prospective studies. Although the ideal method for radiological assessment of the intravascular thrombus is elusive, CT scan provided adequate information for the presence and level of the intravascular thrombus with reasonable accuracy in this study. Study limitations include small sample size, the limited number of events, and lack of multivariate analysis to rule out confounding factors that could influence the observed findings. CONCLUSION: Atrioventricular thrombus and occlusion of IVC represent adverse prognostic factors. The extent of regression, fracture, and viability of thrombus did not affect survival in this study.


Assuntos
Carcinoma de Células Renais , Neoplasias Renais , Trombose , Tumor de Wilms , Carcinoma de Células Renais/cirurgia , Criança , Humanos , Neoplasias Renais/cirurgia , Nefrectomia , Estudos Prospectivos , Estudos Retrospectivos , Trombose/diagnóstico por imagem , Veia Cava Inferior/diagnóstico por imagem , Tumor de Wilms/cirurgia , Tumor de Wilms/terapia
11.
Cytopathology ; 32(1): 20-28, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32853443

RESUMO

OBJECTIVES: To present a comprehensive analysis of cytomorphological features, including clinical scenarios, for 8 cases (4 males, 4 females, aged 17-39 years, average = 28.5) of, retrospectively diagnosed alveolar soft part sarcoma (ASPS), with TFE3 immunostaining in 7 cases. METHODS: Conventional Papanicolaou and May Grunwald-Giemsa (MGG) stained smears and corresponding tissue sections were critically reviewed. Fine needle aspiration cytology was performed for primary diagnosis in 6 cases and for metastatic lesions in 2 cases. TFE3 and other immunohistochemical stains were tested using polymer detection technique. RESULTS: Tumour sites were thigh (n = 6), shoulder (1) and neck (1). Tumour size (n = 6) varied from 5 to 14.5 cm (average = 7.2). Seven out of 8 cases were correctly diagnosed on cytosmears. The smears were mostly hypercellular (5), composed of cohesive clusters (8), including cell balls and pseudopapillae (3) and singly scattered cells (8). Tumour cells were round to oval, containing central to eccentric nuclei (8), abundant granular (8) to finely vacuolated (7) cytoplasm that was ill- to well-defined, intracytoplasmic rod-like or needle-shaped crystals (3) and prominent nucleoli (8), Additionally, there were binucleated cells (7), multinucleation (2), intracytoplasmic inclusions (3), intranuclear inclusions (2), intercellular stroma (5) and bare nuclei (8). Immunohistochemically, 7/8 tumours were positive for TFE3. CONCLUSIONS: This constitutes the largest series describing cytomorphological spectrum of ASPS with TFE3 immunostaining results. Frequently observed features and rod-like/needle-shaped crystals on MGG smears, can help to differentiate ASPS from its mimics. TFE3 immunostaining aids in substantiating diagnoses, in an appropriate clinicoradiological context.


Assuntos
Fatores de Transcrição de Zíper de Leucina e Hélice-Alça-Hélix Básicos/metabolismo , Sarcoma Alveolar de Partes Moles/metabolismo , Sarcoma Alveolar de Partes Moles/patologia , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Biomarcadores Tumorais/metabolismo , Biópsia por Agulha Fina/métodos , Feminino , Humanos , Imuno-Histoquímica/métodos , Masculino , Estudos Retrospectivos , Sarcoma Alveolar de Partes Moles/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Coloração e Rotulagem/métodos , Adulto Jovem
12.
Diagn Cytopathol ; 49(3): E106-E112, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32886445

RESUMO

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PMHE) is a relatively newly described vascular neoplasm, characterized by distinct clinicopathological and molecular features, with no report on its cytomorphological features on smears, till date. A 17-year-old male presented with multiple nodules on his left upper limb. Radiologic imaging disclosed multiple lesions involving the soft tissues, skin, and bones of his left upper limb. Biopsy of one of the lesions on his left ring finger revealed a cellular tumor comprising plump spindle and epithelioid cells, containing moderate to abundant, eosinophilic cytoplasm; mild nuclear atypia, vesicular nuclear chromatin, discernible nucleoli, infrequent mitotic figures, and interspersed neutrophils. Immunohistochemically, tumor cells were positive for AE1/AE3, CD31, and FLI-1, while negative for CD34 and desmin. INI1/SMARCB1 was retained. MIB1/Ki67 highlighted nearly 3% tumor cells (low). Diagnosis of a PMHE was offered. A simultaneous fine needle aspiration cytology smears of the lesions in his left forearm and ipsilateral axillary region revealed spindle and some polygonal shaped cells, arranged in tight and loose clusters, as well as scattered singly, containing abundant cytoplasm with tapering cell membranes, at places; vesicular nuclear chromatin and characteristic intercellular fibrillary stromal material. The present case constitutes the first report on cytomorphological features of a PMHE, diagnosed on cytology, including its differential diagnoses, immunohistochemical features with molecular updates.


Assuntos
Hemangioendotelioma Epitelioide/diagnóstico , Hemangioendotelioma Epitelioide/patologia , Sarcoma/diagnóstico , Sarcoma/patologia , Adolescente , Biópsia por Agulha Fina/métodos , Diagnóstico Diferencial , Humanos , Masculino
13.
Eur J Pediatr Surg ; 31(3): 236-244, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32422675

RESUMO

INTRODUCTION: Treatment guidelines for hepatoblastoma discourage nonanatomic liver resections. However, the evidence for this is inadequate and comes from a study performed almost two decades ago which additionally contained inherent limitations. This study aimed to assess the feasibility and oncologic outcomes of nonanatomic resections (NAR) performed in diligently selected patients and compare the results with anatomic resections (AR). MATERIALS AND METHODS: A total of 120 patients who underwent liver resections for hepatoblastoma between January 2008 and July 2019 were reviewed. Feasibility of NAR was based on postchemotherapy relations to vessels, site of the lesion, and possibility of achieving negative resection margins. RESULTS: AR was performed in 95 patients and 25 had NAR. The NAR cohort had similar International Childhood Liver Tumors Strategy Group (SIOPEL) risk group distribution. Blood loss and operative times were lower in patients undergoing NAR. No differences were noted between the two groups concerning postoperative morbidity and hospitalization. There were no pathologic positive margins or local recurrences in the NAR patients. Relapse free (RFS) and overall survival (OS) was similar in the two groups (p = 0.54 and 0.96, respectively). Subgroup analysis of only posttreatment extent of tumor (POSTTEXT) I and II patients also showed no difference in RFS or OS for the two groups with a persistent significant difference in operative times and blood loss. CONCLUSION: NAR is feasible with clear margins in carefully selected patients. It is not associated with more complications and outcomes are not inferior to AR. NAR is associated with lesser blood loss and operative time.


Assuntos
Hepatectomia/métodos , Hepatoblastoma/cirurgia , Neoplasias Hepáticas/cirurgia , Seleção de Pacientes , Adolescente , Perda Sanguínea Cirúrgica/estatística & dados numéricos , Criança , Pré-Escolar , Bases de Dados Factuais , Estudos de Viabilidade , Feminino , Seguimentos , Hepatoblastoma/mortalidade , Hepatoblastoma/patologia , Humanos , Lactente , Recém-Nascido , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Masculino , Margens de Excisão , Duração da Cirurgia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
14.
J Adolesc Young Adult Oncol ; 10(2): 185-192, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-32706630

RESUMO

Purpose: About 30%-35% of nonmetastatic and 60%-80% of metastatic Ewing Sarcoma (ES) will relapse post-treatment and outcomes after relapse continue to be poor over last several decades. Prognostic factors affecting survival after relapse of ES are also not robustly known. We present outcomes using a novel hybrid salvage protocol of four active chemotherapeutic agents in our cohort of patients after relapse of ES. Methods: This is a retrospective analysis of all consecutive relapsed ES patients treated with curative intent over 4 years (January 2012 to December 2015). All received 12-cycles of hybrid chemotherapy regimen with surgery/radiotherapy done after first 4 cycles. Event-free survival (EFS)/overall survival (OS) estimates were analyzed by Kaplan-Meier product-limit estimator. Cox regression analysis was performed to identify prognostic factors predicting outcome in relapsed ES. Results: Salvage regimen was given to 53/108 relapsed ES patients with the rest having opted for palliation upfront. Median age of the treated patients was 19 years (range: 4-40); male:female ratio was 2.7:1. Median time to first relapse was 18.8 months (range: 2.2-91). While 41/53 patients (77%) completed salvage therapy, 6 (11.3%) progressed and 6 (11.3%) abandoned treatment. Median follow-up of the study cohort is 31 months (range: 4-81). Of the analyzable cohort (n = 47), 30 (64%) had a second relapse or progression on salvage treatment. At last follow-up, 31 patients had died (including one due to toxicity and rest due to disease) and 16 patients were alive (14 with no active disease and 2 with disease). The 4-year EFS and OS are 28% and 37%, respectively, for the entire cohort. While adolescents and young adult patients (AYA) had a better survival (p-0.041), relapsed ES patients with shorter disease-free interval (DFI) (<24 months) had a poorer survival (p-0.004). The type of relapse (local or metastatic or combined) after primary treatment did not affect outcome after salvage therapy. Conclusions: We have used a novel hybrid chemotherapy protocol using four active agents in relapsed ES, which is well tolerated and shows promising results. Older age (≥15 years) and longer DFI (>24 months) portend better survival post-relapse. In our cohort of relapsed ES, AYAs fared better than others and type of relapse after primary treatment did not affect outcome after salvage therapy.


Assuntos
Neoplasias Ósseas , Sarcoma de Ewing , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia/tratamento farmacológico , Prognóstico , Estudos Retrospectivos , Terapia de Salvação , Sarcoma de Ewing/tratamento farmacológico , Adulto Jovem
15.
J Pediatr Surg ; 55(12): 2668-2675, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32854922

RESUMO

BACKGROUND: Despite being mandated by cooperative groups, omission of nodal sampling is the most frequent protocol deviation in surgery for Wilms tumor. The stations as well as the number of nodes that should be sampled are not clearly defined resulting in a marked variation in practices among surgeons. We propose a systematic method for nodal sampling intending to reduce interoperator variation. In this study, we have assessed the feasibility and yield of systematic lymph node sampling and also evaluated the factors influencing nodal metastasis. METHODS: Prospective evaluation of 113 Wilms tumor patients operated at a single tertiary cancer center between 2015 and 2019. All these patients underwent a systematic 5-station nodal sampling. RESULTS: Median lymph node yield was 8 and 13.2% (15/113) patients harbored a histologically positive nodal disease. Of the patients with positive nodal disease, interaortocaval nodes had metastasis in 46.7% (n = 7). They represented isolated sites of nodal disease (skip metastases) in 28.6% (n = 4) of patients. Right-sided tumors had more frequent involvement of interaortocaval nodes and skip disease. Tumors with high-risk histology had 12.5 times more odds of harboring nodal disease as compared to low and intermediate-risk histology Wilms tumor. CONCLUSIONS: The proposed method of systematic station wise sampling provides a template to guide surgeons in performing lymph node harvesting. Interaortocaval nodes sampling should be performed routinely as the incidence of disease at this station is sufficiently high and metastasis may skip hilar nodes. STUDY OF DIAGNOSTIC TEST: Level III evidence.


Assuntos
Neoplasias Renais/diagnóstico , Tumor de Wilms/diagnóstico , Estudos de Viabilidade , Humanos , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Excisão de Linfonodo , Linfonodos/patologia , Metástase Linfática , Estadiamento de Neoplasias , Estudos Prospectivos , Estudos Retrospectivos , Tumor de Wilms/patologia , Tumor de Wilms/cirurgia
16.
Pediatr Blood Cancer ; 67(11): e28604, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32706522

RESUMO

BACKGROUND: The current multidisciplinary approach in the treatment of Ewing sarcoma has improved cure rates, with contemporary dose-dense chemotherapy attaining 5-year event-free survival (EFS) of 73% in localized cases. Dose-intense and dose-dense chemotherapy is difficult in the majority of resource-limited settings with limited access to optimal supportive care. We report on patients with Ewing sarcoma treated on EFT-2001, a nondose-dense chemotherapy protocol. PROCEDURE: A retrospective analysis was conducted of patients (<15 years) with Ewing sarcoma treated with curative intent during January 2013-June 2017 with an institutional ethics committee-approved nondose-dense protocol (EFT-2001). Local therapy was planned after 9-12 weeks of chemotherapy with metastatic sites addressed with radiotherapy. The study assessed outcomes and prognostic factors. RESULTS: We analysed 200 patients with M:F ratio of 1.27:1 and metastases in 41 patients (20.5%). At a median follow up of 41.5 months (range 4.5-81.8 months), respective 3-year EFS and overall survival (OS) of the whole cohort is 65.3% (95% confidence interval [CI]: 58.1-71.7%) and 79.3% (95% CI: 72.8-84.5%); for localized and metastatic cohort, 70.9% (95% CI: 62.9-77.5%) and 82.8% (95% CI: 75.7-89.0%); and for metastatic cohort, 42.8% (95% CI: 28.0-58.6%) and 65.3% (95% CI: 47.7-78.3%). Presence of residual disease (morphologic/metabolic) on positron emission tomography-computed tomography scan done 3 months post definitive radiotherapy (hazard ratio [HR] 7.92 [95% CI: 3.46-18.14]) and delay in any form of local control >4 months (HR 3.42 [95% CI: 1.32-8.89]) affected outcomes. Nonrelapse mortality during treatment was 6.5%, mainly due to cardiomyopathy (3.0%) and bacterial sepsis (1.5%). Cardiotoxicity was seen in 11.5% of patients. CONCLUSIONS: Nondose-dense chemotherapy provides good outcomes with manageable toxicities in a multidisciplinary treatment approach, while reducing cumulative drug exposures in the developing world where dose-intense or dose-dense chemotherapy could potentially increase toxicity, and hence seems a feasible approach in resource-limited settings. Presence of any residual disease post definitive radiotherapy or delay in local control portends poor outcome.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/mortalidade , Sarcoma de Ewing/mortalidade , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/patologia , Taxa de Sobrevida
17.
J Pediatr Urol ; 16(4): 475.e1-475.e9, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32620510

RESUMO

INTRODUCTION: Non-Wilms renal tumors represent a compelling subset of childhood renal tumors. However, their relative rarity renders accurate diagnosis, and therapy challenging which in some instance is inferred from their adult counterparts. OBJECTIVE: To describe the incidence and analyze the diagnostic challenges, therapies and, outcomes of non-Wilms renal tumors at the largest tertiary cancer centre in India. METHODS: All patients with histologically confirmed non-Wilms renal tumours diagnosed in the paediatric oncology unit of Tata Memorial Hospital between 2006 and 2019 were included. Data regarding clinical and radiological features and treatment outcomes were retrieved from the prospectively maintained institutional database. At the outset, histological types were categorised into a high and low-risk group depending on anticipated survival. Survival analysis was performed utilising the Kaplan-Meier method on SPSS software version 24.0. RESULTS: Of the 569 patients with renal tumors, 109 (19%) patients with primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors were included. Histological high-risk group included clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%). The low-risk group comprised of congenital mesoblastic nephroma (CMN) (4.6%), cystic partially differentiated nephroblastoma (2%), and other rare tumors (3%). Diagnostic error occurred in 2 patients in the high-risk group. All low-risk tumours were treated with surgery alone and most (97%) high-risk tumors were operated either upfront (61.5%) or after preoperative chemotherapy (38.4%). Adjuvant therapy based on histology was offered to 70%. The recurrent tumors received various salvage treatments including chemotherapy; radiotherapy; surgery and immunotherapy, however, only 2 patients could be salvaged. The 3-year overall survival for the entire cohort with primary tumors was 59%, and the survival rates were 76.7%, 77.9%, 0.0%, and 52% for CCSK, RCC, MRTK, and rES (summary figure). Low-risk tumors had 100% survival while the recurrent tumors had a median survival of 10.5 months. CONCLUSIONS: Non-Wilms renal tumors constitute a heterogeneous group of tumors, accounting for less than 20% of all renal tumors. Low-risk tumors are associated with excellent outcomes following surgery alone while the high-risk tumours have a variable outcome. MRTK and recurrent non-Wilms tumour have the worst survival. Favourable outcomes for CCSK and RCC and worst outcomes for MRTK were observed in this study. Renal ES have higher incidence of treatment failure and unsatisfactory outcomes. Recurrent non-Wilms tumours have an extremely poor outcome and more alternative or innovative approaches are needed for their treatment.


Assuntos
Neoplasias Renais , Nefroma Mesoblástico , Tumor de Wilms , Criança , Humanos , Incidência , Índia , Lactente , Neoplasias Renais/diagnóstico , Neoplasias Renais/epidemiologia , Neoplasias Renais/terapia , Tumor de Wilms/diagnóstico , Tumor de Wilms/epidemiologia , Tumor de Wilms/terapia
18.
Ann Hepatobiliary Pancreat Surg ; 24(2): 182-187, 2020 May 31.
Artigo em Inglês | MEDLINE | ID: mdl-32457264

RESUMO

Intrahepatic cholangiocarcinoma (ICC) accounts for 8-10% of all malignant liver tumors. Preponderance for elderly males and occurrence of varied morphological patterns in ICC is well known. Recent reports have described a newly recognized variant of thyroid-like cholangiocarcinoma. Herein, we present a hitherto unreported synchronous occurrence of an intrahepatic thyroid-like cholangiocarcinoma and a separate thyroid carcinoma in a 23-year-old post-partum woman. Both tumors displayed striking resemblance to follicular variant of papillary thyroid carcinoma (FVPTC) however exhibited disparate immunohistochemical profiles: the intrahepatic tumor was positive for CK7 and CK19, and negative for TTF-1, PAX-8 and thyroglobulin whereas, the thyroid tumor was positive for TTF-1, thyroglobulin and PAX-8. Young age, female proclivity, large mass at presentation and unique histology in thyroid-like ICC hint towards a distinctive subset of ICC. Awareness and recognition of this rare entity is essential, not only for accurate diagnosis, but also for gathering information on its biology and clinical behavior. Synchronous occurrence with a FVPTC is a challenging scenario that can simulate metastatic disease and mislead subsequent patient management. Whether morphologic similarity points to an underlying linkage between the two different tumors needs exploration.

19.
Clin Nucl Med ; 45(2): e120-e121, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31490315

RESUMO

Ga prostate-specific membrane antigen (PSMA) PET/CT is used in the staging, evaluation of biochemical recurrence, and response assessment of patients with prostate cancer. In addition to the PSMA-expressing prostate cancer cells, Ga-PSMA binds to the neovasculaature of various other solid tumors and benign lesions. We report a case of a 72-year-old man with recently diagnosed adenocarcinoma of prostate, incidentally found to have pleomorphic sarcoma on the staging Ga-PSMA PET/CT.


Assuntos
Adenocarcinoma/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias da Próstata/diagnóstico por imagem , Sarcoma/diagnóstico por imagem , Adenocarcinoma/complicações , Idoso , Isótopos de Gálio , Radioisótopos de Gálio , Humanos , Achados Incidentais , Masculino , Glicoproteínas de Membrana , Compostos Organometálicos , Neoplasias da Próstata/complicações , Compostos Radiofarmacêuticos , Sarcoma/complicações
20.
Ann Diagn Pathol ; 44: 151440, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31862519

RESUMO

Chondroblastoma is a relatively uncommon, primary benign bone tumor, frequently identified in young individuals. Despite its classical radiologic and histopathological features, at times, it is fraught with a diagnostic challenge, especially differentiating it from a giant cell tumor of bone (GCTB); an osteosarcoma and a chondrosarcoma. Lately, few studies have shown the diagnostic utility of immunohistochemical (IHC) expression DOG1 antibody in chondroblastomas. The present study was undertaken to evaluate IHC expression of S100 protein, DOG1 and p63 in 36 chondroblastomas. From January 2013 to July 2019 (6-year duration), 106 chondroblastomas were diagnosed, with IHC staining performed in 36 cases. Conventional Hematoxylin and Eosin stained microsections and IHC stained sections were reviewed in 36 cases. IHC staining of p63 (intranuclear), S100 protein (nuclear and cytoplasmic) and DOG1 (cytoplasmic membranous) was recorded in various cases. Seventy-four tumors occurred in males and 32 in females, within age-range of 7-55 years (average = 18.6), frequently in tibia (33/106; 31.1%), followed by femur (26, 24.5%) humerus (22, 20.7%), calcaneum (5) and scapula (4). IHC staining for S100P was positive in 33/36cases (91.7%); DOG1 in 16/19 (84.2%) cases and p63 in 10/15cases (66.6%). DOG1 immunostaining was negative in 25 various other tumors. Sensitivity and specificity for S100P, DOG1and p63 in chondroblastomas was (91.6%, 59.3%); (84.2%, 100%) and (66.6%, 46.6%), respectively. P63 was positively expressed in 15/27 (55.5%) GCTBs. S100 protein and DOG1 can be utilized for a confirmatory diagnosis of a chondroblastoma, especially for differentiating it from its other differentials, such as GCTB, in view of certain associated therapeutic implications. P63 is not useful in that scenario.


Assuntos
Biomarcadores Tumorais/metabolismo , Neoplasias Ósseas/patologia , Condroblastoma/patologia , Tumor de Células Gigantes do Osso/patologia , Osteossarcoma/patologia , Adolescente , Adulto , Anoctamina-1/metabolismo , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/metabolismo , Criança , Condroblastoma/diagnóstico por imagem , Condroblastoma/metabolismo , Feminino , Tumor de Células Gigantes do Osso/diagnóstico por imagem , Tumor de Células Gigantes do Osso/metabolismo , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Proteínas de Neoplasias/metabolismo , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/metabolismo , Proteínas S100/metabolismo , Sensibilidade e Especificidade , Adulto Jovem
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