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1.
Chirurgia (Bucur) ; 117(2): 222-229, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35535785

RESUMO

Malignant extramucosal esophageal tumors are rare. We publish a case of an extramucosal esophageal tumor which turned out to be a yolk sac tumor. This type of tumor is extremely rare. We present the initial and also late management of this case (from the diagnostic to the management of the late complications). The patient was admitted for dysphagia. Contrast thoraco-abdominal CT scan revealed an important retrocardiac mass; echoendoscopy with biopsy confirmed one malignant extra-mucosal esophageal tumor. We performed an esophagectomy through triple approaches (McKeown) â?" right thoracotomy, laparotomy, and left cervicotomy for anastomosis. The pathological report raised the suspicion of a mediastinal tumor with germ cells or a poorly differentiated carcinoma. The immunohistochemistry tests confirmed a yolk sac tumor. The patient was referred to the oncologist and was treated with etoposide and cisplatin chemotherapy. Subsequently, the patient developed respiratory failure secondary to giant hiatal hernia which was surgically treated via laparoscopy.


Assuntos
Tumor do Seio Endodérmico , Neoplasias Esofágicas , Neoplasias do Mediastino , Cisplatino , Tumor do Seio Endodérmico/diagnóstico , Tumor do Seio Endodérmico/patologia , Tumor do Seio Endodérmico/cirurgia , Neoplasias Esofágicas/cirurgia , Humanos , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Resultado do Tratamento
2.
J Cardiothorac Surg ; 17(1): 67, 2022 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-35382841

RESUMO

BACKGROUND: Mediastinal teratomas are rare tumors that frequently occur in the anterior mediastinum. The majority of these tumors are benign and slow growing. Due to their low malignant potential, the treatment for these tumors is surgical resection. More recently, the surgical management has shifted from invasive approaches such as a sternotomy to minimally invasive ones such as robotic-assisted thoracoscopic resections utilizing lung isolation ventilation. We present a rare case of a locally advanced mediastinal teratoma requiring resection, which was initially attempted thoracoscopically using double lung ventilation. CASE PRESENTATION: A 43 year-old female was found to have an anterior mediastinal mass during work-up for an intermittent cough in 2009. Chest imaging and biopsy at the time showed evidence of a cystic teratoma without concerning features. She underwent imaging surveillance until 2018, when repeat chest imaging showed increasing growth and worrisome radiologic features concerning for malignant degeneration. She underwent an elective robotic-assisted thoracoscopic resection utilizing double lung ventilation, but due to extensive involvement of the right lung, pericardium, superior vena cava, and right phrenic nerve the patient had to be repositioned and started on single lung ventilation mid-procedure to facilitate a safe and complete resection. CONCLUSIONS: Anterior mediastinal teratomas can be successfully removed by robotic-assisted thoracoscopic resections utilizing single lung ventilation. Though robotic-assisted thoracoscopic resection utilizing double lung ventilation can be effective in performing certain procedures such as lung wedge resections, thymectomy, pleural biopsies and minimally invasive cardiac procedures, it is limited in removing locally advanced mediastinal tumors.


Assuntos
Neoplasias do Mediastino , Procedimentos Cirúrgicos Robóticos , Teratoma , Neoplasias do Timo , Adulto , Feminino , Humanos , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Procedimentos Cirúrgicos Robóticos/métodos , Teratoma/patologia , Teratoma/cirurgia , Veia Cava Superior/patologia
3.
Radiat Oncol ; 17(1): 58, 2022 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-35346279

RESUMO

BACKGROUND: The low incidence of primary mediastinal seminomas has precluded the development of clinical trials on mediastinal seminomas. We investigated the clinicopathologic characteristics, prognosis of patients with primary mediastinal seminomas as well as the efficiency of nonsurgical treatments compared with treatments containing surgery. METHODS: We retrospectively collected data on the clinicopathologic characteristics, treatments, toxicities, and survival of 27 patients from a single center between 2000 and 2018. Patients were divided into two groups according to whether they received operation. Survivals were assessed using the Kaplan-Meier method. Univariate analysis was performed using the log-rank test. RESULTS: The median age was 28 (13-63) years. The most common symptoms were chest pain (29.6%), cough (25.9%), and dyspnea (22.2%). There were 13 and 14 patients in surgery and non-surgery group. Patients in the non-surgical group were more likely to be with poor performance scores (100% vs. 76.9%) and disease invaded to adjacent structures (100% vs. 76.9%) especially great vessels (100% vs. 46.2%).The median follow-up period was 32.23 (2.7-198.2) months. There was no significant difference of overall survival (5-year 100% vs. 100%), cancer-specific survival (5-year 100% vs. 100%), local regional survival (5-year 91.7% vs. 90.0%, p = 0.948), distant metastasis survival (5-year 90.9% vs. 100.0%, p = 0.340) and progression-free survival (82.5% vs. 90.0%, p = 0.245) between patients with and without surgery. CONCLUSIONS: Primary mediastinal seminoma was with favorable prognosis, even though frequently invasion into adjacent structures brings difficulties to surgery administration. Chemoradiotherapy is an alternative treatment with both efficacy and safety.


Assuntos
Neoplasias do Mediastino , Seminoma , Neoplasias Testiculares , Adolescente , Adulto , Quimiorradioterapia , Humanos , Masculino , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/terapia , Pessoa de Meia-Idade , Estudos Retrospectivos , Seminoma/patologia , Seminoma/radioterapia , Neoplasias Testiculares/terapia , Adulto Jovem
4.
Zentralbl Chir ; 147(1): 99-120, 2022 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-35235970

RESUMO

If mediastinal tumours cause symptoms these are related to their anatomical localization or a paraneoplastic syndrome. The differential diagnosis is based on the clinical situation with finding the lesion, and, furthermore, taking into account the age and sex of the patient, and the mediastinal compartment where the lesion is located. Cross-sectional radiographic diagnostic is essential for defining the therapeutic strategy. The anterior mediastinum is dominated by thymic tumours, mediastinal lymphomas, germ cell tumours and ectopic mediastinal poiters. The middle mediastinal compartment is the most frequent place of mediastinal cystic tumours, whereas the posterior mediastinum is the domain of neurogenic tumours. For selected cases a tissue biopsy is required. Surgery is the mainstay for most mediastinal tumours. Median sternotomy is the most frequent conventional surgical technique while minimally invasive surgery with thoracoscopic and above all robot assisted operation techniques are increasingly frequent. Combined chemotherapy and modern radiotherapy are essential components of the comprehensive treatment for mediastinal tumours.


Assuntos
Neoplasias do Mediastino , Timoma , Neoplasias do Timo , Estudos Transversais , Diagnóstico Diferencial , Humanos , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Timoma/diagnóstico , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/cirurgia
5.
Leuk Lymphoma ; 63(4): 834-844, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35075971

RESUMO

Few data exist concerning circulating tumor DNA (ctDNA) relevance in primary mediastinal B-cell lymphoma (PMBL). To explore this topic, we applied a 9-gene next-generation sequencing pipeline to samples from forty-four PMBL patients (median age 36.5 years). The primary endpoint was a similarity between paired biopsy/plasma mutational profiles. We detected at least one variant in 32 plasma samples (80%). The similarity between the biopsy and ctDNA genetic profiles for the 30 patients with paired mutated biopsy/plasma samples was greater than or equal to 80% in 19 patients (63.3%). We then compared PMBL ctDNA features with those of a cohort of Hodgkin lymphoma patients (n = 60). The top three mutated genes were SOCS1, TNFAIP3, and B2M in both lymphoma types. PMBL displayed more alterations in TNFAIP3 (71.9% vs. 46.3%, p = 0.029) and GNA13 (46.9% vs. 17.1%, p = 0.013) than cHL. Our 9-gene set may delineate tumor genotypes using ctDNA samples from both lymphoma types.


Assuntos
DNA Tumoral Circulante , Doença de Hodgkin , Linfoma de Células B , Linfoma Difuso de Grandes Células B , Neoplasias do Mediastino , Adulto , DNA Tumoral Circulante/genética , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/genética , Doença de Hodgkin/patologia , Humanos , Linfoma de Células B/genética , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/patologia , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/genética , Neoplasias do Mediastino/patologia , Estudos Retrospectivos
6.
J Forensic Sci ; 67(2): 795-801, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34585399

RESUMO

The literature on pediatric sudden unexpected death (SUD) due to unrecognized mediastinal neoplasms is limited to a small number of case reports with several cases confirmed to be secondary to T-cell lymphoblastic lymphoma (T-cell LBL). Mediastinal T-cell LBL can be rapidly progressive and potentially fatal due to the compression and obstruction of the airway and/or the great vessels. The clinical presentation is nonspecific with a predominance of respiratory symptoms that are more apparent when the patient is supine. We presented three cases of pediatric SUD attributed to forensic autopsy-diagnosed anterior mediastinal T- cell LBL. Case 1 involved a 2-year-old girl who presented with 9 days of cough and dyspnea. Postmortem examination revealed a firm rubbery mass surrounding the heart and compressing the bronchi. Case 2 involved a 3-year-old girl who suffered from a respiratory tract infection over several days. Autopsy revealed a firm nodular mass compressing the superior vena cava. Case 3 involved a 2-year-old boy who was found unresponsive, lying prone in his crib. He had cold-like symptoms for several days before his death. Postmortem examination revealed a firm, rubbery anterior mediastinal neoplasm surrounding the superior vena cava and great arteries. These three cases demonstrate the importance of identifying children with mediastinal masses that could potentially lead to life-threatening presentations and pediatric SUD. The forensic pathologist should consider a hematologic neoplasm at the time of autopsy in a previously healthy child who dies suddenly.


Assuntos
Neoplasias do Mediastino , Leucemia-Linfoma Linfoblástico de Células T Precursoras , Criança , Pré-Escolar , Morte Súbita/etiologia , Feminino , Humanos , Masculino , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/patologia , Leucemia-Linfoma Linfoblástico de Células T Precursoras/complicações , Linfócitos T/patologia , Veia Cava Superior/patologia
7.
Histopathology ; 80(2): 381-396, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34506648

RESUMO

AIMS: Primary mediastinal germ cell tumours (PMGCTs) are rare mediastinal neoplasms, and their diagnosis can be challenging, owing to small biopsy samples. The aim of this study was to develop a diagnostic algorithm using immunohistochemical staining, with a focus on novel markers, and molecular analysis of isochromosome 12p [i(12p)]. METHODS AND RESULTS: Paraffin-embedded tissues of 32 mediastinal tumours were analysed with immunohistochemical staining for sal-like transcription factor 4 (SALL4), Lin-28 homologue A (LIN28), octamer-binding transcription factor 3/4 (OCT3/4), D2-40, cluster of differentiation 117 (CD117), sex-determining region Y-box 17, sex-determining region Y-box 2 (SOX2), cluster of differentiation 30, the ß-subunit of human chorionic gonadotropin (ß-hCG), GATA-binding protein 3 (GATA3), forkhead box protein A2 (FOXA2), glypican-3 (GPC3), α-fetoprotein (AFP), terminal deoxynucleotidyl transferase (TdT), nuclear protein of the testis (NUT), and pan-cytokeratin. Quantitative real-time polymerase chain reaction was performed to investigate the i(12p) status. Fifteen seminomas, seven teratomas, one yolk sac tumour, one choriocarcinoma and seven mixed PMGCTs were diagnosed. Each entity had different immunohistochemical staining patterns, which helped to distinguish them: OCT3/4, D2-40, CD117 and TdT for seminoma; OCT3/4 and SOX2 for embryonal carcinoma; FOXA2, GPC3 and AFP for yolk sac tumour; and ß-hCG and GATA3 for choriocarcinoma. Mature teratomas stained positively for pan-cytokeratin in epithelial components and focally for SALL4, SOX2, GATA3, D2-40, and FOXA2. Furthermore, a NUT carcinoma mimicking a PMGCT was diagnosed, showing strong nuclear SOX2 staining and speckled nuclear NUT staining. i(12p) was detected in 24 of 27 PMGCTs (89%). CONCLUSION: A diagnostic algorithm is of great importance for a reliable diagnosis of PMGCT in, usually small, tissue biopsy samples. Therefore, a combination of three to four antibodies to identify the correct histological subtype is usually necessary, in addition to morphological features. The i(12p) status serves as an additional option to indicate a germ cell origin in selected cases.


Assuntos
Neoplasias do Mediastino/diagnóstico , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Adolescente , Adulto , Idoso , Algoritmos , Biomarcadores Tumorais/metabolismo , Criança , Feminino , Humanos , Imuno-Histoquímica , Masculino , Neoplasias do Mediastino/metabolismo , Neoplasias do Mediastino/patologia , Pessoa de Meia-Idade , Neoplasias Embrionárias de Células Germinativas/metabolismo , Neoplasias Embrionárias de Células Germinativas/patologia , Patologia Molecular , Adulto Jovem
9.
Gastrointest Endosc ; 95(4): 642-649.e2, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34875257

RESUMO

BACKGROUND AND AIMS: Primary mediastinal cysts are infrequent lesions derived from a variety of mediastinal organs or structures. Complete surgical resection is the treatment of choice even in asymptomatic patients to prevent severe adverse events (AEs) and to establish the diagnosis. Transesophageal endoscopic resection of benign mediastinal tumors has been proven feasible. The aim of this study was to evaluate the feasibility, safety, and efficacy of transesophageal endoscopic surgery for mediastinal cysts. METHODS: From January 2016 to May 2021, patients with mediastinal cysts who underwent transesophageal endoscopic resection were retrospectively included. Clinicopathologic characteristics, procedure-related parameters, AEs, and follow-up outcomes were analyzed. RESULTS: Ten patients with mediastinal cysts were included in this study. The mean cyst size was 3.3 ± 1.3 cm. Histopathology revealed 3 bronchogenic cysts (30.0%), 4 esophageal duplication cysts (40.0%), 2 gastroenteric cysts (20.0%), and 1 lymphatic cyst (10.0%). All procedures were performed uneventfully without conversion to traditional surgery. En-bloc resection was achieved in 6 patients (60.0%). Aggressive resection was avoided to prevent damage to the surrounding vital organs. Mean resection time and suture time were 58.0 ± 36.4 minutes and 5.4 ± 1.0 minutes, respectively. No major pneumothorax, bleeding, mucosal injury, or fistula occurred. One patient had a transient febrile episode (>38.5°C). Mean postoperative hospital stay was 2.7 ± .9 days. No residual or recurrent lesions were observed in any patient during a mean follow-up period of 29.8 ± 19.5 months. CONCLUSIONS: Transesophageal endoscopic surgery appears to be a feasible, safe, effective, and much less invasive approach for mediastinal cyst resection. Larger prospective studies are required to fully assess the efficacy and safety of this novel technique.


Assuntos
Cisto Broncogênico , Cisto Mediastínico , Neoplasias do Mediastino , Cisto Broncogênico/diagnóstico , Cisto Broncogênico/patologia , Cisto Broncogênico/cirurgia , Humanos , Cisto Mediastínico/diagnóstico , Cisto Mediastínico/patologia , Cisto Mediastínico/cirurgia , Neoplasias do Mediastino/patologia , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
10.
Am Surg ; 88(2): 212-218, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33522269

RESUMO

OBJECTIVE: Mediastinal masses are commonly encountered by the thoracic surgeon. Few studies have reported on the frequency and characteristics of symptoms at presentation. The primary objective of this study is to determine how often patients present with symptoms from a mediastinal mass. The secondary objective is to determine if the presence of symptoms has an effect on outcomes after surgery. METHODS: A retrospective review of an institutional database was performed. All patients who underwent surgical resection of a mediastinal mass from 2013 to 2019 were included in the analysis. Medical records were reviewed for the presence or absence of symptoms preoperatively, and these cohorts were compared. Multivariable analysis was performed, adjusting for clinical variables to assess for differences between these cohorts. RESULTS: 70 patients underwent surgery for a mediastinal mass. The average age was 49.2 years, and 46 patients (65.7%) presented with symptoms. There were no significant differences in demographics between the symptomatic and asymptomatic groups. The most common symptom was dyspnea in 18 patients (22%), followed by chest pain (15 patients, 19%) and dysphagia (8 patients, 10%). When comparing symptomatic and asymptomatic patients, symptomatic patients had a larger tumor size (5.8 cm vs 3.8 cm, P = .04) and a longer length of stay (2.0 days vs 1.2 days, P = .02). CONCLUSIONS: The majority of patients with mediastinal masses present with symptoms, with the most common symptom being dyspnea. Symptomatic patients are more likely to have a larger tumor and tend to have a longer length of hospital stay postoperatively compared to asymptomatic patients.


Assuntos
Dor no Peito/etiologia , Transtornos de Deglutição/etiologia , Dispneia/etiologia , Neoplasias do Mediastino/complicações , Doenças Assintomáticas , Bases de Dados Factuais , Feminino , Humanos , Tempo de Internação , Masculino , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Avaliação de Sintomas , Carga Tumoral
13.
Medicine (Baltimore) ; 100(46): e27873, 2021 Nov 19.
Artigo em Inglês | MEDLINE | ID: mdl-34797329

RESUMO

RATIONALE: Sclerosing thymoma (ST) is quite a rare disease, as denoted in previous literature. Less than 20 cases of ST have been reported to date. However, the combined thymoma, composed of both type AB thymoma and ST, has never been described before. PATIENT CONCERNS: The subject, a 49-year-old woman, came in with the chief complaint of cough for 10 days. DIAGNOSES: Both the contrast-enhanced computed tomography scan and the ultrasonography showed a huge mass located in the right thoracic cavity with inhomogeneous contrast accompanied by the invasion of the pericardium and pleura. Subsequently, computed tomography-guided core-needle biopsy revealed type B2 thymoma, and type AB thymoma could not be excluded. Based on postsurgical histopathology and immunohistochemical finding, this tumor was given the final diagnosis of ST and type AB thymoma. INTERVENTIONS: After 6 months of adjuvant chemotherapy and local radiotherapy, total thymectomy was performed. OUTCOMES: The patient has been duly followed up for 1 year without any tumor recurrence. LESSONS: ST is a very rare mediastinal neoplasm. Moreover, ST in combination with AB thymoma and affecting a large area, is unprecedented. Whether radiotherapy and chemotherapy have a certain effect on ST requires further investigation. In addition, due to the unclear recurrence rate of ST, long-term follow-up evaluation seems necessary.


Assuntos
Tosse/etiologia , Neoplasias do Mediastino/patologia , Timoma/patologia , Neoplasias do Timo/patologia , Biópsia , Quimioterapia Adjuvante , Feminino , Humanos , Pessoa de Meia-Idade , Radioterapia , Timectomia , Timoma/terapia , Neoplasias do Timo/terapia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia
14.
Chest ; 160(4): e357-e363, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34625184
15.
Medicine (Baltimore) ; 100(43): e27617, 2021 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-34713845

RESUMO

RATIONALE: Mediastinal non-seminomatous germ cell tumors (MNSGCTs) are rare malignancies. Chemotherapy followed by surgical resection has been regarded as the standard management, but treatment options for chemotherapy-refractory patients or those with unresectable tumors are limited, resulting in a very poor prognosis. PATIENT CONCERNS: An 18-year-old female presented with symptoms of cough, chest tightness, and shortness of breath for 2 months, and the symptoms gradually worsened. DIAGNOSIS: Computed tomography (CT) revealed a large mediastinal mass invading the pericardium and great blood vessels. Serum human chorionic gonadotropin (HCG) and α-fetoprotein (AFP) levels were normal. Histopathological examination of biopsy specimens revealed mixed MNSGCT with embryonal carcinoma and immature teratoma components. INTERVENTIONS: The patient achieved complete remission (CR) and long-term survival after multimodal therapy comprising chemotherapy, positron emission tomography/CT (PET/CT)-guided volumetric-modulated arc therapy (VMAT), and anti-angiogenic targeted therapy. OUTCOMES: The patient was followed up for more than 4 years without recurrence, metastasis, or treatment-related adverse effects. LESSONS: The case presented here highlights the importance of multidisciplinary diagnosis and treatment, providing evidence that radiotherapy and anti-angiogenic therapy may play an important role in unresectable or residual tumors after failure of conventional treatments of MNSGCT. Percutaneous biopsy is necessary for diagnosis if the tumor is unresectable, and serum AFP and HCG levels are normal. Additionally, PET/CT is an effective method for evaluation of efficacy and radiotherapy guidance for patients with MNSGCTs.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias do Mediastino/terapia , Neoplasias Embrionárias de Células Germinativas/terapia , Radioterapia de Intensidade Modulada/métodos , Neoplasias Testiculares/terapia , Adolescente , Antineoplásicos/uso terapêutico , Cisplatino/uso terapêutico , Terapia Combinada , Etoposídeo/uso terapêutico , Feminino , Humanos , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Embrionárias de Células Germinativas/patologia , Piridinas/uso terapêutico , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/patologia
16.
Thorac Cancer ; 12(21): 2949-2952, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34581018

RESUMO

Here, we report a case of carbohydrate antigen (CA) 19-9-producing mediastinal neuroendocrine tumor (NET) (atypical carcinoid). A 54-year-old woman with no specific relevant medical history was referred to our hospital because of increased CA19-9 (95.3 U/ml) detected on health screening. Chest computed tomography (CT) revealed an anterior mediastinal mass without localized lymphadenopathy. Thoracic surgery was performed and the histopathological diagnosis was thymic CA19-9-positive NET. The patient developed mediastinal lymph node metastasis at 1 year (CA19-9: 413 U/ml) and multiple bone metastases 4 years (CA19-9: 2303 U/ml) after surgery. Increased CA19-9 levels paralleled the clinical courses of relapse. To our knowledge, this is the first report of CA19-9-producing thymic NET.


Assuntos
Biomarcadores Tumorais/metabolismo , Neoplasias Ósseas/secundário , Antígeno CA-19-9/metabolismo , Metástase Linfática , Neoplasias do Mediastino/metabolismo , Tumores Neuroendócrinos/metabolismo , Neoplasias do Timo/metabolismo , Feminino , Humanos , Neoplasias do Mediastino/patologia , Neoplasias do Mediastino/cirurgia , Pessoa de Meia-Idade , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Neoplasias do Timo/patologia , Neoplasias do Timo/cirurgia
17.
J Clin Oncol ; 39(33): 3716-3724, 2021 11 20.
Artigo em Inglês | MEDLINE | ID: mdl-34570655

RESUMO

PURPOSE: A dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine and prednisone plus rituximab (DA-EPOCH-R) regimen has been shown to deliver excellent survival for adults with primary mediastinal large B-cell lymphoma (PMLBL) without the use of radiotherapy. No international prospective evaluation of this regimen has previously been reported in children and adolescents. PATIENTS AND METHODS: We conducted an international single-arm phase II trial involving patients younger than age 18 years with PMLBL who were to receive six courses of DA-EPOCH-R. The primary end point was event-free survival (EFS). Overall survival and toxicity were also assessed. This trial was registered (ClinicalTrials.gov identifier: NCT01516567). RESULTS: Analyses were based on 46 patients. The median age was 15.4 years (interquartile range: 14-16 years). The median follow-up was 59.0 months (interquartile range: 52.6-69.2 months). Fourteen events were observed (eight relapses or progressions (including three parenchymal CNS relapses), four residual lymphoma, and two second malignancies). The 4-year EFS was 69.6% (95% CI, 55.2 to 80.9), which did not differ from the rate observed historically (P = .59). Seven deaths occurred (six disease-related and one second malignancy). The overall survival was 84.8% (95% CI, 71.8 to 92.4). Twenty-two patients (48%) reached dose levels ≥ 4. Nonhematologic adverse events grade ≥ 3 or cardiac adverse events grade ≥ 2 occurred in 47 of 276 (17%) courses and 30 of 46 patients (65%). CONCLUSION: DA-EPOCH-R did not improve the EFS compared with a historical control in this first prospective multisite international study of children and adolescents with PMLBL. Further studies are required to determine the optimum therapy for children and adolescents with this lymphoma.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma Folicular/tratamento farmacológico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias do Mediastino/tratamento farmacológico , Adolescente , Criança , Ciclofosfamida/administração & dosagem , Relação Dose-Resposta a Droga , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Seguimentos , Humanos , Linfoma Folicular/patologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Neoplasias do Mediastino/patologia , Prednisona/administração & dosagem , Prognóstico , Estudos Prospectivos , Rituximab/administração & dosagem , Vincristina/administração & dosagem
18.
Int J Mol Sci ; 22(17)2021 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-34502216

RESUMO

Background and case: An adolescent male presented with a second mediastinal tumor 1.5 years after treatment of a proven malignant germ-cell tumor in that location. The differential diagnosis included a recurrent germ-cell tumor or a non-germ cell malignancy. Serum tumor markers alpha-fetoprotein (AFP) and human chorionic gonadotrophin (HCG) were negative. The first biopsy was not informative, and the second biopsy gave a broad differential diagnosis including secondary non-germ cell malignancy using histology and immunohistochemistry. DNA methylation profiling, RNA sequencing, and targeted microRNA371a-3p profiling was subsequently performed, without a supportive result. After resection of the tumor the definitive diagnosis yielded two secondary non-germ cell malignancies in the form of a leiomyosarcoma and a solitary neuro endocrine carcinoma (NEC). In spite of the differences between the molecular profiles of the initial germ-cell tumor, the leiomyosarcoma and large-cell NEC are clonally related, as determined by the presence of identical chromosomal breakpoints. The copy number profiles suggest an initial polyploidization step, followed by various independent chromosomal gains and losses. This case demonstrates that germ-cell tumors must be evaluated carefully, including molecularly, in which the non-germ cell malignancy is negative for miR-371a-3p, both in tissue as well as in serum, in contrast to the primary tumor. We conclude that the patient presented with a primary type II mediastinal GCT and, a year and a half later, followed by a leiomyosarcoma and a large-cell NEC presenting as two secondary somatic-type malignancies clonally related to the original GCT. Conclusions: Malignant germ-cell tumors are known to recur as a somatic-type malignancy in very rare cases. This case report illustrates the challenges faced in defining the nature and clonality of the secondary somatic-type malignancies.


Assuntos
Biomarcadores Tumorais/genética , Neoplasias do Mediastino/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Embrionárias de Células Germinativas/patologia , Adolescente , Humanos , Masculino , Neoplasias do Mediastino/genética , Neoplasias do Mediastino/terapia , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/terapia , Neoplasias Embrionárias de Células Germinativas/genética , Neoplasias Embrionárias de Células Germinativas/terapia , Prognóstico
20.
Diagn Pathol ; 16(1): 76, 2021 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-34419077

RESUMO

BACKGROUND: First described in 1955 Primary mediastinal seminomas are rare. Only 1-4% of mediastinal tumours are germ cell tumors; majority of which are teratomas. They typically present in men aged between 20 and 40 years. Very few cases are reported in the literature. Florid follicular lymphoid hyperplasia can obscure the malignant cells and is a rarer finding still. We present a rare case of a 48 year old man with a primary mediastinal seminoma with florid follicular lymphoid hyperplasia; found following excision of a clinically presumed thymoma. CASE PRESENTATION: A 48 year old man was referred for excision of a thymic mass. The presumed diagnosis was a thymoma; following preoperative investigations. The mass was incidentally found on a radiological imaging. However, the patient did report mid-sternal discomfort on lying flat and breathlessness. The patient underwent a thymectomy via a partial median sternotomy with good recovery. Histological assessment was that the mass was in fact a primary mediastinal seminoma with florid follicular lymphoid hyperplasia. A primary testicular malignancy was excluded and the patient required no further oncological treatment. CONCLUSIONS: Only 11 cases have previously been reported of primary mediastinal seminoma with florid follicular lymphoid hyperplasia. Although rare, a primary mediastinal seminoma should be considered as a differential diagnosis for presentations with a thymic mass. Tumour markers can be helpful, however are only positive in third of cases. Ultrasound imaging of the gonads is essential to exclude a primary gonadal lesion. Pure seminomas are radiotherapy and chemotherapy sensitive however the mainstay treatment of primary mediastinal seminomas remains surgical excision. Radiotherapy is reserved postoperatively for incomplete surgical margins.


Assuntos
Neoplasias do Mediastino/patologia , Seminoma/patologia , Adulto , Biomarcadores Tumorais/análise , Humanos , Hiperplasia , Masculino , Neoplasias do Mediastino/química , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/cirurgia , Pessoa de Meia-Idade , Seminoma/química , Seminoma/diagnóstico por imagem , Seminoma/cirurgia , Resultado do Tratamento , Adulto Jovem
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