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South Dakota Medicine : the Journal of... Aug 2023Testicular embryonal carcinoma is a type of nonseminomatous germ cell tumor (NSGCT) that commonly presents with scrotal swelling due to testicular mass. About half of...
Testicular embryonal carcinoma is a type of nonseminomatous germ cell tumor (NSGCT) that commonly presents with scrotal swelling due to testicular mass. About half of patients with NSGCTs will present with metastases at initial diagnosis. Rarely, testicular embryonal carcinoma can present primarily in the mediastinum. Treatment is well-studied and effective: chemotherapy with bleomycin, etoposide, and cisplatin. Post-chemotherapy retroperitoneal lymph node dissection (RPLND) is common adjuvant therapy. In this report we present a case of testicular embryonal carcinoma in a 32-year-old Caucasian man. The rarity of the case resides in its presentation: supraclavicular lymphadenopathy and no testicular mass on palpation or scrotal ultrasound.
Topics: Male; Humans; Adult; Mediastinum; Carcinoma, Embryonal; Testicular Neoplasms; Neoplasms, Germ Cell and Embryonal; Etoposide
PubMed: 37734076
DOI: No ID Found -
Fetal and Pediatric Pathology Aug 2023Commonly, pediatric solid tumors occur independently. Only two patients with synchronous hepatoblastoma (HBL) and neuroblastoma (NBL) have been reported. Two Chinese...
Commonly, pediatric solid tumors occur independently. Only two patients with synchronous hepatoblastoma (HBL) and neuroblastoma (NBL) have been reported. Two Chinese infants presented with abdominal mass at 10 and 8 months. Computed tomography (CT) scans in both revealed hepatic masses with additional mediastinal or adrenal masses. Pathology confirmed synchronous HBLs in the liver and NBLs in the mediastinum and adrenal. Next generation sequencing (NGS) found no remarkable germline mutations. Both patients received gross total resections with chemotherapy before or after surgery. They were followed up for 36 and 8 months, and recovered well. These two cases of synchronous HBL and NBL tumors lacked significant genetic alterations.
Topics: Humans; Infant; East Asian People; Hepatoblastoma; Liver Neoplasms; Mutation; Neuroblastoma; Neoplasms, Multiple Primary; Abdominal Neoplasms; Adrenal Gland Neoplasms; Mediastinal Neoplasms
PubMed: 36930961
DOI: 10.1080/15513815.2023.2186696 -
Histopathology Jan 2024The mediastinal compartment harbours vital organs and structures, including the heart, great vessels, major airways, and thymus. These structures are embedded in and... (Review)
Review
The mediastinal compartment harbours vital organs and structures, including the heart, great vessels, major airways, and thymus. These structures are embedded in and associated with soft-tissue elements consisting of adipose and fibro-collagenous tissue in which soft-tissue tumours may develop. A detailed inventory of soft-tissue tumours that may be encountered in the mediastinum based on the WHO 2013 classification was published in 2015. In addition, several comprehensive reviews on mediastinal soft-tissue pathology are available, including reviews focusing specifically on a single tumour type. This review will focus on primary neurogenic and spindle cell tumours of the somatic soft tissue of the posterior mediastinum and provide a discussion of the pertinent differential diagnoses.
Topics: Humans; Mediastinum; Diagnosis, Differential; Soft Tissue Neoplasms; Mediastinal Neoplasms
PubMed: 37771117
DOI: 10.1111/his.15045 -
The Journal of Surgical Research Aug 2023The purpose of this study was to perform a population-based investigation to assess the disease characteristics and prognosis of children and adolescents with malignant...
Clinical Features and Survival Outcomes in Children and Adolescents With Malignant Mediastinal Germ Cell Tumors Based on Surveillance, Epidemiology, and End Results Database Analysis.
INTRODUCTION
The purpose of this study was to perform a population-based investigation to assess the disease characteristics and prognosis of children and adolescents with malignant mediastinal germ cell tumors (MMGCT).
METHODS
Data on the demographics, treatment, and survival outcomes of children and adolescents with MMGCT from January 1, 2000 to December 31, 2018 were obtained. To compare survival curves, the log-rank test was employed. The generation of survival curves based on different parameters was done using Kaplan-Meier estimations. Cox proportional hazards regression was performed to determine the variables linked to disease-specific survival.
RESULTS
The selection criteria were met by 152 MMGCT patients, 130 of whom were male. Fifty three cases of mixed germ cell tumors (GCTs), 41 cases of malignant teratomas, 26 cases of yolk sac tumors, 14 cases of seminoma, 13 cases of choriocarcinomas, and five cases of embryonal carcinoma were reported. Overall survival at 3 and 5 y for all patients was 63.1% and 61.2%, respectively. Malignant teratoma, yolk sac tumors, and mixed GCTs in children and adolescents had comparable survival rates, while those with choriocarcinoma and embryonal carcinoma showed the worst prognosis. Embryonal carcinoma, malignant teratoma, mixed GCTs, and choriocarcinoma were found as risk factors by multivariate Cox proportional hazards analysis. In contrast, surgery and younger age were protective factors. However, chemotherapy alone showed no survival benefits.
CONCLUSIONS
Our population-based evidence showed that MMGCT had worse prognosis in older children and adolescents. Choriocarcinomas and embryonal carcinomas had the worst prognosis. Surgery can prolong survival time. Chemotherapy and radiotherapy were not associated with improved prognosis.
Topics: Pregnancy; Female; Humans; Male; Child; Adolescent; Carcinoma, Embryonal; Endodermal Sinus Tumor; Neoplasms, Germ Cell and Embryonal; Teratoma; Mediastinal Neoplasms; Choriocarcinoma; Testicular Neoplasms
PubMed: 37062232
DOI: 10.1016/j.jss.2023.03.018 -
Journal of Cardiothoracic Surgery Nov 2023Thymic carcinomas are rare and aggressive tumours. They constitute a heterogeneous group of tumours with various histological patterns and subtypes resembling epithelial...
BACKGROUND
Thymic carcinomas are rare and aggressive tumours. They constitute a heterogeneous group of tumours with various histological patterns and subtypes resembling epithelial tumours arising from other organs.
CASE PRESENTATION
We hereby represent a case of primary thymic carcinoma with adenoid cystic carcinoma-like features (TCACC) which is an extremely rare variant of thymic adenocarcinoma. To date and to the best of our knowledge, there are nine reported cases in literature and ours is the tenth. Our case was treated surgically but the implementation of adjuvant chemoradiotherapy has been reported in few of the published cases.
CONCLUSIONS
TCACC constitutes a rare entity of thymic adenocarcinoma with limited available literature. The current data is derived from few case reports and case series. The histological overlap of these tumours and primary ACC of salivary glands poses a diagnostic challenge. Radiological investigations, immunohistochemical phenotyping and genetic analysis are crucial in establishing the diagnosis.
Topics: Humans; Carcinoma, Adenoid Cystic; Thymus Gland; Adenocarcinoma; Thymoma; Thymus Neoplasms
PubMed: 37946236
DOI: 10.1186/s13019-023-02432-w -
Surgical Endoscopy Dec 2023Lung cancer poses a significant challenge with high mortality rates. Minimally invasive surgical approaches, including the uniportal thoracoscopic technique, offer...
Radicality and safety of mediastinal lymphadenectomy in lung resection: a comparative analysis of uniportal thoracoscopic, multiportal thoracoscopic, and thoracotomy approaches.
BACKGROUND
Lung cancer poses a significant challenge with high mortality rates. Minimally invasive surgical approaches, including the uniportal thoracoscopic technique, offer potential benefits in terms of recovery and patient compliance. This study focuses on evaluating the radicality of mediastinal lymphadenectomy during uniportal thoracoscopic lung resection, specifically assessing the reachability of established lymphatic stations.
METHODS
A comparative study was conducted at the University Hospital Ostrava from January 2015 to July 2022, focusing on the evaluation of radicality in mediastinal lymphadenectomy across three patient subgroups: uniportal thoracoscopic approach, multiportal thoracoscopic approach, and thoracotomy approach. The study implemented the routine identification and excision of 8 lymph node stations from the respective hemithorax to assess the radicality of lymph node harvesting.
RESULTS
A total of 428 patients were enrolled and evaluated. No significant differences were observed in the number of lymph nodes removed between the subgroups. The mean number of lymph nodes removed was 6.50 in the left hemithorax and 6.49 in the right hemithorax. The 30-day postoperative morbidity rate for the entire patient population was 27.3%, with 17.5% experiencing minor complications and 6.5% experiencing major complications. Statistically significant differences were observed in major complications between the uniportal approach and the thoracotomy approach (3.5% vs 12.0%, p = 0.002). The overall mortality rate in the study population was 3%, with a statistically significant difference in mortality between the uniportal and multiportal approaches (1.0% vs 6.4%, p = 0.020).
CONCLUSIONS
The uniportal approach demonstrated comparable accessibility and lymph node yield to multiportal and thoracotomy techniques. It is equivalent to established methods in terms of postoperative complications, with fewer major complications compared to thoracotomy. While our study indicates a potential for lower mortality following uniportal lung resection in comparison to multiportal lung resection, and demonstrates comparable outcomes to thoracotomy, it is important to approach these findings cautiously and refrain from drawing definitive conclusions.
Topics: Humans; Thoracotomy; Pneumonectomy; Thoracic Surgery, Video-Assisted; Retrospective Studies; Lung Neoplasms; Lymph Node Excision; Lung
PubMed: 37857921
DOI: 10.1007/s00464-023-10476-1 -
Veterinary Radiology & Ultrasound : the... Nov 2023A geriatric dog presented for lethargy, dyspnea, and urinary incontinence. Thoracic radiographs demonstrated a large, mixed fat, and soft tissue opaque axillary mass and...
A geriatric dog presented for lethargy, dyspnea, and urinary incontinence. Thoracic radiographs demonstrated a large, mixed fat, and soft tissue opaque axillary mass and a pulmonary mass. Computed tomography (CT) further characterized these masses and revealed innumerable fat-attenuating hepatic masses and cranial mediastinal lymphadenopathy. Histopathology of the axillary and hepatic masses confirmed grade two primary axillary liposarcoma with hepatic metastasis. Cytology of the pulmonary mass was consistent with a pulmonary carcinoma. This is the first published CT description of fat-attenuating metastatic hepatic liposarcoma in a dog.
Topics: Animals; Dogs; Liposarcoma; Tomography, X-Ray Computed; Liver Neoplasms; Lung Neoplasms; Dog Diseases
PubMed: 37582509
DOI: 10.1111/vru.13286 -
Cancer Jul 2024Small-cell lung cancer (SCLC) is characterized by rapid proliferation and early dissemination. The objective of this study was to examine the demographic trends and...
BACKGROUND
Small-cell lung cancer (SCLC) is characterized by rapid proliferation and early dissemination. The objective of this study was to examine the demographic trends and outcomes in SCLC.
METHODS
The authors queried the National Cancer Institute's Surveillance, Epidemiology, and End Results database to assess the trends in incidence, demographics, staging, and survival for SCLC from 1975 to 2019. Trends were determined using joinpoint analysis according to the year of diagnosis.
RESULTS
Among the 530,198 patients with lung cancer, there were 73,362 (13.8%) with SCLC. The incidence per 100,000 population peaked at 15.3 in 1986 followed by a decline to 6.5 in 2019. The percentage of SCLC among all lung tumors increased from 13.3% in 1975 to a peak of 17.5% in 1986, declining to 11.1% by 2019. There was an increased median age at diagnosis from 63 to 69 years and an increased percentage of women from 31.4% to 51.2%. The percentage of stage IV increased from 58.6% in 1988 to 70.8% in 2010, without further increase. The most common sites of metastasis at diagnosis were mediastinal lymph nodes (75.3%) liver (31.6%), bone (23.7%), and brain (16.4%). The 1-year and 5-year overall survival rate increased from 23% and 3.6%, respectively, in 1975-1979 to 30.8% and 6.8%, respectively, in 2010-2019.
CONCLUSIONS
The incidence of SCLC peaked in 1988 followed by a gradual decline. Other notable changes include increased median age at diagnosis, the percentage of women, and the percentage of stage IV at diagnosis. The improvement in 5-year overall survival has been statistically significant but clinically modest.
Topics: Humans; Small Cell Lung Carcinoma; Female; Male; Lung Neoplasms; Middle Aged; Aged; SEER Program; Incidence; United States; Neoplasm Staging; Adult; Aged, 80 and over; Survival Rate
PubMed: 38470453
DOI: 10.1002/cncr.35281 -
Modern Pathology : An Official Journal... Jun 2024GLI1(12q13.3) amplification is identified in a subset of mesenchymal neoplasms with a distinct nested round cell/epithelioid phenotype. MDM2 and CDK4 genes are situated...
GLI1(12q13.3) amplification is identified in a subset of mesenchymal neoplasms with a distinct nested round cell/epithelioid phenotype. MDM2 and CDK4 genes are situated along the oncogenic 12q13-15 segment, amplification of which defines well-differentiated liposarcoma (WDLPS)/dedifferentiated liposarcoma (DDLPS). The 12q amplicon can occasionally include GLI1, a gene in close proximity to CDK4. We hereby describe the first cohort of GLI1/MDM2/CDK4 coamplified WD/DDLPS. The departmental database was queried retrospectively for all cases of WD/DDLPS having undergone next-generation (MSK-IMPACT) sequencing with confirmed MDM2, CDK4, and GLI1 coamplification. Clinicopathologic data was obtained from a review of the medical chart and available histologic material. Four hundred eighty-six WD/DDLPS cases underwent DNA sequencing, 92 (19%) of which harbored amplification of the GLI1 locus in addition to that of MDM2 and CDK4. These included primary tumors (n = 60), local recurrences (n = 29), and metastases (n = 3). Primary tumors were most frequently retroperitoneal (47/60, 78%), mediastinal (4/60, 7%), and paratesticular (3/60, 5%). Average age was 63 years, with a male:female ratio of 3:2. The cohort was comprised of DDLPS (86/92 [93%], 6 of which were WDLPS with early dedifferentiation) and WDLPS without any longitudinal evidence of dedifferentiation (6/92, 7%). One-fifth (13/86, 17%) of DDLPS cases showed no evidence of a well-differentiated component in any of the primary, recurrent, or metastatic specimens. Dedifferentiated areas mostly showed high-grade undifferentiated pleomorphic sarcoma-like (26/86,30%) and high-grade myxofibrosarcoma-like (13/86,16%) morphologies. A disproportionately increased incidence of meningothelial whorls with/without osseous metaplasia was observed as the predominant pattern in 16/86 (19%) cases, and GLI1-altered morphology as described was identified in a total of 10/86 (12%) tumors. JUN (1p32.1), also implicated in the pathogenesis of WD/DDLPS, was coamplified with all 3 of MDM2, CDK4, and GLI1 in 7/91 (8%) cases. Additional loci along chromosomal arms 1p and 6q, including TNFAIP3, LATS1, and ESR1, were also amplified in a subset of cases. In this large-scale cohort of GLI1 coamplified WD/DDLPS, we elucidate uniquely recurrent features including meningothelial whorl-like and GLI-altered morphology in dedifferentiated areas. Assessment of tumor location (retroperitoneal or mediastinal), identification of a well-differentiated liposarcoma component, and coamplification of other spatially discrete genomic segments (1p and 6q) might aid in distinction from tumors with true driver GLI1 alterations.
Topics: Humans; Male; Liposarcoma; Female; Middle Aged; Aged; Zinc Finger Protein GLI1; Adult; Cyclin-Dependent Kinase 4; Aged, 80 and over; Gene Amplification; Retrospective Studies; Proto-Oncogene Proteins c-mdm2; Biomarkers, Tumor
PubMed: 38621503
DOI: 10.1016/j.modpat.2024.100494 -
Zhonghua Jie He He Hu Xi Za Zhi =... Apr 2024To analyze the clinical characteristics and prognosis of 17 patients with pathologically confirmed SMARCA4-deficient chest tumors. Seventeen patients with... (Review)
Review
To analyze the clinical characteristics and prognosis of 17 patients with pathologically confirmed SMARCA4-deficient chest tumors. Seventeen patients with SMARCA4-deficient thoracic tumors diagnosed by pathology in the Affiliated Hospital of Jining Medical University from September 2021 to January 2023 were collected through Results Query System of Pathology Department, and the patients' general conditions, clinical symptoms, tumor markers, imaging features, treatment and regression were retrospectively analyzed, and literature review was performed. A total of 17 patients were included in this study. Their clinical characteristics were characterized as follows: male/female=16/1, age 42-74 years, mean (64.0±5.7)years. Only 1 female had no clear smoking history, and 16 males had a smoking history, of whom 1 had 5 smoking pack-years, and the remaining 15 case had a smoking history of 20-100 smoking pack-years, with a mean of (68.5±44.5) smoking pack-years. Clinical symptoms were mainly cough and sputum, followed by chest tightness, hemoptysis and chest pain. Tumor markers CYFRA19-9 was elevated in 9 cases (3.79-16.61 ng/ml), CEA was elevated in 8 cases (5.37-295.93 ng/ml), and NSE was elevated in 6 cases (17.18-70.37 ng/ml). Imaging manifestations were intrapulmonary or mediastinal mass shadows, and the tumor involved the mediastinum in 9 cases, the upper lobe of the right lung in 6 cases, the upper lobe of the left lung in 5 cases, the lower lobe of the right lung in 3 cases, the lower lobe of the left lung in 3 cases; cervical or supraclavicular lymph node metastasis in 8 cases, pleural metastasis in 4 cases, hepatic metastasis in 3 cases, cerebral metastasis in 3 cases, bone metastasis in 2 cases, and subcutaneous metastasis in 1 case. Combining immuno-histochemistry and pathology, there were 6 cases of SMARCA4-deficient NSCLC and 11 cases of SMARCA4-deficient undifferentiated tumor. Eight patients were treated with platinum-contained chemotherapy agents, four of which were combined with immune checkpoint inhibitors, and one was treated with enzatinib; only one of the 9 patients achieved partial remission after treatment, and the remaining eight had progression of the tumors on chest CT after treatment. Five patients abandoned the treatment, and died in 6-month of follow-up. Three patients underwent surgery for resection, and there was no significant progression in the three patients in the 6 months of follow-up. Clinically, middle-aged and elderly men with a history of heavy smoking should be given high priority, especially in patients whose imaging mostly showed intrapulmonary, especially in upper lobes, and/or mediastinal masses, rapid lesion progression, and early distant metastasis, and who should be alerted to the possibility of SMARCA4-deficient thoracic tumors. Late clinical stage is a high risk factor for poor overall patient survival, and platinum-containing chemotherapy agents combined with immune checkpoint inhibitor therapy may be effective, and early surgery may improve patient prognosis.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Biomarkers, Tumor; DNA Helicases; Lung Neoplasms; Nuclear Proteins; Platinum; Prognosis; Retrospective Studies; Thoracic Neoplasms; Transcription Factors
PubMed: 38599807
DOI: 10.3760/cma.j.cn112147-20230927-00202