Retrospective Analysis of Pediatric Hepatoblastoma With Tumor Rupture: Experience From a Single Center.
Ontology highlight
ABSTRACT: Purpose
Hepatoblastoma (HB) tumor rupture is currently considered as a high-risk factor in some risk stratification systems. This study aimed to investigate the value of HB tumor rupture in predicting the poor prognosis.Methods
The clinical data from children with high-risk HB or HB tumor rupture at our institution from October 2008 to 2017 were retrospectively reviewed and analyzed.Results
Together, 34 children with high-risk HB or HB tumor rupture were retrospected, including 25 in the high-risk group and nine in tumor rupture group. The 3-year overall survival (OS) rate in tumor rupture group was significantly higher than that of the high-risk group (100 vs. 64%, p = 0.0427). In tumor rupture group, seven (77.8%) of nine patients had a hemoglobin level ≤ 8 g/L and 3 of them (33.3%) had ≤ 6 g/L at the time of diagnosis. Peritoneal perfusion with interleukin-2 was implemented for each patient. At the end of the treatment, seven (77.8%) of nine patients achieved complete response (CR). No patient died at the last follow-up.Conclusions
HB tumor rupture might not be predictive of poor prognosis with the risk of peritoneal dissemination/relapse, in which peritoneal perfusion with interleukin-2 could play a role.
SUBMITTER: Zhang YT
PROVIDER: S-EPMC8980550 | biostudies-literature | 2022
REPOSITORIES: biostudies-literature
Publications
Retrospective Analysis of Pediatric Hepatoblastoma With Tumor Rupture: Experience From a Single Center.
Zhang Yu-Tong YT Zhao Yu-Fei YF Yang Dian-Fei DF Chang Jian J
Frontiers in pediatrics 20220322
<h4>Purpose</h4>Hepatoblastoma (HB) tumor rupture is currently considered as a high-risk factor in some risk stratification systems. This study aimed to investigate the value of HB tumor rupture in predicting the poor prognosis.<h4>Methods</h4>The clinical data from children with high-risk HB or HB tumor rupture at our institution from October 2008 to 2017 were retrospectively reviewed and analyzed.<h4>Results</h4>Together, 34 children with high-risk HB or HB tumor rupture were retrospected, inc ...[more]
Similar Datasets
Project description:In preterm premature rupture of membranes (PPROM), a decision between early delivery with prematurity complications and pregnancy prolongation bearing the risk of chorioamnionitis has to be made. To define disadvantages of delayed prolongation, latency duration of PPROM in expectantly managed pregnancies was investigated. We included those PPROMs > 48 h leading to preterm birth prior 37 weeks' gestation and retrospectively analyzed 84 preterm infants fulfilling these criteria. The association between latency duration/appearance of PPROM and respiratory outcome (primary outcomes) and neurological outcome (secondary outcomes) was investigated. The study showed that latency duration of PPROM is not associated with clinical or histological chorioamnionitis (p = 0.275; p = 0.332). As the numerous clinical parameters show multicollinearity between each other, we performed a multiple regression analysis to consider this fact. Respiratory distress syndrome is significantly associated with gestational age at PPROM (p < 0.001), and surfactant application is significantly associated with PPROM duration (p = 0.014). The other respiratory parameters including steroids and diuretics therapy, bronchopulmonary dysplasia, and the neurological parameters (intraventricular hemorrhage, Bayley II testing at a corrected age of 24 months) were not significantly associated with PPROM duration or gestational age at PPROM diagnosis.Conclusion: Latency duration of PPROM was not associated with adverse neonatal outcome in expectantly and carefully managed pregnancies, but respiratory distress syndrome was pronounced. The observed effect of pronounced respiratory distress syndrome can be treated with surfactant preparations and was not followed by increased rate of bronchopulmonary dysplasia. What is Known: • In case of preterm premature rupture of membranes, a decision between pregnancy prolongation with the risk of chorioamnionitis and early delivery with prematurity complications has to be made. • Chorioamnionitis is a dangerous situation for the pregnant woman and the fetus. • Impaired neurodevelopmental outcome is strongly correlated with pronounced prematurity due to the increased rate of serious complications. What is New: • Respiratory distress syndrome is significantly associated with gestational age at PPROM, and surfactant application is significantly associated with PPROM duration. • Latency duration of PPROM is not associated with adverse respiratory neonatal outcome (therapy with continuous positive airway pressure, therapy with diuretics and/or steroids, bronchopulmonary dysplasia) in expectantly and carefully managed pregnancies. • Intraventricular hemorrhage and Bayley II testing at a corrected age of 24 months are not associated with latency duration of PPROM when pregnancies are carefully observed.
| S-EPMC8821059 | biostudies-literature
Project description:Iatrogenic tracheal ruptures are rare but severe complications of medical interventions. The main goal of this study was to explore prognostic factors for all-cause mortality and rupture-related (adjusted) mortality. We retrospectively analyzed patients admitted to an academic referral center over a 15-year period (2004-2018). Fifty-four patients met the inclusion criteria, of whom 36 patients underwent surgical repair and 18 patients were treated conservatively. In a 90-day follow-up, the all-cause mortality was 50%, while the adjusted mortality was 13%. Rupture length was identified as a predictor for all-cause mortality (area under the curve, 0.84; 95% confidence interval (CI) 0.74-0.94) with a cutoff rupture length of 4.5 cm (sensitivity, 0.70; specificity, 0.81). Multivariate analysis confirmed rupture length as a prognostic factor for all-cause mortality (adjusted hazard ratio (HR) 1.5; 95% CI 1.2-1.9; p = 0.001), but not for adjusted mortality (HR 1.5; 95% CI 0.97-2.3; p = 0.068), while mediastinitis predicted adjusted mortality (HR 5.8; 95% CI 1.1-31.7; p = 0.042), but not all-cause mortality (HR 1.6; 95% CI 0.7-3.5; p = 0.243). The extent of iatrogenic tracheal rupture and mediastinitis might be relevant prognostic factors for all-cause mortality and adjusted mortality, respectively.
| S-EPMC7074133 | biostudies-literature
Project description:ObjectivePrimary adrenal insufficiency (PAI) is a rare but potentially life-threatening condition. In childhood, PAI is usually caused by monogenic diseases. Although congenital adrenal hyperplasia (CAH) is the most common cause of childhood PAI, numerous non-CAH genetic causes have also been identified.MethodsPatients aged 0-18 years and diagnosed with PAI between 1998 and 2019 in a tertiary care hospital were retrospectively evaluated. After the etiologic distribution was determined, non-CAH PAI patients were evaluated in detail.ResultsSeventy-three PAI patients were identified. The most common etiology was CAH (69.9%, n=51). Non-CAH etiologies accounted for 30.1% (n=22) and included adrenoleukodystrophy (ALD; n=8), familial glucocorticoid deficiency (n=3), Triple A syndrome (n=5), autoimmune adrenalitis (n=1), adrenal hypoplasia congenital (n=1), IMAGe syndrome (n=1), and other unknown etiologies (n=3). The median age at the time of AI diagnosis for non-CAH etiologies was 3.52 (0.03-15.17) years. The most frequent symptoms/clinical findings at onset were hyperpigmentation of skin (81.8%), symptoms of hypoglycemia (40.9%), and weakness/fatigue (31.8%). Hypoglycemia (50.0%), hyponatremia (36.4%) and hyperkalemia (22.7%) were prominent biochemical findings. Diagnosis of specific etiologies were proven genetically in 13 of 22 patients. A novel p.Q301* hemizygous frameshift mutation of the DAX1 gene was identified in one patient.ConclusionEtiology was determined in 86.3% of children with non-CAH PAI through specific clinical and laboratory findings with/ without molecular analysis of candidate genes. ALD was the most common etiology. Currently, advanced molecular analysis can be utilized to establish a specific genetic diagnosis for PAI in patients who have no specific diagnostic features.
| S-EPMC7947721 | biostudies-literature
Project description:Cutaneous T-cell lymphomas (CTCL) represent rare non-Hodgkin lymphomas (NHL) with an incidence less than 1 per 100,000 inhabitants. The most common type of CTCL is mycosis fungoides (MF), which represents approximately 60% of all CTCL, followed by Sézary syndrome (SS), approximately 5%. We retrospectively analyzed the outcome of 118 patients with MF (n=96) and SS (n=22) treated between the years 1998 and 2021 at the Charles University General Hospital in Prague, Czech Republic. The ratio between men and women was 1.2:1 (62 men, and 56 women). The median age at diagnosis was 62 years (23 to 92 years). From the MF cohort 48 patients (50% out of MF cohort) presented with advanced stage disease. Ninety patients (77%) received a systemic treatment at any time from the diagnosis; the median number of therapy lines was two. At the time of database lock, the overall survival (OS) of 96 patients with MF reached 17.7 years with the median follow-up 4.0 years. With the median follow-up 2.6 years, the median OS of 22 patients with SS was 3.5 years. The most common type of systemic therapy for MF included low-dose methotrexate (61%), interferon-alpha (58%), bexarotene (28%), and chlorambucil (25%). The most common type of therapy for SS included bexarotene (64%), extracorporeal photopheresis (50%), and interferon-alpha (45%). Only the minority of patients received innovative targeted agents including brentuximab vedotin, mogamulizumab, or pembrolizumab. Besides the retrospective analysis of the CTCL cohort, current standards and future perspectives of selected innovative agents are summarized and discussed. The analyzed cohort represents the largest cohort of CTCL patients in the Czech Republic. Overall, the survival parameters of our CTCL cohort are comparable to those previously published by other groups. In conclusion, our analysis of 118 real world cohort of consecutive CTCL patients treated at the single center confirmed the efficacy of immune response modifiers and underlines the urgent need for ample implementation of innovative agents and their combinations into earlier lines of therapy.
| S-EPMC9210166 | biostudies-literature
Project description:BACKGROUND:Fibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Doppler ultrasound (US), magnetic resonance angiography (MRA), computed tomography angiography (CTA), and catheter-based angiography (angiography) are currently used to help make a clinicoradiological diagnosis of FMD. The main aim of the study was to compare the efficacy of imaging modalities which can allow for earlier and improved detection. Furthermore, an anatomical mapping of the location of lesions can help determine the best treatment modalities. METHODS:All patients with non-syndromic non-inflammatory renovascular hypertension were recruited from the Nephrology Department at the Children's Hospital of Philadelphia (CHOP) and enrolled in the U.S. FMD Registry maintained at the University of Michigan. Clinical presentation and imaging findings on US, CT, and MRI of children diagnosed with FMD were evaluated. RESULTS:Mean age at diagnosis was 7?±?4.9 years (4 months-17 years). Family history of hypertension (HTN) (52%), FMD (8.7%), Caucasian (60%), headache (48%), and HTN (80%) were the most prevalent symptom and sign at presentation. Bruits were 100% specific for renal artery stenosis (RAS) diagnosis but were heard in the minority of patients (3 patients, 12%). FMD was mainly unifocal within a single site (68%) or multiple sites (28%) and involved the main or first order renal branch in about 68% of children. Isolated distal lesions beyond the second order branches were found in about 25% of children. US imaging was significantly less sensitive than angiography (28%, p?=?0.003). MRA had a better sensitivity (62.5%, p?=?0.3) than US. Overall, CTA had the best sensitivity (84.2%, p?=?0.4) compared to angiography; however, only angiography showed distal vessel disease. CONCLUSIONS:Limitations of the study include the sample size and biases-only patients diagnosed with FMD were included in this study and most patients were referred to a pediatric nephrologist for unexplained hypertension. Angiography should be performed as part of the initial work-up of any child suspected of having renovascular FMD, regardless of the findings seen on US, MRA, or CTA.
| S-EPMC6082421 | biostudies-literature
Project description:Background/Aim:Appendix tumors are mostly incidentally identified in patients who were operated with the diagnosis of acute appendicitis. They are detected in approximately 1% of appendectomy specimens. Neuroendocrine tumors (NETs) account for over 50% of appendix neoplasms. NETs appearing in the appendix can cause carcinoid syndrome. In our study, we aimed to retrospectively examine the clinical features of patients who underwent appendectomy with the diagnosis of acute appendicitis and diagnosed with appendix NET in the postoperative period. Materials/Methods. The records of 4026 patients who were operated with the diagnosis of acute appendicitis between January 2008 and January 2020 at the Department of General Surgery at the Sakarya University Faculty of Medicine, were evaluated retrospectively. Clinical findings, demographic data, surgical findings, and results of the patients with appendix NET, as a result of histopathology, were examined in detail. Results:16 of 4026 patients were reported as NET. Nine of the patients were male, and seven were female. The average age was 33 (19-49). Any of the patients had no signs and symptoms of carcinoid syndrome. All tumors were located at the tip of the appendix, and the mean tumor diameter was 0.85?cm (0.3-2.5?cm). As a result of pathology, one patient had mesoappendix and one patient had serosa invasion. Right hemicolectomy was applied to both patients. In other patients, meso, serosa, and lymphatic invasion were not detected. Tumor size was 2.5?cm in one of the patients, 1.5?cm in one, and 1.4?cm in the other, and the others were below 1?cm. In the postoperative follow-up, all the patients were discharged on average 2.71 (2-6 days) days without any complications. Conclusion:Appendix NETs are mostly asymptomatic and localized in a distal third of the appendix. Symptoms are mostly related to tumor size and distant metastases. Clinical behavior and prognosis can best be predicted by tumor size. Complementary hemicolectomy is recommended for tumors larger than 2?cm and tumors smaller than 1 to 2?cm, such as mesoappendix invasion, positive or uncertain surgical margin, high proliferative rate, and angioinvasion. For tumors whose diameter is less than 1?cm, simple appendectomy alone is sufficient.
| S-EPMC7486639 | biostudies-literature
Project description:Papillary tumor of the pineal region (PTPR) is a rare entity. Its clinical presentation is diverse, and establishing an accurate and timely diagnosis may be challenging. Treatment recommendations are based on the evidence level of case series. Recently, several key advances have been made for immunohistochemical characterization, molecular diagnostics, and neurosurgical treatment of PTPR. Here, we describe our single-center experience.
| S-EPMC7571506 | biostudies-literature
Project description:BackgroundGastrointestinal stromal tumor (GIST) is known for its wide variability in biological behaviors and it is difficult to predict its malignant potential. The aim of this study is to explore the characteristics and prognostic factors of GIST.MethodsClinical and pathological data of 497 GIST patients in our center between 1997 and 2012 were reviewed.ResultsPatients were categorized into very low-, low-, intermediate- and high-risk groups according to modified National Institutes of Health (NIH) consensus classification system. Among the 401 patients untreated with imatinib mesylate (IM), 5-year overall survival (OS) in very low-, low-, intermediate- and high-risk groups was 100%, 100%, 89.6% and 65.9%; and 5-year relapse-free survival (RFS) was 100%, 98.1%, 90.9% and 44.5%, respectively. Univariate analysis revealed that sex, tumor size, mitotic rate, risk grade, CD34 expression, and adjacent involvement were predictors of OS or RFS. COX hazard proportional model (Forward LR) showed that large tumor size, high mitotic rate, and high risk grade were independent risk factors to OS, whereas high mitotic rate, high risk grade and adjacent organ involvement were independent risk factors to RFS. The intermediate-high risk patients who received IM adjuvant therapy (n = 87) had better 5-year OS and RFS than those who did not (n = 188) (94.9% vs. 72.1; 82.3% vs. 56.3%, respectively). Similarly, advanced GIST patients underwent IM therapy (n = 45) had better 3-year OS and 1-year progression-free survival (PFS) than those who didn't (n = 42) (75.6% vs. 6.8%; 87.6% vs. 12.4%, respectively).ConclusionsVery low- and low-risk GISTs can be treated with surgery alone. Large tumor size, high mitotic rate, high risk grade, and adjacent organ involvement contribute to the poor outcome. IM therapy significantly improves the survival of intermediate-high risk or advanced GIST patients.
| S-EPMC4254179 | biostudies-literature
Project description:Rare pediatric tumors are heterogeneous group containing a variety of histopathological diseases, they represent approximately 10% of all childhood cancers. These rare tumors had a diversity of histology and clinical behaviors that pose different challenges to the investigators. Exploring different pediatric rare tumors. The data were reviewed, retrospectively, through the medical records of seven rare pediatric diseases between 2012 and 2019. Giant cell fibroblastoma (GCF) presented as painless swelling in the trunk, positive for CD34 with PTEN gene mutation. Neuroglial heterotopic tissue presented in 7 days old girl with facial asymmetry and bulging in the oral cavity, maximal de-bulking was done, histopathology was positive for GFAP and S100p. Left side neck mass, surgically excised revealed non-metastatic salivary grand mucoepidermoid carcinoma. Follow up without any chemotherapy or radiotherapy for 5 years with complete remission. Mesenchymal chondrosarcoma (MCS) presented in maxillofacial bones by persistent nasal bleeding, HEY1-NCOA2 fusion gene confirmed the diagnosis. Extra-osseous Ewing sarcoma (EES) presented as rubbery painless swelling in the scalp with fusion transcript involving EWSR1-FL11. Juvenile xanthogranuloma (JXG) presented by butter fly like skin patch in the face with foamy histiocytes in upper dermis with few Touton giant cells, extensive systemic involvement of lung and bone marrow. Metastatic ovarian choriocarcinoma with choriocarcinoma syndrome received induction two different lines of chemotherapy and consolidated with autologous stem cell transplant. Seven pediatric rare tumors, with different aspects of challenges in diagnosis and management, despite the absence of formal protocols and rarity of other center experiences.
| S-EPMC7907711 | biostudies-literature
Project description:ObjectiveAortopulmonary window (APW) is a rare congenital heart defect. It occurs as an isolated cardiac lesion or in association with other cardiac anomalies and rarely with abnormal coronary arteries. The spectrum of cardiovascular anomalies associated with APW and overall management and outcome in the current era were reviewed.MethodsBetween 2001 and 2018, all patients diagnosed with APW were included. Based on associated cardiovascular anomalies, those patients were divided into 2 groups: simple APW group and complex APW group (APW with associated other cardiovascular anomalies). All cases were followed longitudinally. The outcomes are described.ResultTwenty patients underwent APW repair including 2 (10%) in simple APW group and 18 (90%) in complex APW group. Their mean age and weight were 4.8 ± 1.8 months and 4 ± 0.4 kg, respectively. APW Type I was confirmed in 65% followed by Type III in 20% and then Type II in 15% of the patients. In the complex APW group, atrial septal defect was the commonest associated cardiac lesion occurring in 8/20 (40%), followed by ventricular septal defect, interrupted aortic arch, and pulmonary artery anomalies in 25% of each. The presence of patent ductus arteriosus (PDA) was found in 40% of APW cases with 2/3rd of them in association with interrupted aortic arch. Two patients (10%) had unusual coronary anomalies that required repair, both with APW Type I. Associated non-cardiac anomalies were found in 30% of cases. Risk Adjustment for Congenital Heart Surgery (RACHS-1) score frequencies were between 2 and 4. Only one patient had reactive pulmonary hypertension related to chronic lung disease. All patients underwent surgical correction with median age of 2 month at the time of repair (interquartile range, 2 weeks to 4.5 months). Mean duration of mechanical ventilation, pediatric cardiac ICU and hospital length of stay were 2.8 ± 0.5, 9 ± 3 and 26 ± 6 days, respectively. All patients survived with no residual APW with mean follow-up duration of 4.5 years.ConclusionMajority of APW are associated with other cardiovascular anomalies (90%) including coronary abnormalities (10%). Early surgical repair of APW and associated lesions showed excellent survival rate, freedom from re-intervention need within an average of 4.5 years of follow up and no evidence of persistent pulmonary hypertension post repair.
| S-EPMC7640542 | biostudies-literature