Cell Tumors Of Childhood Research Articles

Conservative management of gynecomastia in Peutz-Jeghers syndrome: Case series and review of the literature.

Peutz-Jeghers syndrome (PJS) is a childhood-onset cancer predisposition syndrome that is associated with oral freckling and gastrointestinal polyposis. Male patients with PJS are at risk for large-cell calcifying Sertoli cell tumors in childhood. These tumors are estrogen-producing and can cause symptoms of precocious puberty, gynecomastia, and growth acceleration. Here we discuss our experience with spontaneous resolution or stabilization of breast enlargement without medical intervention in three patients with PJS and gynecomastia. These cases indicate that a watchful waiting approach can be considered in the management of gynecomastia in male children with PJS.

Ovarian germ cell tumors in children and adolescents (literature review)

Ovarian germ cell tumors account for 30 % of germ cell tumors of other localizations and 70 % of all ovarian neoplasms. The aim of this review is to study and systematize clinical manifestations, diagnosis and treatment of ovarian germ cell tumors in childhood and adolescence on the basis of current foreign and domestic studies.

GCT-14. TREATMENT APPROACH FOR METASTATIC INTRACRANIAL GERMINOMA: A MULTI-INSTITUTIONAL EXPERIENCE

Abstract BACKGROUND Germinoma is the most common CNS germ cell tumor in children. Treatment with 24Gy craniospinal irradiation (CSI) with boost to 40Gy alone results in excellent survival irrespective of disease stage. For localized germinoma, recent studies have demonstrated the efficacy of neoadjuvant chemotherapy in decreasing the field/dose of radiation therapy (RT) needed for cure. For metastatic germinoma however, the optimal RT approach when combined with chemotherapy is unclear. Herein, we present our experience in treating patients with metastatic germinoma. METHODS We performed a retrospective, IRB-approved study of patients with metastatic germinoma diagnosed between 2001-2023 at Boston Children’s Hospital, Dana-Farber Cancer Institute, Brigham and Women’s Hospital, and Mass General Hospital. Clinical features, treatment plan, and outcomes were collected and analyzed using the Kaplan-Meier method and Fisher’s exact test. RESULTS Twenty-eight patients with metastatic germinoma were identified. Median age at diagnosis was 15.9 years (range:6.2-25.9). Metastasis was intracranial in 20, craniospinal in three and CSF-only in five patients. Six patients received CSI only. Twenty-two patients received neoadjuvant chemotherapy and CSI. Median number of chemotherapy cycles was 4 (range:3-6). Seventeen patients had complete response, four patients had partial response, and one patient had progressive disease with chemotherapy. All patients went on to receive CSI, with median dose of 21Gy (range:18-36Gy) with median IF boost to 36Gy (range:30-51.6Gy). Median duration of follow-up was 11 years (range:0.3-25.3). There were no recurrences or deaths within the cohort. Analysis of long-term outcomes data revealed no significant differences in frequency of endocrinopathies, CNS vasculopathies, or memory loss between the doses of RT. CONCLUSIONS Our data show that neoadjuvant chemotherapy followed by CSI is associated with excellent overall survival in patients with metastatic germinoma. Importantly, our experience suggests that in combination with neoadjuvant chemotherapy, 21Gy CSI may be sufficient as a curative dose in this patient population.

Keratin-Positive Giant Cell Tumor of Bone and Soft Tissue With HMGA2::NCOR2 Fusion in Children Under 10 With Response to Imatinib Therapy: A Case Series.

HMGA2::NCOR2 keratin-positive giant cell tumors in children with response to imatinib in an infant.

Antenatal teratoma sacrococcygeal in a three month old children in Indonesia: a rare case report

Sacrococcygeal teratoma is a type of germinal cell tumor in children, usually observed in the prenatal or antenatal phases. In either phase, sacrococcygeal teratoma may cause complications that lead to increased risk of morbidity and mortality. A proper screening and management for sacrococcygeal in children are mandatory for the best outcome. Currently, surgical resection is the most promising treatment to manage antenatal sacrococcygeal teratoma in children. Three-month-old female children were brought to the hospital with a growing 8.4 cm mass since birth in her groin. The mass was painless and soft in consistency. Computed tomography (CT) scan revealed a dominant solid lesion accompanied by multiloculated fluid density arising from right gluteal muscle. The patient planned for surgical excision. Hyperkalemia was observed prior to the operation; hence an appropriate correction was needed before surgery. Germ cell tumors, though uncommon, may develop beyond the gonadal region, such as in the brain, neck, mediastinum, and retroperitoneum. In neonates and fetuses, these tumors frequently originate in the sacrococcygeal area, possibly resulting from the migration of primordial cells and the proliferation of totipotent stem cells in that region. Optimal disease burden outcomes for sacrococcygeal teratoma are often achieved through surgical resection. Sacrococcygeal teratoma, although rare, might possess a mortal threat to patient, hence proper diagnosis and treatment must be done to ensure the best clinical outcome.

Unusual imaging findings associated with abdominal pediatric germ cell tumors.

Germ cell tumors of childhood are tumors arising from germline cells in gonadal or extragonadal locations. Extragonadal germ cell tumors are characteristically located in the midline, arising intracranially or in the mediastinum, retroperitoneum, or pelvis. These tumors are generally easily diagnosed due to typical sites of origin, characteristic imaging findings, and laboratory markers. However, germ cell tumors can be associated with unusual clinical syndromes or imaging features that can perplex the radiologist. This review will illustrate atypical imaging/clinical manifestations and complications of abdominal germ cell tumors in childhood. These features include unusual primary tumors such as multifocal primaries; local complications such as ovarian torsion or ruptured dermoid; atypical presentations of metastatic disease associated with burned-out primary tumor, growing teratoma syndrome, and gliomatosis peritonei; endocrine manifestations such as precocious puberty and hyperthyroidism; and antibody mediated paraneoplastic syndrome such as anti-N-methyl-D-aspartate-receptor antibody-mediated encephalitis. This review aims to illustrate unusual imaging features associated with the primary tumor, metastatic disease, or distant complications of abdominal germ cell tumors of childhood.

Intracranial non-germinomatous germ cell tumors in children and adolescents: how can the experience from an uppermiddle-income country contribute to the worldwide effort to improve outcomes?

Non-germinomatous germ cell tumors (NGGCT) accounts for one third of intracranial GCT. While the germinoma group have an excellent overall survival, the standard of practice for children with NGGCT is still under evaluation. Describe the results of the of the Brazilian consortium protocol. Since 2013, 15 patients with a diagnosis of NGGCT by histopathology and/or serum/cerebrospinal fluid (CSF) tumor markers, βHCG >200mlU/ml and/or positive alpha-fetoprotein were treated with neoadjuvant chemotherapy with carboplatin, cyclophosphamide and etoposide followed by ventricular radiotherapy (RTV) of 18Gy with boost (32Gy) to the primary site. Metastatic patients underwent craniospinal irradiation (CSI) and "slow responders" to the four initial cycles of CT, to autologous stem cell transplantation (ASCT) followed by CSI. Mean age, 13.1 years. Thirteen males. Primary sites: pineal (n=12), suprasellar (n=2) and bifocal (n=1). Four patients were metastatic at diagnosis. Eight patients had CSF and/or serum alpha-fetoprotein levels > 1,000ng/ml. Tumor responses after chemotherapy demonstrated complete in six cases and partial in seven, with "second-look" surgery being performed in five cases, and two patients presenting viable lesions being referred to ASCT. The main toxicity observed was hematological grades 3/4. Two patients with metastatic disease, one with Down Syndrome and AFP > 1,000ng/ml and the other with choriocarcinoma and pulmonary metastases, developed progressive disease resulting in death, as well as two other patients without evidence of disease, due to endocrinological disorders. Event-free and overall survival at 2 and 5 years were 80% and 72.7%, respectively, with a mean follow-up of 48 months (range, 7-107). Despite the small number of patients, in our series, treatment with six cycles of chemotherapy and RTV with focal boost for localized disease (n=11) and ACST for identified slow responders (n=2) seem to be effective strategies contributing to the overall effort to improve outcomes of this group of patients.

Development and validation of a nomogram for preoperative prediction of immature teratoma in children with teratoma: a retrospective, multicenter, diagnostic study.

Teratomas are the most common germ cell tumors in children, and histologically classified as mature teratomas (MTs) and immature teratomas (ITs). Preoperative IT identification can affect the surgical approach, the type of procedure, and future possible reproductive health. However, there is no complete diagnostic criterion for ITs nowadays. We aimed to establish and validate a nomogram based on clinical and computed tomography (CT) features for preoperative prediction of ITs in children. We retrospectively reviewed 519 teratoma patients from hospital I for training (n=364) and validation (n=155), and 113 patients from hospital II for external validation. Univariate and multivariate logistic regression analyses were performed on the training set to screen risk factors, including alpha-fetoprotein (AFP), age, gender, tumor site, size, tumor composition, calcification and fat. Then, a nomogram was established based on identified risk factors and validated on the validation set. The performance of the nomogram was evaluated in terms of discrimination, calibration and the clinical usefulness. Multivariate logistic regression showed that tumor composition, AFP, age, calcification and fat were independent risk factors for preoperative prediction of IT. The area under the receiver operating characteristic (ROC) curves (AUCs) for the nomogram on the training set, internal and external validation set were 0.92 (0.88-0.96), 0.91 (0.84-0.97) and 0.92 (0.86-0.97), respectively. The model demonstrated sensitivity of 80%, specificity of 90% at the cut-off value of 0.262. Whatever the set, the calibration curve indicated good calibration. Decision curve analysis (DCA) curves demonstrated that the nomogram had greater net benefits than either the treat-all tactics or the treat-none tactics within a large scope of threshold. The nomogram established based on clinical and CT findings had the favorable accuracy for the preoperative prediction of IT, and may help in clinical decision-making and risk stratification.

Extracranial Germ Cell Tumors in Children: Ten Years of Experience in Three Children's Medical Centers in Shanghai.

The aim was to describe the clinical features of extracranial germ cell tumors (GCTs) in pediatrics and study the clinical risk factors related to survival for malignant germ cell tumors (MGCTs) in order to optimize therapeutic options. The clinical data of children with extracranial GCTs in three children's medical centers in Shanghai were retrospectively analyzed. In total, 1007 cases of extracranial GCTs diagnosed between 2010 and 2019 were included in this study, including teratomas (TERs) 706 (70.11%) and MGCTs 301 (29.89%). There were twice as many TER cases as MGCT cases. Approximately 50% of children with GCTs were <3 years old (43.39% for TERs, 67.13% for MGCTs). GCTs in children of different ages show differences in tumor anatomical locations and pathological subtypes. The 5-year event-free survival (EFS) and overall survival (OS) of all patients with MGCTs were 82.33% (95% CI, 77.32%, 86.62%) and 94.13% (95% CI, 90.02%, 96.69%), respectively. The multivariate Cox regression analysis identified a primary site in the mediastinum and alpha fetoprotein (AFP) levels ≥10,000 ng/mL as independent adverse prognostic factors (p < 0.0.0001, χ2 = 23.6638, p = 0.0225, χ2 = 5.2072.). There were no significant differences in OS among children receiving various chemotherapy regimens, such as the BEP, PEB, JEB and other regimens (VBP/VIP and AVCP/IEV) (p < 0.05). The clinical features of GCTs in Chinese pediatrics are similar to those reported in children in Europe and America. The age distribution of pathological types and primary sites in GCTs reflect the developmental origin of type I and type II GCTs transformed from mismigration primordial germ cells (PGCs). Optimizing the current platinum-based chemotherapy regimens and exploring the treatment strategies for MGCTs of the mediastinum are future research directions.

Testicular Germ Cell Tumors in Children and Adolescents Treated With Bleomycin, Etoposide, and Cisplatin (BEP) Protocol: A Survival Analysis

Testicular Germ Cell Tumors in Children and Adolescents Treated With Bleomycin, Etoposide, and Cisplatin (BEP) Protocol: A Survival Analysis

Localized tenosynovial giant cell tumor in children.

To investigate the clinical characteristics and surgical efficacy of localized tenosynovial giant cell tumors in children. The clinical data, surgery, and follow-up results of 17 children with localized tenosynovial giant cell tumors who visited our hospital from 2011 to 2021 were collected for statistical analysis. The median patient age was 7 years and 8 months, and the ratio of males to females was 1.43 (10/7). The predilection of disease was similar in hands and feet, and the common presenting symptom was mass. One patient experienced recurrence after surgery, and one child had postoperative functional limitations. Extremities are common sites of localized tenosynovial giant cell tumors in children. Complete surgical resection helps reduce the recurrence rate. Level III.

A Testicular Leydig Cell Tumor; An Uncommon Cause of Precocious Puberty: A Case Report With Secondary Central Precocious Puberty

Sexual precocity in boys is defined as any sign of secondary sexual characteristics present before the age of 9. Leydig cell tumors of the testes are a rare cause of peripheral precocious puberty in boys. Here, we report 8 years and 4-month-old boys with signs of peripheral precocious puberty because of a testicular Leydig cell tumor that developed true precocious puberty after surgical removal. Examination of genitalia showed Tanner 4 hair growth. The penis length was 14.5 cm with a 2.5 cm width. The right testis was enlarged but the left testis was measured at 2cm in length and 1 cm in width. Laboratory results showed low serum gonadotropin levels and increased androgen levels. Testicular sonography reported one solid mass measured 31×28×15 millimeters. With a presumptive diagnosis of Leydig cell tumor, the patient underwent radical orchiectomy. Pathologic evaluation confirmed it. Two months after surgery, the diagnosis of central precocious puberty was confirmed according to physical examination and rising of serum gonadotropins. We started treatment with a Gonadotropin-releasing hormone (GnRH) agonist. Leydig cell tumor in children is an uncommon cause of precocious puberty. In every boy with the sign of peripheral precocious puberty and asymmetrical testicular enlargement, the testicular tumor should be considered. It may induce central precocious puberty after surgical resection and this diagnosis should be considered in the patient’s follow-up in the next visits.

Predictors of Relapse and Survival in Testicular Germ cell Tumors in Children

Background: Testicular germ cell tumors are common solid organ malignancies in children with a survival rate of more than 90 %. This study aims to assess the predictors of relapse and survival in testicular germ cell tumors in children.&#x0D; Methodology: A retrospective review was conducted on children up to the age of 18-years from January 2010 to December 2020 with a diagnosis of primary testicular germ cell tumors. Factors related to relapse and survival like age, baseline levels of tumor markers in serum and on relapse, stage at diagnosis, histological type, tumor laterality &amp; size of the tumor in testicular germ cell tumors were analyzed. The data was entered into SPSS version 20. Statistical significance was set at a p-value ≤0.05.&#x0D; Results: A total of 115 patients with a mean age of 5.42+ 1.54 years having testicular germ cell tumor were treated. Seventeen patients (14.7 %) had relapse of disease. Relapse was highest in patients with stage I disease (64.7 %). Yolk sac tumor was the most common pathology that was noted in twelve (70 %) patients. The most common site of relapse was the retroperitoneum (70 %). Age of patient, stage of disease, and lymphovascular invasion were significant predictors of relapse and survival in testicular germ cell tumors.&#x0D; Conclusion: Management of patients with testicular germ cell tumors requires standardized follow-up protocol for early detection and treatment of disease relapse. Complete surgical excision with meticulous control of the residual disease is critical to prevent disease relapse.&#x0D; Keywords: Chemotherapy, Children, Outcome, Relapse, Testicular Tumor&#x0D; &#x0D;

Blastic plasmacytoid dendritic cell tumor in children.

Blastic plasmacytoid dendritic cell tumor in children.

Extracranial Germ Cell Tumor in Children: A Three-year Experience of Pediatric Hematology/Oncology Center, Medical City

Extracranial Germ Cell Tumor in Children: A Three-year Experience of Pediatric Hematology/Oncology Center, Medical City

Primary central nervous system germ cell tumors in children and young adults: A review of controversies in diagnostic and treatment approach

Primary central nervous system (CNS) germ cell tumors (GCT) are a rare heterogenous group of cancers, arising most commonly in the second decade of life. Through several clinical trials conducted around the world by various groups, the treatment approach for CNS GCT has advanced substantially with generally improved overall outcomes. In recent years, the goal of clinical trials has been focused on reduction of the radiotherapy burden and minimization of long-term toxicity. This review summarizes the current diagnostic and treatment regimens for CNS GCT, examines the controversies associated with these approaches, gaps in contemporary knowledge, and underscores the challenges we face. We also explore future directions in the management of CNS GCT with the ultimate overall aim of preserving curative outcomes, identifying novel biomarkers, and mitigating neurocognitive, endocrine, and psychological toxicity through prospective clinical studies.

Identification and verification of microRNA signature and key genes in the development of osteosarcoma with lung metastasis.

Osteosarcoma (OS) is a heterogeneous malignant spindle cell tumor in children under the age of 20. This study aims to research the association between Solute Carrier Family 7 Member 8 (SLC7A8) as well as related genes and OS. OS and normal samples (GSE38698 and GSE85537) were downloaded from Gene Expression Omnibus dataset. The bioinformatics analysis was performed to distinguish 2 differentially expressed genes, prognostic candidate genes and functional enrichment pathway. Immunohistochemistry and quantitative real-time PCR were utilized for further study. There were 5 DEMs and 10 differentially expressed genes in cancer tissues compared to normal tissues. According to the km-plot software, ARHGEF3, BSN, PQLC3, and SLC7A8 were significantly related to the overall survival of patients with OS. Furthermore, Multivariate analysis included that SLC7A8 was independent risk factors for OS patients. Furthermore, immunohistochemistry and quantitative real-time PCR outcomes indicated that the expression level of SLC7A8 and hsa-miR-506 was differentially expressed in lung metastasis OS tissues and non-metastasis tissues. The prognostic model based on the miRNA-mRNA network could provide predictive significance for prognosis of OS patients, which would be worthy of clinical application. Our results concluded that SLC7A8 may play a key role in the development of OS.

Late mortality reduction among survivors of germ cell tumors in childhood and adolescence in Europe: A report from the PanCareSurFup cohort.

Data on late mortality from pediatric germ cell tumors (GCTs) are limited to small case series. Our population-based study aimed to investigate excess risk of death in survivors of GCT in childhood and adolescence, whether long-term mortality changed over time and by period of diagnosis. The PanCare Childhood and Adolescent Cancer Survivor Care and Follow-Up Studies (PanCareSurFup) cohort includes 2773 five-year survivors diagnosed under 21years of age with gonadal and extragonadal GCT (from 1940 to 2008). We calculated standardized mortality ratios (SMRs) and absolute excess risks (AERs). We fitted a Cox's model to assess the impact of treatment period. We estimated 10-year survival and calculated average percentage changes between periods of diagnosis (1970-1979, 1980-1989, 1990-1999) to assess whether late mortality decreased. GCT survivors had an almost four-fold excess risk of dying compared to general population. The risk of death for patients treated after 1980 was nearly halved compared to patients treated before 1980. Survivors diagnosed in 1990-1999 had a 10-year survival rate of 99%, which was 2.4% and 1.1% higher than for patients treated in 1970-1979 and 1980-1989, respectively. This is the largest population-based study in Europe and showed a decrease in long-term mortality for survivors of GCTs in childhood and adolescence over the last decades. After the introduction of platinum compound in 1980, which is a paradigm of success compared to the previous treatments, no major changes in drug therapies have been made to treat GCTs in the last 40years. However, GCT survivors maintain an excessive risk of death that requires long-term care.

Outcome of Germ Cell Tumors in Children after Surgical Management; A Longitudinal Study

Aim: To assess the outcome of germ cell tumors in children after surgical management.&#x0D; Study Design: A longitudinal study.&#x0D; Place and duration: Pediatric surgery and oncology department of the national institute of child health Karachi from January 2017 to December 2018.&#x0D; Methodology: A total of 79 children between the ages of 0 to 12 years were included in the study. Baseline, ultrasound, CT scan, tumor markers, and biopsy for tissue diagnosis were done. After surgical excision, they were followed up for 12 months with tumor markers, ultrasound and CT Scans for any recurrence. We just observed patients with mature teratoma and did surveillance for immature variety. The rest of the subtypes received JEB chemotherapy (carboplatin, etoposide, and bleomycin) as per UK-based chemotherapy protocol according to their disease stage.&#x0D; Results: Most common age of presentation was &lt; 1 month old. The antenatal diagnosis was made in 2 (2.5%) children. Sacrococcygeal region 54 (68.3%) was the most commonly involved region followed by gonads in 17 (21.5%) cases. According to histology mature teratoma was present in 32 (40.5%) children. Complete resection was done in 52 (65.8%) children. Recurrence occurred in one child and 3 children expired.&#x0D; Conclusion: In the current study, the general outcome was good in terms of complete excision in most of the patients. Worst outcome noted in yolk sac tumor. There was a minimal recurrence rate.

Shattering Glass Ceilings: Three Black Women Surgeons Ascend to Chairs of Departments of Surgery.

Shattering Glass Ceilings: Three Black Women Surgeons Ascend to Chairs of Departments of Surgery.