Oncology

2020 ◽  
pp. 203-213
Author(s):  
Roy L. Kao ◽  
Maritza E. Ruiz ◽  
Moran Gotesman ◽  
James H. Ch’ng
Keyword(s):  
Pediatric Oncology ◽  
Hematopoietic Cell Transplantation ◽  
Wilms Tumor ◽  
Tumor Lysis Syndrome ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Cancer Predisposition Syndrome ◽  
Pediatric Cancers ◽  
Questions And Answers ◽  
Board Review

Pediatric oncology focuses on the diagnosis and management of cancer in infants, children, and adolescents. Leukemias, lymphomas, and other hematological malignancies are the most common group of pediatric cancers, followed by brain tumors. Other important pediatric cancers include Wilms tumor, neuroblastoma, sarcomas, histiocytosis, and germ cell tumors. Certain cancers can present with oncologic emergencies, such as tumor lysis syndrome or spinal cord compression, or as part of a cancer predisposition syndrome. With increased survival rates, short- and long-term complications of chemotherapy, radiation, surgery, and hematopoietic cell transplantation are also prevalent and important to recognize. This chapter reviews these key concepts in pediatric oncology using board review–style questions and answers.

scholarly journals Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

PLoS ONE ◽  
2022 ◽  
Vol 17 (1) ◽  
pp. e0261729
Author(s):  
Prakriti Roy ◽  
Sophie E. van Peer ◽  
Martin M. de Witte ◽  
Godelieve A. M. Tytgat ◽  
Henrike E. Karim-Kos ◽  
...  
Keyword(s):  
The Netherlands ◽  
Pediatric Oncology ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Survival Rates ◽  
Renal Tumors ◽  
Childhood Malignancies ◽  
Netherlands Cancer Registry ◽  
Central Pathology ◽  
Centralized Care

Around 6% of all childhood malignancies represent renal tumors, of which a majority includes Wilms tumor (WT). Although survival rates have improved over the last decades, specific patients are still at risk for adverse outcome. In the Netherlands, since 2015, pediatric oncology care for renal tumors has been centralized in the Princess Máxima Center for Pediatric Oncology. Here, we describe experiences of the first 5 years of centralized care and explore whether this influences the epidemiological landscape by comparing data with the Netherlands Cancer Registry (NCR). We identified all patients <19 years with a renal mass diagnosed between 01-01-2015 and 31-12-2019 in the Princess Máxima Center. Epidemiology, characteristics and management were analyzed. We identified 164 patients (including 1 patient who refused consent for registration), in our center with a suspicion of a renal tumor. The remaining 163 cases included WT (n = 118)/cystic partially differentiated nephroblastoma (n = 2)/nephrogenic rests only (n = 6) and non-WT (n = 37). In this period, the NCR included 138 children, 1 17-year-old patient was not referred to the Princess Máxima Center. Central radiology review (before starting treatment) was performed in 121/163 patients, and central pathology review in 148/152 patients that underwent surgery. Treatment stratification, according to SIOP/EpSSG protocols was pursued based on multidisciplinary consensus. Preoperative chemotherapy was administered in 133 patients, whereas 19 patients underwent upfront surgery. Surgery was performed in 152 patients, and from 133 biomaterial was stored. Centralization of care for children with renal tumors led to referral of all but 1 new renal tumor cases in the Netherlands, and leads to referral of very rare subtypes not registered in the NCR, that benefit from high quality diagnostics and multidisciplinary decision making. National centralization of care led to enhanced development of molecular diagnostics and other innovation-based treatments for the future.

The Outcome of Wilms’ Tumor in Infants, Italy 1970-79

1982 ◽  
Vol 68 (2) ◽  
pp. 133-136
Author(s):  
G. Pastore ◽  
L. Cordero Di Montezemolo ◽  
A. Brach Del Prever ◽  
G. Bartolozzi ◽  
M. Carli ◽  
...  
Keyword(s):  
Pediatric Oncology ◽  
Wilms Tumor ◽  
Postoperative Radiotherapy ◽  
Survival Rates ◽  
Group Iii ◽  
Group I ◽  
Group Ii

Thirty-four infants under 1 year of age with Wilms’ tumor were diagnosed and treated in 14 Italian pediatric oncology units during 1970-79. The 3-year survival rates decreased with higher group unilateral tumors: 95% in group I Wilms’ tumor, 75% in group II and 20% in group III. The survival rates for children with group I and II Wilms’ tumor were similar for those who were treated with surgery and chemotherapy and those who also received postoperative radiotherapy. During 1975-79 fewer patients with group I Wilms’ tumor received radiotherapy (1 of 11) than during 1970-74 (4 of 6, p < 0.05). All these children are alive at this writing.

scholarly journals Commonly Reported Adverse Events Associated With Pediatric Immunotherapy: A Systematic Review From the Children’s Oncology Group

2020 ◽  
Vol 38 (1) ◽  
pp. 16-25
Author(s):  
Janice S. Withycombe ◽  
Aimee Carlson ◽  
Carly Coleman ◽  
Sharon L. Leslie ◽  
Micah Skeens ◽  
...  
Keyword(s):  
Systematic Review ◽  
Adverse Events ◽  
Pediatric Oncology ◽  
Nursing Practice ◽  
Evaluation Criteria ◽  
Brentuximab Vedotin ◽  
Educational Materials ◽  
Eligibility Criteria ◽  
Assessment Development ◽  
Pediatric Cancers

Background: Immunotherapy is a new and promising approach to treating pediatric cancers. These types of therapies have unique mechanisms of action for identifying and fighting cancer, as compared with traditional chemotherapy, and therefore are associated with different therapy-related adverse events (AEs). The purpose of this systematic review was to review available evidence to: (a) identify commonly reported AEs associated with immunotherapy agents frequently used in pediatric oncology and (b) generate recommendations for nursing practice. Method: A clinical question was developed and used to guide the systematic literature review. Five immunotherapy agents (dinutuximab, blinatumomab, rituximab, inotuzumab ozogamicin, brentuximab vedotin) were selected for inclusion secondary to their high relevance to pediatric oncology. A literature search was conducted to locate articles published between January 1, 2003 and October 31, 2018. Results: Seventeen articles met eligibility criteria for inclusion and were evaluated using the Grading of Recommendations Assessment, Development, and Evaluation criteria. The most commonly reported AEs for the selected immunotherapy agents were identified and summarized. Strong recommendations are made for nurses to become familiar with the unique AE profiles associated with individual immunotherapy agents. Agent-specific recommendations for nursing practice regarding AEs associated with dinutuximab and rituximab were generated. Conclusions: Immunotherapy is rapidly emerging as an effective therapy for pediatric cancers. Nurses need to be aware of the breadth of agent-specific, immunotherapy-related AEs to appropriately monitor and manage patients receiving these therapies. Additional work is needed to confidently profile immunotherapy-related AEs in pediatric oncology and to develop agent-specific educational materials for patients/families.

scholarly journals GCT-30. TREATMENT OF PRIMARY INTRACRANIAL GERM CELL TUMORS: SINGLE INSTITUTION EXPERIENCE OF 74 CASES WITHOUT HISTOLOGICAL CONFIRMATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii334-iii334
Author(s):  
Chengcheng Guo ◽  
Qunying Yang ◽  
Jian Wang ◽  
Yonggao Mou ◽  
Zhongping Chen
Keyword(s):  
Germ Cell ◽  
Tumor Therapy ◽  
Survival Rates ◽  
Gamma Knife Radiosurgery ◽  
Germ Cell Tumors ◽  
Cancer Center ◽  
Chemotherapy Group ◽  
Tumor Group ◽  
Intracranial Germ Cell Tumors ◽  
Histological Confirmation

Abstract BACKGROUND AND OBJECTIVE Primary intracranial germ cell tumors (PIGCTs) are a group of heterogeneous tumors. It is very difficult to treat those patients without pathological diagnosis. This study retrospectively analyzed the clinical data and outcomes of patients with clinically diagnosed (without histologically confirmed) PIGCTs in SunYat-sen University Cancer Center. METHODS Patients who were clinically diagnosed as PIGCTs without histological diagnosis through surgical resection or biopsy were included in this study. Patients were analyzed for clinical characteristics, treatment patterns, outcomes and adverse effects. RESULTS From May 2002 to July 2014, 74 patients clinically diagnosed with PIGCTs received chemotherapy and/or radiotherapy at the Sun Yat-sen University Cancer Center. The median age was 16.5 years old (4–45 years old, majority was teenagers). The most of tumors were found in male, and located in the pineal and suprasellar regions. When the patients were grouped into diagnostic chemotherapy group (57 cases), diagnostic radiotherapy group (5 cases) and gamma knife radiosurgery group (12 cases) based on their initial anti-tumor therapy. The 5-year survival rates were 84.3%, 75.0% and 75.0%, respectively. There was a trend that the chemotherapy group got a better survival. Patients were allocated to secretory tumor group (49 cases) and non-secretory tumor group (25 cases) based on their levels of tumor makers (α-FP and β-hCG). The 5- year survival rates were 80% and 77.8% (P value = 0.966), respectively. CONCLUSION Clinical diagnosed PIGCT (without histological confirmation) patients may obtain good responses when receiving comprehensive treatments of chemotherapy combined with radiotherapy.

scholarly journals Malignant Rhabdoid Tumor of the Lung in the Young Adult: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Adel Attia ◽  
Moosa Suleman ◽  
Hesham Mosleh
Keyword(s):  
Case Report ◽  
Young Adult ◽  
Pediatric Oncology ◽  
Wilms Tumor ◽  
Rhabdoid Tumor ◽  
Malignant Rhabdoid Tumor

Malignant rhabdoid tumor (MRT) is one of the most aggressive and lethal malignancies in pediatric oncology. Malignant rhabdoid tumor was initially described in 1978 as a rhabdomyosarcomatoid variant of a Wilms tumor because of its occurrence in the kidney and because of the resemblance of its cells to rhabdomyoblasts. The absence of muscular differentiation led Haas and colleagues to coin the term rhabdoid tumor of the kidney in 1981, Haas et al..

scholarly journals Impact of CMV reactivation on relapse of acute myeloid leukemia after HCT is dependent on disease stage and ATG

2021 ◽  
Author(s):  
Amin T. Turki ◽  
Nikolaos Tsachakis-Mück ◽  
Saskia Leserer ◽  
Pietro Crivello ◽  
Tobias Liebregts ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Hematopoietic Cell Transplantation ◽  
Advanced Disease ◽  
Survival Rates ◽  
Disease Stage ◽  
The Impact ◽  
Cmv Reactivation ◽  
Acute Myeloid

Cytomegalovirus (CMV) reactivation is a frequent complication after allogeneic hematopoietic cell transplantation (HCT), whose impact on clinical outcome, in particular on leukemic relapse is controversial. We retrospectively analyzed 687 HCT recipients with acute myeloid leukemia (AML) and ciclosporin-based immunosuppression to better understand the differential impact of CMV on transplant outcomes depending on AML disease stage and in-vivo T-cell depletion with anti-thymocyte globulin (ATG). Without ATG, CMV reactivation associated with significantly reduced relapse, yet its effect was more pronounced for advanced disease AML (p=0.0002) than for patients in first complete remission (CR1, p=0.0169). Depending on the disease stage, ATG exposure abrogated relapse protection following CMV reactivation in advanced stages (p=0.796), while it inverted its effect into increased relapse for CR1 patients (p=0.0428). CMV reactivation was associated with significantly increased non-relapse mortality in CR1 patients without ATG (p=0.0187), but not in those with advanced disease and ATG. Following CMV reactivation, only patients with advanced disease had significantly higher event-free survival rates as compared to patients without CMV. Overall, our data suggest that both ATG and disease stage modulate the impact of post-HCT CMV reactivation in opposite directions, revealing a level of complexity that warrants future studies regarding the interplay between anti-virus and anti-tumor immunity.

scholarly journals Ten Reasons Why People With Down Syndrome are Protected From the Development of Most Solid Tumors -A Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Marta Pilar Osuna-Marco ◽  
Mónica López-Barahona ◽  
Blanca López-Ibor ◽  
Águeda Mercedes Tejera
Keyword(s):  
Down Syndrome ◽  
Solid Tumors ◽  
Tumor Suppressor Genes ◽  
Wilms Tumor ◽  
Extra Chromosome ◽  
Germ Cell Tumors ◽  
Chromosome 21 ◽  
Testicular Tumors ◽  
Unique Pattern ◽  
Increased Risk

People with Down syndrome have unique characteristics as a result of the presence of an extra chromosome 21. Regarding cancer, they present a unique pattern of tumors, which has not been fully explained to date. Globally, people with Down syndrome have a similar lifetime risk of developing cancer compared to the general population. However, they have a very increased risk of developing certain tumors (e.g., acute leukemia, germ cell tumors, testicular tumors and retinoblastoma) and, on the contrary, there are some other tumors which appear only exceptionally in this syndrome (e.g., breast cancer, prostate cancer, medulloblastoma, neuroblastoma and Wilms tumor). Various hypotheses have been developed to explain this situation. The genetic imbalance secondary to the presence of an extra chromosome 21 has molecular consequences at several levels, not only in chromosome 21 but also throughout the genome. In this review, we discuss the different proposed mechanisms that protect individuals with trisomy 21 from developing solid tumors: genetic dosage effect, tumor suppressor genes overexpression, disturbed metabolism, impaired neurogenesis and angiogenesis, increased apoptosis, immune system dysregulation, epigenetic aberrations and the effect of different microRNAs, among others. More research into the molecular pathways involved in this unique pattern of malignancies is still needed.

Malignant Sacrococcygeal Germ Cell Tumors in Children: A 30-year Experience from a Single Institution

2013 ◽  
Vol 99 (1) ◽  
pp. 51-56 ◽  
Author(s):  
Münevver Büyükpamukcu ◽  
Ali Varan ◽  
Serhan Küpeli ◽  
Saniye Ekinci ◽  
Sule Yalcin ◽  
...  
Keyword(s):  
Germ Cell ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Patient Characteristics ◽  
Tumor Stage ◽  
Event Free Survival ◽  
Treatment Results ◽  
Free Survival ◽  
Chemotherapy Protocol

Background Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors. Procedure Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded. Results A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis. Conclusions Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.

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