Palbociclib in Solid Tumor Patients With Genomic Alterations in the cyclinD-cdk4/6-INK4a-Rb Pathway: Results From National Cancer Institute-Children's Oncology Group Pediatric Molecular Analysis for Therapy Choice Trial Arm I (APEC1621I).

Purpose: The National Cancer Institute-Children's Oncology Group Pediatric Molecular Analysis for Therapy Choice trial assigned patients age 1-21 years with relapsed or refractory solid tumors, lymphomas, and histiocytic disorders to phase II treatment arms of molecularly targeted therapies on the basis of genetic alterations detected in their tumor. Patients with tumors that harbored prespecified genomic alterations in the cyclinD-CDK4/6-INK4a-Rb pathway with intact Rb expression were assigned and treated with the cdk4/6 inhibitor palbociclib.

Methods: Patients received palbociclib orally once daily for 21 days of 28-day cycles until disease progression, intolerable toxicity, or up to 2 years.

Role of Fine Needle Aspiration Cytology in Paratesticular Neoplasms-A Case Reports-Based Systematic Review.

Introduction: The cytology features of neoplastic paratesticular lesions are mostly documented as case reports. Thus, we conducted a case report-based literature review to identify the characteristics of paratesticular neoplasms and tried to determine the significance of FNAC in these tumors.

Methods And Materials: The studies were searched using PubMed and Scopus.

Successful treatment of rare case of rhabdomyosarcoma of urinary bladder in adult, experience from tertiary hospital. Case report.

Introduction And Importance: Rhabdomyosarcoma [RMS] is a malignant soft-tissue neoplasm characterized by skeletal muscle differentiation. It accounts for 7 % of childhood malignancies and is, by a wide margin, the most common sarcoma of childhood (Pappo, 1996). Approximately 20 % of cases of childhood rhabdomyosarcoma occur in the genitourinary tract (vagina, urinary bladder, prostate, paratestis, and uterus), and they are most commonly observed in the head and neck region.

Cytological Features and Mimickers of Thyroid Gland Sarcomas: A Case-Based Study.

Sarcomas of thyroid glands represent a distinctive subset of rare and perplexing anomaly that present a challenges in the field of thyroid pathology. Thyroid sarcomas, primary or secondary, are exceptionally rare with only a handful of case reports documented so far. The challenges lie in the fact that certain primary thyroid malignancies of epithelial origin may exhibit spindle cell morphology, making them difficult to differentiate from thyroid sarcomas.

Improving Individualized Rhabdomyosarcoma Prognosis Predictions Using Somatic Molecular Biomarkers.

Purpose: Molecular markers, such as fusion genes and and mutations, increasingly influence risk-stratified treatment selection for pediatric rhabdomyosarcoma (RMS). This study aims to integrate molecular and clinical data to produce individualized prognosis predictions that can further improve treatment selection.

Patients And Methods: Clinical variables and somatic mutation data for 20 genes from 641 RMS patients in the United Kingdom and the United States were used to develop three Cox proportional hazard models for predicting event-free survival (EFS).

Orbital Lesions: A bird's eye view of series of 2068 cases in 27 years in a tertiary care hospital in Pakistan.

Objective: To determine the relative frequency of orbital lesions based on the site of origin and histopathology at a Tertiary care hospital (Mayo Hospital, Lahore Pakistan) from 1996 till 2022 (27 years).

Methods: This descriptive case series included 2651 patients of all age groups presenting with orbital lesions who initially got enrolled at Institute of Ophthalmology Mayo Hospital, Lahore from 1996 till 2022. Of these, 583 patients left against medical advice.

A rare case of perianal alveolar rhabdomyosarcoma.

Perianal alveolar rhabdomyosarcoma is a rare sarcoma that requires a high index of suspicion along with tissue biopsy for accurate diagnosis. Successful treatment, even in the setting of recurrence, requires a multidisciplinary approach.

Evaluation of Hi-C Sequencing for Detection of Gene Fusions in Hematologic and Solid Tumor Pediatric Cancer Samples.

Hi-C sequencing is a DNA-based next-generation sequencing method that preserves the 3D genome conformation and has shown promise in detecting genomic rearrangements in translational research studies. To evaluate Hi-C as a potential clinical diagnostic platform, analytical concordance with routine laboratory testing was assessed using primary pediatric leukemia and sarcoma specimens. Archived viable and non-viable frozen leukemic cells and formalin-fixed paraffin-embedded (FFPE) tumor specimens were analyzed.

Whole Lung Irradiation in Rhabdomyosarcoma With Lung Metastases: A Report From the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

Purpose: Patients with rhabdomyosarcoma with metastatic disease have a poor prognosis despite therapy intensification. The aim of this study was to investigate the efficacy of whole lung irradiation (WLI) in patients with rhabdomyosarcoma and lung metastases.

Methods: Patients with rhabdomyosarcoma with lung metastases enrolled on four Children's Oncology Group protocols (D9802, D9803, ARST08P1, ARST0431) were retrospectively reviewed.

Late dental and bone alterations in patients after orbital rhabdomyosarcoma treatment.

Purpose: Orbital rhabdomyosarcoma is a rare soft tissue sarcoma in childhood but with a good prognosis. Treatment usually includes surgery, chemotherapy, and radiotherapy. This study aimed to evaluate long-term alterations in teeth and cranial bones in children, adolescents, and young adults after oncologic treatment for childhood orbital rhabdomyosarcoma.

Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes.

Introduction: Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST.

Embryonal Rhabdomyosarcoma of the Bile Ducts Causing Obstructive Jaundice in a Child: A Case Report.

Jaundice in older children can occur when any obstruction is found within the bile ducts, either from bile stones, parasites, choledochal cysts and rarely, secondary to tumors. We present a previously well, 10-year-old Filipino boy with three-week history of progressive jaundice and tea-colored urine, and was initially assessed to have biliary ascariasis. Ultrasound showed a heterogeneous focus spanning the gallbladder neck and confirmed on endoscopic retrograde cholangiopancreatography as an exophytic mass at the ampulla of Vater.

New dual inducible cellular model to investigate temporal control of oncogenic cooperating genes.

The study of cooperating genes in cancer can lead to mechanistic understanding and identifying potential therapeutic targets. To facilitate these types of studies, we developed a new dual-inducible system utilizing the tetracycline- and cumate-inducible systems driving HES3 and the PAX3::FOXO1 fusion-oncogene, respectively, as cooperating genes from fusion-positive rhabdomyosarcoma. With this model, we can independently induce expression of either HES3 or PAX3::FOXO1, as well as simultaneously induce expression of both genes.

Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee.

Purpose: To describe clinical features, risk factors, and outcomes of patients with perineal and perianal rhabdomyosarcoma.

Methods: The records of 51 patients (38 perineal and 13 perianal) enrolled on Children's Oncology Group clinical trials between 1997 and 2012 were reviewed.

Results: At presentation, 53% were female, 65% were older than 10 years of age, 76% were alveolar histology, 76% were more than 5 cm, 84% were invasive, 65% were regional node positive by imaging, 49% were metastatic, only 16% were grossly resected upfront, and 25% of patients had a delayed excision.

The utility of DNA methylation profiling in the diagnosis of un-, de- and trans-differentiated melanoma: a series of 11 cases.

Aims: Melanomas are recognised for their remarkable morphological plasticity. Some tumours may lose conventional features and/or acquire non-melanocytic characteristics, referred to as undifferentiated, dedifferentiated and transdifferentiated melanoma. Despite this phenotypical variability, melanomas typically maintain their cancer driver aberrations, affecting genes such as BRAF, NRAS and NF1.

The Impact of Radiation Therapy on Metastatic Rhabdomyosarcoma: Results From the EpSSG MTS 2008 Study.

Purpose: Radiation oncologists use radiation variably for children with metastatic rhabdomyosarcoma (RMS). Data from the European paediatric Soft tissue sarcoma Study Group (EpSSG) MTS 2008 study were retrospectively analyzed to validate the previous observation that the use of radiation is associated with improved outcomes and guide future recommendations on radiation use in this patient group.

Methods And Materials: The radiation delivered to 216 patients aged 0 to 21 years with metastatic RMS was retrospectively reviewed and classified as radical (all sites of disease irradiated within the protocol parameters), partial (some sites irradiated within the protocol parameters), and none (no radiation or delivered outside the protocol parameters).