Oncology Commons^™

Amplification Of The Plag-Family Genes-Plagl1 And Plagl2-Is A Key Feature Of The Novel Tumor Type Cns Embryonal Tumor With Plagl Amplification, Michaela-Kristina Keck, Martin Sill, Andrea Wittmann, Piyush Joshi, Damian Stichel, Pengbo Beck, Konstantin Okonechnikow, Philipp Sievers, Annika K. Wefers, Nasir Uddin

Department of Pathology and Laboratory Medicine

Pediatric central nervous system (CNS) tumors represent the most common cause of cancer-related death in children aged 0-14 years. They differ from their adult counterparts, showing extensive clinical and molecular heterogeneity as well as a challenging histopathological spectrum that often impairs accurate diagnosis. Here, we use DNA methylation-based CNS tumor classification in combination with copy number, RNA-seq, and ChIP-seq analysis to characterize a newly identified CNS tumor type. In addition, we report histology, patient characteristics, and survival data in this tumor type. We describe a biologically distinct pediatric CNS tumor type (n = 31 cases) that is characterized by focal …

Aplastic Anemia: Clinicohaematological Features, Treatment And Outcome Analysis, Rabia Wali, Zehra Fidoo, Salman Adil, Muhammad Ahmed Naqvi

Department of Pathology and Laboratory Medicine

Objective: To determine the clinicohaematological features, treatment and outcome of children diagnosed with aplastic anemia at a single institution.

Study Design: Observational study.

Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 1999 till December 2008.

Methodology: Medical records of children aged less than 15 years of age diagnosed with aplastic anemia were reviewed. Clinicohaematological features, treatment and its response to therapy and outcome were recorded. Results were described in percentages.

Results: Ninety patients were diagnosed to have aplastic anemia (AA); 65 were male during the study period. Age ranged from 1 to 15 years. Fever …

Orbital Embryonal Rhabdomyosarcomain Karachi (1998-2002), Yasmin Bhurgri, A. Mazhar, H. Bhurgri, A. Usman, J. Malik, A. Bhurgri, R. Ahmed, S. Muzaffar, N. Kayani, Shahid Pervez, S. H. Hasan

Department of Pathology and Laboratory Medicine

Objective: To study the epidemiology of ocular Rhabdomyosarcoma (ORMS) in Karachi.

Methods: Incident ORMS cases resident of Karachi, registered at Karachi Cancer Registry (KCR) during 1st January 1998 to 31st December 2002 were included in the study. The data were classified using ICD-O2; computerized with Canreg-3, and analyzed using SPSS 10.0.

Results: Ten cases of ORMS were reported to KCR during 1998-2002. RMS originated in the orbit in eight cases, conjunctiva in one and eyelid in one. Nine cases presented with proptosis, associated with conjunctivitis in four cases. One case presented with eyelid swelling. The crude annual incidence rate was …

Flow Cytometric Analysis Of Childhood Leukemias, R. Z. Yusuf, Shahid Pervez, S. A. Aziz, Mohammad Khurshid

Department of Pathology and Laboratory Medicine

Objective: To collect demographic data for childhood (less than 15 years) leukemias in Karachi, describe the accuracy of the cell surface markers routinely used in the flow cytometric analysis of leukemic cells and arrive at an ideal panel of antibodies for analyzing leukemic samiples.

Materials and Methods: Data from 62 consecutive cases of childhood leukemias referred to the Department of Pathology, Aga Khan University Hospital. (AKUH) between January 1995 and December 1998 was analyzed using Epi Info Version 6. Flow cytometry on all samples was performed using standard protocols.

Results: The mean age of patients was 8.2 years and 49 …

Immunohistochemical Evaluation Of Small Round Cell Tumors Of Childhood, Sajid H. Shah, Irshad N. Soomro, M. Shahid Siddiqui, Shahid Pervez, Sheema H. Hassan

Department of Pathology and Laboratory Medicine

Objective:

This study was done to evaluate the pediatric undifferentiated small round cell tumors with immunohistochemical staining.

Setting:

The present study included consecutive cases of small round cell tumors which were diagnosed in children (<15 years) in the section of Histopathology at the Aga Khan University Hospital, Karachi during the period of two years.

Methods:

The group of undifferentiated small round cell tumors were evaluated immunohistochemically by using a panel of antibodies on sections from routinely processed, formalin fixed, paraffin embedded tissue blocks.

Results:

The category of undifferentiated small round cell tumors included rhabdomyosarcoma (23.2%), primitive neuroectodermal tumor (17.9%), non-Hodgkin’s lymphoma (16.1%), neuroblastoma (14.2%), Ewing’s sarcoma (10.7%) in order of frequency. Osteosarcoma (Small cell variant), retinoblastoma and medulloblastoma comprised …