SMALL ROUND CELL TUMORS PRESENTING AS ORBITAL PROPTOSIS IN CHILDREN WITH BONE MARROW INVOLVEMENT: A STUDY OF HAEMATO- MORPHOLOGICAL FEATURE.

Abstract

Introduction: Childhood proptosis is an interesting clinical presentation of underlying hematological and non-hematological disorders. Malignant proptosis is most commonly caused by retinoblastoma, rhabdomyosarcoma, leukemia/lymphomas and other metastatic tumors such as neuroblastoma. Bone marrow examination is a commonly used diagnostic and staging modality in the evaluation of hematological and non- hematological disorders in pediatric population. Patients with proptosis present with subtle changes in hemogram which point towards metastatic or hematologic etiology and hence a detailed hematologic work-up is mandatory. A descriptive cross-sectional study wasAim/objective: conducted to study the clinical, hematological and pathological data of interesting pediatric malignancies presenting as orbital proptosis with or without bone marrow involvement at the time of diagnosis from July 2020 to June 2021. All cases of orbital proptosis presenting withMethods: cytopenias referred for bone marrow examination were included in the study. Primary diagnosis of was made on histopathology/ne needle aspiration cytology combined with clinico-radiological features. Bone marrow procedure was done from posterior superior iliac spine. The informed consents were taken from the patients in the vernacular language after explaining the procedures. All procedures performed in the current study were approved by Institutional ethical committee. Aspirate smears and biopsy sections were stained by May-Grunwald-Giemsa and Hematoxylin and eosin respectively as per standard protocols. Special stains Myeloperoxidase (MPO), Alcin blue, PAS (perioidic acid and Schiff) and reticulin were performed when required. Samples were also processed for molecular genetics studies in acute leukaemias. The descriptive analysis of the data was done, and the results were presented as mean, frequencies, and percentages. Results: Of total 20 cases presenting as orbital-proptosis, only 11 cases showed bone marrow involvement as acute myeloid leukemia (45%) and as a metastatic site (55%). The most common cause of bilateral orbital proptosis was metastatic malignancies followed by acute myeloid leukaemias. Anaemia and thrombocytopenia were signicantly (p<0.05) associated with marrow inltration. The present analysis showedConclusion: different types of pattern bone marrow inltration had prognostic implications. Pancytopenias/bicytopeinias were most commonly seen in association with solid malignancies. Acute leukaemias presented with leukocytosis along with reduction in red cells and megakaryocytic lineages. Some cases primary diagnosis was initially made from bone marrow examination only. The present study highlights the signicance of bone marrow examination in patients with proptosis wherein subtle changes in hemogram point toward metastatic or hematologic etiology.