Juvenile rhabdomyosarcomas in Port Harcourt, Nigeria: A twelve year review

Abstract

Background: Juvenile rhabdomyosarcoma (JRMS) though rare, is the most common soft tissue malignancy of childhood that exhibits bimodal age distribution pattern.

Objective: Review the pattern of JRMS in Port Harcourt based on sex, age, tumor sites and histologic types.

Design: A retrospective descriptive study.

Setting: The study was conducted in the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt, Nigeria over 12 years.

Methodology: We studied 21 juvenile rhabdomyosarcomas during the period under review. The hematoxylin and eosin stained histological slides were retrieved and reviewed to confirm previous diagnosis and histologically typed for the study. The sex, age and tumor sites were retrieved from the histology consultation forms,

Result: These tumors were diagnosed from patients aged 0 - 20 years with the highest frequencies (38.1% each) occurring at 0 - 5 and 16 - 20 years age group. Males are more affected with a ratio 3.2:1. The trunk is the most common site of occurrence (47.7%) of which the genitourinary system is the most affected (23.8%) in this study. The most common histologic type is the embryonal rhabdomyosarcoma (71.5%). Alveolar rhabdomyosarcoma accounted for (19%) and the sarcoma botryoides (9.5%).

Conclusion: The age of presentation and anatomic sites of the tumor are important in the diagnosis of these tumors. If a tumor histologically shows as small round blue cells, rhabdomyosarcoma should be considered as a differential diagnosis.