Open AccessBotryoid rhabdomyosarcoma of the conjunctiva in a young boy
Author(s) -
Justin D Pennington,
R Joel Welch,
Sara E. Lally,
Jerry A. Shields,
Ralph C. Eagle,
Carol L. Shields
Publication year - 2018
Publication title -
middle east african journal of ophthalmology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.357
H-Index - 25
eISSN - 0975-1599
pISSN - 0974-9233
DOI - 10.4103/meajo.meajo_8_18
Subject(s) - medicine , fornix , rhabdomyosarcoma , conjunctiva , radiation therapy , pathology , surgery , sarcoma , hippocampus
Primary conjunctival rhabdomyosarcoma (RMS) is a rare entity that can present with or without papillomatous features. A 5-year-old Asian boy was referred for a rapidly growing conjunctival tumor in the superior fornix of the left eye. Surgical excision yielded a 28 mm multilobulated papillomatous specimen that exhibited histopathologic and immunohistochemical features consistent with embryonal (botryoid) RMS. Molecular analysis revealed the absence of the PAX3/FOXO1 fusion gene, indicating favorable prognosis. After surgery, he was promptly treated with systemic chemotherapy and proton beam radiotherapy.