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High clinical suspicion urged

What was the cause of a painless swelling of a young boy's upper lid?

Soosan Jacob

Posted: Saturday, September 1, 2018

A five-year-old boy was referred by a general practitioner to the department of ophthalmology at University Hospital Galway (UHG) for right eye preseptal cellulitis, reported a poster presentation at the Irish College of Ophthalmologists Annual Conference in Kilkenny, Ireland.
This referral was made after he had experienced two weeks of right upper-lid swelling, reported Nora Mohd and Gerry Fahy, UHG.
The child’s mother believed the swelling was caused following blunt to trauma to the lid by his brother while the two were playing. He did not complain of any pain or vision loss, however.
Upon examination, doctors found a firm, fixed lump measuring 3x2cm, which caused mild limitation of eye movement when looking up.
Clinical differential diagnosis suggested a medial angular dermoid cyst, lymphangioma, rhabdomyosarcoma or neuroblastoma. An excision biopsy of the lesion showed fibro adipose tissue infiltrated by rhabdomyosarcoma with a solid growth pattern.
Although rare, orbital rhabdomyosarcoma is the most common primary malignancy of the orbit in children. It tends to develop in the soft tissue of the orbit. Most parents will notice either ptosis or proptosis, or both.
A CT scan will show the orbital mass and in advanced cases there maybe destruction of the bone. In this case the orbital mass was well circumscribed in the anteromedial orbit.
Treatment consisted of chemotherapy and consideration will be given to orbital x irradiation depending on response to chemotherapy. Cure rates have markedly improved with 
use of chemotherapy and x irradiation. Long term follow-up is required.
The patient received chemotherapy IVA (ifosfamide, vincristine, actinomycin) in Our Lady’s Children’s Hospital, Crumlin, every three weeks, as well as vincristine alone in UHG every week. This began in November 2017 and continued until May 2018. He has had an excellent response to chemotherapy.
The doctors concluded that rhabdomyosarcoma may appear as a benign process in its early stages. It is an important diagnosis to consider when presented with a child with an enlarging orbital lesion.
The authors recommend a high level of clinical suspicion, prompt orbital imaging and prompt biopsy to establish the diagnosis of rhabdomyosarcoma.