Question

An eight year old boy presents with swelling in the left eye of 3 months duration. Examination reveals proptosis of the left eye with preserved vision. The right eye is normal. CT scan reveals an intraorbital, extraconal mass lesion. Biopsy reveals embryonal rhabdomyosarcoma. Metastatic workup is normal. The standard line of treatment is

• A. Chemotherapy only
• B. Wide local excision
• C. Enucleation
• D. Chemotherapy and radiotherapy

Hint:

Orbital rhabdomyosarcoma responds well to chemo and radiation together, so the eye and vision can usually be saved without surgery.

Answer 1

The Correct Option is D

Step 1: Understanding the Question:
A child has a biopsy-proven embryonal rhabdomyosarcoma confined to the orbit, with normal vision and no spread elsewhere, and we need the standard treatment for this exact situation.

Step 2: Key Formula or Approach:
Modern pediatric oncology protocols for localized rhabdomyosarcoma favor organ-sparing treatment whenever the tumor is chemosensitive and radiosensitive, saving radical surgery for tumors that cannot be controlled any other way.

Step 3: Detailed Explanation:
Orbital rhabdomyosarcoma responds strongly to combination chemotherapy, which shrinks the tumor and covers micrometastatic disease, and to radiotherapy, which mops up residual local disease in the orbit. Together these achieve excellent local control while keeping the eye and useful vision intact, which matches this boy's presentation of preserved vision. Chemotherapy alone leaves local disease under-treated. Wide excision is not feasible in the confined orbital space without major functional loss, and it is unnecessary given how well this tumor responds to chemoradiation. Enucleation is kept in reserve for a blind, disfigured, or unsalvageable eye, not for a case where vision is still good.

Step 4: Final Answer:
The standard line of treatment for this boy's localized orbital embryonal rhabdomyosarcoma is chemotherapy combined with radiotherapy.