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: Identify the tumor and its behavior.
Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma of the orbit in children. It grows fast and causes proptosis, but it stays localized to the orbit for a long time before spreading, which fits this boy's normal metastatic workup.

Step 2: Recall the modern treatment philosophy.
Because orbital rhabdomyosarcoma is very chemosensitive and radiosensitive, and because the eye and vision can often be preserved, treatment protocols moved away from radical surgery decades ago. The standard approach for localized, non-metastatic orbital disease is multi-agent chemotherapy combined with radiotherapy to the orbit.

Step 3: Explain why chemotherapy alone is not enough.
Chemotherapy shrinks the tumor and treats any microscopic spread, but local control of the residual orbital disease still needs radiotherapy, so chemotherapy alone is an incomplete plan for a localized orbital mass.

Step 4: Explain why surgery is not the answer here.
Wide local excision is not practical in the tightly packed orbit without badly damaging vision and surrounding structures, and it is not needed since the tumor responds well to chemoradiation. Enucleation, removing the eye, is reserved for cases where the globe itself is destroyed or blind, which is not the case here since vision is preserved; the whole point of the combined chemoradiation approach is to spare the eye.

Step 5: Final answer.
\[ \boxed{\text{Chemotherapy and radiotherapy}} \]