Orbital fleshy tumors rank fourth in the incidence of orbital tumors. Among pediatric patients, rhabdomyosarcoma is the most common, while malignant tumors originating from the lymphatic system are more prevalent in adult patients. Liposarcoma, fibrosarcoma, synovial sarcoma, and leiomyosarcoma are rare.

bubble_chart Clinical Manifestations

Orbital rhabdomyosarcoma is more common in males than in females, with 80% of cases occurring in children under 10 years old. The onset is rapid and progression is swift. Proptosis is a very common symptom, followed by the appearance of a mass at the orbital rim. In younger patients with rapid progression, the mass can quickly enlarge, leading to eyelid conjunctival edema and presenting an inflammatory appearance of the orbit. When the tumor originates in the superior orbit, it often infiltrates the superior rectus and levator palpebrae muscles, resulting in drooping of the upper eyelid (blepharoptosis) and impaired eye movement. Tumors arising within the retrobulbar muscle cone not only cause proptosis but may also show signs of posterior pole compression on fundus examination, such as optic disc congestion and edema, macular striae, and in severe cases, retinal detachment. Early X-rays may show no changes in the orbit, but in advanced stages, orbital enlargement and/or bone destruction may be observed. The mass at the orbital rim may appear well-defined and slightly elastic, easily mistaken for a benign tumor, or it may adhere to the orbital rim and be tender, mimicking orbital pseudotumor. Tumors originating beneath the conjunctiva often present with a polypoid appearance. If the tumor invades the intracranial space, it can cause severe headache accompanied by symptoms of increased intracranial pressure, such as nausea and vomiting.

1. Exenteration of the orbital contents. This tumor is highly malignant, and once diagnosed, immediate exenteration of the orbital contents should be performed, supplemented with radiotherapy and chemotherapy.

2. Radiotherapy. This tumor is relatively sensitive to radiation, and the postoperative dose (⁶⁰Co) is 40–60Gy.

3. For patients unsuitable for surgery, treatment with vincristine and cyclophosphamide may be attempted. Continuous treatment for one year can improve the cure rate.