Orbital Rhabdomyosarcoma: Causes, Symptoms, and Treatment

Orbital Rhabdomyosarcoma

A rhabdomyosarcoma is a relatively rare soft-tissue tumor (sarcoma) that develops under the upper eyelid near the nose (superonasal orbit). Although this type of cancer is most frequently diagnosed in children, orbital rhabdomyosarcomas can affect people of all ages.

Tampa General Hospital’s Neuroscience Institute and Ear, Nose and Throat (ENT) Institute offer internationally acknowledged expertise in the diagnosis and treatment of orbital rhabdomyosarcomas and other complex brain tumors. Through our highly acclaimed institutes, our patients have access to a world-class team of neurosurgeons and ENT surgeons who are skilled and experienced in performing the latest open, endoscopic and minimally invasive surgical techniques. In recognition of our unwavering commitment to excellence, U.S. News & World Report has given TGH a “high performing” designation for Neurology and Neurosurgery for 2023-24.

What Can Cause an Orbital Rhabdomyosarcoma?

Most orbital rhabdomyosarcomas develop with no identifiable cause. However, scientists have linked this type of cancer to several uncommon genetic syndromes, including:

  • Neurofibromatosis type 1
  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Costello syndrome
  • Noonan syndrome

Some research studies suggest that the risk of developing an orbital rhabdomyosarcoma may increase with exposure to X-rays before birth, but those findings are inconclusive.

What Are the Symptoms of an Orbital Rhabdomyosarcoma?

In many cases, the first noticeable sign of an orbital rhabdomyosarcoma is a bulging or protruding eyeball (proptosis). Other symptoms can include:

  • A red, swollen or droopy eyelid (ptosis)
  • Difficulty moving the affected eye
  • Vision changes or loss of vision
  • Discoloration of the iris
  • Nasal congestion
  • Bleeding in the nose, throat or ears

In some cases, an orbital rhabdomyosarcoma is first discovered by an ophthalmologist during a routine eye examination.

How Is an Orbital Rhabdomyosarcoma Diagnosed?

If an orbital rhabdomyosarcoma is suspected based on the symptoms, a physician will typically perform a physical examination and order an imaging study, such as a:

The resulting images may reveal a mass near or attached to one of the ocular or orbital muscles.

Like all types of cancer, an orbital rhabdomyosarcoma cannot be conclusively diagnosed without a biopsy, which involves collecting a sample of the suspicious cells for analysis by a pathologist under a microscope. The types of biopsy procedures that may be used to diagnose an orbital rhabdomyosarcoma include:

  • Needle biopsy – The physician will insert a thin needle through the skin, guide it into the tumor and remove a small amount of tissue.
  • Surgical biopsy – The physician will make an incision in the skin and remove either the entire tumor (excisional biopsy) or a portion of the tumor (incisional biopsy).

What Are the Treatment Options for an Orbital Rhabdomyosarcoma?

Treatment for an orbital rhabdomyosarcoma usually involves a combination of skull base surgery, radiation therapy and chemotherapy. The goal is to surgically remove as much of the tumor as is safely possible, then destroy any remaining cancer cells.

Choose TGH for World-Class Care

In addition to extensive expertise, the experts at the TGH Skull Base Surgery Center have the resources necessary to meet the unique needs of children and adults with orbital rhabdomyosarcomas. If would like to discuss your symptoms with a specialist at TGH, contact us at (800) 822-3627 to schedule an appointment.

Take the Next Step

Learn more about Tampa General Hospital’s Skull Base Surgery Center and find out if our approach to care is right for you.