The Diseases

Embryonal Rhabdomyosarcoma

What is it?

Rhabdomyosarcoma is the most common pediatric cancer of the orbit and has mesenchymal origin (muscle, fibroblast, cartilage, fat, etc). There seems to be almost no familial correlation; however, those who have inherited a general oncogene (cancer-causing gene) are more likely to get it. When associated with the orbit, the swelling that we observed can occur over a few days or weeks.¹

Prevalence:

There are about 250-340 cases per year in the United States, about 34 of which deal with the orbit. The mean age for diagnosis is about 8 years old.²,³

Methods of treatment:

Radiation, chemotherapy, surgery.

Survival rates:

Seer.cancer.gov lists children under the age of 5 as 79% survival. Others say up to 94% 5-year survival. However, one group reported that infants under the age on 1 have less than 50% survival (this may include alveolar rhabdomyosarcoma, which is more severe than embryonal).²,³,⁴

(Langerhans cell) Histiocytosis

What is it?

This is a complicated one. It is not technically a tumor or cancer but it acts a lot like one. It can grow in size and metastasize, but the difference is that it is not uncontrolled cell growth caused by a mutation. Rather, histiocytosis occurs when Langerhans cells (a type of white blood cell usually found in the skin) for an unknown reason migrate to a specific site in the body and attack the tissue. This autoimmune pseudotumor often attacks bone. There are obvious problems with this as the inflammation can destroy surrounding tissue. The biggest concern is that, similar to a cancer, it can spread to other organs and inhibit their functions. The prognosis for those with histiocytosis in one location is good while those with multiple affected organs are quite poor. However, sometimes this disease will disappear on its own.

Prevalence:

Histiocytosis accounts for about 1-3% of pediatric orbital tumors.¹

Methods of treatment:

An Australian paper used about 50% surgery alone, sometimes with methotrexate, vinblastine, and or prednisone. Radiation is also used, but less often for fear of secondary tumorogenesis.⁵

Survival rates:

The same Australian paper surveyed 34 patients 6 of whom had a relapse following primary treatment, 2 of those 6 had multi-organ relapse. 30 out of the 34 were alive and disease free, 2 were unknown, and the 2 were the multi-organ relapse, still with disease. The moral of the story is that this disease seems to be the easiest to treat and has the best survival. ⁵

Neuroblastoma

What is it?

Neuroblastoma is an embryonal cancer, which originates from the sympathetic nervous system.

Prevalence:

There are about 650 neuroblastomas diagnosed each year; however most of those are located at sites such as the adrenal gland.

Methods of treatment:

Surgery, chemotherapy

Survival rates:

Seer.cancer.gov lists children under the age of 1 as 83%³

Citations

1. Castillo BV, Kaufman L. Pediatric tumors of the eye and orbit. Pediatr Clin N Am. 2003;50(1):149–172.

2. Shields JA, Shields CL. Rhabdomyosarcoma:: Review for the Ophthalmologist**. Survey of ophthalmology. 2003;48(1):39–57.

3. SEER Web Site. Available at: http://seer.cancer.gov/ [Accessed October 29, 2010].

4. Kodet R, Newton WA, Hamoudi AB, et al. Orbital rhabdomyosarcomas and related tumors in childhood: relationship of morphology to prognosis--an Intergroup Rhabdomyosarcoma study. Med Pediatr Oncol. 1997;29(1):51-60.

5. Maccheron LJ, McNab AA, Elder J, et al. Ocular adnexal Langerhans cell histiocytosis clinical features and management. Orbit. 2006;25(3):169–177.