The Biopsy

This last Thursday Andrew went under anesthesia again so that the surgeon could remove a piece of his tumor for biopsy. The actual surgery took about 20 minutes. He made an incision in the outside corner of his eye, pulled the skin back a little, and debulked a decent bit of the tumor. He said that there was very little bleeding, which means that there was not a whole lot of vasculature around the tumor (most cancers are covered with vasculature). He also told us that the tumor was whitish-yellow, firm and well-defined. Just by physical observation, the surgeon said that the tumor did not look like a normal cancer and that histiocytosis may be a more likely option.

Normally the surgeon sends the sample down to pathology during the surgery and they send back a preliminary assessment within the hour and a full diagnosis within a few days. Unfortunately, pathology had shut down their equipment for the night. Our surgeon was told that they would not analyze the sample until at least Monday, if not later. So we are just waiting and reading about what each possibility involves.

We have appreciated your prayers and support in our behalf. We’ll try to post the diagnosis as soon as we know.

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.

Monday

As many of you know, Andrew has had a swollen eye since he was three months old. After a number of doctors and several diagnoses crossed off the list, we went in for an MRI and a CT scan this last Monday and were shocked with the news that there is a sizeable tumor covering a large area of his face.

We initially thought he was having an allergic reaction or a blocked tear duct. We were referred from a nurse practitioner to a pediatrician to a pediatric ophthalmologist to another ophthalmologist specializing in ultrasounds and finally to the radiologist who did the scans. For at least a month, we were sure it was a vascular lesion or hemangioma, which is a very treatable condition that usually goes away on its own or can be treated with a laser and/or steroidal injections. Our ophthalmologist told us that an ultrasound on his eye would pretty definitively show us a hemangioma if that was the problem. Andrew was the right age, he had the right presentation, and even the ultrasound seemed to initially point that way. We knew something was wrong when the day after the ultrasound, we got a call from our ophthalmologist's colleague who told me they would schedule the extra scans right away and gave me his pager number.

After the scans, the ophthalmologist told us it looked like one of three things: rhabdomyosarcoma, histiocytosis, or neuroblastoma. We asked him which disease was the most likely candidate. Rhabdomyosarcoma. He gave us his pager number again and told us we should talk to the radiologist. Stew had his computer handy, so he googled 'Rhabdomyosarcoma'. Fast-growing. Highly malignant. 50% 5-year mortality rate. Needless to say, we got pretty emotional after that.

The radiologist showed us the tumor, which extended from near the inside corner of Andrew's eye to the outside corner of his eyebrow and down through his zygomatic (cheek) bone. It reached back into his head past the length of his eye. He also showed us the CT scan showing the bone destruction from the tumor. There was no evidence of it in his brain, and Stew remembers someone saying that the local lymph nodes weren't enlarged. After explaining the extent of the tumor and his observations, he asked where our family was. We told him Ukraine and Indiana, but we had extended family close by. "Do you want to be part of our family?" We thanked him for his kindness. We told him that no matter what happens, Andrew is sealed to us.

Soon after, an Asian man walked in the room followed by a few residents. This was Dr. Kim, the man who was going to do a biopsy to figure out what exactly Andrew has. The radiologist did a quick run-down with this surgeon, who said it would be a simple procedure that he had done before. He took a look at Andrew and told us that the incision would be small, almost unnoticeable, minimal scarring. This was honestly the last thing on our minds. He soon left and we were left with Andrew.

Even a few days later, the details of the next few hours are fuzzy. We did a lot of praying, singing hymns, and Stew began his fast. We started to read in Alma 2 where the sons of Mosiah are teaching King Lamoni's father about God. We came upon one of my very favorite verses where his Father is praying, "O God, Aaron hath told me that there is a God; and if there is a God, and if thou art God, wilt thou make thyself known unto me, and I will give away all my sins to know thee." We realized that we had two choices--that we could either curse God, lose our testimonies and become embittered by this experience or we could turn to God and repent and find peace despite our adversity. We would certainly need to repent in this experience by turning our minds and wills toward God.

We know that we as his parents are able to do very little against cancer. We would need to put Andrew into the doctor’s hands, but really, we would need to put him in God’s hands. It was a comforting thought, imagining Andrew being taken care of by much more capable hands, as he is so close to God. We thought a lot about how much God loves Andrew, so much more than we do. After many hours of prayer and a priesthood blessing from the bishop, we both eventually felt and have clinged to the peace that comes only from the Atonement of Jesus Christ. We don’t know what will happen next, but we know that if we put our trust in God, things will work out.