Danilo's Page

Surgery will be June 16th at CHLA.

Here are some upcoming appointments:

5/26 - bone scan, meeting with oncology Dr. Anderson
6/2 - MIBG injection
6/3 - MIBG
6/7 - appointment with Dr. Mitchell, neurology
6/8 - appointment with Dr. Shaul, surgeon

The tumor surgery (tumorectomy??) will be on either 6/11 or 6/16.

I tagged along with Danilo and Oge to their appointment on Tuesday at Children's hospital. We were supposed to meet with the surgeon, but he had been called into emergency surgery. So, we talked to his associate, a paediatric surgeon named Kasper Wang.

We thought that Danilo would get scheduled for surgery to remove the neuroblastoma. However, Dr. Wang said Danilo needs an MIBG scan first. The cat scan is not super clear. He thinks the MIBG scan, which is sensitive to certain types of neural tissue, may reveal other tumors if the thing has spread. Also, a clearer picture of where the neuroblastoma is will help them know how to approach it. Since the mass appears near the diaphragm, they will first attempt to get it out using a laparoscope and several half millimeter incisions (in the back I think). If they can't get it with the scope, they may need to go through the abdomen from the front or the chest cavity from the side with larger incisions. He says the T11 (vertabrae?)that the mass is adjacent to should be accessible through the abdomen. If they go through the chest cavity, he said they would have to leave a tube inside Danilo for a couple of days to re-expand the lung. He said some stuff about suction, but my notes aren't clear.Obviously there are more risks from bleeding the more they have to cut. In any event, Danilo will need to be in the hospital a couple of days.

He said the anaesthesiologist may recommend an epidural catheter (I guess this would be in addition to general anaesthesia).

When they do the surgery, they will take bone marrow and a sample of "bone matrix" from Danilo's hip. They need to check the bone marrow in case the tumor metastasized in there. However, Wang said the cat scan appears to show normal marrow.

He looked at Danilo's latest cat scan with us and showed us the small dark blob that is the neuroblastoma. He then looked at the scan done in 2003 and found the same dark mass that the last pathologist didn't notice.(Grrrrrrr) It's hard to tell, but he said the mass looked to be about the same size. Oge is going to try to get the earliest one done at Kaiser to compare.

I asked the doctor about the possibility of weird complications from the surgery and risks of infection since Danilo's immune system is suppressed with ACTH. He said there is not a significant risk of infection, since Danilo will get antiobiotics and IV medications. He said possibly they might talk to an endocrinologist about supplementing Danilo's steroid level, since the ACTH changes it, but wasn't sure how critical it was.

I was worried about somebody's hand slipping because the neuroblastoma is supposed to be close to the spine. Wang said it's possible nerves coming out from the spine will need to be cut in the area of the mass. However, he says those nerves are involved purely in sensation. So, if they need to cut them, the worst result would be an area of numbness along the rib.

I asked how often Danilo will need to be rechecked for neuroblastomas after the surgery. He said typically kids get an MIGB and cat scan every three months, but says we need to ask the oncologist, Dr. Anderson about this.

Oge asked about donating blood in advance of the surgery. He recommended donating one unit of blood a week before.We don't know Danilo's blood type though. He didn't have it. I don't even know my blood type. I guess we need to figure this out, but Oge's gotta get the MIBG scan scheduled first.

Here are some excerpts I stole from some online information sites. Danilo's tumor is very tiny, by the way ... :

Neuroblastoma is predominantly a tumor of early childhood, with two thirds of the cases presenting in children younger than 5 years. In rare cases, neuroblastoma can be discovered prenatally by fetal ultrasonography. Neuroblastoma originates in the adrenal medulla or the paraspinal sites where sympathetic nervous system tissue is present. The most common symptoms are due to a tumor mass or to bone pain from metastases. Proptosis and periorbital ecchymosis are common and arise from retrobulbar metastasis. Extensive bone marrow metastasis may result in pancytopenia. Abdominal distention with respiratory compromise due to massive liver metastases occurs in infants. Because they originate in paraspinal ganglia, neuroblastomas may invade through neural foramina and compress the spinal cord, causing paralysis. Fever, anemia, and hypertension are found occasionally. Multifocal neuroblastoma occurs rarely, usually in infants, and generally has a good prognosis. Rarely, children may have severe watery diarrhea due to the secretion of vasoactive intestinal peptide by the tumor.

Children with neuroblastoma rarely present with paraneoplastic neurologic findings including cerebellar ataxia or opsoclonus/myoclonus. The opsoclonus/myoclonus syndrome appears to be caused by an immunologic mechanism that is not yet fully defined. Unlike other neuroblastomas, the primary tumor usually is diffusely infiltrated with lymphocytes. Patients who present with this syndrome often have neuroblastomas with favorable biologic features and are likely to survive, although tumor-related deaths have been reported. Neurologic dysfunction is most often a presenting symptom but may arise after removal of the tumor. Opsoclonus/myoclonus is frequently associated with pervasive and permanent neurologic and cognitive deficits, including psychomotor retardation. Some patients may clinically respond to removal of the neuroblastoma, but improvement may be slow and partial; symptomatic treatment is often necessary. Adrenocorticotropic hormone (ACTH) treatment is thought to be effective, but some patients do not respond to ACTH. Various drugs, plasmapheresis, and intravenous gamma-globulin have been reported to be effective in selected cases. It has been suggested that the long-term neurologic outcome may be superior in patients treated with chemotherapy, possibly by means of its immunosuppressive effects.

***

Your child has a cancer called neuroblastoma. this develops from cells which make up a part of the nervous system called the sympathetic nervous system. The commonest place for this tumour to develop is just above one of the kidneys in the tummy, but it can occur anywhere along the sympathetic chain (this is a special group of nerves which travel down from the neck to the pelvis next to the spine). Neuroblastoma can present as a mass in the tummy or neck, or if it starts in the chest it might present as a problem with breathing. The outcome of treatment depends on how big the tumour is when it is first noticed and how much the tumour has spread into other parts of the body. If the tumour is small and has not spread, more than 90% of children will survive and will only need an operation to remove the tumour. If the tumour is larger, such that an operation is not possible to take it out, but it has not spread to other parts of the body, the chance of cure with correct treatment is about 60%. However, if the tumour has spread to other parts of the body, such as the bone marrow, the chance of cure is about 25%.

His eyes are bloody, but the strabismus appears to be gone. We saw the ophthalmologist this morning and she checked his vision and it's pretty darn good so he should heal up fine.

We had an appointment with the oncologist this morning. He said that Danilo's tumor is technically a neuroblastoma, but they are not as feared these days as they used to be. He is not expecting Danilo's tumor to be very aggressive and he's not expecting to have to fight it with chemotherapy. They are excited about removing it and doing all kinds of lab experiments on it though. The surgery is not scheduled yet, but we have an appointment to see the surgeon next Tuesday. They will also need to get some of his bone marrow and blood to study as well. The neuroblastomas are still very mysterious so they hope to eventually figure out what causes them to become aggressive as they sometimes do.

Eye surgery will be Thursday ...

Tuesday he had his CT scans at Children's Hospital. They found a tiny peanut sized mass. I talked to Dr. Mitchell today. She says the mass is located in the T11 area on the right, above the diaphragm. They will go in with a scope to remove it. I also talked to the pediatrician today. He contacted Dr. Kimiko at Cedars who looked at his scans last year. Kimiko claims that he looked at it again and didn't see it. Probably they didn't scan small enough. Dr. Mitchell asked me to try to get copies of the scans from Cedars and Kaiser on disk so she can look at them. She is interested to see if it was there before or not. They say, usually it would get smaller after 2 years rather than larger, but it's not unheard of that it got larger. The surgery will be soon. They are working on all the authorizations and scheduling. He'll be in there for a couple of days.

He has eye surgery next Thursday.