High risk NB relapsed in the bones, seeking help and suggestions

Xinyi is a five year old girl from China diagnosed with Stage 4 high risk neuroblastoma in June 2015 in China.
She started 3 cycles of chemo.First & second cycles: Vincristine, Cyclophosphamide,pirarubicin respectively. Third cycle: Etoposide, Cisplatin,
Then we came to Singapore for surgical excision of the mass. Histopathology confirmed undifferentiated neuroblastoma, genetics negative for Nmyc amplification, 1p deletion, 11 q deletion. Bone marrow aspiration was negative for disease.
She received two more cycles of chemotherapy. Vincristine, Cyclophosphamide,pirarubicin and Etoposide, Cisplatin.
This was followed by consolidation high dose therapy with busulfan and melphalan, followed by autologous stem cell infusion on 16 November 2015.
Three months later, 10 times radiation was done on the tumor part.
Approximately 140 days from transplant she started immunotherapy with Dinutiximab beta (European medicine, 10 days infusion, without IL2 and GCSF). After the fourth cycle, MIBG scan showed multiple areas of uptake of the bones.

She relapsed.
With this, Xinyi started chemotherapy with Topotecan and Cyclophosphamide on September 7 2016 with three cycles. The fourth and fifth cycle of chemotherapy with irinotecan and temozomide. Response has been decrease in bone pain and in the urinary catecholamine levels.
Bone marrow result: No tumor was found in the bone marrow. Right and Left Sections show bone marrows with approximately 50% cellularities.
After relapse, it usually takes 6 or 7 weeks for her blood count recover after each cycle of chemo. Platelets is usually around 50 (reference interval 200-490) at most.
17 March 2017, MIBG scan showed the uptake parts almost the same with last time which did on 26 August 2016.

I am seeking suggestions and advice on treatment methods for my little girl. Even though we know the possibility for fully cure is very slim, we still hope miracle happen on her and she may stay with us longer. Million thanks for your help.

Hi Zheng Yi,

I am really sorry to hear about your daughter and wish you all the best…
May be you already explored another topic in this forum about a bivalent vaccine:

The very first results look promising, but this option is a quite costly and a kid should reach a remission first.

There is also another option - DFMO, but I don’t know any numbers for relapse cases… I only saw that update:

http://lucacan.com/a-normal-life/

For relapsed neuroblastoma, 50% of the participants remain in remission after two years. A number that gives us a great deal of hope.

But this is just unofficial numbers…

So sorry to hear your story, I wish you lots of luck

Have you investigating MIBG therapy?

Have you done any genetic testing for ALK, PHOX2B and so on? If she is ALK positive there are some very promising treatments around the corner.

There is a COG clinical trial that attempt to combine immunotherapy drugs with chemotherapy that has had some positive press that may be worth exploring. ANBL1221: A Phase II Randomized Trial of Irinotecan/Temozolomide with Temsirolimus or Chimeric 14.18 Antibody (ch14.18) in Children with Refractory, Relapsed or Progressive Neuroblastoma

It attempt to combine irinotecan, temozolomide and ch14.18.

There are also some other trials available in the US with humanized antibodies at St.Judes and MSKCC but travel and treatment would be enormously expensive.

Wishing you lots and lots of luck!

Hi, Zheng Yi,

I second Sam’s suggestions.

I would definitely look into COG’s ANBL1221. Check out Irinotecan Hydrochloride and Temozolomide With Temsirolimus or Dinutuximab in Treating Younger Patients With Refractory or Relapsed Neuroblastoma - Full Text View - ClinicalTrials.gov, They have seen successes in stubborn cases. You can get this at almost all hospitals in US, and a few in Australia, Canada, etc.

MIBG therapy has also shown effectiveness. Check out http://www.nant.org/Patients_and_Families/instmap.php to get a list of the hospitals which offer MIBG treatment.

Talk to MSKCC and CHOP.

Best wishes!
Chunwei Wang

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Thank you so much for giving me advice. the ch14.18 + chemo clinical trail, I reviewed the detailed requirement.
Subjects who have previously received anti-GD2 monoclonal antibodies for biologic therapy or for tumor imaging are eligible unless they have had progressive disease while receiving prior anti-GD2 therapy;

I think my daughter is uneligible, she got relapsed while receiving the 4th cycle of GD2.

As to MIBG treatment, her bone Marrow is not good enough, we are not sure, the stem cell can be good enough to rescue after the treatment.

The big problem is her blood count is low.

I am very grateful for your immediate reply and so much information. Thank you so much. I will ask doctor the do ALK test first. By the way, if is positive, what medicine can be used?

As to the vaccine and humanized antibodiese, we cannot afford the expensive treatment.

Hi Zheng Yi,

ALK+: There are several drugs that are in trials. Most U.S. NB doctors can tell you about them. Dr. Mosse at CHOP leads the most number of ALK trials.
Blood count: Which one(s)? Only platelets? It doesn’t hurt to consult with one or more U.S. doctors.

Best of luck!
Chunwei

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