Treating the primary only, after ruling out lymphnode mets. - considering the very low incidence of lymph node mets in this subsite.

http://onlinelibrary.wiley.com/doi/10.1002/1097-0142%2819870815%2960:4%3C910::AID-CNCR2820600433%3E3.0.CO;2-8/abstract

some literature on this site.

http://www.ncbi.nlm.nih.gov/pubmed/15817347?dopt=Abstract

http://www.ncbi.nlm.nih.gov/pubmed/17854490?dopt=Abstract

http://www.ncbi.nlm.nih.gov/pubmed/18455321?dopt=Abstract

http://www.ncbi.nlm.nih.gov/pubmed/18501529?dopt=Abstract

Typically head-neck RMS do not have as high a incidence of lymph nodal involvement as against a Bladder/ Prostate/ Paratesticular RMS. I think, if the CT is not concerning and if PET has been done and is negative, it probably is reasonable to just treat the primary (that too with conservative margins) in a 14 year old girl (or for that matter any age). From my understanding, this is what most ongoing protocols specify. This is probably related to the extreme toxicity of the treatment when combined with concurrent RMS type chemotherapy as against a standard head-neck with cisplatin based therapy. Also, there is a higher chance of distant relapse followed by persistent/ progressive/ recurrent disease at primary site rather than nodal relapse alone.

The AMORE protocol for advanced non-orbital RMS infact just uses brachytherapy after a massive surgical resection and reconstruction.
[http://www.ncbi.nlm.nih.gov/pubmed/19250759]

Int J Radiat Oncol Biol Phys. 2009 Aug 1;74(5):1555-62. Epub 2009 Feb 26.
The AMORE protocol for advanced-stage and recurrent nonorbital rhabdomyosarcoma in the head-and-neck region of children: a radiation oncology view. Blank LE, et al. Department of Radiation Oncology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

Just a quick point, I am sure your pathologists are great, but a RMS not responding at all to four cycles of chemo is not very common. You may consider getting a pathological review if feasible.

Will be interested to know what others feel.

post-op —>4 cycles of adjuvant chemotherapy (VAC/VAI)

plan to start radiotherapy at the earliest omitting AMD and Etoposide from her chemotherapy in consultation with the medical oncologist since she has a) large residual tumor b) orbital extension c) limited intracranial extension in ipsilateral temporal lobe —she is IRSG post-surgical Group 3 .

Planning CT scan showed the residual tumor with extension as mentioned above ( image attached)and we are trying to protect as much of the visual pathway and brain as achievable.

Pre-op CT/MRI showed no evidence of lymph node involvement -not sampled surgically.

WOULD YOU INCLUDE IPSILATERAL NODAL STATIONS IN THE CTV —IF SO , WHAT LEVELS? I could not find literature supporting prophylactic nodal irradiation in this scenario .

I am planning to deliver 50.4 Gy @ 1.8 in 28 # .

1. ONG restricted to orbit without significant progression on imaging willl not require treatment in most cases and will regress sometimes without treatment
2. ONG with chiasmal involvement, suprasellar involvement, diencephalic syndrome and rapidly progressive visual loss do require treatment
3. RT is the treatment of choice but reserved for those aged > 8 -10 years to avoid the risks of SMN/ Neurocognitive/ cerebrovascular/ skeletal abnormalities in long term f/u
4. CCT is recommended to restrict the growth of tumor till RT can be given safely
5. CCT utilises Carboplatin/vincristine/etoposide in most protocols.
6. You can expect CCT will stabilize the leison in 60% over a 3 year time span but about 21% will progress during CCT
7. RT however has the best PFS rates approaching 90% at 10 years.
8. If the child has NF1 then the prognosis is better - so testing for same can be useful for prognostication.

Overall I think a close follow up with radiological and visual field testing is mandated provided the parents understand that no treatment is not a free ticket to come back 10 years with a proptosed eye.

Case Capsule:
Age: 21/2 yrs
Optic glioma, dimination of vision on one side
Case disscussed by Neurosurgeon on phone/no surgical intervention
My opinion was: to wait at least 6mths (should we wait for completion of 3yrs)
Nsx:what we will do if it progresses on other side meanwhile.

Regards!

Dr.Shailesh S. Shende

MBBS, MD (Radiation Oncology)

Consultant Radiation Oncologist,

Vimal Lalchand Mutha Cancer Centre,

Deenanath Mangeshkar Hospital & Research Center,

Erandwane, Pune-411004

Phone no - +91-(20)-66023000, Extn 2910

Fax - +91-(20)-25420104

Mobile No - 09890609830
Email - oc.liamffider|ednehshseliahs#oc.liamffider|ednehshseliahs

http://www.scribd.com/doc/37727021/Textbook-of-Neuroblastoma-by-Cheung-Springer-Publications

Santam has answered the first question: CT scan is now regarded as one of the Std Investigations in developed worlds incorporated into all new Wilms protocols.

2) If there is a relapse in lungs after completing prior treatment for abdominal disease - what is the management?

Salvage rates after relapse are dismal, with the majority of children dying from recurrent disease.
If solitary lung nodule relapse —> Early surgical intervention followed by second line chemo (Adjuvant)
Depending on disease free interval there are options on the choice of salvage chemo (re-challenge with previous chemo, ICE, etc )

3) If after chemotherapy the relapsed lung metastasis disappear radiologically is it required to give WLI in relapsed cases?

One of the recent SIOP studies suggested that whole-lung irradiation may not be necessary for successful therapy of patients with stage IV, favorable histology Wilms' tumor if the metastases completely respond to chemotherapy.
Nearly 10% children will develop RT induced interstitial pneumonitis, and a quarter of which died from this. Further more , there is a 93% incidence of musculoskeletal and soft tissue growth abnormalities in patients treated with whole-lung irradiation after more than 10 years of follow-up.

However, in relapsed cases the benefit Vs Harm ratio is in favour of giving WLI since their prognosis is quite dismal and the competing cause of mortality is the disease itself

The basic idea - Give RT for pulmonary relapse

When I was in my MD and had prepared for a seminar (which is available at http://www.slideshare.net/santam/management-of-wilms-tumors) I had read that using CT doesnot add much over and above use of X rays to detect Pulmonary nodules in terms of "outcome" [Based on data by the study from Green et al - http://www.jco.ascopubs.org/content/9/10/1776.abstract?ijkey=93b527ec37cc6015629fe2cd7ec0f6eefe337bd9&keytype2=tf_ipsecsha].
That data was based on retrospective reviews. Thanks to your question I searched the net again. Turns out CT should now be indeed considered as the imaging modality of choice for detecting pulmonary metastasis. There are two issues when u consider the use of CT for detection of pulmonary mets:.

1. There is significant interobserver variability in quantification of radiological findings in presence of lung mets
2. Despite the high sensitivity of CT scans its often doubtful if the lung nodules are benign or malignant

The more recent study on patients on patients treated in the UKW2 trial [http://www.jco.ascopubs.org/content/20/12/2768.full#R6-109705] seems to suggest that CT scan does add to information in stage I patients (at the time of the protocol NWTS recommendation was to stage patients according to abdominal findings in presence of lung nodules on CT in absence of CXR). These patients were treated with vincristine only however. However they did find patients ending up with relapse had a poorer prognosis. Interestingly stage I with pulmonary nodules on CT had poorer EFS as compared to stage IV patients.