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Tumor Discovery CRMO presenting as multifocal bone LCH

treatment paradigm. At present, there are no consensus the high uptake observed in LCH, and the corresponding
12
guidelines on the treatment of CRMO, and treatment is CT imaging showed sclerosis, which is also unusual in
mostly empirical. NSAIDs are usually used as first-line malignant disorders.
13
agents. The Childhood Arthritis and Rheumatology
Research Alliance (CARRA), a North American 4. Conclusion
organization comprised of pediatric rheumatologists and CRMO, or CNO, should always be considered in children
researchers, has put down various treatment regimens that presenting with features of multifocal bone LCH. It is
may be used for NSAID-refractory disease. The consensus a diagnosis of exclusion. Although whole-body MRI
treatment plans for the 1 year of therapy suggested are is the investigation of choice, biopsy should always be
st
methotrexate or sulfasalazine, TNF-α inhibitors with or recommended in patients in whom the findings are not
without methotrexate, and bisphosphonates. CARRA has typical.
14
also suggested the baseline evaluations that are mandatory
at diagnosis and some guidelines on the criteria that may Acknowledgments
be used for defining treatment failure after 3 months of The authors would like to acknowledge the children and
initiation of treatment. Experts have suggested the need their parents for giving consent for publishing images in
for a randomized controlled trial as currently, treatment is
given based on personal experience. 15 the manuscript.
Various NSAIDS have been used in the treatment Funding
of CRMO, such as celecoxib, diclofenac, ibuprofen, None.
indomethacin, naproxen, and piroxicam. Methotrexate
at a dosage of 15 mg/m , either orally or subcutaneously Conflict of interest
2
weekly once, or sulfasalazine at a dosage of 50 mg/kg/day
divided overdoses, is the most preferred second-line agent The authors declare no conflicts of interest.
in NSAID-refractory disease. TNF-α inhibitors such as Author contributions
adalimumab, etanercept, or infliximab have also been
advocated by experts treating children with CRMO, though Conceptualization: Yamini Krishnan
the cost is restrictive in low/middle-income country Methodology: Yamini Krishnan, Gazel Sainulabdin
settings. Another cost-effective option recommended is Writing – original draft: All authors
pamidronate intravenously at a dosage of 1 mg/kg every Writing – review & editing: Yamini Krishnan, Gazel
month or zoledronic acid at a dosage of 0.05 mg/kg every Sainulabdin
3 months. Since interleukins have been implicated in
15
the pathogenesis of this condition, theoretically, anti-IL1 Ethics approval and consent to participate
antibody treatments such as canakinumab or recombinant Ethics approval (Approval ID: IEC/2022/II/06) for this
IL1 receptor antagonists (e.g., anakinra) are likely to be retrospective study was obtained from the Institution
effective. RANK ligand inhibitors (e.g., denosumab), Review Board of MVR Cancer Centre and Research
which have an inhibitory effect on osteoclasts, may also Institute.
have some activity in CRMO. 3
Consent for publication
The major limitation of our analysis is that it is a
retrospective case series with a small number of subjects. Consent was obtained from the subjects or their guardians
All children received treatment at various centers with for the publication of images used in the article.
rheumatological expertise, and the follow-up evaluation
was conducted telephonically. None of the children Availability of data
underwent evaluation for genetic susceptibility. There Data used in this work are available from the corresponding
is very little awareness about this condition among the author on reasonable request.
medical community, and it should always be kept in the
differential diagnosis of a child presenting with multifocal References
bone disease, although the most common presentation of 1. Hedrich CM, Hahn G, Girschick HJ, Morbach H. A clinical
CRMO is solitary bone disease. All our patients underwent and pathomechanistic profile of chronic nonbacterial
a PET-CT at our center as multifocal LCH was our osteomyelitis/chronic recurrent multifocal osteomyelitis
diagnosis at the first evaluation of these children. The low and challenges facing the field. Expert Rev Clin Immunol.
FDG uptake in these children was significant as opposed to 2013;9(9):845-854.

Volume 3 Issue 3 (2024) 5 doi: 10.36922/td.3102