Patients and methods
We performed a retrospective analysis of case records of children less
than 18 years of age, diagnosed to have IMFT in the department of
pediatric hemato-oncology, from January 2014 till May 2020. The
diagnosis was confirmed through morphology and immunohistochemistry
(IHC) of an image-guided trucut biopsy of the lesion or review of the
excised tissue in those who underwent surgery. The extent of disease
required imaging with either computed tomography (CT) or Positron
emission tomography-computed tomography (PET-CT) as indicated. All cases
were discussed in a multidisciplinary tumor board before initiating
treatment. Written informed consent was obtained from all families, and
the institutional review board has approved the study.
The oral combination chemotherapy regimen included prednisolone at 1
milligram/kilogram/day (mg/kg) in two to three divided doses,
6-mercaptopurine (6-MP) started at 25 mg once a day in all children and
dosage titrated based upon serial absolute neutrophil counts (ANC) such
as to maintain an ANC of 750 – 1500/cu.mm, methotrexate at 20 mg/square
meter once a week and celecoxib at 50 mg once a day in those between
10-25 kgs, and 100 mg once a day in those weighing >25kg.
All children were monitored for adverse effects with regular assessment
for complete blood counts, liver enzymes, and serum creatinine. Response
assessment was performed for all children at four weeks of initiating
the above chemotherapy. When required, crizotinib was administered at a
dose of 280 mg/square meter/day (5).