Results
Five children were included with the youngest being two years six months
and the oldest being 15 years of age, with a prolonged history of fever
in all and weight loss in one child. Two children presented with a
mediastinal mass and features of superior mediastinal syndrome, and one
child each presented with an adrenal mass, midline mass over the bladder
and a sigmoid polyp. All five children had localized disease on imaging.
Upfront surgery was performed in the three children with abdomen
disease, where the polyp was completely excised with negative margins,
and 90% of the tumor was resected in the other two. In both the
children with mediastinal mass, image-guided trucut biopsy was
performed. Morphology was consistent with IMFT for all five children
with predominantly myofibroblastic spindle cells in a background of
plasma cells, lymphocytes, and eosinophils. Four of the five children
were positive for anaplastic lymphoma kinase (ALK) on IHC.
All children received a combination of chemotherapy, which was well
tolerated. One patient (child#2) had methotrexate-induced toxicity in
the form of raised serum bilirubin around three months after initiation
of therapy. As he was in remission, we stopped methotrexate and
continued 6MP. Child#5 was kept on the above chemotherapy for three
years, given local recurrence on a taper. He is now two years off
chemotherapy and is in remission. Child#2 and 3 were on the medications
at the time of this publication.
In both the children with residual abdominal disease post-surgery,
localized disease recurrence was noted on imaging, following the initial
response. They were both started on Crizotinib, which resulted in
complete resolution. Crizotinib continued for one year for child # 1
and child # 4 was on the drug for ten months at the time of this
publication.
Overall survival in our cohort is 100%, with all children in remission.
The longest follow up is five years and the shortest being six months.
The details of the five children with their presentations and treatment
are elaborated in Table 1. Response evaluations for the children are
depicted in Figures 1 and 2. Histopathology and IHC from lung biopsy
sample for child#3 are represented in Figure 3.