CASE HISTORY

This 2-year-old boy from Venezuela presented with a large right neck soft tissue mass.

 A biopsy was performed. The tumor was initially thought to represent classic Hodgkin lymphoma. Given the unusual age for this diagnosis, it was subsequently submitted via courier for a second opinion.

Medium power view shows a soft tissue mass composed of spindle (elongated) cells arranged in intersecting fascicles, in a focally sclerotic appearing stroma.

Higher power view shows moderately atypical cells containing irregular nuclei with open vesicular chromatin and occasional prominent nucleoli.

In areas, a mixed inflammatory background containing abundant eosinophils, plasma cells, lymphocytes and neutrophils was noticed.

In view of these histologic findings, an inflammatory myofibroblastic tumor was strongly suspected. The diagnosis was further confirmed by the expression of smooth muscle actin (SMA) and ALK. 

Following his revised diagnosis, the patient underwent surgery alone to remove his tumor, obviating a need for chemo and/or radiotherapy at this time. In addition, given its underlying molecular rearrangement involving the ALK1 gene, these tumors can be potentially treated with targeted therapies (i.e. tyrosine kinase inhibitors), if they don't respond to conventional treatments following their recurrence.