An Unusual Case of Systemic Inflammatory Myofibroblastic Tumor with Successful Treatment with ALK-Inhibitor

Abstract EN

Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity.

A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness.

PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas.

MRI of the abdomen demonstrated two 6-7 cm masses in the liver.

MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease.

A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation.

Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1).

Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver.

FISH analysis of these lesions demonstrated an ALK (2p23) gene rearrangement.

The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission.

We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation.

This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy.