Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumor with sarcomatous morphology and distant metastases: An unusual histomorphology and behavior.
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Date
2015-10
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Abstract
Inflammatory myofibroblastic tumor (IMT), an intermediate-grade neoplasm
of myofibroblastic/fibroblastic differentiation, occurs commonly in children and
young adults. It is characterized by anaplastic lymphoma kinase (ALK) gene
rearrangement and overexpression of ALK-protein. However, aggressive
behavior is more commonly associated with ALK-negativity rather than ALKpositivity.
Pulmonary involvement is most common visceral location and carries
minimal potential for distant metastasis. We present a case of 49-year-old female
with pulmonary IMT of spindle cell sarcomatous histomorphology. Frequent
mitoses and necrosis with characteristic cytoplasmic immunoreactivity for ALK-1
protein and ALK-gene rearrangement on fluorescence in-situ hybridization were
noted. This case is unusual for occurrence in higher age-group of fifth decade,
sarcomatous histomorphology at presentation (rather than transformation)
and metastases to distant sites despite ALK-protein overexpression and gene
rearrangement.
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Keywords
Anaplastic lymphoma kinase gene, anaplastic lymphoma kinase-protein, crizotinib, fluorescence in-situ hybridization, inflammatory myofibroblastic sarcoma
Citation
Sethi Bhawna, Pai Trupti, Allam Abhishek, Epari Sridhar. Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumor with sarcomatous morphology and distant metastases: An unusual histomorphology and behavior. Indian Journal of Pathology & Microbiology. 2015 Oct-Dec 58(4): 509-512.