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Indian Journal of Pathology and Microbiology
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CASE REPORT
Year : 2015  |  Volume : 58  |  Issue : 4  |  Page : 509-512

Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumor with sarcomatous morphology and distant metastases: An unusual histomorphology and behavior


1 Department of Pathology, Tata Memorial Centre, Mumbai, Maharashtra, India
2 Sri Venkateswara Medical Colleges, Tirupati, Andhra Pradesh, India

Correspondence Address:
Dr. Sridhar Epari
Department of Pathology, Tata Memorial Centre, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.168866

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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 ALK-positivity. 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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