Open AccessAnaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumor with sarcomatous morphology and distant metastases: An unusual histomorphology and behavior
Author(s) -
Bhawna Sethi,
Trupti Pai,
Abhishek Allam,
Sridhar Epari
Publication year - 2015
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/0377-4929.168866
Subject(s) - anaplastic lymphoma kinase , pathology , fluorescence in situ hybridization , gene rearrangement , biology , immunohistochemistry , lymphoma , sarcoma , medicine , cancer research , lung cancer , gene , biochemistry , malignant pleural effusion , chromosome
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.