Cytopathologic features of epithelioid inflammatory myofibroblastic sarcoma with correlation of histopathology, immunohistochemistry, and molecular cytogenetic analysis
Journal
Cancer Cytopathology
Journal Volume
123
Journal Issue
8
Pages
495-504
Date Issued
2015
Author(s)
Abstract
BACKGROUND Epithelioid inflammatory myofibroblastic sarcoma (E-IMS) is a recently established rare variant of inflammatory myofibroblastic tumor. It is characterized by a distinctive constellation of clinical, pathological, and molecular features, including a nearly exclusive intraabdominal location, strong male predilection, aggressive clinical course, predominance of epithelioid tumor cells, and Ran-binding protein 2 (RANBP2)-anaplastic lymphoma kinase (ALK) fusion in the majority of cases. To the authors' knowledge, the cytologic features of E-IMS have not been described to date. METHODS Cases of E-IMS that had corresponding cytology were searched. Six cytology samples (1 fine-needle aspiration sample, 2 imprint samples, and 3 effusion fluids) containing tumor cells were identified in 5 patients with E-IMS. RESULTS The cytomorphology included large monotonous epithelioid cells arranged in loose aggregates or singly, with admixed myxoid stroma, and an inflammatory background rich in neutrophils. The tumor cells had a large, round, eccentric nucleus with vesicular chromatin, prominent nucleoli, and moderate amounts of pale cytoplasm. Delicate thin-walled branching vessels traversing tumor aggregates was a prominent feature in a fine-needle aspiration sample. Immunohistochemically, ALK was positive in all 5 tumors, with a nuclear membranous staining pattern noted in 3 cases and a cytoplasmic pattern observed in the other 2 cases. ALK rearrangement was confirmed in all 5 tumors by molecular genetic studies. CONCLUSIONS The cytologic features of E-IMS recapitulate its histologic characteristics. E-IMS merits inclusion in the differential diagnosis of any intraabdominal, large epithelioid cell neoplasm. Confirmation of ALK rearrangement is advisable because patients may benefit from targeted therapies. Cancer (Cancer Cytopathol) 2015;123:495-504. ? 2015 American Cancer Society.
SDGs
Other Subjects
anaplastic lymphoma kinase; anaplastic lymphoma kinase inhibitor; CD30 antigen; cell protein; crizotinib; desmin; hybrid protein; Ran binding protein 2; smooth muscle actin; unclassified drug; anaplastic lymphoma kinase; nuclear protein; protein tyrosine kinase; ran-binding protein 1; adolescent; adult; aged; ALK gene; anisonucleosis; Article; ascites fluid; cancer mortality; cancer radiotherapy; cancer recurrence; cancer surgery; cancer survival; cell nucleus; cell structure; chromatin; chromosome analysis; clinical article; cytopathology; cytoplasm; cytoreductive surgery; diagnostic error; disease course; eosinophil; eosinophil count; epithelioid cell; epithelioid inflammatory myofibroblastic sarcoma; female; fine needle aspiration biopsy; fluorescence in situ hybridization; gene; hepatic encephalopathy; histiocyte; histopathology; human; human cell; human tissue; immunohistochemistry; immunophenotyping; inflammatory cell; intermethod comparison; lymphocytic infiltration; male; mesothelium cell; molecular imprinting; molecularly targeted therapy; multinuclear cell; myxoid stroma; neutrophil; nucleolus; plasma cell; plasma cell granuloma; pleura effusion; priority journal; protein expression; reverse transcription polymerase chain reaction; spindle cell; stroma; tumor cell; tumor localization; tumor necrosis; tumor volume; chromosome analysis; cytodiagnosis; epidemiology; genetics; immunohistochemistry; liver tumor; lung tumor; metabolism; middle aged; myofibroblast; pathology; procedures; real time polymerase chain reaction; register; sarcoma; sensitivity and specificity; Adolescent; Adult; Biopsy, Fine-Needle; Cytodiagnosis; Cytogenetic Analysis; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Liver Neoplasms; Lung Neoplasms; Male; Middle Aged; Myofibroblasts; Nuclear Proteins; Real-Time Polymerase Chain Reaction; Receptor Protein-Tyrosine Kinases; Registries; Sampling Studies; Sarcoma; Sensitivity and Specificity
Type
journal article