Inflammatory myofibroblastic tumor with thrombocytosis and a unique chromosomal translocation With ALK rearrangement.
We describe a case of inflammatory myofibroblastic tumor with an unusual constellation of clinical, pathologic, and genetic findings. A 7-year-old girl had an 11-cm abdominopelvic mass accompanied by thrombocytosis, anemia, elevated erythrocyte sedimentation rate, and elevated C-reactive protein. The inflammatory myofibroblastic tumor displayed unusual histologic features of zonal coagulative necrosis, high cellularity with a herringbone pattern, and tumor-associated osteoclast-like giant cells. The complex tumor karyotype included a translocation t(1;2)(q21; p23). Following resection, the laboratory abnormalities resolved. The patient is well and free of recurrence at 3 years following resection. This case raises interesting questions about clinical, pathologic, prognostic, and molecular genetic interrelationships in inflammatory myofibroblastic tumor.
Cole
Bonnie
B
University of Utah, School of Medicine, Salt Lake City, USA.
Zhou
Holly
H
McAllister
Nancy
N
Afify
Zeinab
Z
Coffin
Cheryl M
CM
D000008:Abdominal Neoplasms / Q000150:complications / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics*; D002889:Chromosomes, Human, Pair 2 / Q000235:genetics*; D004273:DNA, Neoplasm / Q000032:analysis; D018572:Disease-Free Survival; D005260:Female; D015321:Gene Rearrangement; D006104:Granuloma, Plasma Cell / Q000150:complications / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D011505:Protein-Tyrosine Kinases / Q000235:genetics* / Q000378:metabolism; D020794:Receptor Protein-Tyrosine Kinases; D013922:Thrombocytosis / Q000150:complications / Q000235:genetics / Q000473:pathology*; D014178:Translocation, Genetic / Q000235:genetics*
D004273:DNA, Neoplasm; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases