Abstract
Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.
Highlights
Inflammatory malignant fibrous histiocytoma (IMFH), known as undifferentiated pleomorphic sarcoma (UPS) inflammatory variant, was initially identified as a separate category by Kyriakos and Kempson in 1976 [1]
If the white blood cell count (WBC) was less than 50 K/mm3, but a severe left shift was present, early myeloid cells were seen in circulation, polyclonality was identified, and there were no signs of maturation arrest; it was considered as a leukemoid reaction
Inflammatory malignant fibrous histiocytoma\undifferentiated pleomorphic sarcoma inflammatory variant was first described over 30 years ago; due to the rarity of this entity, information about its presentation, clinical course, and outcome is very scarce
Summary
Inflammatory malignant fibrous histiocytoma (IMFH), known as undifferentiated pleomorphic sarcoma (UPS) inflammatory variant, was initially identified as a separate category by Kyriakos and Kempson in 1976 [1]. Its name is derived from its distinct histological pattern; an intense inflammatory infiltrate that may be composed of neutrophils, eosinophils, and/or lymphocytes without a recognized source of infection. These tumors are usually bulky, and their clinical course is characterized by multiple local recurrences, metastasis, and death. Patients typically present with fever and other constitutional symptoms mimicking an infectious process, and/or a rapidly growing painful mass. In cases of retroperitoneal IMFH, patients can present with additional signs/symptoms of a space-occupying lesion [2,3,4,5,6]
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