Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma‐like component presenting a leukemoid reaction

Abstract

A rare case of dedifferentiated liposarcoma (well-differentiated liposarcoma with an inflammatory malignant fibrous histiocytoma (MFH)-like anaplastic component) occurring in a 69-year-old male is presented. The patient had noticed a dull pain in his left loin and thigh for about 1 month. Computed tomography examination revealed a low-density mass lesion, measuring about 6 cm in diameter, in the left iliopsoas muscle, and it was surgically removed. Grossly, the lesion was composed of an encapsulated, soft, whitish mass and an adjacent, well-demarcated, yellowish hard nodule, measuring about 2.5 cm in diameter. Microscopically, both lesions showed features of an inflammatory variant of MFH and a sclerosing type of well-differentiated liposarcoma, respectively. To our knowledge, only two cases of dedifferentiated liposarcoma combined with inflammatory MFH as a dedifferentiated component have been recorded in the literature. The salient feature of the present case is a systemic inflammatory reaction, as shown by prominent leukocytosis (up to 73,900/mm3) and the elevated serum value of C reactive protein (up to 26.0 mg/dL), which were transiently reduced after surgery. The inflammatory reaction was suggested to be induced by cytokines, such as granulocyte colony-stimulating factor and interleukin-6, which were probably produced by the tumor cells in the present case, because the elevated serum values of those cytokines were decreased after surgery.

FluorescenceIn SituHybridization forMDM2Amplification as a Routine Ancillary Diagnostic Tool for Suspected Well-Differentiated and Dedifferentiated Liposarcomas: Experience at a Tertiary Center

“…The significance of this finding of MDM2 amplification in 21.9% is uncertain, since up to 40% of other soft tissue sarcomas can harbor amplified MDM2 [21, 22, 39, 40]. This and the fact that DDL can have a variety of appearances ranging from bland fibromatosis-like to UPS-like and exhibit several different types of heterologous differentiation [6, 7, 43–46] mean that unless there is an antecedent history of WDL or adjacent WDL component, a diagnosis of DDL cannot always be proven, even at sites where it is likely. Coindre et al have previously shown that most retroperitoneal UPS represent DDL [43], although of our 30/64 sarcomas that were retroperitoneal, only 9 showed MDM2 amplification, a lower figure than expected.…”

Clinical implications of serum C‐reactive protein levels in malignant fibrous histiocytoma

“…2 A molecular study has revealed that MFH and leiomyosarcomas share very similar comparative genomic hybridization imbalance profiles, suggesting that MFH is a morphologic modulation in the tumorigenesis of other sarcomas, particularly leiomyosarcoma. 16 In addition, Hisaoka et al 17 reported a dedifferentiated liposarcoma with an inflammatory MFHlike component presenting with a leukemoid reaction. Taken together, these results suggest that PNS in MFH might be a manifestation of advanced sarcoma tumorigenesis.…”

Malignant Fibrous Histiocytoma of Soft Tissue Producing Granulocyte Colony-Stimulating Factor.

“…G-CSF-producing tumors originating in non-epithelial tis- sues are rare. Wefound several cases of G-CSF-producing MFH in the Japanese literature (8)(9)(10)(11)(12)(13)(14) and in the literature of other countries (15)(16)(17)(18)(19) from the retroperitoneum with a white blood cell count of 44,500/|il. They diagnosed it as a G-CSF-producing tumor because the serum G-CSF was 400 pg/ml (normal: <100 pg/ml) and the white blood cell count was normalized after removal of the tumor.…”

“…They diagnosed it as a G-CSF-producing tumor because the serum G-CSF was 400 pg/ml (normal: <100 pg/ml) and the white blood cell count was normalized after removal of the tumor. Hisaoka et al (13) reported in 1997, a case of MFHof soft tissue with a white blood cell count of 73,900/jj,1 and a high level of serum G-CSF (109 pg/ml; normal: 2.2-30.9 pg/ml). They diagnosed it as a G-CSF-producing tumor because the white blood cell count decreased to 14,800/|J,l, and the serum G-CSF level decreased to 2.8 pg/ml after removal of the tumor, but increased again after the patient suffered a relapse of the tumor.…”