PMID- 30955263
OWN - NLM
STAT- MEDLINE
DCOM- 20190411
LR  - 20211204
IS  - 0529-5807 (Print)
IS  - 0529-5807 (Linking)
VI  - 48
IP  - 4
DP  - 2019 Apr 8
TI  - [Dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like 
      features: a clinicopathological analysis of five cases].
PG  - 282-287
LID - 10.3760/cma.j.issn.0529-5807.2019.04.004 [doi]
AB  - Objective: To investigate the clinicopathological features, diagnosis and 
      differential diagnosis of dedifferentiated liposarcoma (DDLPS) with inflammatory 
      myofibroblastic tumor (IMT)-like features. Methods: Five cases of DDLPS with 
      IMT-like features were collected from the First Affiliated Hospital of Nanjing 
      Medical University, the Affiliated Hospital of Nanjing University of Traditional 
      Chinese Medicine and the First People's Hospital of Qinzhou between 2013 and 
      2018. EnVision method and fluorescence in situ hybridization (FISH) were used to 
      detect the immunophenotype of the tumor cells and the profile of MDM2 gene 
      amplification respectively. Results: All five cases were male and the median age 
      was 61 (range 53 to 65) years. The clinical symptoms were mainly related to the 
      space-occupying lesions. The tumors were located in duodenal mesentery (two 
      cases), intestinal wall (one case), retroperitoneum (one case), and spermatic 
      cord (one case). Grossly, the tumors were not well encapsulated, ranging from 3 
      to 13 cm (median 6.7 cm) in diameter, with tan to gray and firm cut surface. 
      Histologically, the dedifferentiated component closely resembled inflammatory 
      myofibroblastic tumor (IMT), with spindle/polygonal/stellate-shaped cells 
      arranged in storiform, sheet-like, or random pattern, with varying degrees of 
      chronic inflammation and fibrosis. All three major patterns seen in IMT (myxoid, 
      cellular and hypocellular fibrous) were observed, the hypocellular fibrous 
      pattern was the most common. Well-differentiated liposarcomatous component was 
      found in the peripheral areas of all the tumors. One case had high grade 
      dedifferentiated component. Four cases were strongly positive for MDM2 and p16. 
      Two cases were positive for SMA, and one case was focally positive for desmin and 
      one for CD34. None of the cases stained for ALK-1. FISH demonstrated MDM2 gene 
      amplification in all five cases. Clinical follow-ups were available in all five 
      cases and the interval ranged from 3 to 66 months (median 23 months). Two 
      patients developed recurrences and one patient had metastasis. The remaining two 
      patients were alive with no evidence of tumor recurrence at 3 and 14 months after 
      surgery respectively. Conclusions: DDLPS with IMT-like features is a more 
      aggressive neoplasm than its histological mimic (IMT), and should not be 
      misdiagnosed as other intermediate or low-grade malignant tumors, such as IMT, 
      sclerosing liposarcoma, inflammatory liposarcoma, aggressive fibromatosis, 
      solitary fibrous tumors, low-grade myofibroblastic sarcoma, and low-grade 
      fibrosarcoma.
FAU - Si, H P
AU  - Si HP
AD  - Department of Pathology, the Affiliated Hospital of Nanjing University of 
      Traditional Chinese Medicine, Nanjing 210029, China.
FAU - Wang, Z
AU  - Wang Z
AD  - Department of Pathology, First Affiliated Hospital of Nanjing Medical University, 
      Nanjing 210029, China.
FAU - Fan, Q H
AU  - Fan QH
AD  - Department of Pathology, First Affiliated Hospital of Nanjing Medical University, 
      Nanjing 210029, China.
FAU - Zhang, Y F
AU  - Zhang YF
AD  - Department of Pathology, the Affiliated Hospital of Nanjing University of 
      Traditional Chinese Medicine, Nanjing 210029, China.
FAU - Yang, D Q
AU  - Yang DQ
AD  - Department of Pathology, the First People's Hospital of Qinzhou, Guangxi 
      Autonomous Region, Qinzhou 535000, China.
FAU - Zhang, Z H
AU  - Zhang ZH
AD  - Department of Pathology, First Affiliated Hospital of Nanjing Medical University, 
      Nanjing 210029, China.
FAU - Gong, Q X
AU  - Gong QX
AD  - Department of Pathology, First Affiliated Hospital of Nanjing Medical University, 
      Nanjing 210029, China.
LA  - chi
PT  - Journal Article
PL  - China
TA  - Zhonghua Bing Li Xue Za Zhi
JT  - Zhonghua bing li xue za zhi = Chinese journal of pathology
JID - 0005331
RN  - 0 (CDKN2A protein, human)
RN  - 0 (Cyclin-Dependent Kinase Inhibitor p16)
RN  - 0 (MAS1 protein, human)
RN  - 0 (Proto-Oncogene Mas)
RN  - EC 2.3.2.27 (MDM2 protein, human)
RN  - EC 2.3.2.27 (Proto-Oncogene Proteins c-mdm2)
SB  - IM
MH  - Aged
MH  - Cyclin-Dependent Kinase Inhibitor p16/analysis/genetics
MH  - Diagnosis, Differential
MH  - Duodenal Neoplasms/genetics/*pathology
MH  - Fibrosarcoma/genetics/*pathology
MH  - Gene Amplification
MH  - Genital Neoplasms, Male/genetics/*pathology
MH  - Humans
MH  - Immunophenotyping
MH  - In Situ Hybridization, Fluorescence
MH  - Intestinal Neoplasms/genetics/*pathology
MH  - Liposarcoma/genetics/*pathology
MH  - Male
MH  - Middle Aged
MH  - Neoplasm Recurrence, Local
MH  - Proto-Oncogene Mas
MH  - Proto-Oncogene Proteins c-mdm2/genetics
MH  - Retroperitoneal Neoplasms/genetics/*pathology
MH  - Tumor Burden
OTO - NOTNLM
OT  - Cell dedifferentiation
OT  - Inflammatory myofibroblastic tumor
OT  - Liposarcoma
OT  - Proto-oncogene proteins c-mdm2
OT  - Soft tissue neoplasms
EDAT- 2019/04/09 06:00
MHDA- 2019/04/12 06:00
CRDT- 2019/04/08 06:00
PHST- 2019/04/08 06:00 [entrez]
PHST- 2019/04/09 06:00 [pubmed]
PHST- 2019/04/12 06:00 [medline]
AID - 10.3760/cma.j.issn.0529-5807.2019.04.004 [doi]
PST - ppublish
SO  - Zhonghua Bing Li Xue Za Zhi. 2019 Apr 8;48(4):282-287. doi: 
      10.3760/cma.j.issn.0529-5807.2019.04.004.