PMID- 27460384
OWN - NLM
STAT- MEDLINE
DCOM- 20170210
LR  - 20220419
IS  - 1746-1596 (Electronic)
IS  - 1746-1596 (Linking)
VI  - 11
IP  - 1
DP  - 2016 Jul 27
TI  - Epithelioid inflammatory myofibroblastic sarcoma: a clinicopathological, 
      immunohistochemical and molecular cytogenetic analysis of five additional cases 
      and review of the literature.
PG  - 67
LID - 10.1186/s13000-016-0517-z [doi]
LID - 67
AB  - BACKGROUND: To explore the clinical characteristics and pathological features of 
      epithelioid inflammatory myofibroblastic sarcoma (EIMS) with emphasis on the 
      diagnostic spectrum. METHODS: The clinical data and histological features in 5 
      additional cases of EIMS were retrospectively reviewed. Immunohistochemical study 
      and interphase fluorescence in situ hybridization (FISH) analysis were carried 
      out. RESULTS: There were 2 males and 3 females with age at presentation ranging 
      from 15 to 58 years (mean, 37 years). All 5 tumors were intra-abdominal with 2 
      arising in the mesentery and 1 each in the omentum, rectum and transverse colon. 
      The tumor size ranged from 5 to 20 cm in maximum diameter (mean, 10.7 cm). 
      Histologically, all 5 tumors were composed predominantly of large epithelioid 
      cells possessing vesicular nuclei, prominent nucleoli, and amphophilic cytoplasm. 
      Mitotic figures were easily identified (mean, 20/10HPF). Tumor cells were 
      arranged in clusters or sheets embedded in a myxoid stroma containing prominent 
      neutrophils. A minor component of spindle cells was present in focal areas. By 
      immunohistochemistry, all 5 cases were positive for anaplastic lymphoma kinase 
      (ALK) with a nuclear membrane pattern in 4 and cytoplasmic staining with 
      perinuclear accentuation in 1. Besides ALK, tumor cells stained variably for 
      desmin (4/5), alpha smooth muscle actin (2/5), muscle-specific actin (1/2) and 
      pan-cytokeratin (1/4). FISH analysis demonstrated the presence of ALK 
      rearrangement in all 5 cases. Of 5 patients, 3 developed local recurrence, 1 died 
      of disease 8 months after surgery. CONCLUSION: EIMS represents a highly 
      aggressive variant of inflammatory myofibroblastic tumor characterized by 
      epithelioid morphology, prominent neutrophilic infiltrate, and nuclear membrane 
      staining of ALK with ALK rearrangement. As patients with ALK-rearrangement tumors 
      may benefit from targeted therapy, accurate diagnosis of EIMS is very important. 
      Familiar with the characteristic features of EIMS will help pathologists avoid 
      misdiagnosing the tumor as other malignancies.
FAU - Yu, Lin
AU  - Yu L
AD  - Department of Pathology, Fudan University Shanghai Cancer Center, Fudan 
      University, 270 Dong An Road, Shanghai, 200032, China.
AD  - Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 
      200032, China.
FAU - Liu, Jinguo
AU  - Liu J
AD  - Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University and 
      Shanghai Respiratory Research Institute, Shanghai, 200032, China.
FAU - Lao, I Weng
AU  - Lao IW
AD  - Department of Pathology, Fudan University Shanghai Cancer Center, Fudan 
      University, 270 Dong An Road, Shanghai, 200032, China.
AD  - Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 
      200032, China.
FAU - Luo, Zhiguo
AU  - Luo Z
AD  - Department of Medical Oncology, Fudan University Shanghai Cancer Center, Fudan 
      University, Shanghai, 200032, China.
AD  - Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 
      200032, China.
FAU - Wang, Jian
AU  - Wang J
AD  - Department of Pathology, Fudan University Shanghai Cancer Center, Fudan 
      University, 270 Dong An Road, Shanghai, 200032, China. softtissuetumor@163.com.
AD  - Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 
      200032, China. softtissuetumor@163.com.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20160727
PL  - England
TA  - Diagn Pathol
JT  - Diagnostic pathology
JID - 101251558
RN  - 0 (Biomarkers, Tumor)
RN  - EC 2.7.10.1 (ALK protein, human)
RN  - EC 2.7.10.1 (Anaplastic Lymphoma Kinase)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Anaplastic Lymphoma Kinase
MH  - Biomarkers, Tumor/*metabolism
MH  - Cytogenetic Analysis
MH  - Epithelioid Cells/metabolism/pathology
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Inflammation
MH  - Male
MH  - Middle Aged
MH  - Myofibroblasts/metabolism/pathology
MH  - Neoplasms, Muscle Tissue/*diagnosis/metabolism
MH  - Receptor Protein-Tyrosine Kinases/metabolism
MH  - Retrospective Studies
MH  - Sarcoma/*diagnosis/metabolism
MH  - Young Adult
PMC - PMC4962498
OTO - NOTNLM
OT  - Fluorescence in situ hybridization
OT  - Gastrointestinal tract
OT  - Inflammatory myofibroblastic tumor
OT  - RANBP2-ALK
OT  - Soft tissue tumor
EDAT- 2016/07/28 06:00
MHDA- 2017/02/12 06:00
PMCR- 2016/07/27
CRDT- 2016/07/28 06:00
PHST- 2016/04/27 00:00 [received]
PHST- 2016/07/19 00:00 [accepted]
PHST- 2016/07/28 06:00 [entrez]
PHST- 2016/07/28 06:00 [pubmed]
PHST- 2017/02/12 06:00 [medline]
PHST- 2016/07/27 00:00 [pmc-release]
AID - 10.1186/s13000-016-0517-z [pii]
AID - 517 [pii]
AID - 10.1186/s13000-016-0517-z [doi]
PST - epublish
SO  - Diagn Pathol. 2016 Jul 27;11(1):67. doi: 10.1186/s13000-016-0517-z.