Ask about this productRelated genes to: PLEKHH2 Blocking Peptide
- Gene:
- PLEKHH2 NIH gene
- Name:
- pleckstrin homology, MyTH4 and FERM domain containing H2
- Previous symbol:
- -
- Synonyms:
- KIAA2028, PLEKHH1L
- Chromosome:
- 2p21
- Locus Type:
- gene with protein product
- Date approved:
- 2004-07-30
- Date modifiied:
- 2016-10-05
Related products to: PLEKHH2 Blocking Peptide
Related articles to: PLEKHH2 Blocking Peptide
- Mesenchymal neoplasms characterized by ALK fusions mainly include inflammatory myofibroblastic tumors (IMTs) and epithelioid fibrous histiocytomas (EFHs). More recently, ALK-rearranged mesenchymal tumors that are not IMTs or EFHs, characterized by S100 and CD34 coexpression, have been reported in a few small series and isolated case reports. The neoplasms present a broad clinicopathological spectrum and variable biological behavior. - Source: PubMed
Gao ShengWu JunhuaFan JunChang XiaonaHuang BoLuo DanjuHe JunShi HeshuiDong XiaochuanNie Xiu - Heart failure (HF) represents the end stage of cardiovascular disease and is the leading cause of mortality. The objective of this study was to identify potential biomarkers and elucidate the mechanisms underlying the development of HF across diverse populations and among different genders. - Source: PubMed
Publication date: 2025/10/14
Yu YueXue ChentianJi DongSheng WeiGao XiangWu XizeWu Chengyan - rearrangements are rarely documented in superficial soft tissue neoplasms exhibiting an infantile fibrosarcoma-like spindle cell tumor (IFS) pattern or stromal, resembling Neurotrophic Tyrosine Kinase Receptor()rearranged spindle cell tumors. Here, we present two cases of pediatric cutaneous soft tissue tumors with an IFS pattern, in which fusions involving related partner genes were identified. The tumors in both cases demonstrated similar morphology and consisted of ovoid and spindle cells with infiltrative boundaries. The spindle cells exhibited either a fascicular growth pattern or a haphazard pattern and stromal hyalinization. Both cases involved inflammatory cell infiltration, brisk mitosis, and CD34, S100, and ALK-D5F3 immunoreactivity. Next-generation sequencing identified fusion with different partner genes ( and ). The fluorescence hybridization break-apart assay confirmed ALK rearrangements in both cases. In case 1, no indications of disease progression or metastasis was observed within the limited follow-up (36 months). However, the patient in case 2 experienced a rapid recurrence and metastasis. - Source: PubMed
Publication date: 2025/05/29
Ouyang QiGuo XiaohongMao RongjunCao Zhixing - Anaplastic lymphoma kinase ( ALK ) rearrangements drive most examples of epithelioid fibrous histiocytoma (EFH) and have been reported in an emerging family of receptor tyrosine kinase (RTK) fusion-positive mesenchymal neoplasms, including superficial ones described under the rubric of "superficial ALK -rearranged myxoid spindle cell neoplasm" (SAMS). Here, we describe 35 superficial tumors with SAMS morphology, which occurred in 18 females (51%) and 17 males at a median age at presentation of 39 years (range: 6 to 82 y). Most tumors occurred on the lower extremity (25 tumors; 71%), followed by upper extremity (5; 14%), trunk (3; 9%), and face (2; 6%). Nine tumors were reported to have grown slowly before presentation, including >10 years in 2 cases. Tumors occurred primarily in the dermis (32 tumors; 91%) or subcutis (3; 9%); 8 dermal tumors extended into the subcutis. Median tumor size was 1.3 cm (range: 0.5 to 8.0 cm). Clinical follow-up was available for 12 patients (34%; range: 2 mo to 21 y; median: 2.7 y), none of whom experienced metastasis. One incompletely resected tumor recurred locally at 19 months, and no other patients experienced recurrence. Histologically, tumors were characterized by bland spindle-to-ovoid cells showing whorled growth and myxoid-to-collagenous stroma. Recurrent features included an epidermal collarette (19/30; 63%), perivascular hyalinization (20/35; 57%), amianthoid collagen (14/35; 40%), and metaplastic ossification (2/35; 6%). Immunohistochemistry (IHC) demonstrated expression of ALK (24/31; 77%), CD34 (15/21; 71%), EMA (17/28; 61%), and S-100 (9/32; 28%). Eleven tumors showed hybrid morphologic features between EFH and SAMS; 9 of them (82%) showed cytomorphology typical of EFH but with whorled growth, myxoid stroma, and/or regions of spindle cell morphology. Two hybrid tumors showed sharp transitions between a region characteristic of EFH and a region characteristic of SAMS, with a concomitant sharp transition in EMA, CD34, and S-100 expression by IHC. Sequencing revealed ALK fusions in 15 of 19 tumors: 2 each with fusion partners FLNA , SQSTM1 , and VCL , and 1 each with COL1A2, DCTN1, EML4, FXR1, MPRIP , PLEKHH2, PRKAR1A, SPECC1L , and TLN2 . Thirteen of 14 ALK -rearranged tumors expressed ALK by IHC. Three tumors negative for ALK fusions instead harbored alternate RTK fusions ( NCOA4::RET , TRIM27::RET , and VIM :: NTRK3 ), and 1 tumor was negative for RTK alterations. CDKN2A / B deletions were found in 2 tumors with ALK fusions and both tumors with RET fusions. SAMS is on a morphologic and molecular genetic spectrum with EFH, with a similar body site distribution, frequent clinical presentation as an exophytic skin tumor, and invariably benign outcomes; we conclude that SAMS should be considered a histologic variant of EFH. Some morphologically typical examples harbor alternate RET and NTRK3 fusions, such that SAMS is not an appropriate designation for this morphologic class; instead, to highlight the clinicopathologic similarities to EFH, we propose the diagnostic term "myxoid spindle cell variant of epithelioid fibrous histiocytoma." - Source: PubMed
Publication date: 2024/09/27
DeSimone Mia SOdintsov IgorTsai Harrison KDickson Brendan CAlomari Ahmed KHornick Jason LFletcher Christopher D MPapke David J - The majority of dermatofibrosarcoma protuberans (DFSP) harbour PDGFB or PDGFD rearrangements. We encountered ALK expression/rearrangement in a PDGFB/D-negative CD34-positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of ALK-rearrangements in DFSP and plaque-like CD34-positive dermal fibroma (P-LDF). - Source: PubMed
Publication date: 2024/06/13
Agrawal ShrutiAmeline BaptisteFolpe Andrew LAzzato ElizabethAstbury CarolineMentzel ThomasKnapp CalvinRütten ArnoCreytens DavidSukov WilliamBaumhoer DanielBillings Steven DFritchie Karen J