NTRK-rearranged Spindle Cell Neoplasm | NTRK基因重排 梭形细胞肿瘤 |
This provisional category includes a group of rare soft tissue tumors defined by NTRK gene fusions, encompassing a wide spectrum of histologies and clinical behavior (ranging from benign neoplasms to aggressive sarcomas); infantile fibrosarcoma is excluded from this category. | 这个暂定种类是一组由NTRK基因融合定义的罕见软组织肿瘤，包括广泛的组织学和临床行为（从良性肿瘤到侵袭性肉瘤）；此类肿瘤不包括婴儿型纤维肉瘤。 |
These tumors frequently demonstrate monotonous spindle cell morphology, infiltrative growth, and coexpression of CD34 and S100 protein by IHC. | 这类肿瘤常表现为单一梭形细胞形态，浸润性生长，以及IHC共表达CD34和S100蛋白。 |
This category includes the recently described lipofibromatosislike neural tumor and tumors that resemble peripheral nerve sheath tumors; many of these tumor subsets most often affect children. | 这一种类包括最近描述的脂肪纤维瘤样神经肿瘤和类似外周神经鞘肿瘤的肿瘤；其中这类肿瘤的许多亚型最好发于儿童。 |
The varied histologic features include haphazard and infiltrative monomorphic spindle cell morphology, a solid pattern with stromal bands and perivascular rings of keloid-like collagen (Fig. 7), a pattern with zonation resembling MPNST, and a particularly uncommon pattern with a myopericytomalike architecture. | 不同的组织学特征包括无序和浸润性单一梭形细胞形态，具有基质条束状和血管周围环绕的瘢痕疙瘩样胶原的实性模式（图7），类似MPNST的带状模式，以及具有肌周细胞瘤样结构的特别罕见模式。 |
In addition to CD34 and S100 protein co-expression, tumors retain H3K27me3 (in contrast to many MPNSTs), and the majority are reactive with a panTRK monoclonal antibody, although the latter shows imperfect specificity; tumors with NTRK1 fusions show the most consistent and strong staining (Fig. 7D). | 除了CD34和S100蛋白共表达外，肿瘤保留H3K27me3（与许多MPNST相反），大多数肿瘤表达panTRK单克隆抗体，尽管后者显示出不完全的特异性；NTRK1融合的肿瘤显示出最一致和最强的染色（图7D）。 |
Most tumors in this category harbor NTRK1 fusions, with a variety of partners, although rare NTRK2 and NTRK3 fusions have also been reported; those resembling peripheral nerve sheath tumors may instead harbor RAF1 or BRAF fusions. | 这类肿瘤大多有NTRK1融合，有多种伙伴基因，尽管也有报道罕见的NTRK2和NTRK3融合；类似周围神经鞘肿瘤的肿瘤可能存在RAF1或BRAF基因融合。 |
NTRK fusions form chimeric proteins containing aberrant oncogenic receptor tyrosine kinases (TRK-A, TRK-B, or TRK-C), which are therapeutic targets; detection of NTRK fusions is currently mandatory for systemic therapy. | NTRK融合形成包含异常致癌受体酪氨酸激酶（TRK-A、TRK-B或TRK-C）的嵌合蛋白，这些蛋白是治疗的靶点；检测NTRK融合目前是系统治疗的强制要求。 |
The prognosis appears to be related to histologic grade, although many NTRK-rearranged spindle cell neoplasms are clinically benign, and firm criteria for malignancy have yet to be established. | 预后似乎与组织学分级有关，尽管许多NTRK重排梭形细胞肿瘤临床上是良性的，而且恶性肿瘤的确切标准尚未建立。 |
