Chopra Shefali, Maloney Nolan, Wang Wei Lien
Keck Medical Center, University of Southern California, Los Angeles, CA, USA.
The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Brain Tumor Pathol. 2022 Jan;39(1):35-42. doi: 10.1007/s10014-021-00416-z. Epub 2021 Nov 6.
Epithelioid inflammatory myofibroblastic sarcomas are an aggressive variant of inflammatory myofibroblastic tumor described primarily in the abdomen and less commonly in pulmonary location. The anaplastic lymphoma kinase (ALK) fusion partners described in this tumor include RANB2, RRBP1 and EML4. While rare examples of inflammatory myofibroblastic tumors have been described in the central nervous system, the epithelioid variant has never been described. The ALK-VCL fusion has been described in renal cell carcinoma, high-grade glioma and epithelioid fibrous histiocytoma but has not been described in epithelioid inflammatory myofibroblastic sarcoma or even inflammatory myofibroblastic tumor. Herein, we report the first case of epithelioid inflammatory myofibroblastic sarcoma in the central nervous system as well as the first case with VCL as the fusion partner for ALK.
上皮样炎性肌成纤维细胞肉瘤是炎性肌成纤维细胞瘤的一种侵袭性变体，主要见于腹部，较少见于肺部。该肿瘤中描述的间变性淋巴瘤激酶（ALK）融合伴侣包括RANB2、RRBP1和EML4。虽然中枢神经系统中曾有罕见的炎性肌成纤维细胞瘤病例报道，但上皮样变体从未被描述过。ALK-VCL融合在肾细胞癌、高级别胶质瘤和上皮样纤维组织细胞瘤中已有报道，但在上皮样炎性肌成纤维细胞肉瘤甚至炎性肌成纤维细胞瘤中尚未见报道。在此，我们报告首例中枢神经系统上皮样炎性肌成纤维细胞肉瘤病例，以及首例以VCL作为ALK融合伴侣的病例。
