Desmoplastic myxoid tumor (DMT), SMARCB1-mutant is a recently proposed brain tumor that occurs in the pineal region of adults. This tumor is characterized by desmoplastic stroma and various degrees of myxoid matrix. Tumor cells with low-grade morphology show polyphenotypic immunoreactivity, and rhabdoid cells are rare. We herein present a case with some uncommon features such as no myxoid stroma and slightly elevated proliferating activity. To date, knowledge on the variety of SMARCB1/INI1-deficient tumors of the central nervous system is gradually increasing, encompassing highly aggressive to slow-growing varieties. DMT, SMARCB1-mutant seems to be relatively benign, but careful attention is necessary because SMARCB1/INI1 deficiency is generally a genetic signature of concern.

促纤维增生性粘液样肿瘤 (DMT)，SMARCB1 突变体是最近提出的一种发生在成人松果体区的脑肿瘤。这种肿瘤的特征是促纤维增生性基质和不同程度的粘液样基质。低级别形态的肿瘤细胞显示多表型免疫反应性，横纹肌细胞很少见。我们在此介绍了一个具有一些不常见特征的病例，例如没有粘液样基质和略微升高的增殖活性。迄今为止，关于中枢神经系统 SMARCB1/INI1 缺陷肿瘤种类的知识正在逐渐增加，包括高度侵袭性到生长缓慢的品种。DMT、SMARCB1 突变体似乎相对良性，但需要仔细注意，因为 SMARCB1/INI1 缺陷通常是值得关注的遗传特征。