■NUT癌(NC)是一种进展迅速且罕见的肿瘤,主要影响年轻患者,生存时间约为1年。这些肿瘤中的大多数表达上皮标志物,没有观察到神经内分泌标志物。回顾性研究表明,由于缺乏共同的临床表现,病理学家和临床医师对该病认识不足,成像,和形态特征。
■一位60岁的女性出现在北京友谊医院,表现出重复性鼻出血,鼻痛,右侧鼻窦、额窦有明显肿块的鼻塞。显微镜分析揭示了两种独特的形态学变化,这在现有文献中尚未报道过:(I)小梭形细胞,细胞质稀疏,细胞核密集染色;(II)大肿瘤细胞,细胞质丰富,一些类似浆细胞的细胞。角质化的突然出现也是一个突出的特征。免疫组织化学染色显示两种细胞形态之间的差异。小梭形细胞同时表达CK5/6和P40,Ki67增殖指数为40%。大圆细胞不表达CK5/6和P40,但突触素呈局灶性阳性,Ki67指数为10%。NUT和P63在两种细胞类型中均强烈表达,荧光原位杂交(FISH)显示BRD4-NUTM1易位。经过20轮术后放射治疗,患者存活,在5个月随访期间未观察到复发或转移.
■我们提供了源自鼻腔的最古老的已知和幸存的NC患者的新信息,具有独特的形态学特征和不同的免疫组织化学结果。NUT抗体检测应在未分化或低分化的恶性肿瘤中进行,特别是那些具有中等大小细胞的细胞质液泡化和突然角质化的细胞,不管患者年龄。
UNASSIGNED: NUT carcinoma (NC) is a rapidly progressing and rare neoplasm that primarily affects younger patients and has a survival time of about 1 year. Most of these neoplasms express epithelial markers with no neuroendocrine markers observed. Retrospective studies have shown that pathologists and clinicians do not have a sufficient understanding of the disease due to the lack of common clinical manifestations, imaging, and morphological features.
UNASSIGNED: A 60-year-old female presented at Beijing Friendship Hospital, showing repetitive epistaxis, nasal pain, and nasal congestion with obvious masses in the right nasal sinus and frontal sinus. Microscope analysis revealed two unique morphological changes which have not been previously reported in the existing literature: (I) small spindle cells with sparse cytoplasm and densely stained nuclei and (II) large tumor cells with abundant cytoplasm, some cells resembling plasma cells. The sudden appearance of keratinization was also a prominent feature. Immunohistochemical staining showed differences between the two cell morphologies. Small spindle cells simultaneously expressed CK5/6 and P40, and the Ki67 proliferation index was 40%. The large round cells did not express CK5/6 and P40 but were focal positive for synaptophysin and the Ki67 index was 10%. NUT and P63 were strongly expressed in both cell types and fluorescence in situ hybridization (FISH) revealed BRD4-NUTM1 translocation. Following 20 rounds of postoperative radiation treatment, the patient was alive and no recurrence or metastasis was observed during a 5-month follow-up.
UNASSIGNED: We present novel information from the oldest known and surviving patient of NC originating in the nasal cavity with unique morphological features and different immunohistochemical results. NUT antibody testing should be performed in undifferentiated or poorly differentiated malignancies, particularly those with either or both cytoplasmic vacuolation of medium-sized cells and abrupt keratinization, irrespective of patient age.