METHODS: We conducted a detailed clinicopathological review of a large retrospective cohort of 30 HN-NUT, aiming to expand its histological and immunohistochemical spectrum.
RESULTS: The median age of patients with HN-NUT was 39 years (range = 17-86). It affected the sinonasal tract (43%), major salivary glands (20%), thyroid (13%), oral cavity (7%), larynx (7%), neck (7%) and nasopharynx (3%). Although most cases of HN-NUT (63%) contained a component of primitive URC tumour, 53% showed other histological features and 37% lacked a URC component altogether. Variant histological features included basaloid (33%), differentiated squamous/squamoid (37%), clear cell changes (13%), glandular differentiation (7%) and papillary architecture (10%), which could co-exist. While most HN-NUT were positive for keratins, p63 and p40, occasional cases (5-9%) were entirely negative. Immunopositivity for neuroendocrine markers and thyroid transcription factor-1 was observed in 33 and 36% of cases, respectively. The outcome of HN-NUT was dismal, with a 3-year disease specific survival of 38%.
CONCLUSIONS: HN-NUT can affect individuals across a wide age range and arise from various head and neck sites. It exhibits a diverse spectrum of histological features and may be positive for neuroendocrine markers, potentially leading to underdiagnosis. A low threshold to perform NUT-specific tests is necessary to accurately diagnose HN-NUT.
方法:我们对30例HN-NUT的大型回顾性队列进行了详细的临床病理回顾,旨在扩大其组织学和免疫组织化学谱。
结果:HN-NUT患者的中位年龄为39岁(范围=17-86)。它影响了鼻窦(43%),主要唾液腺(20%),甲状腺(13%),口腔(7%),喉部(7%),颈部(7%)和鼻咽(3%)。尽管大多数HN-NUT病例(63%)含有原始URC肿瘤的成分,53%显示其他组织学特征,37%完全缺乏URC成分。不同的组织学特征包括基底细胞样(33%),分化鳞状/鳞状(37%),透明细胞变化(13%),腺体分化(7%)和乳头状结构(10%),可以共存。虽然大多数HN-NUT对角蛋白呈阳性,p63和p40,偶发病例(5-9%)完全阴性。在33%和36%的病例中观察到神经内分泌标志物和甲状腺转录因子-1的免疫阳性,分别。HN-NUT的结果令人沮丧,3年疾病特异性生存率为38%。
结论:HN-NUT可以影响广泛年龄范围内的个体,并出现在各种头颈部部位。它表现出不同的组织学特征,并且可能对神经内分泌标志物呈阳性,可能导致诊断不足。执行NUT特定测试的低阈值对于准确诊断HN-NUT是必要的。