Kakkar Aanchal, Antony Vijay Mariadas, Irugu David Victor Kumar, Adhikari Narayan, Jain Deepali
Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India.
Head Neck Pathol. 2018 Jun;12(2):230-236. doi: 10.1007/s12105-017-0858-2. Epub 2017 Sep 25.
NUT midline carcinomas (NMCs) are rare, poorly differentiated tumors with aggressive biological behavior and a characteristic molecular signature. Availability of NUT antibody has facilitated diagnosis of NMC without molecular testing. We report a series of head and neck NMCs diagnosed using NUT IHC at our institute, including one case with an unusual course. Immunohistochemistry for NUT was performed in nasal and sinonasal tumors with diagnoses of undifferentiated carcinoma, poorly differentiated squamous cell carcinoma and malignant neoplasm, not otherwise specified, to identify cases of NMC. Clinicopathological features were reviewed. Five cases of NMC were identified, accounting for 9.6% of poorly differentiated/undifferentiated carcinomas of the sinonasal region. These patients had a sex ratio of 2:3, and ranged in age from of 10 to 31 years (mean: 25.2 years). Patient 4 had previously been diagnosed with basal cell carcinoma arising in left nasolacrimal duct, and inverted papilloma of nasal cavity. She presented to us with a left lacrimal fossa mass extending into nasal cavity, which was diagnosed as NMC. NMC is a rare neoplasm, the awareness of which is imperative for pathologists to identify cases in which NUT IHC should be ordered. NUT IHC should be performed in all cases of a poorly differentiated carcinoma, particularly those with foci of squamous differentiation, irrespective of patient age and unusual tumor location, as seen in one of our cases. Although considered a highly aggressive and lethal neoplasm, NMC can have a more prolonged clinical course on occasion.
NUT中线癌（NMC）是一种罕见的、低分化肿瘤，具有侵袭性生物学行为和特征性分子特征。NUT抗体的可用性有助于在无需分子检测的情况下诊断NMC。我们报告了一系列在我们研究所使用NUT免疫组化诊断的头颈部NMC，包括1例病程不寻常的病例。对诊断为未分化癌、低分化鳞状细胞癌和未另行指定的恶性肿瘤的鼻腔和鼻窦肿瘤进行NUT免疫组化，以识别NMC病例。回顾临床病理特征。共识别出5例NMC，占鼻窦区域低分化/未分化癌的9.6%。这些患者的性别比为2:3，年龄在10至31岁之间（平均：25.2岁）。患者4曾被诊断患有左鼻泪管基底细胞癌和鼻腔内翻性乳头状瘤。她因左泪窝肿块延伸至鼻腔就诊，被诊断为NMC。NMC是一种罕见肿瘤，病理学家必须认识到这一点，以便识别应进行NUT免疫组化的病例。对于所有低分化癌病例，尤其是那些有鳞状分化灶的病例，无论患者年龄和肿瘤位置是否异常，均应进行NUT免疫组化，正如我们其中1例病例所示。尽管NMC被认为是一种高度侵袭性和致命性肿瘤，但偶尔其临床病程可能会更长。
