2,3 It is also known as carcinoma with t(15:19) translocation, 1 because early reports consistently cited identical chromosomal translocation. NUT midline carcinoma - very high mag. NUT carcinoma (NC) is a rare, aggressive subtype of squamous cell carcinoma defined by rearrangement of the NUTM1 (aka NUT) gene. Usually, it is found in the head, neck, and lungs. (Aug 2012). EPIDEMIOLOGY, RISK FACTORS AND SURVIVAL. NUT carcinomas of the gingiva exhibit fragmented tissue and tumor cell clusters, with locally visible bone fragments present within the gingiva (10x) (a). Pathology of Lung Cancer Min Zheng, MD, PhD ... Lymphoepithelioma-like carcinoma NUT carcinoma Salivary gland–type carcinomas Mucoepidermoid carcinoma Adenoid cystic carcinoma Epithelial-myoepithelial carcinoma Mesenchymal tumors, lymphohistiocytic tumors, tumors of … This website is intended for pathologists and laboratory personnel but not for patients. Many undifferentiated tumors occur in the sinonasal tract, the differential diagnoses of which are often challenging. This is a nearly uniformly lethal cancer affecting patients of all ages, but predominantly teens and young adults. Suzuki S, Kurabe N, Ohnishi I, et al. The remaining cases, the fusion of NUTM1 is to an unkn… NUT carcinoma (NC) represents a rare subset of highly aggressive poorly differentiated carcinomas characterized by rearrangement of the NUT (aka NUTM1, nuclear protein in testis) gene, most commonly fused to BRD4.Originally described as a mediastinal/thymic malignancy, NC has been reported at a variety of anatomic regions including the upper and lower aerodigestive tract. https://librepathology.org/w/index.php?title=NUT_carcinoma&oldid=50063, Attribution-NonCommercial-ShareAlike 4.0 International, NUT midline carcinoma, carcinoma with t(15;19) translocation, cohesive malignant cells (poorly differentiated carcinoma), islands of well-differentiated squamous epithelium. ; Tan, LM. "NUT midline carcinomas of the sinonasal tract.". NMC occurs in both children and adults. NUT carcinoma (NC), also known as NUT midline carcinoma, is a type of rare cancer that can grow anywhere in the body. In general, it presents in young adult men and has a poor prognosis. ; Meeuwis, CA. NC is driven by NUT‐fusion oncoproteins resulting from chromosomal translocation, most commonly BRD4‐NUT. ; Moura, Rde D.; Menezes, LM. NUT carcinoma, formerly known as NUT-midline carcinoma (NMC), is a rare and very aggressive form of cancer that can form anywhere in the body, but often forms along the midline structures (head, neck, or lungs). French, CA. NUT midline carcinoma (NMC), an aggressive form of squamous cell carcinoma, is defined by the presence of acquired chromosomal rearrangements involving NUT, usually BRD4-NUT fusion genes and, less commonly, NUT-variant fusion genes involving BRD3 or still-uncharacterized genes. Haack H, Johnson LA, Fry CJ, et al. Case report of a NMC in the parotid gland. A further study points to the potential of a new treatment for NUT carcinoma among one subgroup of patients. Lung tumor - NUT carcinoma (pending) This website is intended for pathologists and laboratory personnel but not for patients. (Sep 2014). Islands of well-differentiated squamous epithelium -. NUT Carcinoma of Lung is an extremely uncommon and aggressive malignancy that is named due to the presence of an abnormality involving the NUT gene (nuclear protein in testis) It is a poorly-differentiated carcinoma of unknown cause. "A review of NUT midline carcinoma.". Basic Facts about NUT Midline Carcinoma; Pathology; Treatment; Recommendations; Consultations; Registry Personnel; Enroll; Clinical Trials; Donate; Publications; Contact : Clinical Trials. Expressed in a broad spectrum of neuroendocrine tumors (including small cell carcinoma, large cell neuroendocrine carcinoma and well differentiated neuroendocrine tumor / carcinoid tumor of various sites, paraganglioma / pheochromocytoma, Merkel cell carcinoma, olfactory neuroblastoma and medullary thyroid carcinoma) Also expressed in normal adult neuroendocrine tissues (adrenal medulla, … We report on a case of NUT midline carcinoma of the mediastinum in a man 27 years of age without any prior malignancy. Due to the NUT carcinoma is an aggressive and invariably fatal carcinoma that generally affects young adults. Tissue/pathology . NC is driven by NUT-fusion oncoproteins resulting from chromosomal translocation, most commonly BRD4-NUT. NUT carcinoma (NC) (formerly NUT midline carcinoma), is a rare genetically defined, very aggressive squamous cell epithelial cancer that usually arises in the midline of the body and is characterized by a chromosomal rearrangement in the nuclear protein in testis gene. In NUT carcinoma staining is nuclear, with a speckled distribution (> 90% of cells are generally positive; the reported sensitivity is 87% and the specificity is 100% (clone: C52, In germ cell tumors staining is nuclear, with an even / uniform distribution and often only focal (, NUT is variably expressed among germ cell tumors, including: dysgerminoma (64 - 93%), embryonal carcinoma (0 - 9%), immature teratoma (75%), seminoma (6 - 74%), spermatocytic seminoma (100%) and yolk sac tumor (7 - 100%) (, NUT associate tumors arising in the soft tissue and viscera (, Germ cell tumors: spermatocytic tumor, other germ cell tumor staining often focal or weak (, Liver (minority of cases may contain cytoplasmic and nuclear blush), Nuclear expression in > 90% of cells with a speckled pattern, Nuclear expression in > 90% of cells with an even / uniform pattern, Cytoplasmic expression in < 90% of cells with a speckled pattern, Cytoplasmic expression in < 90% of cells with an even / uniform pattern. Background and Genetic Characteristics. ; Westra, WH. NUT midline carcinoma (NMC) is a rare and life-threatening type of cancer. Synonyms : NUT midline carcinoma, carcinoma with t(15;19) translocation "NUT midline carcinoma.". Nuclear protein of the testis (NUT) carcinoma (NC) (formerly known as NUT midline carcinoma) is an aggressive pleomorphic squamous cell carcinoma with a dismal prognosis. (Mar 2011). NUT carcinoma is a rare and aggressive solid tumor. Li, W.; Chastain, K. (Jul 2018). Diagnosis of NUT midline carcinoma using a NUT-specific monoclonal antibody. (WC). ; Antonescu, CR. Karakuş E(1), Poyraz A(2), Oğuz Erdogan AS(3), Emir S(4), Özyörük D(4). NUT carcinoma (NC) is a rare, aggressive subtype of squamous cell carcinoma defined by rearrangement of the NUTM1 (aka NUT) gene. Am J Surg Pathol 2009; 33:984. These tumors overexpress the nuclear protein in the testis (NUT), most commonly due to a … However, we cannot answer medical or research questions or give advice. NC grows very quickly and can spread to other parts of the body. "Demystified molecular pathology of NUT midline carcinomas.". NUT carcinoma (NC) is a rare, aggressive subtype of squamous cell carcinoma defined by rearrangement of the NUTM1 (aka NUT) gene. Basic Facts of NUT Midline Carcinoma. Nuclear protein in testis (NUT) carcinoma is one such tumor, and is considered a rare subtype of squamous cell carcinoma .It generally expresses the BRD4-NUT fusion gene, and patients have a very poor outcome. NMC is a rare subtype of squamous cell carcinoma defined by rearrangements of the NUT, aka NUTM1, gene on chromosome 15.q14.Most commonly the rearrangement is a translocation between BRD4 (chr. Abstract: NUT midline carcinoma (NMC) is a rare and aggressive subtype of squamous carcinoma that typically arises from midline supradiaphragmatic structures, frequently from the head and neck area. "Expression of P16 in NUT carcinomas with no association with human papillomavirus (HPV).". Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. Primary NC tumors are commonly located in the chest or head and neck regions. ; Bacchi, CE. Surgical pathology of the sinonasal tract (nasal cavity and paranasal sinuses) is extremely challenging due in part to the tremendous diversity of tumor types that may arise in this region. ; Beverloo, BH. (Nov 2010). Pathological diagnosis is usually regarded as the final diagnosis driving tailored management for a particular patient. [1] It is also known as NUT midline carcinoma, abbreviated NMC. NUT midline carcinoma (NMC) is a rare and aggressive genetically defined subtype of squamous cell carcinoma characterized by chromosomal rearrangement of the nuclear protein in testis (NUT) gene (also known as NUTM1 or Chr15orf55). Zhu, S.; Schuerch, C.; Hunt, J. NUT midline carcinomas (NMCs) comprise a group of highly aggressive tumors that have been reported primarily in the head, neck, and mediastinum of younger individuals. "NUT midline carcinoma of the parotid gland with mesenchymal differentiation.". NUT midline carcinomas (NMC) comprise a group of highly aggressive tumors that have been reported primarily in the head, neck, and mediastinum of younger individuals. It typically, but not always, affects the body’s “midline,” including the nose, mouth, sinuses, trachea, upper airways, chest, and thymus. Imaging plays an … We welcome suggestions or questions about using the website. These tumors share several overlapping histologic … (Jan 2015). "Review and updates of immunohistochemistry in selected salivary gland and head and neck tumors.". (Apr 2014). Poorly differentiated tumor cells with large nuclei are evident in dense clusters separated by fibrous stroma in another region of … den Bakker, MA. French, CA. NC grows from the squamous cells in the body, which are cells that make up the skin and lining of some organs, like the lungs and stomach. from tumor resection or biopsy A. Operative report B. Addendum and/or comments on tissue/pathology report ... NUT carcinoma 8023* Midline carcinoma of children and young adults with NUT rearrangement NUT midline carcinoma : Head and Neck Solid Tumor Rules NSD3-NUT-expressing midline carcinoma of the lung: first characterization of primary cancer tissue. Usually midline - as the name of the tumour suggests. 1. Department of Oncology and Hematology, University Hospital of Modena, Modena, Italy. NUT carcinoma (formerly known as NUT midline carcinoma) is a rare, aggressive cancer, 1,2 which is defined by fusions of the nuclear protein in testis (NUT) gene. Pathol Res Pract 2015; 211:404. NUT carcinoma is characterized by the fusion of NUTM1 with another gene (e.g., BRD4, BRD3 or NSD3, among others); these proteins bind acetylated chromatin; these domains result in dysregulated transcription of genes, including cMYC and TP63, which promote tumor growth (Genes Dev 2015;29:1507, Proc Natl Acad Sci U S A 2017;114:E4184) NUT Midline Carcinoma of the Lung in a Six-Year-Old Child. Improved diagnostic tests reveal that although rare, NMCs occur in people of any age and may be … (Jun 2010). Not specific to any tissue type or organ. Pathology is a medical specialty, which provides a tissue diagnosis using a wide array of the tools of laboratory medicine, including histology, cytology, immunohistochemistry, molecular biology, etc. (2)Institute of Pathology, Heidelberg University Hospital, University of Heidelberg, Im Neuenheimer Feld 224, 69120 Heidelberg, Germany. Defining NUT carcinoma. NUT carcinoma is a rare tumour of the head and neck. Introduction. NMC is genetically driven by a chromosomal rearrangement involving the NUT gene, which forms oncoproteins … ; Westra, WH. NUT carcinoma arising in the sinonasal tract: H&E, NUT carcinoma arising in the sinonasal tract: IHC, See Elsevier's new video about their Clinic Review Articles on our homepage or click, 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). Author information: (1)a Ankara Children's Hematology and Oncology Research and Training Hospital , Pathology Department , Ankara , Turkey. French, in Advances in Cancer Research, 2016 4.1 NUT Midline Carcinoma. (Aug 2009). ; van den Heuvel-Eibrink, MM. "NUT midline carcinoma with leukemic presentation mimicking CD34-positive acute leukemia.". In approximately 75% of cases, the coding sequence of NUTM1 on chromosome 15q14 is fused to BRD4 or BRD3, which creates a chimeric gene that encodes the BRD-NUT fusion protein. NUT carcinoma is an undifferentiated or poorly differentiated squamous cell carcinoma, meaning the cancer begins in squamous cells in the body. NUT carcinoma is a rarely diagnosed, poorly differentiated subtype of squamous cell carcinoma, defined by chromosomal rearrangements of the gene encoding nuclear protein of the testis (NUT). Bishop, JA. NC is driven by NUT‐fusion oncoproteins resulting from chromosomal translocation, most commonly BRD4‐NUT. Stelow, EB. Originally called thymic carcinomas with t(15;19) translocation, NUT midline carcinoma was first described in Japan in the early 1990s [1, 2].The term “NUT mid-line carcinoma” is used because this cancer is defined by rearrangement and trans-location of the NUT (NUTM1) gene, and most of these tumors (≈ 90%) arise from midline anatomic sites []. Bishop, JA. 1 The characteristic somatic t(15:19) translocation that positions NUT in frame with bromodomain containing 4 (BRD4), a ubiquitously expressed … Salles, PG. 2 The most common fusion seen is that of the NUT gene with the bromodomain-containing protein 4 (BRD4) gene, which is a regulator of chromatin activity, 2 but BRD3-NUT … It occurs all over the world in both adults and children of both sexes. One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. ; French, CA. A new study was able to develop a model categorizing the disease, and a treatment’s likelihood of success, based on the tumor’s site of origin and its molecular irregularity. This page was last edited on 9 April 2019, at 20:48. ; van Leenders, GJ. © Copyright PathologyOutlines.com, Inc. Click, NUT carcinoma is characterized by the fusion of, Diagnosis of NUT carcinoma is predicated on immunohistochemical demonstration of nuclear NUT staining (or molecular confirmation of, NUT is expressed in a subset of germ cell tumors (, Normal overexpression of this biomarker is limited to testicular and ovarian germ cells (, Function of the NUT protein remains poorly understood. "SMARCB1 (INI-1)-deficient carcinomas of the sinonasal tract.". 1 As a clinicopathologic entity, it has, to our knowledge, only been described as recent as the early 1990s. The WHO calls this tumour carcinoma with t(15;19) translocation. Compounding the difficulty, a number of new sinonasal tumor entities have been recently described, and patholo … Summary: Nuclear protein of the testis (NUT) midline carcinoma can present in the head, neck, and medi-astinum. C.A. ; Johnson, LA. Squamous cells normally line hollow organs in the body, such as the respir…
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