TY - JOUR
T1 - Sinonasal Undifferentiated Carcinoma (SNUC)
T2 - From an Entity to Morphologic Pattern and Back Again-A Historical Perspective
AU - Agaimy, Abbas
AU - Franchi, Alessandro
AU - Lund, Valerie J.
AU - Skálová, Alena
AU - Bishop, Justin A.
AU - Triantafyllou, Asterios
AU - Andreasen, Simon
AU - Gnepp, Douglas R.
AU - Hellquist, Henrik
AU - Thompson, Lester D.R.
AU - Rinaldo, Alessandra
AU - Ferlito, Alfio
PY - 2020/3/1
Y1 - 2020/3/1
N2 - Since the first description of sinonasal undifferentiated carcinoma (SNUC) as a distinctive highly aggressive sinonasal neoplasm with probable origin from the sinonasal mucosa (Schneiderian epithelium), SNUC has been the subject of ongoing study and controversy. In particular, the SNUC category gradually became a "wastebasket" for any undifferentiated or unclassifiable sinonasal malignancy of definite or probable epithelial origin. However, with the availability of more specific and sensitive immunohistochemical antibodies and increasing implementation of novel genetic tools, the historical SNUC category became the subject of progressive subdivision leading to recognition of specific genetically defined, reproducible subtypes. These recently recognized entities are characterized by distinctive genetic aberrations including NUTM1-rearranged carcinoma (NUT carcinoma) and carcinomas associated with inactivation of different members of the SWI/SNF chromatin-remodeling gene complex such as SMARCB1-deficient and less frequently SMARCA4-deficient carcinoma. The ring became almost closed, with recent studies highlighting frequent oncogenic IDH2 mutations in the vast majority of histologically defined SNUCs, with a frequency of 82%. A review of these cases suggests the possibility that "true SNUC" probably represents a distinctive neoplastic disease entity, morphologically, phenotypically, and genetically. This review addresses this topic from a historical perspective, with a focus on recently recognized genetically defined subsets within the SNUC spectrum.
AB - Since the first description of sinonasal undifferentiated carcinoma (SNUC) as a distinctive highly aggressive sinonasal neoplasm with probable origin from the sinonasal mucosa (Schneiderian epithelium), SNUC has been the subject of ongoing study and controversy. In particular, the SNUC category gradually became a "wastebasket" for any undifferentiated or unclassifiable sinonasal malignancy of definite or probable epithelial origin. However, with the availability of more specific and sensitive immunohistochemical antibodies and increasing implementation of novel genetic tools, the historical SNUC category became the subject of progressive subdivision leading to recognition of specific genetically defined, reproducible subtypes. These recently recognized entities are characterized by distinctive genetic aberrations including NUTM1-rearranged carcinoma (NUT carcinoma) and carcinomas associated with inactivation of different members of the SWI/SNF chromatin-remodeling gene complex such as SMARCB1-deficient and less frequently SMARCA4-deficient carcinoma. The ring became almost closed, with recent studies highlighting frequent oncogenic IDH2 mutations in the vast majority of histologically defined SNUCs, with a frequency of 82%. A review of these cases suggests the possibility that "true SNUC" probably represents a distinctive neoplastic disease entity, morphologically, phenotypically, and genetically. This review addresses this topic from a historical perspective, with a focus on recently recognized genetically defined subsets within the SNUC spectrum.
KW - Biomarkers, Tumor/genetics
KW - Carcinoma/diagnosis
KW - DNA Helicases/genetics
KW - Humans
KW - Maxillary Sinus Neoplasms/diagnosis
KW - Nuclear Proteins/genetics
KW - SMARCB1 Protein/genetics
KW - Transcription Factors/genetics
UR - http://www.scopus.com/inward/record.url?scp=85079535693&partnerID=8YFLogxK
U2 - 10.1097/pap.0000000000000258
DO - 10.1097/pap.0000000000000258
M3 - Review
C2 - 31876536
AN - SCOPUS:85079535693
SN - 1072-4109
VL - 27
SP - 51
EP - 60
JO - Advances in Anatomic Pathology
JF - Advances in Anatomic Pathology
IS - 2
ER -