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Sinonasal Large Cell Neuroendocrine Carcinoma

MedGen UID:
1772715
•Concept ID:
C5420035
•
Neoplastic Process
Synonym: Nasal Cavity and Paranasal Sinus Large Cell Neuroendocrine Carcinoma

Definition

A large cell neuroendocrine carcinoma that arises from the nasal cavity and paranasal sinuses. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSinonasal Large Cell Neuroendocrine Carcinoma

Professional guidelines

PubMed

Management of olfactory neuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma involving the skullbase.
Mehta GU, Raza SM, Su SY, Hanna EY, DeMonte F
J Neurooncol 2020 Dec;150(3):367-375. Epub 2020 May 18 doi: 10.1007/s11060-020-03537-1. PMID: 32424573
Sinonasal Neuroendocrine Neoplasms: Current Challenges and Advances in Diagnosis and Treatment, with a Focus on Olfactory Neuroblastoma.
Bell D
Head Neck Pathol 2018 Mar;12(1):22-30. Epub 2018 Feb 9 doi: 10.1007/s12105-018-0887-5. PMID: 29427030Free PMC Article
Sinonasal undifferentiated carcinoma: clinical and pathologic features and a discussion on classification, cellular differentiation, and differential diagnosis.
Ejaz A, Wenig BM
Adv Anat Pathol 2005 May;12(3):134-43. doi: 10.1097/01.pap.0000163958.29032.56. PMID: 15900114

Recent clinical studies

Etiology

Proceedings of the 2023 North American Society of Head and Neck Pathology Companion Meeting, New Orleans, LA, March 12, 2023: Navigating New Developments in High Grade Sinonasal Neuroendocrine and Neuroectodermal Neoplasms.
Rooper LM
Head Neck Pathol 2023 Jun;17(2):299-312. Epub 2023 May 15 doi: 10.1007/s12105-023-01548-8. PMID: 37184733Free PMC Article
IDH2 Mutation Analysis in Undifferentiated and Poorly Differentiated Sinonasal Carcinomas for Diagnosis and Clinical Management.
Riobello C, López-Hernández A, Cabal VN, García-Marín R, Suárez-Fernández L, Sánchez-Fernández P, Vivanco B, Blanco V, López F, Franchi A, Llorente JL, Hermsen MA
Am J Surg Pathol 2020 Mar;44(3):396-405. doi: 10.1097/PAS.0000000000001420. PMID: 31876581
Sinonasal Neuroendocrine Neoplasms: Current Challenges and Advances in Diagnosis and Treatment, with a Focus on Olfactory Neuroblastoma.
Bell D
Head Neck Pathol 2018 Mar;12(1):22-30. Epub 2018 Feb 9 doi: 10.1007/s12105-018-0887-5. PMID: 29427030Free PMC Article
Large Cell Neuroendocrine Carcinoma of the Head and Neck: A Clinicopathologic Series of 10 Cases With an Emphasis on HPV Status.
Thompson ED, Stelow EB, Mills SE, Westra WH, Bishop JA
Am J Surg Pathol 2016 Apr;40(4):471-8. doi: 10.1097/PAS.0000000000000580. PMID: 26735857Free PMC Article
Lymphoepithelial-like carcinomas of the head and neck.
Wenig BM
Semin Diagn Pathol 2015 Jan;32(1):74-86. Epub 2014 Dec 19 doi: 10.1053/j.semdp.2014.12.004. PMID: 25804344

Diagnosis

SMARCA4/BRG1-Deficient Sinonasal Carcinoma.
Kakkar A, Ashraf SF, Rathor A, Adhya AK, Mani S, Sikka K, Jain D
Arch Pathol Lab Med 2022 Sep 1;146(9):1122-1130. doi: 10.5858/arpa.2021-0001-OA. PMID: 34871352
Management of olfactory neuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma involving the skullbase.
Mehta GU, Raza SM, Su SY, Hanna EY, DeMonte F
J Neurooncol 2020 Dec;150(3):367-375. Epub 2020 May 18 doi: 10.1007/s11060-020-03537-1. PMID: 32424573
SMARCA4-deficient Sinonasal Carcinoma.
Agaimy A, Weichert W
Head Neck Pathol 2017 Dec;11(4):541-545. Epub 2017 Feb 7 doi: 10.1007/s12105-017-0783-4. PMID: 28176137Free PMC Article
Neuroendocrine neoplasms of the sinonasal region.
Bell D, Hanna EY, Weber RS, DeMonte F, Triantafyllou A, Lewis JS Jr, Cardesa A, Slootweg PJ, Stenman G, Gnepp DR, Devaney KO, Rodrigo JP, Rinaldo A, Wenig BM, Westra WH, Bishop JA, Hellquist H, Hunt JL, Kusafuka K, Perez-Ordoñez B, Williams MD, Takes RP, Ferlito A
Head Neck 2016 Apr;38 Suppl 1:E2259-66. Epub 2015 Jul 18 doi: 10.1002/hed.24152. PMID: 26041714
Lymphoepithelial-like carcinomas of the head and neck.
Wenig BM
Semin Diagn Pathol 2015 Jan;32(1):74-86. Epub 2014 Dec 19 doi: 10.1053/j.semdp.2014.12.004. PMID: 25804344

Therapy

Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses.
Babin E, Rouleau V, Vedrine PO, Toussaint B, de Raucourt D, Malard O, Cosmidis A, Makaeieff M, Dehesdin D
J Laryngol Otol 2006 Apr;120(4):289-97. Epub 2006 Mar 10 doi: 10.1017/S0022215106000594. PMID: 16526967

Prognosis

IDH2 -Mutated Sinonasal Tumors: A Review.
Alzumaili B, Sadow PM
Adv Anat Pathol 2023 Mar 1;30(2):104-111. Epub 2022 Dec 20 doi: 10.1097/PAP.0000000000000391. PMID: 36537260Free PMC Article
Sinonasal Neuroendocrine Neoplasms: Current Challenges and Advances in Diagnosis and Treatment, with a Focus on Olfactory Neuroblastoma.
Bell D
Head Neck Pathol 2018 Mar;12(1):22-30. Epub 2018 Feb 9 doi: 10.1007/s12105-018-0887-5. PMID: 29427030Free PMC Article
Poorly differentiated sinonasal tract malignancies: A review focusing on recently described entities.
Agaimy A
Cesk Patol 2016;52(3):146-53. PMID: 27526015
Neuroendocrine neoplasms of the sinonasal region.
Bell D, Hanna EY, Weber RS, DeMonte F, Triantafyllou A, Lewis JS Jr, Cardesa A, Slootweg PJ, Stenman G, Gnepp DR, Devaney KO, Rodrigo JP, Rinaldo A, Wenig BM, Westra WH, Bishop JA, Hellquist H, Hunt JL, Kusafuka K, Perez-Ordoñez B, Williams MD, Takes RP, Ferlito A
Head Neck 2016 Apr;38 Suppl 1:E2259-66. Epub 2015 Jul 18 doi: 10.1002/hed.24152. PMID: 26041714
Lymphoepithelial-like carcinomas of the head and neck.
Wenig BM
Semin Diagn Pathol 2015 Jan;32(1):74-86. Epub 2014 Dec 19 doi: 10.1053/j.semdp.2014.12.004. PMID: 25804344

Clinical prediction guides

IDH2 R172 Mutations Across Poorly Differentiated Sinonasal Tract Malignancies: Forty Molecularly Homogenous and Histologically Variable Cases With Favorable Outcome.
Glöss S, Jurmeister P, Thieme A, Schmid S, Cai WY, Serrette RN, Perner S, Ribbat-Idel J, Pagenstecher A, Bläker H, Keber U, Stadelmann C, Zechel S, Johann PD, Hasselblatt M, Paulus W, Thomas C, Dohmen H, Baumhoer D, Frank S, Agaimy A, Schüller U, Vasudevaraja V, Snuderl M, Liu CZ, Pfister DG, Jungbluth AA, Ghossein RA, Xu B, Capper D, Dogan S
Am J Surg Pathol 2021 Sep 1;45(9):1190-1204. doi: 10.1097/PAS.0000000000001697. PMID: 34265800Free PMC Article
Molecular epidemiology of IDH2 hotspot mutations in cancer and immunohistochemical detection of R172K, R172G, and R172M variants.
Dogan S, Frosina D, Geronimo JA, Hernandez E, Mohanty A, Bale T, Hechtman JF, Arcila ME, Hameed MR, Jungbluth AA
Hum Pathol 2020 Dec;106:45-53. Epub 2020 Oct 2 doi: 10.1016/j.humpath.2020.09.013. PMID: 33017591Free PMC Article
DNA methylation-based classification of sinonasal undifferentiated carcinoma.
Dogan S, Vasudevaraja V, Xu B, Serrano J, Ptashkin RN, Jung HJ, Chiang S, Jungbluth AA, Cohen MA, Ganly I, Berger MF, Momeni Boroujeni A, Ghossein RA, Ladanyi M, Chute DJ, Snuderl M
Mod Pathol 2019 Oct;32(10):1447-1459. Epub 2019 Jun 11 doi: 10.1038/s41379-019-0285-x. PMID: 31186531Free PMC Article
INSM1 is a Sensitive and Specific Marker of Neuroendocrine Differentiation in Head and Neck Tumors.
Rooper LM, Bishop JA, Westra WH
Am J Surg Pathol 2018 May;42(5):665-671. doi: 10.1097/PAS.0000000000001037. PMID: 29438167
Lymphoepithelial-like carcinomas of the head and neck.
Wenig BM
Semin Diagn Pathol 2015 Jan;32(1):74-86. Epub 2014 Dec 19 doi: 10.1053/j.semdp.2014.12.004. PMID: 25804344

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