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Neurofibroma with Atypia

MedGen UID:
1793874
•Concept ID:
C5557433
•
Neoplastic Process
Synonym: Ancient Neurofibroma

Definition

A neurofibroma characterized by the presence of scattered atypical or bizarre nuclei and smudgy chromatin in the absence of other worrisome features. (WHO 2020) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeurofibroma with Atypia

Professional guidelines

PubMed

The 2019 Genitourinary Pathology Society (GUPS) White Paper on Contemporary Grading of Prostate Cancer.
Epstein JI, Amin MB, Fine SW, Algaba F, Aron M, Baydar DE, Beltran AL, Brimo F, Cheville JC, Colecchia M, Comperat E, da Cunha IW, Delprado W, DeMarzo AM, Giannico GA, Gordetsky JB, Guo CC, Hansel DE, Hirsch MS, Huang J, Humphrey PA, Jimenez RE, Khani F, Kong Q, Kryvenko ON, Kunju LP, Lal P, Latour M, Lotan T, Maclean F, Magi-Galluzzi C, Mehra R, Menon S, Miyamoto H, Montironi R, Netto GJ, Nguyen JK, Osunkoya AO, Parwani A, Robinson BD, Rubin MA, Shah RB, So JS, Takahashi H, Tavora F, Tretiakova MS, True L, Wobker SE, Yang XJ, Zhou M, Zynger DL, Trpkov K
Arch Pathol Lab Med 2021 Apr 1;145(4):461-493. doi: 10.5858/arpa.2020-0015-RA. PMID: 32589068
Field cancerization: Treatment.
Cornejo CM, Jambusaria-Pahlajani A, Willenbrink TJ, Schmults CD, Arron ST, Ruiz ES
J Am Acad Dermatol 2020 Sep;83(3):719-730. Epub 2020 May 6 doi: 10.1016/j.jaad.2020.03.127. PMID: 32387663
The 2017 Bethesda System for Reporting Thyroid Cytopathology.
Cibas ES, Ali SZ
Thyroid 2017 Nov;27(11):1341-1346. doi: 10.1089/thy.2017.0500. PMID: 29091573

Recent clinical studies

Etiology

Papillary neoplasms of the breast including upgrade rates and management of intraductal papilloma without atypia diagnosed at core needle biopsy.
Brogi E, Krystel-Whittemore M
Mod Pathol 2021 Jan;34(Suppl 1):78-93. Epub 2020 Oct 26 doi: 10.1038/s41379-020-00706-5. PMID: 33106592
Fibroepithelial lesions revisited: implications for diagnosis and management.
Tan PH
Mod Pathol 2021 Jan;34(Suppl 1):15-37. Epub 2020 May 27 doi: 10.1038/s41379-020-0583-3. PMID: 32461622
Polycystic Ovary Syndrome.
Azziz R
Obstet Gynecol 2018 Aug;132(2):321-336. doi: 10.1097/AOG.0000000000002698. PMID: 29995717
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
PEComa: morphology and genetics of a complex tumor family.
Thway K, Fisher C
Ann Diagn Pathol 2015 Oct;19(5):359-68. Epub 2015 Jun 9 doi: 10.1016/j.anndiagpath.2015.06.003. PMID: 26144278

Diagnosis

Apocrine lesions of the breast.
Quinn CM, D'Arcy C, Wells C
Virchows Arch 2022 Jan;480(1):177-189. Epub 2021 Sep 18 doi: 10.1007/s00428-021-03185-4. PMID: 34537861Free PMC Article
Fibroepithelial tumours of the breast-a review.
Lerwill MF, Lee AHS, Tan PH
Virchows Arch 2022 Jan;480(1):45-63. Epub 2021 Sep 10 doi: 10.1007/s00428-021-03175-6. PMID: 34505197
Polycystic Ovary Syndrome.
Azziz R
Obstet Gynecol 2018 Aug;132(2):321-336. doi: 10.1097/AOG.0000000000002698. PMID: 29995717
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article

Therapy

Uterine serous carcinoma.
Bogani G, Ray-Coquard I, Concin N, Ngoi NYL, Morice P, Enomoto T, Takehara K, Denys H, Nout RA, Lorusso D, Vaughan MM, Bini M, Takano M, Provencher D, Indini A, Sagae S, Wimberger P, Póka R, Segev Y, Kim SI, Candido Dos Reis FJ, Lopez S, Mariani A, Leitao MM Jr, Raspagliesi F, Panici PB, Di Donato V, Muzii L, Colombo N, Scambia G, Pignata S, Monk BJ
Gynecol Oncol 2021 Jul;162(1):226-234. Epub 2021 Apr 30 doi: 10.1016/j.ygyno.2021.04.029. PMID: 33934848Free PMC Article
Levonorgestrel-releasing intrauterine system for endometrial hyperplasia.
Mittermeier T, Farrant C, Wise MR
Cochrane Database Syst Rev 2020 Sep 6;9(9):CD012658. doi: 10.1002/14651858.CD012658.pub2. PMID: 32909630Free PMC Article
Field cancerization: Definition, epidemiology, risk factors, and outcomes.
Willenbrink TJ, Ruiz ES, Cornejo CM, Schmults CD, Arron ST, Jambusaria-Pahlajani A
J Am Acad Dermatol 2020 Sep;83(3):709-717. Epub 2020 May 7 doi: 10.1016/j.jaad.2020.03.126. PMID: 32387665
Field cancerization: Treatment.
Cornejo CM, Jambusaria-Pahlajani A, Willenbrink TJ, Schmults CD, Arron ST, Ruiz ES
J Am Acad Dermatol 2020 Sep;83(3):719-730. Epub 2020 May 6 doi: 10.1016/j.jaad.2020.03.127. PMID: 32387663
Metformin for endometrial hyperplasia.
Clement NS, Oliver TR, Shiwani H, Sanner JR, Mulvaney CA, Atiomo W
Cochrane Database Syst Rev 2017 Oct 27;10(10):CD012214. doi: 10.1002/14651858.CD012214.pub2. PMID: 29077194Free PMC Article

Prognosis

Fibroepithelial tumours of the breast-a review.
Lerwill MF, Lee AHS, Tan PH
Virchows Arch 2022 Jan;480(1):45-63. Epub 2021 Sep 10 doi: 10.1007/s00428-021-03175-6. PMID: 34505197
Fibroepithelial lesions revisited: implications for diagnosis and management.
Tan PH
Mod Pathol 2021 Jan;34(Suppl 1):15-37. Epub 2020 May 27 doi: 10.1038/s41379-020-0583-3. PMID: 32461622
Field cancerization: Definition, epidemiology, risk factors, and outcomes.
Willenbrink TJ, Ruiz ES, Cornejo CM, Schmults CD, Arron ST, Jambusaria-Pahlajani A
J Am Acad Dermatol 2020 Sep;83(3):709-717. Epub 2020 May 7 doi: 10.1016/j.jaad.2020.03.126. PMID: 32387665
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article

Clinical prediction guides

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
The 2017 Bethesda System for Reporting Thyroid Cytopathology.
Cibas ES, Ali SZ
Thyroid 2017 Nov;27(11):1341-1346. doi: 10.1089/thy.2017.0500. PMID: 29091573
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article
A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process.
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ; Peritoneal Surface Oncology Group International
Am J Surg Pathol 2016 Jan;40(1):14-26. doi: 10.1097/PAS.0000000000000535. PMID: 26492181
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Recent systematic reviews

Management of phyllodes tumor: A systematic review and meta-analysis of real-world evidence.
Yu CY, Huang TW, Tam KW
Int J Surg 2022 Nov;107:106969. Epub 2022 Oct 31 doi: 10.1016/j.ijsu.2022.106969. PMID: 36328344
A Systematic Review of Atypical Endometriosis-Associated Biomarkers.
Bartiromo L, Schimberni M, Villanacci R, Mangili G, Ferrari S, Ottolina J, Salmeri N, Dolci C, Tandoi I, Candiani M
Int J Mol Sci 2022 Apr 17;23(8) doi: 10.3390/ijms23084425. PMID: 35457244Free PMC Article
Endometrial hyperplasia as a risk factor of endometrial cancer.
Nees LK, Heublein S, Steinmacher S, Juhasz-Böss I, Brucker S, Tempfer CB, Wallwiener M
Arch Gynecol Obstet 2022 Aug;306(2):407-421. Epub 2022 Jan 10 doi: 10.1007/s00404-021-06380-5. PMID: 35001185Free PMC Article
Levonorgestrel-releasing intrauterine system for endometrial hyperplasia.
Mittermeier T, Farrant C, Wise MR
Cochrane Database Syst Rev 2020 Sep 6;9(9):CD012658. doi: 10.1002/14651858.CD012658.pub2. PMID: 32909630Free PMC Article
Metformin for endometrial hyperplasia.
Clement NS, Oliver TR, Shiwani H, Sanner JR, Mulvaney CA, Atiomo W
Cochrane Database Syst Rev 2017 Oct 27;10(10):CD012214. doi: 10.1002/14651858.CD012214.pub2. PMID: 29077194Free PMC Article

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