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Epithelial-myoepithelial carcinoma(EMC)

MedGen UID:
220892
Concept ID:
C1275278
Neoplastic Process
Synonyms: Adenomyo-epithelioma; CHONDROSARCOMA, EXTRASKELETAL MYXOID; Clear cell adenoma; Clear cell carcinoma; EMC; Glycogen-rich adenocarcinoma; Glycogen-rich adenoma; Monomorphic clear cell tumor; Tubular solid adenoma
Modes of inheritance:
Somatic mutation
MedGen UID:
107465
Concept ID:
C0544886
Cell or Molecular Dysfunction
Source: HPO
An alteration in DNA that occurs after conception. Somatic mutations can occur in any of the cells of the body except the germ cells (sperm and egg) and therefore are not passed on to children. These alterations can (but do not always) cause cancer or other diseases.
SNOMED CT: Extraskeletal myxoid chondrosarcoma (404079008); Chondroid sarcoma (404079008)
 
Genes (locations): NR4A3 (9q31.1); TAF15 (17q12)
OMIM®: 612237
Orphanet: ORPHA209916

Definition

Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm of chondroblastic origin. The tumors are most commonly found in middle-aged and elderly individuals, are more common among men, and are often detected as deep-seated lesions in the extremities. Despite their relatively low-grade malignancy, recurrence and metastasis may appear many years after the initial diagnosis. Histologic tissue section examination reveals a mixture of cellular and myxoid stromal components (Panagopoulos et al., 2002). [from OMIM]

Clinical features

Chondrosarcoma
MedGen UID:
3054
Concept ID:
C0008479
Neoplastic Process
A slowly growing malignant neoplasm derived from cartilage cells.
Chondrosarcoma
MedGen UID:
3054
Concept ID:
C0008479
Neoplastic Process
A slowly growing malignant neoplasm derived from cartilage cells.

Recent clinical studies

Etiology

Vázquez A, Patel TD, D'Aguillo CM, Abdou RY, Farver W, Baredes S, Eloy JA, Park RC
Otolaryngol Head Neck Surg 2015 Oct;153(4):569-74. Epub 2015 Jul 20 doi: 10.1177/0194599815594788. [Epub ahead of print] PMID: 26195572
Dimitrijevic MV, Tomanovic NR, Jesic SD, Arsovic NA, Mircic AL, Krstic AM
Arch Iran Med 2015 Apr;18(4):218-22. doi: 015184/AIM.005. PMID: 25841941
Mann JM, Kellman RM, Hahn SS, de la Roza GL, Gajra A
Head Neck 2015 Aug;37(8):E96-8. Epub 2015 May 26 doi: 10.1002/hed.23873. [Epub ahead of print] PMID: 25242451
Sainathan S, Pugalenthi A, Danovich S, Arif F, Andaz S
Am Surg 2012 May;78(5):E290-1. PMID: 22546111
Yang S, Chen X
Int J Oral Maxillofac Surg 2012 Jul;41(7):810-3. Epub 2011 Dec 29 doi: 10.1016/j.ijom.2011.12.012. [Epub ahead of print] PMID: 22209183

Diagnosis

Vázquez A, Patel TD, D'Aguillo CM, Abdou RY, Farver W, Baredes S, Eloy JA, Park RC
Otolaryngol Head Neck Surg 2015 Oct;153(4):569-74. Epub 2015 Jul 20 doi: 10.1177/0194599815594788. [Epub ahead of print] PMID: 26195572
Dimitrijevic MV, Tomanovic NR, Jesic SD, Arsovic NA, Mircic AL, Krstic AM
Arch Iran Med 2015 Apr;18(4):218-22. doi: 015184/AIM.005. PMID: 25841941
Davidoss NH, Khaleel Z, Low TH
BMJ Case Rep 2015 Mar 25;2015 doi: 10.1136/bcr-2014-204068. PMID: 25809427
Fonseca I, Bell A, Wani K, Bell D
Genes Chromosomes Cancer 2015 Apr;54(4):249-59. Epub 2014 Dec 29 doi: 10.1002/gcc.22238. [Epub ahead of print] PMID: 25546727Free PMC Article
Mann JM, Kellman RM, Hahn SS, de la Roza GL, Gajra A
Head Neck 2015 Aug;37(8):E96-8. Epub 2015 May 26 doi: 10.1002/hed.23873. [Epub ahead of print] PMID: 25242451

Therapy

Mann JM, Kellman RM, Hahn SS, de la Roza GL, Gajra A
Head Neck 2015 Aug;37(8):E96-8. Epub 2015 May 26 doi: 10.1002/hed.23873. [Epub ahead of print] PMID: 25242451
Zhang J, Zhen L, Zhang JG, Yu GY
Int J Oral Maxillofac Surg 2014 Dec;43(12):1431-5. Epub 2014 Sep 16 doi: 10.1016/j.ijom.2014.08.007. [Epub ahead of print] PMID: 25240907
Yamazaki H, Ota Y, Aoki T, Kaneko A
J Oral Maxillofac Surg 2013 Jan;71(1):220-6. Epub 2012 Jun 12 doi: 10.1016/j.joms.2012.03.031. [Epub ahead of print] PMID: 22695014
Esposito E, Cassiano B, Cinquegrani F
Acta Otorhinolaryngol Ital 2009 Jun;29(3):156-9. PMID: 20140162Free PMC Article
Pierard S, Gregoire V, Weynand B, Machiels JP
Eur Arch Otorhinolaryngol 2006 Dec;263(12):1158-60. Epub 2006 Jul 29 doi: 10.1007/s00405-006-0125-8. [Epub ahead of print] PMID: 16896751

Prognosis

Dimitrijevic MV, Tomanovic NR, Jesic SD, Arsovic NA, Mircic AL, Krstic AM
Arch Iran Med 2015 Apr;18(4):218-22. doi: 015184/AIM.005. PMID: 25841941
Fonseca I, Bell A, Wani K, Bell D
Genes Chromosomes Cancer 2015 Apr;54(4):249-59. Epub 2014 Dec 29 doi: 10.1002/gcc.22238. [Epub ahead of print] PMID: 25546727Free PMC Article
Baker AR, Ohanessian SE, Adil E, Crist HS, Goldenberg D, Mani H
Int J Surg Pathol 2013 Oct;21(5):514-9. Epub 2012 Dec 5 doi: 10.1177/1066896912468153. [Epub ahead of print] PMID: 23222809
Sainathan S, Pugalenthi A, Danovich S, Arif F, Andaz S
Am Surg 2012 May;78(5):E290-1. PMID: 22546111
Yang S, Chen X
Int J Oral Maxillofac Surg 2012 Jul;41(7):810-3. Epub 2011 Dec 29 doi: 10.1016/j.ijom.2011.12.012. [Epub ahead of print] PMID: 22209183

Clinical prediction guides

Seethala RR, Barnes EL, Hunt JL
Am J Surg Pathol 2007 Jan;31(1):44-57. doi: 10.1097/01.pas.0000213314.74423.d8. PMID: 17197918
Wilson RW, Moran CA
Hum Pathol 1997 May;28(5):631-5. PMID: 9158714
Witterick IJ, Noyek AM, Chapnik JS, Heathcote JG, Bedard YC
J Otolaryngol 1993 Jun;22(3):176-9. PMID: 8396653
Fonseca I, Soares J
Arch Pathol Lab Med 1993 Oct;117(10):993-5. PMID: 8105767
Makek M, Grant JW
Int J Oral Maxillofac Surg 1988 Apr;17(2):134-7. PMID: 3133424

Recent systematic reviews

Wang B, Brandwein M, Gordon R, Robinson R, Urken M, Zarbo RJ
Arch Pathol Lab Med 2002 Jun;126(6):676-85. doi: 10.1043/0003-9985(2002)126<0676:PSCCTA>2.0.CO;2. PMID: 12033955

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