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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
Sources: HPO, OMIM
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.
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
Somatic mutation (HPO, OMIM)
not inherited (Orphanet)
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:
505973
Concept ID:
CN005888
Finding
A slowly growing malignant neoplasm derived from cartilage cells.
Chondrosarcoma
MedGen UID:
505973
Concept ID:
CN005888
Finding
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. 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. PMID: 25242451
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. PMID: 22209183
Teppo H, Paronen I
J Craniofac Surg 2008 Nov;19(6):1689-91. doi: 10.1097/SCS.0b013e318189723a. PMID: 19098583

Diagnosis

Oh HJ, Do NY, Kee KH, Park JH
J Med Case Rep 2016 Feb 27;10:45. doi: 10.1186/s13256-016-0824-8. PMID: 26922486Free PMC Article
Molnar SL, Zarka MA, De Las Casas LE
Diagn Cytopathol 2016 May;44(5):422-5. Epub 2016 Jan 22 doi: 10.1002/dc.23434. PMID: 26798976
Hattori K, Murai N, Kusano J, Takahashi Y
Head Neck 2016 Mar;38(3):E73-5. Epub 2015 Aug 28 doi: 10.1002/hed.24179. PMID: 26314935
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. 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. 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. 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. 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. PMID: 16896751

Prognosis

Gonçalves AC, de Lima PP, Monteiro ML
Ophthal Plast Reconstr Surg 2016 Mar-Apr;32(2):e42-4. doi: 10.1097/IOP.0000000000000225. PMID: 25025382
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. 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. PMID: 23222809
Seethala RR, Richmond JA, Hoschar AP, Barnes EL
Arch Pathol Lab Med 2009 Jun;133(6):950-9. doi: 10.1043/1543-2165-133.6.950. PMID: 19492889

Clinical prediction guides

Hattori K, Murai N, Kusano J, Takahashi Y
Head Neck 2016 Mar;38(3):E73-5. Epub 2015 Aug 28 doi: 10.1002/hed.24179. PMID: 26314935
Shinozaki A, Nagao T, Endo H, Kato N, Hirokawa M, Mizobuchi K, Komatsu M, Igarashi T, Yokoyama M, Masuda S, Sano K, Izumi M, Fukayama M, Mukai K
Am J Surg Pathol 2008 Jun;32(6):913-23. doi: 10.1097/PAS.0b013e318160852a. PMID: 18425042
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
Fonseca I, Soares J
Arch Pathol Lab Med 1993 Oct;117(10):993-5. PMID: 8105767

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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