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Soft Tissues: Malignant Myoepithelioma

Written2005-08Eva van den Berg
Clinical cytogeneticist, Dept. of Clinical Genetics, Academic Hospital Groningen, Ant. Deusinglaan 4, 9713 AW Groningen, the Netherlands

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ICD-Topo C470-C476,C478-C479,C490-C496,C498-C499 CONNECTIVE & SOFT TISSUE
ICD-Morpho 9373/1 8940/1 8982/1 Parachordoma Mixed tumour Myoepithelioma
Atlas_Id 5206
Phylum Soft Tissue Tumors:Uncertain differentiation:Mixed tumor/Myoepithelioma/Parachordoma

Clinics and Pathology

Disease In the recent WHO classification of tumors of soft tissue and bone, myoepithelioma is considered to be part of a spectrum that also embrases mixed tumor and parachordoma.
Soft tissue myoepithelioma is a rare tumor displaying myoepithelial elements within a hyalinized to chondromyxoid stroma and lacking obvious ductal differentiation. The histogenesis is unclear and most are benign, but some behave in a malignant fashion.


Note To date there are no cytogenetic data on soft tissue myoepithelioma as such except for one case: the stemline is described as 82,XXYY,+Y,-1,add(1)(p13),-3,del(3)(p21),-4,del(4)(q27q31),del(5)(q11q34),-6,-6,add(7)(p21), der(9)t(1;9)(q25;p22) or (q31;p23)x2,+der(9)t(9;9)(p13;q22),-10,-11,-13,-14,-18,-21,-22[4] and the sideline as 86,idem,+7,+8,+9,-der(9)t(9;9),+15,i(20)(q10),+21,add(22)(p1)[4].
This case shares some cytogenetic aberrations described in pleiomorphic adenomas of the salivary gland and basal cell (myoepithelial) adenocarcinoma of the lung especially rearrangements of chromosome 1 and 9 and some numerical chromosomal aberrations mentioned in chordomas, especially losses of chromosomes 3, 4, and to al lesser extent, 10 and 13.
These cytogenetic aberrations seem unrelated to previously reported chromosome changes usually seen in closely related entities like parachordoma and intramuscular mixed tumor in which cases mostly loss of material from the short arm of chromosome 17 was detected.

It might be that soft tissue myoepithelioma is a distinct entity with some resemblance to (para)-chordoma on the one hand and myoepithelioma on the other.
It was recently observed that benign human myoepithelial tumors of diverse sources exhibit common mRNA expression profiles indicative of a tumor-suppressor phenotype.


Myoepithelial mRNA expression profiling reveals a common tumor-suppressor phenotype.
Barsky SH
Experimental and molecular pathology. 2003 ; 74 (2) : 113-122.
PMID 12710942
Basal cell (myoepithelial) adenocarcinoma of the lung. First case with cytogenetic findings.
Damiani S, Magrini E, Farnedi A, Pession A
Histopathology. 2004 ; 45 (4) : 422-424.
PMID 15469487
Parachordoma exists--but what is it?
Fisher C
Advances in anatomic pathology. 2000 ; 7 (3) : 141-148.
PMID 10809219
Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma.
Folpe AL, Agoff SN, Willis J, Weiss SW
The American journal of surgical pathology. 1999 ; 23 (9) : 1059-1067.
PMID 10478665
Tumours of uncertain differentiation: Mixed tumour/Myoepithelioma/Parachordoma.
Kilpatrick SE, Limon J
Parachordoma: a rare sarcoma with clonal chromosomal changes.
Limon J, Babińska M, Denis A, Ryś J, Niezabitowski A
Cancer genetics and cytogenetics. 1998 ; 102 (1) : 78-80.
PMID 9530345
Clonal chromosome aberrations in three sacral chordomas.
Mertens F, Kreicbergs A, Rydholm A, Willén H, Carlén B, Mitelman F, Mandahl N
Cancer genetics and cytogenetics. 1994 ; 73 (2) : 147-151.
PMID 8174090
Intramuscular mixed tumour with clonal chromosomal changes.
Pauwels P, Dal Cin P, Roumen R, van den Berghe H, Sciot R
Virchows Archiv : an international journal of pathology. 1999 ; 434 (2) : 167-171.
PMID 10071252
Cytogenetic analysis of a parachordoma.
Tihy F, Scott P, Russo P, Champagne M, Tabet JC, Lemieux N
Cancer genetics and cytogenetics. 1998 ; 105 (1) : 14-19.
PMID 9689924
Cytogenetic analysis of a primary salivary gland myoepithelioma.
el-Naggar AK, Lovell M, Callender DL, Ordonez NG, Killary AM
Cancer genetics and cytogenetics. 1999 ; 113 (1) : 49-53.
PMID 10459346
Cytogenetics of a soft tissue malignant myoepithelioma.
van den Berg E, Zorgdrager H, Hoekstra HJ, Suurmeijer AJ
Cancer genetics and cytogenetics. 2004 ; 151 (1) : 87-89.
PMID 15120917


This paper should be referenced as such :
van den Berg, E
Soft tissue tumors: Malignant myoepithelioma
Atlas Genet Cytogenet Oncol Haematol. 2006;10(1):33-33.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes EWSR1

Translocations implicated (Data extracted from papers in the Atlas)

 t(1;22)(q23;q12) EWSR1/PBX1
 t(19;22)(q13;q12) EWSR1/ZNF444
 t(6;22)(p21;q12) EWSR1/POU5F1

External links

Mitelman database t(1;22)(q23;q12) EWSR1/PBX1 [Case List]    t(1;22)(q23;q12) EWSR1/PBX1 [Association List] Mitelman database (CGAP - NCBI)
Mitelman database t(19;22)(q13;q12) EWSR1/ZNF444 [Case List]    t(19;22)(q13;q12) EWSR1/ZNF444 [Association List] Mitelman database (CGAP - NCBI)
Mitelman database t(6;22)(p21;q12) EWSR1/POU5F1 [Case List]    t(6;22)(p21;q12) EWSR1/POU5F1 [Association List] Mitelman database (CGAP - NCBI)
arrayMap Topo ( C47,C49) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
Mitelman databaseEWSR1/PBX1[MCList]    EWSR1 (22q12.2) PBX1 (1q23.3)   t(1;22)(q23;q12)
TICdbEWSR1/PBX1    EWSR1 (22q12.2) PBX1 (1q23.3)
COSMIC_fusionEWSR1 (22q12.2) POU5F1 (6p21.33)    [fusion1305] [fusion1306] [fusion322] [fusion323] [fusion324]
Mitelman databaseEWSR1/POU5F1[MCList]    EWSR1 (22q12.2) POU5F1 (6p21.33)   t(6;22)(p21;q12)
TICdbEWSR1/POU5F1    EWSR1 (22q12.2) POU5F1 (6p21.33)
COSMIC_fusionEWSR1 (22q12.2) ZNF444 (19q13.43)    [fusion1342] [fusion1343] [fusion1345]
Mitelman databaseEWSR1/ZNF444[MCList]    EWSR1 (22q12.2) ZNF444 (19q13.43)   t(19;22)(q13;q12)
TICdbEWSR1/ZNF444    EWSR1 (22q12.2) ZNF444 (19q13.43)
Disease databaseSoft Tissues: Malignant Myoepithelioma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed

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