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Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres

MedGen UID:
234154
•Concept ID:
C1333068
•
Neoplastic Process
Synonyms: CCMMT; Clear Cell Myomelanocytic Tumor; Clear Cell Myomelanocytic Tumors; Clear-Cell Myomelanocytic Tumor; Clear-Cell Myomelanocytic Tumors; Myomelanocytic Tumor, Clear-Cell; Myomelanocytic Tumors, Clear-Cell; Tumor, Clear-Cell Myomelanocytic; Tumors, Clear-Cell Myomelanocytic

Definition

A very rare, usually benign neoplasm with perivascular epithelioid cell differentiation characterized by the presence of clear spindle cells arranged in fascicles and nests that usually affects young girls. Patients usually present with a painful abdominal mass. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres

Recent clinical studies

Etiology

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
PEComas of the kidney and of the genitourinary tract.
Martignoni G, Pea M, Zampini C, Brunelli M, Segala D, Zamboni G, Bonetti F
Semin Diagn Pathol 2015 Mar;32(2):140-59. Epub 2015 Feb 24 doi: 10.1053/j.semdp.2015.02.006. PMID: 25804448
Multifocal PEComa (PEComatosis) of the female genital tract and pelvis: a case report and review of the literature.
Yang W, Li G, Wei-qiang Z
Diagn Pathol 2012 Mar 9;7:23. doi: 10.1186/1746-1596-7-23. PMID: 22404894Free PMC Article
Clear cell 'sugar' tumor of the breast: another extrapulmonary site and review of the literature.
Govender D, Sabaratnam RM, Essa AS
Am J Surg Pathol 2002 May;26(5):670-5. doi: 10.1097/00000478-200205000-00014. PMID: 11979098
Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW
Am J Surg Pathol 2000 Sep;24(9):1239-46. doi: 10.1097/00000478-200009000-00007. PMID: 10976698

Diagnosis

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
Malignant perivascular epithelioid cell tumor of the gallbladder: a case report and review of literature.
Zhao L, Anders KH
Arch Pathol Lab Med 2014 Sep;138(9):1238-41. doi: 10.5858/arpa.2013-0092-CR. PMID: 25171708
Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres.
Tan Y, Zhang H, Wang XC
Indian J Pathol Microbiol 2014 Jul-Sep;57(3):453-5. doi: 10.4103/0377-4929.138766. PMID: 25118744
Multifocal PEComa (PEComatosis) of the female genital tract and pelvis: a case report and review of the literature.
Yang W, Li G, Wei-qiang Z
Diagn Pathol 2012 Mar 9;7:23. doi: 10.1186/1746-1596-7-23. PMID: 22404894Free PMC Article
Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW
Am J Surg Pathol 2000 Sep;24(9):1239-46. doi: 10.1097/00000478-200009000-00007. PMID: 10976698

Prognosis

Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW
Am J Surg Pathol 2000 Sep;24(9):1239-46. doi: 10.1097/00000478-200009000-00007. PMID: 10976698

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