A myxoid liposarcoma is a
malignant
Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer.
A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
adipose tissue neoplasm of
myxoid appearance histologically.
Myxoid liposarcomas are the second-most common type of
liposarcoma
Liposarcomas are the most common subtype of soft tissue sarcomas, accounting for at least 20% of all sarcomas in adults. Soft tissue sarcomas are rare neoplasms with over 150 different histological subtypes or forms. Liposarcomas arise from the ...
, representing 30–40% of all liposarcomas in the limbs, occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist. They occur in the intermuscular
fascial planes or deep-seated areas. They present as a large, slow-growing, painless mass.
The neoplastic cells in these neoplasms contain
chromosomal translocation
In genetics, chromosome translocation is a phenomenon that results in unusual rearrangement of chromosomes. This includes "balanced" and "unbalanced" translocation, with three main types: "reciprocal", "nonreciprocal" and "Robertsonian" transloc ...
s which create one of two
fusion gene
In genetics, a fusion gene is a hybrid gene formed from two previously independent genes. It can occur as a result of translocation, interstitial deletion, or chromosomal inversion. Fusion genes have been found to be prevalent in all main types ...
s: the ''
FUS-
DDIT3'' in ~90% and the ''
EWSR1-DDIT3'' fusion gene in up to 10% of myxoid liposarcoma cases.
The ''FUS-DDIT3'' fusion gene forms by a merger of part of the
FUS FET gene family gene normally located at
band 11.2 on the
short (or "p") arm of
chromosome 16 with part of the ''DDIT3''
ETS transcription factor family
In the field of molecular biology, the ETS (E26 transformation-specific or Erythroblast Transformation Specific) family is one of the largest families of transcription factors and is unique to animals. There are 28 genes in humans, 27 in the mous ...
gene normally located at band 13.3 on the long (or "q") arm of
chromosome 12
Chromosome 12 is one of the 23 pairs of chromosomes in humans. People normally have two copies of this chromosome. Chromosome 12 spans about 133 million base pairs (the building material of DNA) and represents between 4 and 4.5 percent of the tot ...
. This fusion gene is notated as t(12;16)(q13;p11).
, i.e. laboratory studies, suggest that the ''FUS-DDIT3'' fusion gene may act as an
oncogene
An oncogene is a gene that has the potential to cause cancer. In tumor cells, these genes are often mutated, or expressed at high levels. to promote the development of myxoid liposarmas.
The ''EWSR1-DDIT3'' fusion gene forms by a merger of the ''
EWSR1'' FET gene family gene located at band 12.2 on the q arm of
chromosome 22
Chromosome 22 is one of the 23 pairs of chromosomes in human cells. Humans normally have two copies of chromosome 22 in each cell. Chromosome 22 is the second smallest human chromosome, spanning about 51 million DNA base pairs and representing b ...
with part of the ''DDIT3'' gene. This fusion gene is notated as t(12;22)(q13;12).
Additional images
Myxoid liposarcoma (06).JPG, Micrograph
A micrograph is an image, captured photographically or digitally, taken through a microscope or similar device to show a magnify, magnified image of an object. This is opposed to a macrograph or photomacrograph, an image which is also taken ...
of myxoid liposarcoma. H&E stain
Hematoxylin and eosin stain ( or haematoxylin and eosin stain or hematoxylin–eosin stain; often abbreviated as H&E stain or HE stain) is one of the principal tissue stains used in histology. It is the most widely used stain in medical diag ...
HG_mixoid_liposarcoma,_MRI,_2019,_10,_13.png, MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
of myxoid liposarcoma of high grade, in left axillary region of 40 year old man. Horizontal section.
See also
*
Lipoma
A lipoma is a benign tumor made of adipose tissue, fat tissue. They are generally soft to the touch, movable, and painless. They usually occur just under the skin, but occasionally may be deeper. Most are less than in size. Common locations inc ...
*
Trabectedin
Trabectedin, sold under the brand name Yondelis, is an antitumor chemotherapy medication for the treatment of advanced soft-tissue sarcoma and ovarian cancer.
The most common adverse reactions include nausea, fatigue, vomiting, constipation, de ...
References
External links
Myxoid liposarcoma: a rare soft-tissue tumor with a misleading benign appearanceEfficacy of first-line doxorubicin and ifosfamide in myxoid liposarcoma. 2012
{{Chromosomal abnormalities
Sarcoma