Clear-cell sarcoma
Encyclopedia
Clear-cell sarcoma is a rare form of cancer. It is a type of sarcoma
.
Recurrence is common.
It has been associated with a EWSR1-ATF1
fusion gene.
Clear cell sarcoma of the soft tissues in adults is not related to the pediatric tumor known as clear cell sarcoma of the kidney.
these tumors show some similarities to traditional skin melanomas, and are characterized by solid nests and fascicles of tumor cells with clear cytoplasm and prominent nucleoli. The clear cell sarcoma has a uniform and distinctive morphological pattern which serves to distinguish it from other types of sarcoma.
is also necessary. Furthermore, a chest CT, a bone scan and positron emission tomography (PET) may be part of the tests in order to evaluate areas where metastasizes occurs.
or hard mass. It causes either pain or tenderness but only until it becomes large enough. This kind of tumor is commonly found in the extremities especially around the knee
, feet and ankle
. It affects people of all ages, including young adults and even children.
and local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are the bone
, lymphnode and lungs.
in the first place in order to remove the tumor. The surgical procedure is then followed by radiation
and chemotherapy
.
Sarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
.
Recurrence is common.
It has been associated with a EWSR1-ATF1
ATF1
Cyclic AMP-dependent transcription factor ATF-1 is a protein that in humans is encoded by the ATF1 gene.-Interactions:ATF1 has been shown to interact with CSNK2A2, Casein kinase 2, alpha 1 and BRCA1. EWS,- External links :...
fusion gene.
Clear cell sarcoma of the soft tissues in adults is not related to the pediatric tumor known as clear cell sarcoma of the kidney.
Pathology
Under a microscopeMicroscope
A microscope is an instrument used to see objects that are too small for the naked eye. The science of investigating small objects using such an instrument is called microscopy...
these tumors show some similarities to traditional skin melanomas, and are characterized by solid nests and fascicles of tumor cells with clear cytoplasm and prominent nucleoli. The clear cell sarcoma has a uniform and distinctive morphological pattern which serves to distinguish it from other types of sarcoma.
Diagnosis
Imaging studies such as X-rays, computed tomography scans, or MRI may be required to diagnose clear-cell sarcoma together with a physical exam. Normally a biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
is also necessary. Furthermore, a chest CT, a bone scan and positron emission tomography (PET) may be part of the tests in order to evaluate areas where metastasizes occurs.
Signs and symptoms
It presents as a slow growing mass that especially affects tendons and aponeuroses and it is deeply situated. Patients often perceive it as a lumpSwelling (medical)
In medical parlance, swelling is the transient enlargement or protuberance in the body and may include tumors. According to cause, it may be congenital, traumatic, inflammatory, neoplastic or miscellaneous....
or hard mass. It causes either pain or tenderness but only until it becomes large enough. This kind of tumor is commonly found in the extremities especially around the knee
Knee
The knee joint joins the thigh with the leg and consists of two articulations: one between the fibula and tibia, and one between the femur and patella. It is the largest joint in the human body and is very complicated. The knee is a mobile trocho-ginglymus , which permits flexion and extension as...
, feet and ankle
Ankle
The ankle joint is formed where the foot and the leg meet. The ankle, or talocrural joint, is a synovial hinge joint that connects the distal ends of the tibia and fibula in the lower limb with the proximal end of the talus bone in the foot...
. It affects people of all ages, including young adults and even children.
Prognosis
When the tumor is large and there is presence of necrosisNecrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
and local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are the bone
Bone
Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...
, lymphnode and lungs.
Treatment
Treatment depends upon the site and the extent of the disease. Clear cell sarcoma is usually treated with surgerySurgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
in the first place in order to remove the tumor. The surgical procedure is then followed by radiation
Radiation
In physics, radiation is a process in which energetic particles or energetic waves travel through a medium or space. There are two distinct types of radiation; ionizing and non-ionizing...
and chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
.
See also
- MelanomaMelanomaMelanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye...
- Clear-cell sarcoma of the kidneyClear-cell sarcoma of the kidneyClear cell sarcoma of the kidney is a rare type of kidney cancer. Clear cell sarcoma can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body....
- List of cutaneous conditions