In the present study, one case was attributed to hemangioma at initial examination. In alveolar soft part sarcomas, solid tumor tissues are surrounded by vascular tissues and blood flow wash-out is slow. In contrast, A-V malformations are comprised of pure vascular tissue without accompanying tissues, and blood flow wash-out is rapid, which enables the differentiation of these two conditions [ 14 - 16 ]. Since most soft tissue sarcomas lack defined radiological characteristics, it is not easy to differentiate sarcomas, even by MRI.
Nonetheless, alveolar soft part sarcomas do have specific imaging results, and thus, imaging tests may play a decisive diagnostic role. The general characteristics of most sarcomas are that they grow quickly, are located deep within tissue, and are relatively large. As shown by the present study, the characteristics of some types of soft tissue sarcomas contrast with the traditionally held opinion that they are small slow-growing masses with a superficial location.
Fortunately, non-specific soft tissue sarcomas are uncommon, and if preconceived notions are avoided, they can be diagnosed early. Synovial sarcoma cases occur primarily in adults in the vicinity of the knee joint and in the lower leg, and the tumors grow slowly and occur superficially.
On the other hand, alveolar soft part sarcomas occur in adolescents and young adults, and are found preferentially around the knee joint and have specific MRI characteristics. According to this study, some soft tissue tumors that grow slowly are painless, and some that occur in the superficial limbs may be highly malignant. The most important characteristic of soft tissue sarcomas is their non-specific nature. Thus, even when a slow growing, painless superficial mass is encountered in the limbs, after eliminating absolutely benign possibilities, such as, a ganglion of the wrist or lipoma of the back, physicians must consider the possibility of sarcoma and conduct a careful examination.
All authors read and approved the final manuscript. National Center for Biotechnology Information , U. World J Surg Oncol. Published online Sep Author information Article notes Copyright and License information Disclaimer. Corresponding author. Jong Hoon Park: rk. Received Jun 27; Accepted Sep This article has been cited by other articles in PMC. Abstract Purpose To evaluate the characteristics of highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.
Materials and methods The clinical and radiological characteristics of 18 cases of highly malignant soft tissue sarcomas of the extremity with a delayed diagnosis were determined. Results Ten men and eight women of mean age Conclusions Contrary to general expectations, some soft tissue tumors that grow slowly are painless, and those that occur in superficial limbs may be highly malignant.
Materials and methods Of 31 cases with a diagnosis of highly malignant Grade III according to the WHO classification soft tissue sarcoma of the limb treated at our hospital between July and December , we analyzed 18 cases in which the delay between patient awareness of the tumor and diagnosis exceeded 1 year. Results Seven cases of synovial sarcoma, three cases each of alveolar soft part sarcoma and malignant fibrous histiocytoma, two cases each of myxofibrosarcoma and high-grade leiomyosarcoma, and one case of clear cell sarcoma were diagnosed.
Open in a separate window. Figure 1. Figure 2. Discussion Some types of soft tissue sarcoma that develop in the limbs or axial skeleton grow slowly over several years, or remain the same size for years or even decades, and then suddenly start to grow. Table 1 Clinical data of all materials. Conclusions According to this study, some soft tissue tumors that grow slowly are painless, and some that occur in the superficial limbs may be highly malignant.
Competing interests The authors declare that they have no competing interests. Residual disease following unplanned excision of soft tissue sarcoma of an extremity. J Bone Joint Surg. The management of soft-tissue sarcomas of the extremities. Adult soft tissue sarcomas. A pattern of care survey of the American College of Surgeons. Ann Surg. Soft tissue tumors. Louis, Missouri; Synovial sarcoma, A clinicopathologic study. Orthop Rev. Liposarcomas, one of the more common types of soft tissue sarcoma, are cancerous tumors that develop from fat tissue, most often in the thighs or deep within the abdomen.
Symptoms include a painless lump under the skin. Some liposarcomas are very slow growing and respond well to treatment with surgery. Others grow rapidly and require an aggressive treatment plan that includes surgery, radiation therapy, or possibly chemotherapy.
Our doctors use genetic and molecular testing of the tumor to determine what type of liposarcoma is present and how best to treat it. Most angiosarcomas are cancerous tumors arising from the inner lining of the blood vessels. They can occur in the skin, the spleen, the liver, or breast tissue. When they develop on the skin, they sometimes look like a bruise or an infection, or they may appear as a soft, small red spot.
They may occur on the skin after radiation therapy, often in women who are in remission from breast cancer. They can also develop on the head and face, usually in men older than age 60, or in the soft tissue of younger adults. The location of the tumor often guides individualized treatment plans, which may include surgery, chemotherapy, and radiation therapy.
Although this growth was once described as slow growing and not likely to spread, many solitary fibrous tumors, also known as SFTs, can spread to other parts of the body after treatment.
Many are found on the lining of the lungs, but they can occur elsewhere in the body. Treatment often includes surgery and radiation therapy, depending on the size and location of the tumor and how quickly it is growing. Leiomyosarcoma is an aggressive cancer that develops from smooth muscles, the muscles of the body that you cannot control.
Leiomyosarcoma of the uterus is sometimes mistaken for a noncancerous tumor called leiomyoma. Surgically removing a suspected leiomyoma using a procedure called a morcellation, in which small pieces of the growth are removed one at a time, can inadvertently cause the spread, or seeding, of what is actually a leiomyosarcoma.
The chemotherapy drugs prescribed for leiomyosarcoma are different from those used for other soft tissue sarcomas, requiring the guidance of our medical oncologists. Dermatofibrosarcoma protuberans, also called DFSP, is a slow growing tumor consisting of long, narrow cells with tapered ends.
Because of their appearance, doctors call them spindle cells. These tumors form in the skin or just below it, and surgery can lead to a remission.
Although DFSPs infrequently spread to other organs, they may recur in the area in which they originally grew, even if the border of tissue around the tumor was clear of cancer cells. Low-grade fibromyxoid sarcomas are slow growing but also have the potential to spread to other parts of the body many years after diagnosis.
They can appear on the trunk, arms, or legs as a painless lump. These tumors contain a gene mutation, which doctors use to diagnose the cancer. Surgery can successfully treat the condition, but most of these tumors tend to come back after several years.
Malignant peripheral nerve sheath tumors are cancerous sarcomas that arise in the cells surrounding the nerves in the peripheral nervous system, meaning those outside the spinal cord and brain. This form of sarcoma can develop spontaneously or in people with a genetic condition called neurofibromatosis type 1 , who can receive care at the NYU Langone Comprehensive Neurofibromatosis Center.
Symptoms of these tumors can include pain, weakness, or numbness in the muscles near the tumor. Treatment can include surgery, along with radiation therapy and chemotherapy. Both fibromatosis and desmoid tumors are usually slow growing and benign, or noncancerous, sarcomas that do not spread. But some can grow fast and become large enough to damage surrounding vital organs, such as the intestines, liver, lungs, and nerves.
Another option is treating with radiation therapy after surgery. This lowers the chance of the cancer coming back. If the tumor is not in a limb, for example it's in the head, neck, or abdomen , it can be harder to take out the entire tumor with enough normal tissue around it.
For these tumors, radiation with or without chemo may be given before surgery. This may be able to shrink the tumor enough to remove it entirely with surgery. If radiation is not used before surgery, it may be given after surgery to lessen the chance that the tumor will come back. They tend to grow and spread quickly. Some stage III tumors have already spread to nearby lymph nodes. Even when these sarcomas have not yet spread to lymph nodes, the risk of spread to lymph nodes or distant sites is very high.
These tumors also tend to grow back in the same area after they're removed. This is called local recurrence. Lymph nodes will also be removed if they contain cancer. Radiation may be given after surgery. If the tumor is large or in a place that would make surgery difficult, but not in lymph nodes, the patient may be treated with chemo , radiation, or both before surgery.
For large tumors in the arms or legs, giving chemo by isolated limb perfusion is also an option.
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