What are the risk factors for soft tissue sarcomas?
Wojcik, J. Primary sclerosing epithelioid fibrosarcoma of bone: Analysis of a series [Abstract]. The American Journal of Surgical Pathology , 38 11 , — MLA Wessels, Dan. MediLexicon, Intl. APA Wessels, D. MNT is the registered trade mark of Healthline Media. Any medical information published on this website is not intended as a substitute for informed medical advice and you should not take any action before consulting with a healthcare professional.
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Symptoms & Diagnosis
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It is a common misconception that sarcomas are named after the tissue compartment in which they arise. For example, we often see a soft tissue sarcoma in a skeletal muscle referred to as a rhabdomyosarcoma. For most subtypes of sarcoma the specific cause is unknown. However, there are some factors thought to be associated with specific sarcomas. Genetic syndromes such as neurofibromatosis and Li-Fraumeni cancer syndrome are associated with an increased risk of sarcoma.
Malignant peripheral nerve sheath tumors are almost always seen in patients with neurofibromatosis type 1, an autosomal dominant syndrome that is related to an abnormality of a pericentromeric gene on chromosome The product of this gene, neurofibromin, is a ubiquitous protein with tumor-suppressor activity. Well-differentiated liposarcomas very rarely metastasize; however, local recurrence can be a difficult problem if they are inadequately resected.
Pathologic examination of a malignant tumor will determine its grade, which is an attempt to predict the probability of metastasis. Most soft tissue sarcomas are derived from a mesodermal cell origin, with the exception of the malignant peripheral nerve sheath tumors, which are neuroectodermal in origin. Soft tissue sarcomas are grouped according to their presumed cell of origin e. Nevertheless, the biological activity of soft tissue sarcomas is similar for almost all cell types, and thus for purposes of diagnosis and management this group of tumors has historically been discussed as a whole,[ 10 ] although finer-grained approaches are becoming more feasible with better pathologic techniques.
Clinical assessment All soft tissue masses should be approached with the awareness that they may represent a sarcoma. While there are no absolute clinical indicators that will distinguish benign from malignant soft tissue masses, several clinical features exist that will suggest the possibility of a malignancy to an alert clinician. Failure to recognize these features at initial assessment can lead to misdiagnosis and interventions that may compromise survival or limb salvage opportunities once the correct diagnosis is made.
For example, in one case a year-old male being treated with anbicoagulants for abrial fibrillation presented with a soft tissue mass. The mass was managed as a spontaneous hematoma with percutaneous drainage for several months. After the lesion became fungating Figure 1 , MR images were obtained.
Subsequent biopsy confirmed malignant fibrous histiocytoma and the patient was referred to BCCA. Rapidly increasing size, particularly in a lesion that was previously stable, suggests malignancy, as does the sudden appearance of a new large mass. Family history is usually negative, although patients should be asked about conditions such as neurofibromatosis.
Exposure to industrial chemicals has not been conclusively proven to be associated with sarcoma. Lesions that are larger than 5 cm in any dimension, deep to the deep fascia, or that are growing rapidly should be assumed to represent soft tissue sarcomas until proven otherwise. In addition, superficial lesions that are smaller than 5 cm in any dimension but are adherent to the deep fascia or surrounding structures should be treated with suspicion. The consistency of a lesion can be misleading, as sarcomas can be quite soft and some benign lesions such as fibromatosis can be quite hard and irregular.
The relationship of a lesion to the deep fascia is often obvious on examination, but can be subtle. Lesions that feel superficial can be further evaluated by first attempting to mobilize the lesion with the patient relaxed, and then again with the underlying musculature tensed.
If the lesion remains mobile, it is most likely not adherent or deep. If the mass does not demonstrate any of these concerning features, serial examination every 3 to 6 months is sufficient management. Systemic examination should not be neglected.
Soft Tissues: Extraskeletal osteosarcoma
While most sarcomas primarily metastasize to the lungs, some sarcomas, such as synovial sarcomas and epithelioid sarcomas, have a predilection for lymphatic metastasis, and the regional lymph nodes should be examined. Lymphomas can present with soft tissue masses resembling sarcomas, and widespread lymphadenopathy may give a clue to this diagnosis. Chest and abdominal examination should be performed.
Further imaging for these lesions is of unclear benefit. An ultrasound examination can confirm the size of the lesion and its relationship to the fascia and is more readily available than MRI. However, ultrasound images will not be able to give any reliable information on the underlying pathology and clincians should not be falsely reassured by ultrasound findings. If a lesion has features suggestive of a sarcoma, then more complete imaging is warranted. It is important to complete all imaging studies prior to any biopsy for two reasons. First, it is impossible to accurately place a biopsy site so that it is consistent with future limb salvage procedures without detailed information about the lesion and its relationship to the normal anatomy that is now distorted by the mass.
This may lead to an amputation being required in a limb that would otherwise have been salvageable. Second, the biopsy itself causes edema and other changes that can interfere with interpretation of any subsequent imaging. MRI is recognized as the standard imaging modality for large or deep soft tissue lesions. However, most lesions will still require some sort of tissue diagnosis regardless of what the MR images reveal.
The sarcoma is deep and large with some stranding A and areas of high signal B , meaning that it is unlikely to be a lipoma. Plain radiographs are rarely helpful when investigating soft tissue masses, although lesions such as hemangiomas, synovial sarcomas, and lipomas can show intralesional calcification. Computed tomography is inferior to MRI but is sometimes used when the patient has a pacemaker or other condition precluding MRI, or where the lesion is near a large metallic implant that would generate a lot of magnetic interference.
PET scanning remains experimental for investigating soft tissue masses, but will most likely develop an important role in the management of soft tissue sarcoma because of its ability to provide information on the biological activity of the tissue. Bone scans have no role in the preoperative investigation of a soft tissue sarcoma. If regional lymphadenopathy is detected clinically, it can be further evaluated with MRI. If the inguinal and pelvic nodes are involved, then a CT scan of the abdomen can reveal para-aortic lymphadenopathy.
PET-CT scanning may be useful in distinguishing reactive from malignant lymphadenopathy, but requires great clinical experience and cannot be recommended for routine use. Staging Once the diagnosis of a soft tissue sarcoma has been made it is important to complete the staging process. In practice it is useful to think of staging as being local and systemic.
Local staging Local staging assesses the extent of the primary tumor mass and gives information on its resectability as well as prognosis. The most useful imaging modality for local staging is MRI. Optimally, this will be used prior to any biopsy, since local trauma from a biopsy can make interpretation of the MR images problematic. In selected cases of vascular compromise secondary to the sarcoma, local radiographs and vascular studies can also make up part of the local staging process.
Systemic staging Systemic staging assesses metastatic disease, and can be delayed until after a biopsy has confirmed the diagnosis. Although soft tissue sarcomas predominantly metastasize to the lung, some, such as synovial sarcoma, have a predilection for lymphatic spread. Imaging of the lungs with a chest X-ray or a CT scan is mandatory, and we recommend obtaining a baseline CT scan once the diagnosis of soft tissue sarcoma has been made.
Lymphatic spread can sometimes be assessed clinically. For this, added MRI or CT scanning can be helpful, combined with biopsy of nodes felt to possibly contain disease. Several staging systems are in common use.
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As the number of adverse factors increase, the probability of systemic metastasis occurring within 5 years also increases. It combines histological grade and compartmental status to predict outcome. Biopsy should be considered following careful clinical assessment and review of the appropriate imaging studies. If there is any doubt about whether or how a biopsy should be performed, the assistance of a surgeon with experience in the management of soft tissue sarcoma should be sought prior to undertaking any intervention on the lesion.