Patients with sarcoma will usually present with a non-tender mass in the limbs or in the torso. Sarcomas may affect nerve structures by compression and cause peripheral neurologic signs like radiculopathies and neuritis. Joints and bursae may also be invaded causing signs of limited mobility and pain. Cystic soft tissue tumors may transilluminate light sources during physical examinations. Late stage sarcomas can involve regional lymph nodes and cause node enlargement and adenopathies. Deep tissue masses should be accurately measured, masses more than 7 centimeters are most likely malignant in most cases of deep tissue sarcomas. Although 30% of subcutaneous soft tissue sarcomas behave less aggressively [5].

• Pain

  And because healthy, active children and teens often have pain and swelling in their arms and legs, osteosarcoma might be mistaken for growing pains or a sports injury. [webmd.com]

  The presence of severe pain and tenderness suggested an inflammatory process. Fasciitis ossificans is a rare form of heterotopic bone formation, commonly presenting with signs of local inflammation or pain. [ncbi.nlm.nih.gov]

  Classic symptoms include chest pain and shortness of breath in tumors that originate in the ribs, and back pain and “sciatic–like” symptoms in patients with tumors in the pelvis. News About Ewing Sarcoma [curesearch.org]

• Swelling

  Soft tissue sarcomas usually start as small, painless lumps under the skin or as a swelling of body tissue. [uabmedicine.org]

  Despite the rarity of femoral aneurysm and the plethora of other causes of swelling in and around its anatomic location, the possibility of its existence must always be borne in mind. [ncbi.nlm.nih.gov]

  For example: swelling under the skin may cause a painless lump that cannot easily be moved around and gets bigger over time swelling in the tummy (abdomen) may cause abdominal pain, a persistent feeling of fullness and constipation swelling near the lungs [nhs.uk]

  They may cause a lump or swelling in the soft tissue. Sometimes they spread and can press on nerves and organs, causing problems such as pain or trouble breathing. No one knows exactly what causes these cancers. [medlineplus.gov]

  Swelling and stiffness in a joint. Swelling of the legs or ankles. Many of the symptoms of sarcoma overlap with other health conditions. If you have any of these symptoms, it's vital to see a doctor right away. [hillman.upmc.com]

• Lymphedema

  Damaged lymph system or lymphedema Chronic lymphedema (where fluid collects in the tissue and causes swelling) after radiation to lymph nodes, or surgical removal of lymph nodes is also a risk factor. [stanfordhealthcare.org]

  Grade 2 late complications included lymphedema (10%), fracture (5%), and fibrosis (5%). At a median follow-up of 36months, the 3-year cumulative incidence of local failure was 41% (95% CI [20-63%]). [ncbi.nlm.nih.gov]

  Lower-extremity lymphangiosarcomas have also been observed in patients with congenital lymphedema or filariasis complicated by chronic lymphedema. [cancernetwork.com]

  Risk factors for developing soft tissue sarcoma include exposure to certain chemicals, previous radiation therapy, and long-term swelling of the arms and legs, called lymphedema. [uabmedicine.org]

  […] there are certain risk factors: Some inherited diseases, such as Li-Fraumeni syndrome Radiation therapy for other cancers Exposure to certain chemicals, such as vinyl chloride or certain herbicides Having swelling in the arms or legs for a long time (lymphedema [mountsinai.org]

• Familial Adenomatous Polyposis

  They include: Familial adenomatous polyposis (FAP) Retinoblastoma Neurofibromatosis type 1 (von Recklinghausen disease or NF1) Li-Fraumeni syndrome Lifestyle Chemical exposure: Exposure to vinyl chloride and dioxin may increase the risk of developing [cancercenter.com]

  These include neurofibromatosis, familial adenomatous polyposis (FAP), Li-Fraumeni syndrome, and retinoblastoma. Katelyn’s Story Learn how Katelyn overcame a rare form of sarcoma at Memorial Sloan Kettering. [mskcc.org]

  Genetic syndromes that increase your risk include hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, neurofibromatosis, tuberous sclerosis and Werner syndrome. Chemical exposure. [mayoclinic.org]

• Fatigue

  Common grade 2 acute toxicities were fatigue (26%), anorexia (17%), and urinary incontinence (13%). There were two grade 3 late wound infections (10%) and one grade 3 late wound complication (5%). [ncbi.nlm.nih.gov]

  Side effects include fever, chills, loss of appetite, headaches, nausea, fatigue and body aches. Preventive measures Preventive measures include practicing safe sex to reduce the risk of acquiring HIV. [news-medical.net]

  Ewing Sarcoma The symptoms of depend on the bone or soft tissue site in which the cancer develops, but they usually include: Pain at the site of the mass, often with swelling Patients may have general symptoms such as loss of appetite, fever, malaise, fatigue [curesearch.org]

  There were three DLT in the first 6 pts at dose level 0 (G3 fatigue in 2 and G4 septic shock) and 1 DLT in the following 10 pts at level -1 (febrile neutropenia). G3/4 toxicity: fatigue 25%, thrombocytopenia 19%, mucositis 13% and neutropenia 13%. [cureasps.org]

  If you have cancer of unknown primary (CUP), symptoms may include swollen lymph nodes, bone, chest or stomach pain, poor appetite, fatigue, headache, confusion and many others. The risk factors vary depending on the location of the carcinoma. [uwmedicine.org]

• Dyspnea

  We report a 57 year-old man who presented to us with left chest pain and progressive dyspnea and was diagnosed to have a pleural metastases of soft tissue sarcoma by thoracoscopic biopsy. [ncbi.nlm.nih.gov]

• Nausea

  Clinically relevant adverse effects in the olaratumab + doxorubicin arm included neutropenia (58%), mucositis (53%), nausea (73%), vomiting (45%), and diarrhea (34%). [ncbi.nlm.nih.gov]

  Side effects include fever, chills, loss of appetite, headaches, nausea, fatigue and body aches. Preventive measures Preventive measures include practicing safe sex to reduce the risk of acquiring HIV. [news-medical.net]

  Sometimes they also have nausea, vomiting and/or weight loss. Treatment: Desmoplastic small round cell tumors are often treated with chemotherapy, radiation, and surgery. [curesearch.org]

  She experienced hair loss, nausea, vomiting and fatigue, but the treatment was unsuccessful. Alicia tried another chemotherapy drug, but it didn’t kill the cancer either. [mdanderson.org]

• Vomiting

  Clinically relevant adverse effects in the olaratumab + doxorubicin arm included neutropenia (58%), mucositis (53%), nausea (73%), vomiting (45%), and diarrhea (34%). [ncbi.nlm.nih.gov]

  A sarcoma in the abdomen could cause abdominal pain, vomiting and constipation. A GIST may also cause bleeding into the bowel. [christie.nhs.uk]

  Possible signs and symptoms of advanced STS include, but are not limited to: blood in the stool or vomit, increasing abdominal pain and/or a new lump or a lump that is growing (anywhere on the body). [novartis.com]

  New treatments protect the heart and kidney, substantially reduce infections and the need for blood transfusions and virtually eliminate nausea and vomiting for most patients. [sarcomaoncology.com]

• Abdominal Pain

  Possible signs and symptoms of advanced STS include, but are not limited to: blood in the stool or vomit, increasing abdominal pain and/or a new lump or a lump that is growing (anywhere on the body). [novartis.com]

  Abdominal pain, constipation and a feeling of fullness may be cancer near the stomach. Coughing and breathlessness may be cancer near the lungs. You should always see your GP if any of these or similar symptoms present themselves. [rnohprivatecare.com]

  A sarcoma in the abdomen could cause abdominal pain, vomiting and constipation. A GIST may also cause bleeding into the bowel. [christie.nhs.uk]

  For example, tumors found in the following areas of the body may develop these symptoms: Arm, leg, or trunk—uncomfortable swelling in the affected limb Chest—cough and breathlessness Abdomen—abdominal pain, vomiting, and constipation Uterus—bleeding from [wkhs.com]

  For example: swelling under the skin may cause a painless lump that cannot easily be moved around and gets bigger over time swelling in the tummy (abdomen) may cause abdominal pain, a persistent feeling of fullness and constipation swelling near the lungs [nhs.uk]

• Constipation

  Abdominal pain, constipation and a feeling of fullness may be cancer near the stomach. Coughing and breathlessness may be cancer near the lungs. You should always see your GP if any of these or similar symptoms present themselves. [rnohprivatecare.com]

  A sarcoma in the abdomen could cause abdominal pain, vomiting and constipation. A GIST may also cause bleeding into the bowel. [christie.nhs.uk]

  For example, tumors found in the following areas of the body may develop these symptoms: Arm, leg, or trunk—uncomfortable swelling in the affected limb Chest—cough and breathlessness Abdomen—abdominal pain, vomiting, and constipation Uterus—bleeding from [wkhs.com]

  For example: swelling under the skin may cause a painless lump that cannot easily be moved around and gets bigger over time swelling in the tummy (abdomen) may cause abdominal pain, a persistent feeling of fullness and constipation swelling near the lungs [nhs.uk]

  For example, a tumour near the stomach may cause abdominal (tummy) pain, a feeling of fullness and constipation, whereas a tumour near the lungs may cause a cough or breathlessness. [nhsinform.scot]

• Diarrhea

  Clinically relevant adverse effects in the olaratumab + doxorubicin arm included neutropenia (58%), mucositis (53%), nausea (73%), vomiting (45%), and diarrhea (34%). [ncbi.nlm.nih.gov]

  abdominal tumors, accidents, acute diarrhea evoking cholera, and soft tissue infections. ar جوناس) ، لا يمكنك بدء حرب ضد) الناس الذي يفترض بك حمايتهم en This is supposed to be soft tissue, but it's almost as impenetrable as your skin. ar ، أشعر بأنّي [ar.glosbe.com]

  Most of these tumors develop in the stomach or small intestine and may cause nausea, diarrhea, or abdominal swelling or discomfort. [nyulangone.org]

  If radiation targets your belly or pelvis, it may cause diarrhea. If it’s aimed at your head or chest, it may hurt to swallow. As with any type of cancer, it could come back. Doctors call that “recurrent” soft tissue sarcoma. [webmd.com]

  The most common related AEs were nausea (34/48 [70.8%]), vomiting (22/48 [45.8%]), blurred vision (22/48 [45.8%]), diarrhea (20/48 [41.7%]) and fatigue (19/48 [39.6%]). [cureasps.org]

• Confusion

  Nodular fasciitis is a rare but important soft tissue lesion, which can be easily confused with sarcoma. [ncbi.nlm.nih.gov]

  Most liposarcomas develop in the deep layers of soft tissue and are often mistakenly confused with lipomas, which appear in more superficial soft tissue layers. [cancercenter.com]

  If you have cancer of unknown primary (CUP), symptoms may include swollen lymph nodes, bone, chest or stomach pain, poor appetite, fatigue, headache, confusion and many others. The risk factors vary depending on the location of the carcinoma. [uwmedicine.org]

• Radiculopathy

  Sarcomas may affect nerve structures by compression and cause peripheral neurologic signs like radiculopathies and neuritis. Joints and bursae may also be invaded causing signs of limited mobility and pain. [symptoma.com]

The following work-up diagnostic techniques and tests are done in patients suspected of having sarcomas:

• Imaging: In general, imaging studies like computed tomography (CT) and magnetic resonance imaging (MRI) can define the anatomic location, the extent of invasion, and the neighboring structures involved [6]. CT scan is superior in determining the number of pulmonary metastasis and identifying soft tissue tumors in the retroperitoneal space. MRI is able to define contiguous planes in sarcoma and the adjacent anatomic structures that are involved [7].
• Histopathology: The use of fine needle aspiration biopsy in the pathologic identification of sarcoma has been a standard in the diagnosis of soft tissue sarcomas. Histocytologic evaluation and immunocytochemical evaluation of the aberrant cells may be performed on multiple passes under electron microscopy as a confirmatory test [8].

Soft tissue sarcoma under intermediate to high grade classification will benefit significantly with chemotherapy using doxorubicin and ifosfamide by increasing the average survival rate of patients [9]. Localized sarcoma are treated with excision with or without chemotherapy and radiation therapy to prevent recurrence. The radical excision with amputation approach to sarcomas in the extremities affords the lowest recurrence rate among the other surgical approaches used [10]. Non-extremity sarcoma may not be easily accessible for surgical excision especially those tumors within the retroperitoneal space and the mediastinum. These cancers are treated with a combination of surgical resection, chemotherapy, and high dose radiation therapy to avert the high recurrence rating of this kinds of sarcoma.

Sarcomas are graded accordingly in reference to its cellular and subcellular structures found in the tumor. Low-grade tumors carry a good prognosis especially after surgical resection of the sarcoma. However, intermediate grade and high-grade sarcoma offer a grim prognosis despite the treatment combination of surgery and radiotherapy [4]. High-grade tumors are likely to metastasize with high morbidity rating compared to its low-grade counterparts. Osteosarcoma has a survival rating of up to 70% with chemotherapy and only 20% without chemotherapy.

There are compelling evidences that the occurrence of sarcoma is largely influenced by genetic factors like gene mutations. There are other genetic factors that increase the likelihood of the development of sarcoma like the tumor suppressor gene, unique cytogenetic defects, and various oncogenes. The development of fibrosarcoma among patients exposed to radiation therapy has placed radiation exposure as an etiologic factor in the carcinogenesis of sarcoma. Environmental carcinogens like arsenic, thorium, and vinyl chloride have been implicated in the prevalence of hepatic angiosarcoma. The advertent exposure to Epstein Barr virus and Herpes virus type 8 has been linked to the development of soft tissue tumors like Kaposi sarcoma among immunocompromised hosts.

The current incidence of soft tissue sarcoma worldwide reaches up to 35 cases per 1 million population. In the United State, sarcoma has an annual incidence rate of only 15,000 newly diagnosed cases per year [1]. The prevalence rate of sarcoma varies directly with the age of the patient. Soft tissue sarcomas occur twice as often as the primary bone sarcomas. Up to 45% of sarcoma occur in the lower extremities while visceral sarcoma is the most uncommon. Soft tissue sarcomas follow a distinct age distribution pattern like rhabdomyosarcoma that predominates among young children, the synovial sarcoma mostly occurs among young adults, and the malignant fibrous histiocytoma and liposarcoma abound among the elderly age group. Sarcomas have a slight predilection for males than in females.

The majority of soft tissue sarcomas are confined within fascial boundaries and grow centripetally (from lateral to central). These fascial boundaries are only violated during the later stages of cancer. The nearby neurovascular structures in sarcoma are displaced by the neoplasm to sides rather than enveloped as with other invasive cancers. The compressed fibrous region is surgically referred to as the compression zone while the thin layer surrounding this zone is called the reactive zone. These layers will guide surgeons on where to resect the tumor during an en bloc tumor removal or debulking operations.

A sarcoma tumor that has been removed with its capsule will leave behind microscopic tumor particles that usually recure in the same location in 80% of cases within 2 to 3 years from initial operation [2]. The radical use of radiation therapy after surgical resection drastically reduce the incidence of recurrence after tumor removal. High grade and intermediate grade sarcomas are likely to metastasize through the blood and seed in the lungs in the majority of cases. Lung metastasis among high-grade sarcomas occurs in 53% of cases [3].

The prudent avoidance of environmental carcinogens like arsenic, vinyl chloride, and radiation therapy may prevent some soft tissue sarcoma from occurring. The early detection and prompt intervention of sarcoma with excision surgery and adjuvant chemotherapy and radiotherapy can prevent high-grade sarcomas from metastasizing to distant organs.

Soft tissue sarcoma is a clinical term that refers to a broad group of cancers that affect soft connective tissues. Treatment approaches to sarcoma varies on the tumor type, anatomic location, and other patient factors. Sarcoma is conveniently divided into two types based on cell type and includes liposarcoma, leiomyosarcoma, and rhabdomyosarcoma.

Definition

Soft tissue sarcoma is a group of rare cancers that affect the soft tissues that support, surround, and connect organs.

Cause

Genetic mutation is implicated in the development of most sarcomas. Exposure to environmental toxins like vinyl chloride, thorium dioxide, and arsenic are among the popular etiologic agents of the neoplasm. Post-radiation exposure sarcoma has been documented in some patients.

Symptoms

The presence of a non-tender mass tissue in the torso or the extremities. Compression signs are common on the neurovascular structures adjacent to the primary tumor growth.

Diagnosis

A complete physical examination is done to diagnose sarcoma. Imaging studies and histopathologic studies (biopsy) are known confirmatory tests for the diagnosis.

Treatment and follow-up

Surgical excision, adjuvant chemotherapy, and radiotherapy are the therapeutic modalities available for the treatment of sarcoma.

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