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

  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]

  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]

  A sarcoma affecting the womb may cause bleeding from the vagina and pain in the lower part of the abdomen. [christie.nhs.uk]

• Swelling

  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]

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

  If the sarcoma is in an arm or a leg, the most common symptom is an uncomfortable swelling in the affected limb. Occasionally, this swelling may be painful or tender, but it may also be painless. [christie.nhs.uk]

  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]

• Fever

  […] and Symptoms of 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 [curesearch.org]

  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]

  Some kids may need to return to the hospital between treatments if they have side effects, like fever or infection, or if they need a blood transfusion. After chemotherapy, the doctor's goal is to continue to kill tumor cells around the tumor. [kidshealth.org]

  If your child has Ewing sarcoma, the following symptoms may be present: Swelling and soreness around the tumor area (commonly mistaken for a sports injury or the “bumps and bruises” every child gets) A low fever that at first may seem to be caused by [stjude.org]

  Other symptoms may include fatigue, fever, weight loss and anemia. Additionally, the presence of a painless lump which could eventually become sore or painful is something that an oncologist should check. [sarcomaoncology.com]

• 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]

  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]

  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]

  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]

• Anemia

  The results of laboratory tests were significant for a moderate anemia (7.0 g/dL) and a profound thrombocytopenia (3x10/L). The preoperative radiologic findings were suspicious for a soft tissue sarcoma with scapular erosion. [ncbi.nlm.nih.gov]

  Complications after surgery can include: infection slow healing of the wound Chemotherapy-related side effects can include: short-term problems: anemia (low red blood count); bleeding easily; risk of infection; risk of kidney, liver, or heart damage long-term [kidshealth.org]

  Common side effects include: loss of appetite, loss or hair, weakness, anemia, propensity to get infections, nausea, bleeding tendencies, sore mouth ulcers etc. There is another systemic therapy called liposomal chemotherapy. [news-medical.net]

  Other symptoms may include fatigue, fever, weight loss and anemia. Additionally, the presence of a painless lump which could eventually become sore or painful is something that an oncologist should check. [sarcomaoncology.com]

  For example, gastrointestinal stromal tumors (GISTs) present with GI symptoms that are usually indistinguishable from those of the more common adenocarcinomas, such as anemia, melena, abdominal pain, or weight loss. [cancernetwork.com]

• 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]

• 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]

• Constipation

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

  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]

  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]

• 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]

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

  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]

  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]

• Abdominal Mass

  Most patients (80%) present with an abdominal mass, with 50% of patients reporting pain at presentation. [cancernetwork.com]

• 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].

• Decreased Platelet Count

  The most frequent grade 3 or worse adverse events were anaemia (six [14%]), decreased lymphocyte count (five [12%]), prolonged activated partial thromboplastin time (four [10%]), and decreased platelet count (three [7%]) in the bone sarcoma group, and [ncbi.nlm.nih.gov]

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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