Fibrosarcomas of the bone may present with pain and swelling of the affected area and sizable lesions, that are greater than 2 cm involving at least 50% of the bone cortex, may compromise the bones physical integrity leading to fractures. Fibrosarcomas of the femur with lesions affecting the medial calcar also have a high chance of fracturing. Secondary fibrosarcoma may be suspected if patients have a previous bone infarct or have received irradiation in the suspected diseased area.

Fibrosarcoma of the soft tissues are characterized by a painless mass that are relatively large at diagnosis due to their likely origin deep to the muscular fascia.

The majority of fibrosarcomas are located in the proximal arm or femur, around the knee or in the hip. Patients may present with a firm stationary mass or area of tenderness. At the later stages, after invasion and metastasis, vascular and neurologic changes may be observed. Diseases that display similar symptoms include osteosarcoma, Paget sarcoma, fibrous histiocytoma or dysplasia and neurosarcoma.

• Pain

  Here we report a case of primary fibrosarcoma of the kidney in a 70-year-old man who presented with gradually increasing abdominal swelling and pain. [ncbi.nlm.nih.gov]

  In the lungs, symptoms can include pain and trouble breathing. In the abdomen, they may come in the form of pain, nausea, and vomiting. In the limbs, symptoms may include pain, swelling, tenderness, and visible lumps. [study.com]

• Swelling

  This diagnosis should be kept as a possibility in all congenital cervical swellings. These are commonly misdiagnosed as lympho-venous malformations and histopathology is confirmatory. [ncbi.nlm.nih.gov]

  Skin over the swelling was stretched but was normal in color. The swelling was firm and fixed to underlying structures. [jomfp.in]

  The swelling was irregular in shape and the borders were ill defined. Skin over the swelling was stretched, but was normal in color. The swelling was firm and fixed to the underlying structures. [ijdr.in]

• Pathologist

  The case highlights the diagnostic challenges at clinical, radiologic, and pathologic levels in some cases of infantile fibrosarcoma and raises awareness among clinicians and pathologists related to another peculiar pattern that can be encountered in [ncbi.nlm.nih.gov]

  Pathologists have become more cautious in the making the diagnosis of fibrosarcoma and over the years there has been marked decline in this diagnosis. [histopathology-india.net]

  A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a diagnosis. [dovemed.com]

• Weight Loss

  The second case was a 49-year-old male patient who complained of cough, weight loss, excessive sweating, and fatigue. [ncbi.nlm.nih.gov]

  The constitutional symptoms of weight loss, malaise, anorexia, and anemia were legion in this group of patients.* We have recently seen a primary fibrosarcoma of the kidney in a 69-year-old woman and were stimulated to review the literature and to consider [jamanetwork.com]

  Most of the individuals with this type of cancer do not exhibit the common cancer symptoms such as fatigue or weight loss. Diagnosis Primarily, an x-ray can illustrate any problem with the bone. [mybiosource.com]

  Fatigue Unintentional weight loss Fibrosarcoma Causes In general, any cancer is the result of cells dividing and growing uncontrollably. [buoyhealth.com]

  […] immunity, sores on the mouth, nausea and vomiting and weight loss. [epainassist.com]

• Surgical Procedure

  The patient underwent surgical resection and subsequent radiotherapy. FNC diagnosis of FS is reliable and accurate and may be conveniently used in the scheduling of surgical procedures, when needed, avoiding the treatment of benign nodules. [ncbi.nlm.nih.gov]

  Through surgical procedure the mass was removed and submitted to the pathology lab of the Universidad de Nariño, with dimensions of 6x9x3cm, skin and hair, regular surface, a whitish color and hard consistency; for processing and routine dying with hematoxylin-eosin [scielo.org.co]

  […] relief from the symptoms, and reduce blood loss during a surgical procedure Radiation therapy and/or chemotherapy may be used to destroy the tumor cells, based on the assessment by the healthcare provider Post-operative care is important; a minimum activity [dovemed.com]

• Abdominal Mass

  A 14-year-old teenage girl was admitted because of a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass. Salpingo-oophorectomy along with staging procedures were performed. [ncbi.nlm.nih.gov]

  […] enlargement, awareness of abdominal mass (Cancer 1981;47:2663) Laboratory CA125 and CEA in normal range Treatment Surgical resection with or without adjuvant chemotherapy or radiation Gross images Images hosted on other servers: Capsular disruption and [pathologyoutlines.com]

  The patient recovered very well after the operation; however, four months later he presented with abdominal masses. At laparotomy, large retrohepatic masses were noted and these were considered to be unresectable. [heart.bmj.com]

  Three cases were admitted to the hospital for hematuria, two cases for prenatal diagnosis by ultrasound (US), one case for progressive abdominal distension and two cases for an abdominal mass. [doi.org]

• Loss of Appetite

  Patients may have to deal with nausea, vomiting, loss of appetite ( anorexia ), and hair loss ( alopecia ). Many times, chemotherapy as well as radiation therapy are better handled if patients are eating well. [encyclopedia.com]

  There was associated history of difficulty in speech and mastication, loss of appetite with weight loss and fever. [ijdr.in]

• Aggressive Behavior

  Despite the apparent reliability of mitotic counts in differentiating fibrosarcomas from cellular fibrothecomas, a few examples exist in which cellular fibrothecomatous lesions demonstrating low mitotic activity show an unexpected aggressive behavior. [archivesofpathology.org]

• Hematuria

  Hematuria was, unfortunately, a late and inconstant finding. [jamanetwork.com]

  We describe a case of primary renal fibrosarcoma in a 54-year-old male who presented with abdominal lump and intermittent hematuria. [ijpmonline.org]

  Three cases were admitted to the hospital for hematuria, two cases for prenatal diagnosis by ultrasound (US), one case for progressive abdominal distension and two cases for an abdominal mass. [doi.org]

• Confusion

  The presence of a surrounding benign tumor confused and delayed CIFS diagnosis. [ncbi.nlm.nih.gov]

  It is a distinct entity from but similar to malignant fibrous histiocytoma 2 (pleomorphic undifferentiated sarcoma) that may also rarely occur in bone, causing confusion in terminology, as the term "fibrosarcoma" has in the past encompassed a multitude [radiopaedia.org]

  Pediatric Patient Considerations: Hemangioma – May be confused with a congenital infantile fibrosarcoma because of the possible presence of superficial ectatic veins in fibrosarcomas. [visualdx.com]

  A 2014 study warns against confusing it with osteosarcoma, which has similar features. As a result, doctors may carry out a variety of tests, including a biopsy, to ensure a correct diagnosis. [medicalnewstoday.com]

Doctors should initially perform general exams that include patient’s medical history, detailed physical exam, blood test (to identify malignant markers and rule out other diseases) and x rays. X rays can identify the size, shape and location of tumors and be used to define tumor margins. Fibrosarcomas will appear highly destructive on x rays and display a wide transition zone. If fibrosarcoma induces new bone formation, which is rare, the newly formed bone will appear speculated, laminated or Codman triangle. More detailed exams including magnetic resonance imaging (MRI), computed tomography (CT) scans, biopsies and angiograms will be performed if tumors are detected during initial exams. The intraosseous spread and level of soft tissue spread can be determined through MRI. Regardless of the imaging modality used, fibrosarcomas display a diverse non-specific pattern.

• Hypoglycemia

  THE syndrome of hypoglycemia secondary to a nonpancreatic tumor has been reported on several occasions. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 We recently studied a patient with frequent and protracted episodes of hypoglycemia that ceased when a large intrathoracic [nejm.org]

A combination of local and systemic treatments are generally used in fibrosarcoma. Surgical resection with wide margins followed by defect repair will provide adequate local control. Studies have demonstrated that wide margins or radical surgeries greatly reduce recurrence rates. Adjuvant therapy is often used to decrease the chances of metastasis. Radiation is commonly used after surgery to treat fibrosarcomas of the soft tissue while chemotherapy is given on a case by case basis. In general chemotherapy is not used as widely as radiation, however, it is common to administer chemotherapy to patients with fibrosarcoma of the bone. In cases of infantile fibrosarcoma outcomes are generally good with surgery regardless of the grade or stage of the tumor at diagnosis [10]. In one study patients treated for infantile fibrosarcoma had a greater than 90% survival rate regardless of grade, stage or treatment modality [10].

The primary determinant of the outcome of fibrosarcoma patients is the grade of malignancy at diagnosis. High grade fibrosarcomas have a survival rate of 30% after 5 years [4] [5] [6] [7] [8] [9], compared to a 50-80% survival rate in low grade fibrosarcomas [5] [7]. Age is another factor linked to poor survival, with patients over the age of 40 displaying worse outcomes than younger patients [6] [8]. Additionally, a worse prognosis is observed in patients with high disease stage at diagnosis, tumors located in the axial skeleton or eccentric permeative lesions [6] [8].

Recurrence is dramatically decreased with radical surgeries and surgeries that maintain wide margins, as evidenced by the 100% and 93% disease free rate after 5 years, respectively [6]. Fibrosarcoma is controlled equally well by both ablative surgery and limb salvaging radical surgery.

The cause of fibrosarcoma is unknown. The types of mutations associated with fibrosarcoma are most commonly point mutations, allele deletions and translocations.

Fibrosarcoma is primarily diagnosed in patients in the forth decade of life (40-50 years of age) although occurrences are documented at any age. Soft tissue fibrosarcomas are more commonly observed outside of the 40-50 age range than fibrosarcomas affecting the bone. This malignancy accounts for less than 5% of primary bone cancers and around 10% of sarcomas affecting the musculoskeletal system. Fibrosarcomas of the bone and soft tissue are most commonly observed in the lower extremities, particularly the femur and tibia, and thigh and knee, respectively. Patients with fibrosarcomas of the soft tissue usually have large, deep and painless masses with not well defined margins. Although rare, an infantile form of fibrosarcoma exists which affects children aged 10 or younger. Fortunately, the infantile form of fibrosarcoma has a good prognosis, with appropriate resection and adjuvant and neoadjuvant chemotherapy, regardless of the grade or stage of the tumor [1] [2] [3]. Men have a slightly higher chance than women of developing fibrosarcoma of the bone and no links to race have been identified.

The primary determinant of the outcome of fibrosarcoma patients is the grade of malignancy at diagnosis. High grade fibrosarcomas have a survival rate of 30% after 5 years [4] [5] [6] [7] [8] [9], compared to a 50-80% survival rate in low grade fibrosarcomas [5] [7]. Age is another factor linked to poor survival, with patients over the age of 40 displaying worse outcomes than younger patients [6] [8]. Additionally, a worse prognosis is also observed in patients with high disease stage at diagnosis, tumors located in the axial skeleton or eccentric permeative lesions [6] [8].
Recurrence is dramatically decreased with radical surgeries and surgeries that maintain wide margins, as evidenced by the 100% and 93% disease free rate after 5 years, respectively [6]. Fibrosarcoma is controlled equally well by both ablative surgery and limb salvaging radical surgery.

Carcinogens, such as benzene, carbon tetrachloride and alpha-naphthylamine, should be avoided to reduce the risk of cancer. Sources of penetrating radiation, including radioactive materials and x rays, are also associated with a risk of developing cancer, however, in most cases medical x rays have higher benefits than risks.

Fibrosarcoma is a rare malignancy that arises from mutated fibroblasts that reside in fibrous connective tissue. This malignancy occurs most frequently in femur, humerus or mandible of middle aged individuals. Fibrosarcoma may also be found in organs and soft tissue as well and is identified upon palpation even before pain is observed. Surgical resection is often difficult since invasion of the surrounding tissues is often observed. Recurrence and metastasis of fibrosarcoma are common and is highly dependent on the grade and stage at diagnosis. Patients that present normal cell appearance and little spreading (low grade and stage) generally have a more favorable prognosis.

Malignant fibroblasts are immature and display an interlacing pattern. These fibroblasts are highly proliferative and produce an abundance of extracellular matrix proteins, especially collagens, giving fibrosarcomas high collagen content. Fibrosarcomas are most commonly found in the fibrous connective tissue of the trunk, arms and legs, however, these malignant fibroblasts may also arise in other connective tissues, joints, soft tissues (e.g. muscle), blood vessels, fat and bone. Fibrosarcomas that arise in the bone are rare (0.2% of new cases) and usually located in the bone marrow cavities of long bones, most commonly the femur, humerus, hip and pelvis.

Fibrosarcoma is most commonly diagnosed in patients between the ages of 25 and 79 years of age. The cause of fibrosarcoma is unknown, however, a hereditary component may exist based on the observations that certain families have increased incidences and fibrosarcoma risk is higher in individuals with certain other types of sarcomas.

Diagnosis of fibrosarcoma typically involves a detailed physical exam, blood tests and numerous imaging procedures including x rays, magnetic resonance imaging (MRI) and computed tomography (CT). Treatment for fibrosarcoma usually involves a combination of surgery and adjuvant therapies (radiation and/or chemotherapy). Infants typically have a good prognosis with only surgery. Patient outcomes depend on tumor size and spread along with patients’ age and responsiveness to therapy.

The cells responsible for causing fibrosarcoma are fibroblasts which produce a large amount of extracellular matrix proteins that are the major components of connective tissues. Fibrosarcomas most commonly arise in the bone or soft tissues (eg. muscle, fat, blood vessels and joints). Fibrosarcoma of the bone generally occurs in the bones of the arms, legs, pelvis and hips while soft tissue fibrosarcomas usually arise in arms, legs or trunk. Fibrosarcomas are rare and most commonly affect individuals between 25 and 79 years of age with the most cases occurring in individuals between 55 and 69 years of age. Women and men have an equal chance of developing fibrosarcoma and children are less likely to develop this malignancy. Infantile fibrosarcoma (also known as congenital fibrosarcoma) is rare and children affected with this disease have a much better prognosis than adults with around a 90% rate of survival after 5 years.

The exact cause of fibrosarcoma is unknown, however, some evidence indicates a hereditary component. For example, some families have been shown to have increased incidences compared to the normal population and fibrosarcoma incidence is higher in individuals with certain other types of sarcomas. Patients with fibrosarcoma may observes a mass in the truck, arms or legs that is painless or painful if the mass is affecting a muscle or nerve. Symptoms vary widely and are determined primarily by the location, size and extent of tumor spread. Partial paralysis of the affected region may occur resulting in a limp or the inability to use the affected appendage. Along with a detailed physical exam and blood tests physicians will perform numerous imaging procedures. X rays will likely be performed first and will assist in determining the location and size of tumors. CT scans and MRIs will provide a more detailed description of the tissues involved and the spread of a fibrosarcoma. Tumor biopsies are often taken to analyze the appearance of cells within the tumor (grade).

Treatment for fibrosarcoma often includes a combination of local and systemic therapies to prevent metastasis but the type of treatment depends on a number of factors. Infants typically have a good prognosis with only surgery and radiation is generally avoided in infants due to its negative effects on bone growth. Surgery along with radiation and/or chemotherapy is a likely course for older children and adults. Surgical procedures to treat fibrosarcoma may involve collection of tumor biopsies and resection, grafting, limb salvaging and reconstruction procedures (if possible) and potentially amputation. Other surgeries may be used depending on each individual tumors characteristics. Patient outcomes vary dramatically depending on a number of factors, such as tumor size and spread along with patients age and responsiveness to therapy.

1. Russell H, Hicks MJ, Bertuch AA, et al. Infantile fibrosarcoma: clinical and histologic responses to cytotoxic chemotherapy. Pediatr Blood Cancer. 2009; 53(1):23-7.
2. Canale S, Vanel D, Couanet D, et al. Infantile fibrosarcoma: magnetic resonance imaging findings in six cases. Eur J Radiol. 2009; 72(1):30-7.
3. DeComas AM, Heinrich SD, Craver R. Infantile fibrosarcoma successfully treated with chemotherapy, with occurrence of calcifying aponeurotic fibroma and pleomorphic/spindled celled lipoma at the site 12 years later. J Pediatr Hematol Oncol. 2009; 31(6):448-52.
4. Taconis, WK, van Rijssel TG. Fibrosarcoma of long bones. A study of the significance of areas of malignant fibrous histiocytoma. J Bone Joint Surg Br. 1985; 67(1): p. 111-6.
5. Eyre-Brook AL, Price CH. Fibrosarcoma of bone. Review of fifty consecutive cases from the Bristol Bone Tumour Registry. J Bone Joint Surg Br. 1969; 51(1): p. 20-37.
6. Papagelopoulos PJ, Galanis E, Frassica FJ, et al. Primary fibrosarcoma of bone. Outcome after primary surgical treatment. Clin Orthop Relat Res. 2000; (373):88-103.
7. Dahlin DC, Ivins JC. Fibrosarcoma of bone. A study of 114 cases. Cancer. 1969; 23(1):35-41.
8. Taconis WK, Mulder JD. Fibrosarcoma and malignant fibrous histiocytoma of long bones: radiographic features and grading. Skeletal Radiol. 1984; 11(4): p. 237-45.
9. Cunningham MP, Arlen M. Medullary fibrosarcoma of bone. Cancer. 1968; 21(1): p. 31-7.
10. Sulkowski JP, Raval MV, Browne M. Margin status and multimodal therapy in infantile fibrosarcoma. Pediatr Surg Int. 2013; 29(8):771-6.