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Fibrosarcoma

Fibrosarcomas

Fibrosarcoma is cancer of the fibrous connective tissue characterized by dividing undifferentiated mesenchymal cells (fibroblasts).


Presentation

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.

Fatigue
  • The second case was a 49-year-old male patient who complained of cough, weight loss, excessive sweating, and fatigue.[ncbi.nlm.nih.gov]
  • Fatigue Unintentional weight loss Fibrosarcoma Causes In general, any cancer is the result of cells dividing and growing uncontrollably.[buoyhealth.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]
  • Radiotherapy also has its own complications like other cancers, retarded growth of children bones, infertility, fatigue, weight loss, rashes, nausea and vomiting.[epainassist.com]
  • However, these tumors may also present clinically with chest pain, hemoptysis, cough, fever, chills, and fatigue as in the case under study. [5] In children where it presents at any age or as primary malignant congenital tumors, similar symptoms may be[anmjournal.com]
Camping
  • We report a case of intraabdominal sclerosing epithelioid fibrosarcoma (SEF) with a t (11;22)(p11.2;q12.2) Ewing sarcoma breakpoint region 1-cAMP-responsive element-binding protein 3-like 1 translocation.[ncbi.nlm.nih.gov]
  • Genomic profiling by array CGH, followed by RT-PCR and sequencing analysis, revealed a previously not reported EWSR1 translocation partner, cAMP-responsive element-binding protein 3-like 3 (CREB3L3).[ncbi.nlm.nih.gov]
  • Lars Steinstraesser Institute of Cell Biology (Tumor Research), IFZ, University of Essen, Virchowstr. 173, 45122, Essen, Germany Ludger Klein-Hitpass Institute of Pathology, BG University Hospital Bergmannsheil, Ruhr University Bochum, Bürkle de la Camp[bmccancer.biomedcentral.com]
  • CREM (cAMP-responsive element modulator) belongs to a large family of basic leucine zipper (bZIP)-containing proteins, including also CREB3L1 and CREB3L2.[clincancerres.aacrjournals.org]
Axillary Mass
  • The patient underwent resection of the axillary mass. Microscopic examination of the resection specimen showed numerous vascular channels.[ncbi.nlm.nih.gov]
Exertional Dyspnea
  • We report a woman who presented at 20 1 weeks of gestation with a 1-month history of swollen legs, exertional dyspnea, and cough with frothy sputum. Dual-source computed tomography and transthoracic echocardiography showed a left atrial mass.[ncbi.nlm.nih.gov]
Peritoneal Disease
  • Imaging studies revealed a large mesenteric-based mass with extensive omental/peritoneal disease. After resection and cytoreductive surgery, the tumor recurred with metastasis to the lungs; the patient is still alive with disease.[ncbi.nlm.nih.gov]
Intestinal Perforation
  • The four patients shared a stereotyped clinico-pathological presentation with early and acute onset, intestinal perforation, and an infiltration by a highly cellular spindle cell tumor within the dilated intestinal wall exhibiting pathologic features[ncbi.nlm.nih.gov]
Neck Pain
  • We report a case of SEF occurring in the cervicothoracic spine of a 64-year-old man with complaints of neck pain and right hand tingling for 6 months before clinical presentation.[ncbi.nlm.nih.gov]
Scalp Mass
  • Brohi SR, Dilber M (2012) Congenital osteolytic fibrosarcoma of dura presenting as scalp mass. J Coll Physicians Surg Pak 22:531–532 PubMed Google Scholar 6. Goldblum JR, Folpe AL, Weiss SW (2013) Congenital and infantile fibrosarcoma.[doi.org]
Meningism
  • A MEDLINE search was carried out using MESH terms like intracranial fibrosarcoma, intraspinal fibrosarcoma, fibrosarcoma meninges and fibrosarcoma brain. A total of 22 case reports and series reporting a total of 33 cases were identified.[ncbi.nlm.nih.gov]
Adnexal Mass
  • Although ovarian fibrosarcomas are unusual causes of solid masses in postmenopausal women, they should be considered when adnexal masses are examined in these patients.[ncbi.nlm.nih.gov]

Workup

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.

Air Bronchogram
  • A 7 8 cm, irregular limited soft tissue density in the right middle lobe of the lung that showed air bronchogram and cystic regions was detected on thorax CT. Bronchoscopy was performed.[ncbi.nlm.nih.gov]
Erythroblast
  • Five of the 16 had abnormal fusion between erythroblast transformation specific translocation variant 6 and neurotrophin recptor gene neurotrophic tyrosine kinase, receptor, type 3 (ETV6-NTRK3); in another five out of 16 this was absent, and six were[ncbi.nlm.nih.gov]

Treatment

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].

Prognosis

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.

Etiology

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

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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.

Summary

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.

Patient Information

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.

References

Article

  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.

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Last updated: 2019-07-11 22:19