Fibrosarcoma- An Overview

What Are Fibrosarcomas?

Fibrosarcomas are malignant neoplasms made out of fibroblasts with varied quantities of collagen synthesis and a herringbone architecture. Adult fibrosarcomas have been on the decline for several decades as the categorization of fibrosarcoma continues to shrink, and mesenchymal and non-mesenchymal tumors mimic fibrosarcomas.

How Are Fibrosarcomas Classified?

Fibrosarcomas are classified as follows:

• Infantile fibrosarcomas.

• Congenital fibrosarcomas.

• Adult-type fibrosarcomas.

Infantile fibrosarcomas seldom spread, although adult-type fibrosarcomas are extremely dangerous.

What Is the Etiology of Fibrosarcoma?

• Fibrosarcomas often arise from soft tissue fascia and tendons, but they can also develop in bones as a primary or secondary tumor inside the medullary canal or the periosteum.

• In addition, prior bone injury, whether from trauma or radiation, can also result in bone fibrosarcomas.

• Fibrosarcomas are mesenchymal cell tumors made up of spindle-cell fibroblasts that proliferate uncontrollably.

• Fibrosarcomas are most commonly found in tendons and fascias of deep tissues, although they can also arise in the medullary canal or the periosteum of bones.

• Secondary fibrosarcomas of the bone can develop from pre-existing bone lesions or irradiated tissues.

What Is the Epidemiology of Fibrosarcoma?

• Adult fibrosarcoma is more common in middle-aged and older individuals.

• Males have a slight majority, which most usually affects the deep soft tissues of the limbs, trunk, head, and neck.

• A review of adult fibrosarcomas found that 80 percent were ‘high-grade’ (grade 2 or 3), with 25 percent of the remaining low-grade lesions developing into high-grade sarcoma locally over time.

What Are the Symptoms of Fibrosarcoma?

• A thorough neurovascular examination should accompany the regional lymph node examination.

• As fibrosarcomas often develop in deep connective tissue rich in collagen, they are less prevalent in the retroperitoneum, mediastinum, head, and neck.

• Fibrosarcomas are usually spherical, with solid differentiation from surrounding tissue and a hard firmness when palpated.

• Fibrosarcoma tumors range in size from one to three inches.

• Fibrous tumors originate from deep tissue and cause painless soft-tissue swelling, so they are commonly misdiagnosed.

• As a result, the lesion is typically not detected and recognized until signs of a deep tumor's local mass effect appear.

• When a fibrosarcoma is discovered, it is typical to notice reduced blood circulation, nerve compressions, or movement constraints.

• Late fibrosarcoma presentations may include weight loss and anorexia.

• Furthermore, pain from a deep soft tissue mass bigger than five centimeters in size elevates the probability of malignancy and demands referral for extra specialist screening.

How Is Fibrosarcoma Diagnosed?

Contrast-Enhanced MRI (Magnetic Resonance Imaging): It is recommended for evaluating extremities or pelvic cancers. It will offer information on the tumor's size, margins, signal intensity, degree of necrosis, and vascularization.

Computed Tomography (CT) Scan: Can also look for retroperitoneum cancers. Fibrosarcoma manifests as isolated, nonspecific ovoid tumors with somewhat uneven borders.

Fine Needle Aspiration (FNA):

If imaging indicates the existence of soft tissue cancer, a fine needle biopsy is recommended for an accurate first diagnosis. However, if past cytological investigations are available for comparison, fine needle aspiration can be used to monitor progression and identify tumor recurrence or metastasis. Finally, more invasive surgical biopsies may be required in cases where minimally invasive biopsy procedures have failed or are impractical. An excisional biopsy is often indicated when a soft tissue sarcoma of three to five centimeters in size is suspected. However, partial incisional biopsies are suitable when the tumor size surpasses five centimeters.

What Is the Treatment of Fibrosarcoma?

Surgery -

The cornerstone of therapy for localized soft tissue sarcomas is surgical excision. Intramuscular cancers should be excised in a compartmental en-bloc type, with no extra radiation therapy required. If there is extra-compartmental development, a wide-surgical resection to attain tumor-free margins would be suitable. Two millimeters of the buffer is commonly advised. Surrounding structures, including nerves and circulatory systems, must be considered.

Radiation -

Adjuvant radiation therapy is highly recommended for cancers of high grade and greater than five centimeters in size. If clear margins cannot be acquired, reoperation is the best next step in management.

Chemotherapy-

Chemotherapy's efficacy in managing soft tissue sarcomas is unknown, and it is thus not routinely suggested in conventional treatment. Furthermore, fibrosarcomas develop co-resistance to medicines such as Vincristine, Vinblastine, and Etoposide after the treatment of the first-line medication, Doxorubicin. As a result, only individuals with advanced fibrosarcomas who require chemotherapy should be treated with anthracyclines as first-line therapy. The combination of Actinomycin D and Ifosfamide may improve therapy response. However, only 4 to 11 percent of sarcoma patients treated with conventional chemotherapy show an improvement in survival.

Intra-Tumoral Injection-

The intra-tumoral injections of matrix metalloproteinase inhibitors such as TIMP-1-GPI fusion protein have reduced tumor bulk and growth. Furthermore, injection inhibits cell migration while increasing tumor cell death.

What Is the Prognosis of Fibrosarcoma?

• 80 percent of adult fibrosarcomas are high-grade (Grades 2 or 3).

• Furthermore, 25 percent of the remaining low-grade lesions develop into a high-grade sarcoma recurrence.

• These tumors are aggressive, with several local recurrences and lymphatic and parenchymal metastases.

• Adult fibrosarcomas have a two-year survival rate of 70 percent and a five-year survival rate of 55 percent.

What Are the Complications of Fibrosarcoma?

• Surgery is the primary treatment for fibrosarcoma; complications include infection, bleeding, and injury to adjacent tissues or structures.

• Adjuvant radiation treatment can raise the risk of local fibrosis and wound infection. Therefore, chemotherapy may be utilized in advanced fibrosarcomas.

• Each chemotherapy medication has its unique risk profile, although the cornerstone of treatment, Doxorubicin, is often linked with an elevated risk of dilated cardiomyopathy.

Conclusion:

Fibrosarcoma is a rare kind of cancer that attacks fibroblast cells. Fibroblasts are the cells that produce the fibrous tissue present throughout the body. Tendons are fibrous tissues that link muscles to bones. When fibrosarcoma occurs, the body's fibroblasts lose control and proliferate uncontrollably. This either result in forming fibrous tissue where it should not exist or in excessive amounts. As a result, fibrosarcoma, like other tumors, can spread throughout the body.