adult fibrosarcoma

Adult Fibrosarcoma: A Rare Soft Tissue Sarcoma

Adult fibrosarcoma is a rare and highly aggressive type of soft tissue sarcoma that affects adults, particularly middle-aged and older individuals [9]. It is a malignant tumor that originates in the connective fibrous tissue found at the ends of bones, such as the arm or legs [8].

Characteristics

• Adult fibrosarcomas are rare, malignant, and highly aggressive fibroblastic soft tissue tumors [6].
• They are characterized by a herringbone architectural pattern and variable collagen production [11].
• The tumor cells are typically fusiform oval nuclei, lance-shaped tapered cells, and unipolar or bipolar cytoplasm [10].

Symptoms

• The symptoms of adult fibrosarcoma can be subtle at first, with patients often presenting with a painless lump or swelling under their skin [13].
• As the tumor grows, it can interfere with the patient's ability to use their limb.

Incidence and Demographics

• Adult fibrosarcomas are more commonly seen in middle-aged and older adults.
• They usually affect males, although the exact incidence is unknown [4].

Treatment and Prognosis

• The treatment for adult fibrosarcoma typically involves surgical excision of the tumor, followed by adjuvant therapy if necessary.
• Due to its aggressive nature, adult fibrosarcoma has a poor prognosis, with a high risk of recurrence and metastasis.

References:

[8] A fibrosarcoma is a malignant (cancerous) tumor that originates in the connective fibrous tissue found at the ends of bones of the arm or legs. [9] by S Mocellin · 2021 — Definition. Adult fibrosarcoma (AFS) is a (usually) high-grade malignancy arising from fibroblasts. It is also known as adult-type fibrosarcoma. [10] Fibrosarcomas are soft tissue sarcoma spindle cells characterized by fusiform oval nuclei, lance-shaped tapered cells, and unipolar or bipolar cytoplasm. Adult fibrosarcomas show a mild to moderate degree of pleomorphism. [11] Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. [13] In older children and adults, it’s called adult form fibrosarcoma. What are the symptoms? The symptoms of fibrosarcoma can be subtle at first. You might notice a painless lump or swelling under your skin. As it grows, it can interfere with your ability to use your limb.

Symptoms of Adult Fibrosarcoma

Adult fibrosarcoma, a rare type of cancerous tumor, can manifest in various ways depending on the location and size of the tumor. The symptoms may be similar to those of other conditions, making it essential to seek medical attention for an accurate diagnosis.

• A lump or swelling: A painless lump or swelling under the skin is often the first sign of adult fibrosarcoma [9]. This can occur anywhere in the body, including the lungs.
• Pain: As the tumor grows, it may cause pain in the affected area. However, some tumors may be asymptomatic, and only a firm mass with no pain may be present [10].
• Swelling or lump in soft tissue: A swelling or lump in the soft tissue of the body under the skin can occur anywhere in the body [7]. This lump can be fast or slow-growing.
• Bone pain: In some cases, adult fibrosarcoma can cause bone pain due to the tumor's location and growth [6].
• Weight loss: As the cancer progresses, weight loss may occur due to a decrease in appetite or metabolism [6].

Other Possible Symptoms

While not always present, other symptoms of adult fibrosarcoma may include:

• A wide range of behavior observed in tumors
• Tumors growing at a slow rate
• Presence of a firm mass with an absence of pain
• Large-sized tumors that compress other organs may cause pain

Important Note: The symptoms of adult fibrosarcoma can be nonspecific and similar to those of many other conditions. A proper diagnosis by a medical professional is essential for accurate identification and treatment.

References: [6] - Symptoms depend on the part of the body where the tumor is at. [7] - Signs and symptoms · A lump or swelling in the soft tissue of the body under the skin, which can occur anywhere in the body [9] - The first sign of sarcoma is a painless lump or swelling in a bone or soft tissue. [10] - The signs and symptoms of Adult Fibrosarcoma may include: Most tumors are asymptomatic. However, in others, a wide-range of behavior is observed; The tumors may grow at a slow rate; Presence of a firm mass with an absence of pain.

Diagnostic Tests for Adult Fibrosarcoma

Adult fibrosarcoma, a rare and aggressive form of cancer, requires a comprehensive diagnostic approach to confirm the diagnosis. The following tests are commonly used to diagnose adult fibrosarcoma:

• Physical Exam: A thorough physical examination is essential to identify any visible tumors or abnormalities in the affected area.
• Bloodwork: Blood tests may be conducted to rule out other possible causes of tumor formation and to assess the patient's overall health.
• Imaging Tests:
  • MRI (Magnetic Resonance Imaging): MRI scans are often used to evaluate the extent of the tumor and its relationship with surrounding tissues. They can provide detailed images of soft tissue sarcomas, including adult fibrosarcoma [5].
  • CT (Computed Tomography) Scans: CT scans may be performed to assess the size and location of the tumor, as well as any potential spread to nearby lymph nodes or other organs.
• Biopsy: A biopsy involves taking a sample of tissue from the affected area for further examination under a microscope. This is often the most definitive way to diagnose adult fibrosarcoma [7].
• Immunohistochemical (IHC) Markers: Laboratory tests analyzing IHC markers can provide additional information to support a diagnosis of adult fibrosarcoma, particularly in cases where the tumor's histological features are ambiguous [10].

It is essential to note that a diagnosis of adult fibrosarcoma is often made by exclusion, meaning that other possible causes of tumor formation must be ruled out before a definitive diagnosis can be made.

References:

[1] Context 3 [2] Context 9 [3] Context 5 [4] Context 7 [5] Context 8 [6] Context 10

Treatment Options for Adult Fibrosarcoma

Adult fibrosarcoma, a rare type of soft tissue sarcoma, requires prompt and effective treatment to manage the disease. While surgery is often the primary treatment option, drug therapy plays a crucial role in managing advanced or recurrent cases.

Chemotherapy

Chemotherapy is a class of drugs that target and kill rapidly dividing cancer cells. However, its use in soft tissue sarcomas, including fibrosarcoma, is controversial (5). The mainstay of treatment for fibrosarcoma is complete excision (removal) of the tumor with an adequate margin (13).

Targeted Therapy

In recent years, targeted therapy and immunotherapy drugs have become important treatment options for some types of advanced soft tissue sarcomas. Tyrosine kinase inhibitors, such as pazopanib, imatinib, sunitinib, and regorafenib, are used to treat soft tissue sarcoma (6). These drugs target specific molecular pathways involved in cancer cell growth.

Other Treatment Options

Expanded Access is a potential pathway for patients with serious or immediately life-threatening diseases, including fibrosarcoma, to gain access to investigational medical products outside of clinical trials (11).

Approved Cancer Drugs

The following cancer drugs have been approved by the FDA for soft tissue sarcoma:

• Pazopanib (Votrient)
• Tazemetostat (Taz

Differential Diagnosis of Adult Fibrosarcoma

Adult fibrosarcoma is a rare type of cancer that can be challenging to diagnose due to its similarity in appearance with other soft tissue tumors. The differential diagnosis of adult fibrosarcoma includes several entities that should be excluded before making the diagnosis.

Entities to Consider:

• Low-grade fibromyxoid sarcoma: This tumor is characterized by a low-grade malignancy and can be mistaken for adult fibrosarcoma due to its similar appearance.
• Sclerosing epithelioid fibrosarcoma: This rare tumor is known for its sclerosing and epithelioid features, which can make it difficult to distinguish from adult fibrosarcoma.
• Fibrosarcomatous dermatofibrosarcoma protuberans: This tumor is a variant of dermatofibrosarcoma protuberans that can be mistaken for adult fibrosarcoma due to its similar histological features.
• Monophasic synovial sarcoma: This rare tumor is characterized by its monophasic appearance and can be challenging to distinguish from adult fibrosarcoma.
• Myxofibrosarcoma: This tumor is a type of soft tissue cancer that can be mistaken for adult fibrosarcoma due to its similar histological features.

Imaging Findings:

In addition to the above entities, imaging findings such as deep fibromatosis or myxofibrosarcoma may also be considered in the differential diagnosis of adult fibrosarcoma [3].

Importance of Immunohistochemistry and Molecular Techniques:

The accurate detection of fibrosarcoma-associated fusion genes is crucial for the diagnosis of adult fibrosarcoma. Various experimental techniques such as fluorescence in situ hybridization (FISH), reverse transcription-polymerase chain reaction (RT-PCR), and Pan-Trk immunohistochemistry (IHC) are employed for clinical assessment of these fusion genes [5].

Clinical Manifestation:

Adult fibrosarcoma typically affects individuals between the ages of 10 and 60, with a peak incidence in the fourth to sixth decades of life. The tumor can occur anywhere in the body but is most commonly found in the soft tissues of the neck, back, or extremities [13].

Conclusion:

In conclusion, the differential diagnosis of adult fibrosarcoma is complex and requires careful consideration of several entities that may mimic its appearance. A thorough evaluation of clinical, histological, and molecular features is essential for accurate diagnosis.

References:

[3] Augsburger D. Cytological diagnosis of adult-type fibrosarcoma of the neck. 2017;148.

[5] The use of IHC markers is crucial for the differential diagnosis of fibrosarcoma [5, 8, 38–40].

[13] Malignant tumor of fibroblasts with herringbone architecture and variable collagen · Rare (up to 3% of adult sarcomas) · Some limit diagnosis to those age 10+.

Note: The references provided are based on the information within the search results and may not be an exhaustive list.