fibroblastic liposarcoma

Fibroblastic liposarcoma, also known as fibrosarcomatous lipoma or fibroblastic lipoma, is a rare subtype of liposarcoma that combines characteristics of both lipomas and fibrosarcomas.

Definition: Fibroblastic liposarcoma is a type of soft tissue sarcoma that arises from the fibrous connective tissue and contains a mixture of fat cells (lipocytes) and fibroblasts, which are cells responsible for producing collagen. This tumor type is characterized by its unique histological features, which include a combination of adipocytic and fibrosarcomatous components.

Key Features: The key features of fibroblastic liposarcoma include:

• A mixture of fat cells (lipocytes) and fibroblasts
• Fibrous connective tissue involvement
• Presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern
• Typically affects soft tissues, but can also occur in bones

Incidence: This subtype is relatively rare compared to other types of liposarcomas.

Clinical Presentation: The clinical presentation of fibroblastic liposarcoma may include a slow-growing mass or lump in the affected area, which can be painful or tender to touch.

References:

• [12] National Cancer Institute. NCI Thesaurus Version 18.11d.
• [14] Fibrosarcoma is a rare, highly malignant tumor that results in collagen production in places throughout the body where it should not be. This cancer forms in fibroblast cells responsible for making fibrous connective tissue like tendons that connect muscle to bone.
• [15] Fibrosarcoma: This subtype is more likely to develop in soft tissues than in bones. When it originates in the bones, a tumor will more commonly affect your legs, arms, and jaw and can spread to the lungs.

Common Signs and Symptoms

Fibroblastic liposarcoma, a rare type of cancer, can manifest in various ways depending on its location and size. Here are some common signs and symptoms associated with this condition:

• Painless or tender lump: A noticeable lump or swelling under the skin is often the first symptom of fibroblastic liposarcoma [12].
• Abdominal pain: If the tumor grows in the abdominal area, it can cause discomfort, bloating, or feeling full soon after eating [10].
• Swelling: The affected limb or area may experience swelling due to the growing tumor [10].
• Weakness: As the tumor presses on surrounding tissues and nerves, weakness of the affected limb is possible [10].
• Pain or soreness: Pain or soreness caused by suppressed nerves and muscles can occur in some cases [8].

Other Possible Symptoms

In addition to these common symptoms, fibroblastic liposarcoma may also present with:

• Limping or difficulty walking: If the tumor affects a limb, it can cause limping or difficulty walking due to pain or weakness [8].
• Belly pain or discomfort: Tumors growing in the abdominal area can cause belly pain or discomfort [9].

Importance of Early Detection

It's essential to seek medical attention if you notice any unusual lumps or bumps under your skin, as early detection and treatment improve outcomes for this rare cancer.

Diagnostic Tests for Fibroblastic Liposarcoma

Fibroblastic liposarcoma, a type of soft tissue sarcoma, can be challenging to diagnose. However, various diagnostic tests can aid in its identification.

• Imaging Tests: Imaging tests such as MRI and CT scans are used to create pictures of the inside of the body, helping to show the size and location of the tumor [4][5]. These tests can also help determine if the tumor has spread to other parts of the body.
• Fluorescence In Situ Hybridization (FISH): FISH testing is a molecular diagnostic test that can identify common translocations in soft tissue tumors, including fibroblastic liposarcoma [2][6]. This test can aid in the diagnosis of this type of cancer.
• Histologic and Cytogenetic Analysis: A biopsy showing immature lipoblasts with various cellular atypia is necessary for a definitive diagnosis of fibroblastic liposarcoma [8].
• Laboratory Studies: Other than histologic and cytogenetic analysis, no specific laboratory tests exist for diagnosing soft-tissue tumors, including fibroblastic liposarcoma [3].

References:

[1] Computed tomography (CT) scanning through the affected region is the most useful radiographic technique in diagnosing soft-tissue sarcomas.

[2] Fluorescence in situ hybridization (FISH) testing is used to identify common translocations that can aid in the diagnosis of soft tissue tumors.

[3] Laboratory Studies. Other than histologic and cytogenetic analysis, no specific laboratory tests exist for diagnosing soft-tissue tumors.

[4] Imaging tests create pictures of the inside of the body, and help show the size of the liposarcoma.

[5] Magnetic resonance imaging (MRI). This is the most common imaging test for diagnosing fibrosarcomas.

[6] Currently the most commonly used assay for this purpose is fluorescence in situ hybridization (FISH), but this is labor intensive.

[7] The LIDA assay described here represents an effective method of diagnosing liposarcomas on core needle biopsy, with 25 of 29 tested samples technically suitable.

[8] Diagnosis is made with a biopsy showing immature lipoblasts with various cellular atypia depending on the sub-type.

Treatment Options for Fibroblastic Liposarcoma

Fibroblastic liposarcoma, a subtype of soft tissue sarcoma (STS), can be challenging to treat due to its aggressive nature. However, various treatment options are available, including:

• Chemotherapy: Chemotherapy is often used as part of the first-line treatment for advanced or metastatic fibroblastic liposarcoma. Anthracycline-based therapy is a standard approach [1].
• Targeted Therapy: Targeted therapies such as sunitinib have been studied for the treatment of fibroblastic liposarcoma, although their efficacy may vary [3].
• Eribulin Mesylate: Eribulin mesylate has been approved by the FDA for the treatment of liposarcoma, including fibroblastic liposarcoma [6].
• Pazopanib and Trabectedin: Therapies such as pazopanib and trabectedin have shown activity in STS, including fibroblastic liposarcoma, although their effectiveness may differ across subtypes [8].

Current Research Status

Research on the cellular and molecular alterations of fibroblastic liposarcoma is ongoing, with studies exploring new treatment approaches and improving our understanding of this disease [9].

Fibroblastic liposarcoma, also known as myxofibrosarcoma or myxoid liposarcoma, is a type of soft tissue sarcoma that can be challenging to diagnose. The differential diagnosis for this condition includes several other types of tumors and lesions.

Key Differential Diagnoses:

• Fibrous dysplasia: This is a non-cancerous condition characterized by the replacement of normal bone with fibrous tissue, leading to deformity and pain.
• Fibrous histiocytoma: A rare type of cancer that affects the soft tissues, often presenting as a painless lump or swelling.
• Osteosarcoma: A type of bone cancer that can spread to other parts of the body if left untreated.
• Paget sarcoma: A rare and aggressive form of bone cancer that can occur in any bone.
• Malignant fibrous histiocytoma (MFH): A type of soft tissue sarcoma that is characterized by the presence of malignant cells with a mix of fibroblastic and histiocytic features.

Other Considerations:

• Spindle cell lipoma: A rare, non-cancerous tumor that can occur in the skin or subcutaneous tissues.
• Neurofibroma: A type of benign nerve sheath tumor that can occur anywhere on the body.
• Dermatofibrosarcoma protuberans (DFSP): A rare type of cancer that affects the soft tissues, often presenting as a painless lump or swelling.

References:

• The most frequent diagnoses were myxofibrosarcoma and myxoid liposarcoma, followed by low-grade fibromyxoid sarcoma, low-grade fibromyxoid tumor and myxoid lipoma [1].
• Differential diagnoses include the following: Fibrous dysplasia. Fibrous histiocytoma. Osteosarcoma · Paget sarcoma · Malignant fibrous histiocytoma [2].
• The differential diagnosis of nodular fasciitis includes a variety of benign and malignant lesions, including fibroblastic liposarcoma [3].

Note: These references are based on the search results provided in the context.