malignant skin fibrous histiocytoma

Malignant Fibrous Histiocytoma (MFH) Description

Malignant fibrous histiocytoma, also known as undifferentiated pleomorphic sarcoma (UPS), is a rare and aggressive type of cancer that affects the soft tissues. It can occur anywhere in the body, but most commonly develops in the arms, legs, and abdomen.

Characteristics:

• Affects mostly older adults
• Can form in bone or soft tissue
• Typically occurs in the extremities (arms, legs) or abdominal region
• May also occur in other parts of the body, such as the skin, retroperitoneum, or bones

Symptoms:

• Slow-growing lump or mass
• Pain or discomfort in the affected area
• Skin dimpling or puckering on pinching the lesion (dimple or pinch sign)

Types:

• Malignant fibrous histiocytoma (MFH)
• Undifferentiated pleomorphic sarcoma (UPS) - a more recent term for MFH

Treatment and Prognosis:

• Treatment options include surgery, chemotherapy, and radiation therapy
• Prognosis is generally poor due to the aggressive nature of the cancer
• Can spread and return after treatment

References:

• [1] According to search result 11, this type of sarcoma usually affects soft tissues but can also happen in bones.
• [2-5] As per search results 3, 4, 12, and 14, MFH/UPS is a rare and aggressive form of cancer that can affect various parts of the body.
• [6-10] Search results 13, 15, and others describe the characteristics, symptoms, and treatment options for malignant fibrous histiocytoma.

Common Signs and Symptoms

Malignant fibrous histiocytoma (MFH) can manifest in various ways, depending on the location and size of the tumor. Here are some common signs and symptoms:

• Pain at the tumor site: Pain or tenderness is a frequent complaint among patients with MFH.
• Swelling over a bone or joint: Swelling or lump formation near bones or joints can be an indication of MFH.
• A lump that you can feel: A palpable lump or mass is often detected in cases of MFH.
• Weight loss and fatigue: As the tumor grows, patients may experience weight loss and fatigue due
• Difficulty swallowing (dysphasia): In rare cases, MFH can cause difficulty swallowing or speaking.

Other Possible Symptoms

While not as common, other symptoms that have been reported in some cases of MFH include:

• Shortness of breath (dyspnea)
• Rapid tumor enlargement during pregnancy
• Episodic hypoglycemia (low blood sugar)
• Tingling and numbness

It's essential to note that not all patients with MFH will experience symptoms, and some may have no noticeable signs at all. If you suspect you or someone else has MFH, consult a medical professional for proper evaluation and diagnosis.

References:

[4] - Pain at the tumor site is mentioned as a symptom in this search result. [5] - Growing lump or area of swelling is another possible sign of MFH. [6] - Clinically, deep BFH lesions are seen as painless, slowly enlarging masses. [14] - Symptoms such as weight loss and fatigue can present in patients with advanced disease.

Diagnostic Tests for Malignant Fibrous Histiocytoma (MFH)

Malignant fibrous histiocytoma (MFH) is a rare and aggressive type of cancer that can be challenging to diagnose. While there are no specific diagnostic tests for MFH, various imaging studies and laboratory tests can help confirm the diagnosis.

• Imaging Studies:

  • Ultrasound: This non-invasive test uses high-frequency sound waves to create images of the soft tissues inside the body. It can help identify tumors and assess their size and location.
  • Magnetic Resonance Imaging (MRI): MRI is a powerful imaging tool that uses magnetic fields and radio waves to produce detailed images of the internal structures. It can be used to evaluate the extent of tumor spread and involvement of surrounding tissues.
  • Computed Tomography (CT) scans: CT scans use X-rays and computer technology to create cross-sectional images of the body. They are often used to assess the presence or absence of metastatic disease in the chest, abdomen, or pelvis.

• Laboratory Tests:

  • Biopsy: A biopsy involves removing a small sample of tissue from the tumor for microscopic examination. This is the most definitive way to diagnose MFH.
  • Immunohistochemistry (IHC): IHC is a laboratory test that uses antibodies to detect specific proteins in cancer cells. It can help confirm the diagnosis and identify the type of sarcoma.

• Other Diagnostic Tests:

  • Dermoscopic examination: This non-invasive test uses a specialized microscope to examine the skin lesion.
  • Fine-needle aspiration biopsy (FNAB): FNAB involves using a thin needle to collect a sample of cells from the tumor for microscopic examination.

References:

[11] Ultrasound is one type of imaging test that can help diagnose MFH. [12] A CT scan of the chest may be obtained to determine the presence or absence of metastatic disease. [14] The diagnosis of FH (Fibrous Histiocytoma) may be clinically difficult when the lesion is located in the deep tissues, and is frequently confirmed after local excision.

Note: The references provided are based on the search results within the context.

Based on the provided context, it appears that there are various treatment options available for malignant fibrous histiocytoma (MFH), including drug treatments.

• Chemotherapy: According to search result 2, chemotherapy is a standard treatment option for advanced MFH. Regimens primarily based on doxorubicin and ifosfamide are used as first-line treatment, either alone or in combination with other drugs.
• Apatinib: Search result 4 mentions apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor (VEGF). A case study reported partial response to apatinib in advanced MFH.
• Doxorubicin-based regimens: Several clinical trials incorporating doxorubicin have shown trends in improved event-free survival without a major impact on overall survival, as mentioned in search result 8.
• Imatinib therapy: Before starting imatinib therapy, cytogenetic studies to confirm PDGFB gene rearrangement may be necessary for predicting the clinical response, according to search result 9.

It's essential to note that these treatment options might not be suitable or effective for all patients with MFH. A personalized approach and consultation with a healthcare professional are crucial in determining the best course of treatment.

References: [2] [4] [8] [9]

Differential Diagnosis of Malignant Skin Fibrous Histiocytoma

Malignant skin fibrous histiocytoma (MFH) is a rare and aggressive type of cancer that can be challenging to diagnose. The differential diagnosis for MFH includes several other conditions that may present with similar clinical and pathological features.

Key Differential Diagnoses:

• Dermatofibrosarcoma Protuberans (DFSP): A type of skin cancer that is characterized by a slow-growing, firm tumor. While DFSP can be distinguished from MFH based on its lower mitotic activity, it is essential to consider this diagnosis in the differential.
• Atypical Fibroxanthoma (AFX): A rare and aggressive form of skin cancer that can mimic MFH in terms of its clinical and pathological features.
• Plexiform Fibrohistiocytic Tumor: A rare type of soft tissue tumor that can be difficult to distinguish from MFH based on histological examination alone.
• Malignant Melanoma: While less common, malignant melanoma should also be considered in the differential diagnosis for MFH, particularly if there are any suspicious features such as ulceration or regression.

Key Features to Consider:

• Histological Examination: A thorough histological examination is essential to distinguish MFH from other conditions. The presence of fibroblastic and histiocytic elements in varying proportions can help differentiate MFH from DFSP.
• Immunohistochemistry: Immunohistochemical staining for markers such as S100 protein can be helpful in distinguishing MFH from other conditions, particularly AFX.
• Clinical Presentation: A thorough clinical examination is essential to rule out other conditions that may present with similar features.

References:

• [3] Malignant fibrous histiocytoma (MFH) is the most common primary soft tissue sarcoma of late adult life and its occurrence is rare in the pediatric population.
• [5] A dermatofibroma is a skin tumor composed of oval cells appearing similar to histiocytes or spindle cells. For this reason it is also called a common fibrous ...
• [7] Differential Diagnoses · Cutaneous Melanoma · Cutaneous Squamous Cell Carcinoma · Dermatofibrosarcoma Protuberans.
• [9] by J Strong · 2022 · The differential diagnosis of cellular neurothekeoma includes FH, dermal nerve sheath myxoma, and plexiform fibrohistiocytic tumor.