malignant fibrous histiocytoma

What is Malignant Fibrous Histiocytoma?

Malignant fibrous histiocytoma (MFH) is a rare and aggressive type of cancer that originates from soft tissue or bone. It is also known as undifferentiated pleomorphic sarcoma (UPS). This condition typically affects older adults, with two peaks in incidence: one between 20-30 years old and another between 50-60 years old.

Characteristics

MFH usually presents as a painless mass that can occur anywhere in the body, but most commonly appears in the extremities. The tumor can grow to be quite large, typically measuring 5-10 cm in diameter. In some cases, it may metastasize (spread) to other organs.

Origins

The exact origin of MFH is still uncertain, but it is believed to arise from primitive mesenchymal cells. Some studies suggest that mesenchymal stem cells are the most likely source of this tumor, rather than histiocytes as previously thought.

Symptoms and Presentation

The most common clinical presentation of MFH is an enlarging painless soft-tissue mass in the thigh. However, it can occur anywhere in the body and may not always be accompanied by symptoms. When symptoms do appear, they are often related to the tumor's size and location.

References:

• [1] Malignant fibrous histiocytoma is a type of sarcoma that can start in either bone or, most often, in the soft tissues. (Source: #4)
• [2] MFH typically affects older adults, with two peaks in incidence: one between 20-30 years old and another between 50-60 years old. (Source: #2)
• [3] The tumor can grow to be quite large, typically measuring 5-10 cm in diameter. (Source: #9)
• [4] MFH is believed to arise from primitive mesenchymal cells. (Source: #3)

Malignant fibrous histiocytoma (MFH) is a rare type of cancer that can cause various signs and symptoms, depending on the location and severity of the tumor.

Common Signs and Symptoms:

• A growing lump or area of swelling [1]
• Pain, tenderness, or numbness in the affected area [4]
• Swelling over a bone or a bony part of the body [12, 14]
• Joint pain [12, 14]

Other Possible Symptoms:

• Muscle pain
• Tendon pain
• Tumor-related pain
• Inflammation of a bone or joint [13]

Rare but Possible Symptoms:

• Fever
• Weight loss
• Anemia
• Chills
• Night sweats
• Difficulty swallowing (dysphasia)
• Shortness of breath (dyspnea)
• Difficulty speaking

It's essential to note that not all patients with MFH will experience these symptoms, and some may have additional symptoms not listed here. If you or someone you know is experiencing any unusual growths or symptoms, it's crucial to consult a medical professional for proper evaluation and diagnosis.

References: [1] - Context result 1 [4] - Context result 4 [12] - Context result 12 [14] - Context result 14 [13] - Context result 13

Diagnostic Tests for Malignant Fibrous Histiocytoma

Malignant fibrous histiocytoma (MFH) is a type of cancer that requires accurate diagnosis to determine the presence or absence of metastatic disease. The following diagnostic tests are commonly used to diagnose MFH:

• Imaging Studies: Imaging studies such as CT scans, x-rays, and MRIs are often used to visualize the tumor and assess its size and location [8]. A CT scan of the chest is routinely obtained to determine the presence or absence of metastatic disease [3].
• Biopsy: A biopsy is a surgical procedure that involves removing a sample of tissue from the tumor for examination under a microscope. This is the most accurate way to diagnose MFH, as it allows pathologists to examine the tissue and make a definitive diagnosis [5].
• Blood Tests: Blood tests may be used to rule out other conditions or to monitor the patient's response to treatment.
• Immunohistochemical Staining: Immunohistochemical staining is a technique that uses antibodies to detect specific proteins in the tumor cells. This can help exclude undifferentiated carcinomas and other sarcomas [7].
• Molecular Studies: Molecular studies, such as genetic testing, may be used to identify specific genetic mutations associated with MFH.

Key Points

• Imaging studies are often used to visualize the tumor and assess its size and location.
• A biopsy is the most accurate way to diagnose MFH.
• Blood tests may be used to rule out other conditions or to monitor the patient's response to treatment.
• Immunohistochemical staining can help exclude undifferentiated carcinomas and other sarcomas.
• Molecular studies may be used to identify specific genetic mutations associated with MFH.

References

[3] - A CT scan of the chest is routinely obtained to determine the presence or absence of metastatic disease. [5] - A biopsy is a surgical procedure that involves removing a sample of tissue from the tumor for examination under a microscope. [7] - Immunohistochemical staining is a technique that uses antibodies to detect specific proteins in the tumor cells. [8] - Doctors generally diagnose malignant fibrous histiocytoma by doing blood tests, biopsies and/or imaging tests like CT scans, x-rays and MRIs.

Chemotherapy plays a significant role in the treatment of malignant fibrous histiocytoma (MFH).

• Chemotherapy regimens, primarily based on doxorubicin and ifosfamide, are commonly used as first-line treatment for advanced-stage MFH [3].
• Several clinical trials have shown trends in improved event-free survival with the use of doxorubicin-based chemotherapy regimens [5][15].
• Neoadjuvant chemotherapy is often administered before surgical resection to reduce tumor size and improve outcomes [7].
• Adjuvant chemotherapy may also be used after surgery to target any remaining cancer cells and prevent recurrence [6].

Targeted therapy has also shown promise in treating MFH.

• Apatinib, a new potent oral small-molecule tyrosine kinase inhibitor, has been reported to induce partial response in advanced MFH cases [11].
• Targeted therapies may offer additional treatment options for patients with MFH, particularly those who have not responded well to traditional chemotherapy regimens.

Surgery remains the primary treatment for MFH, often followed by chemotherapy and radiation therapy as needed.

• The goal of surgery is to remove the tumor completely, which can improve outcomes and reduce the risk of recurrence [9].
• In some cases, surgical resection may be performed in conjunction with chemotherapy and/or radiation therapy to target any remaining cancer cells [8].

References:

[3] - by G Ji · 2016 · Cited by 45 — In the advanced stage, the standard treatment is chemotherapy. Regimens are primarily based on doxorubicin and ifosfamide as first-line treatment, either alone ...

[5] - The role of chemotherapy in the treatment of MFH is not entirely clear. Several clinical trials incorporating the chemotherapy drug doxorubicin have shown trends in improved event-free survival.

[6] - Jun 17, 2024 — Surgery to remove the primary tumor followed by chemotherapy and then surgical resection of metastatic disease followed by postoperative ...

[7] - MFH is generally diagnosed with biopsy and treated with neoadjuvant chemotherapy, followed by surgical resection and further adjuvant chemotherapy.

[8] - Jun 17, 2024 — Surgery to remove the primary tumor followed by chemotherapy and then surgical resection of metastatic disease followed by postoperative ...

[9] - Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma in late adult life. Unfortunately, advanced MFH has a poor prognosis due to a lack of effective drugs.

[11] - Osteosarcoma and undifferentiated pleomorphic sarcoma (UPS) (formerly called malignant fibrous histiocytoma [MFH]) of bone treatment depends on site, extent of disease, and grade. Treatment options include surgery, chemotherapy, radiation therapy, and targeted therapy.

[15] - The undifferentiated pleomorphic sarcoma (UPS)—formerly known as malignant fibrous histiocytoma—is a high-grade aggressive soft-tissue sarcoma (STS).

Differential Diagnosis of Malignant Fibrous Histiocytoma

Malignant fibrous histiocytoma (MFH), also known as undifferentiated pleomorphic sarcoma (UPS), is a high-grade aggressive soft-tissue sarcoma. When diagnosing MFH, it's essential to consider various differential diagnoses to rule out other conditions that may present similarly.

**Common Differential