Malignant Fibrous Histiocytoma - Etiology, Diagnosis, and Treatment

Introduction:

A malignant fibrous histiocytoma is a cancerous tumor that most commonly develops in soft tissue, such as muscles and tendons. However, it can occur in bones in rare circumstances. Malignant fibrous histiocytoma, also known as pleomorphic undifferentiated sarcoma, is most commonly seen in the arms, legs, or abdomen.

How Is Malignant Fibrous Histiocytoma Classified?

• Histologically, malignant fibrous histiocytoma was classified as a stereotypical pleomorphic sarcoma.

• It was made up of a storiform pattern of extremely abnormal spindle cells, the fibroblastic component interspersed with several giant weird polyhedral cells, which is the histiocytic part.

• Mitoses, including architecturally aberrant kinds, are abundant, as well as necrosis and bleeding, which are often severe.

• However, the first electron microscopic studies of these tumors in the 1970s revealed that the neoplastic cells had ultrastructural features similar to fibroblasts and that some of the cells also contained lysosomes, an organelle characteristic of but not unique to histiocytes, which seemed to support Stout's theory.

• As more information on malignant fibrous histiocytoma became available, various subtypes were identified, including:

  • Storiform-pleomorphic malignant fibrous histiocytoma.

  • Myxoid, inflammatory malignant fibrous histiocytoma.

  • Giant cell-rich malignant fibrous histiocytoma.

  • Angiomatoid forms malignant fibrous histiocytoma.

What Is Malignant Fibrous Histiocytoma of Bone?

The range of fibrous histiocytic tumors has been enlarged to encompass benign dermis and tendon sheath tumors such as dermatofibroma, benign synovioma, and malignant fibrous histiocytoma as soft tissue tumors of intermediate malignancy such as dermatofibrosarcoma protuberans. Bone tumors with a similar histological appearance are classified as benign fibrous histiocytoma of bone or malignant fibrous histiocytoma of bone. Tumors with this morphology are identified as malignant fibrous histiocytoma in more and more places other than the soft tissues as pathologists became more aware of the histological pattern of malignant fibrous histiocytoma.

What Is the Etiology of Malignant Fibrous Histiocytoma?

The specific etiology of malignant fibrous histiocytoma is unknown.

However, this uncommon cancer has been linked to various medical disorders such as Paget's disease. In addition, the chances of having the condition increase for people with a history of radiation therapy or older.

What Are the Signs and Symptoms Indicating Malignant Fibrous Histiocytoma?

Cancer cells replace good bone tissue in malignant fibrous histiocytoma. These cancer cells weaken the bone, increasing the risk of fracture. Other indications and symptoms are as follows:

• Muscle pain.

• Tendon pain.

• Tumor-related pain.

• Inflammation of a bone or joint.

• Feelable lumps beneath the skin.

As these symptoms might be caused by something other than cancer, the doctor will conduct tests to confirm a diagnosis and rule out alternative possibilities.

How Is Malignant Fibrous Histiocytoma Diagnosed?

X-Ray: The first imaging test to reveal if there is a tumor or another medical disease.

Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) Scan: Magnetic resonance imaging (MRI) or computed tomography (CT) scans can offer comprehensive pictures of the tumor and help to determine its extent.

Biopsy: The only way to determine if there is cancer is to take a small sample of the tumor and send it to a lab where it will be examined for cancer cells.

Other Tests: A bone scan X-ray or positron emission tomography scan may be conducted to determine whether cancer has spread. If it applies, it will most likely be discovered in the lungs.

What Is the Treatment for Malignant Fibrous Histiocytoma?

The following factors influence treatment for malignant histiocytoma:

• The tumor's location.

• The tumor's size.

• The extent to which the tumor has spread throughout the body.

• Age and sex

• General health.

• Personal preferences.

Malignant histiocytoma treatment options include:

• Surgery: The primary line of treatment for malignant histiocytoma is surgery to remove the tumor. However, if cancer has spread, there might be a need to remove the neighboring tissues such as muscle, tendons, or bone. The most widely used method has been wide local excision. Unfortunately, achieving adequate margins has frequently resulted in major reconstructive problems in the face of high local recurrence rates. Current treatment involves en bloc resection with a two centimeters margin of uninvolved tissue. However, this method is sometimes limited by closeness to vital organs, particularly in the head and neck. Radiation therapy (RT) is an adjuvant treatment for many cancers, particularly those with no distinct margins.

• Mohs Micrographic Surgery (MMS): Mohs micrographic surgery (MMS) has also been employed for malignant fibrous histiocytoma. Mohs micrographic surgery (MMS) has been found to maximize healthy tissue preservation, allowing for more manageable repair while maximizing adequate tumor clearance in various tumor types. In addition, there are low recurrence rates following Mohs micrographic surgery (MMS) therapy.

• Radiation: Following surgery, the tumor location may be subjected to high-energy X-rays to aid in the death of any cancer cells that remain in the body. In some cases, radiation therapy may be helpful as an adjuvant treatment. A radiation field encompassing the tumor site and at least 5 cm of peripheral tissue are used in effective radiotherapy, with doses ranging from 50 to 65 Gy.

• Chemotherapy: It is usually scheduled following surgery to eliminate any leftover cancer cells. However, chemotherapy may be used to reduce the tumor before surgery if the cancer is too big to be removed surgically. Traditional chemotherapy is typically used only in cases of extensive illness where other treatment modalities have failed to demonstrate a meaningful benefit. Recent discoveries into the molecular characteristics of malignant fibrous histiocytoma have allowed investigation of the possible use of biological medicines in treating advanced illnesses.

• Ipilimumab and Sunitinib, a multifunctional tyrosine kinase inhibitor, are now being used in experiments.

What Is the Follow-up of Malignant Fibrous Histiocytoma(MFH)?

• Following therapy, patients should be regularly monitored with comprehensive skin exams, including palpation of the regional lymph node.

• Visits should occur every three to six months for the first two years, followed by at least yearly visits.

• There are presently no guidelines for follow-up imaging unless there are concerns about the symptoms.

Conclusion:

Malignant fibrous histiocytoma (MFH) is an uncommon tumor with a penchant for severe clinical behavior. Historically, malignant fibrous histiocytoma (MFH) was thought to be a moderately aggressive sarcoma capable of infiltrating soft, skeletal, and retroperitoneal tissues. Even in today's dermatologic literature, these tumors are frequently regarded as a deeper, more aggressive variety of atypical fibroxanthoma (AFX), as they have a nonspecific clinical presentation and a comparable pleomorphic spindle cell morphology under light microscopy.Malignant fibrous histiocytoma (MFH) is now a dangerous neoplasm with distinct molecular, immunohistochemical, and behavioral features.