Plexiform Fibrohistiocytic Tumor Symptoms, Causes, Prognosis, Treatment

Plexiform Fibrohistiocytic Tumor Symptoms, Causes, Prognosis, Treatment

The fibrohistiocytic tumor of the skin refers to a heterogeneous group of subcutaneous or dermal mesenchymal neoplasms. It shows myofibroblastic, fibroblastic, and histiocytic (macrophage-like) differentiation. 

However, plexiform fibrohistiocytic tumor is a rare kind of tumor that usually arises primarily in the upper extremities of young adults and children. This tumor is a rare mesenchymal neoplasm of intermediate malignancy first reported by Zhang and Enzinger in 1988. Plexiform fibrohistiocytic tumor of PFT predicts the young adults and children; however, it can occur irrespective of age. This tumor involves the upper limbs as a painless mass and slow-growing. The tumor has a high local recurrence rate but its metastases occurs rarely. It is a newly described entity that involves a soft tissue neoplasm of low malignant potential. Plexiform fibrohistiocytic tumor is a rare tumor and occurs in the neck and head region.

Plexiform Fibrohistiocytic Tumor Symptoms

Plexiform fibrohistiocytic tumors are usually hard to figure out as they can grow anywhere in the human body and cause symptoms very rarely during the early stages. The first sign begins as a lump or swelling getting big over time pressing against muscles or nerves or causing breathing problems. 

Histologically, the plexiform fibrohistiocytic tumor is characterized; by subcutaneous mass or demarcated dermal. Three distinct recognized growth patterns include; fibroblastic, fibrohistiocytic, and mixed types. The fibrohistiocytic tumor displays uniform immunoreactivity for both CD68 and Vimentin. Ultrastructurally, the cells have similar features like histiocyte-like cells and myofibroblasts.

Plexiform Fibrohistiocytic Tumor Symptoms, Causes, Prognosis, Treatment 

It preferentially involves the upper extremity (64%), particularly the hand, fingers, or wrist (45%). The majority of the patients exhibit a small and average size 2.5 cm painless mass that enlarges over months or years. All tumors involve subcutaneous adipose tissue with an extension of skeletal muscle (14%), dermis (19%), or both (14%). Grossly, the tumor cells are of firm consistency.

Plexiform Fibrohistiocytic Tumor Causes

Plexiform fibrohistiocytic tumor is referred to by other names such as; malignant fibrous cytoma and undifferentiated pleomorphic sarcoma. Sometimes, tumors such as plexiform fibrohistiocytic may link to other medical conditions like Paget disease, past radiation treatments, or chemotherapy treatments. But, it is not what causes them. The tumors can develop at any age; however, these are most frequent in older adults. The cause of malignant fibrohistiocytic tumors is unclear; exposure to radiation or chemicals can play a vital role in cancer development. 

Another cause associated with the plexiform fibrohistiocytic tumor is a genetic component of the disease. Sometimes, it is also on the site of the body that has received radiation therapy in history. Malignant fibrous histiocytomas sometimes quickly grow and spread to other body parts such as the lungs. In older adults, these tumors can sometimes usually occur as secondary cancer in individuals affected with retinoblastoma in the past. 

Plexiform Fibrohistiocytic Tumor Prognosis

Two hundred twenty-seven patients with a localized disease with surgery got reviewed retrospectively; to recognize the clinical outcomes. Fifty-four years was the mean age of the affected individuals. 

Extremities were the primary tumor site in around 62.2% of patients. The total survival rate was 50%, including thirty-eight patients who died of other reasons. Local recurrence occurred in 16% of the patients, distant metastases in 23% of patients, and distant metastases with local recurrence in 11% of the population. However, the most common was distant metastases to the lung. 

The primary tumor indicated a 5-year survival rate. Tumors less than 5 cm showed a survival rate of 82%, 5 to 10 cm, 68%, and greater than 10 cm, 51%. Rates of survival were influenced; by size. 

Plexiform Fibrohistiocytic Tumor Treatment

The treatment option for plexiform fibrohistiocytic tumors involves surgery to eradicate the cancerous cells. Some other options are chemotherapy, immunotherapy, radiation therapy, and drug treatments. The well-suited treatment method depends on the location and size of the tumor. 

Chemotherapy most often helps in treating fibrohistiocytic tumors that come back. This strategy is also used before surgery to cause the cancerous cells to shrink so that their removal gets easy. The technique of immunotherapy is reserved; for people with advanced cancer. 

Radiation therapy may involve either of the ways, that is, external beam radiation and intraoperative radiation therapy (IORT). However, targeted drug treatments emphasize specific anomalies present within the tumor cells. Targeted drug treatment methods can cause cancer cells to die by blocking these anomalies. For patients with plexiform fibrohistiocytic tumors, this drug treatment may be combined; with chemotherapy. The cancer cells of an affected individual may assist in whether these drugs are beneficial for you or not. 

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