Plexiform fibrohistiocytic tumor

Plexiform fibrohistiocytic tumor (PFHT) is a rare type of soft tissue tumor that typically occurs in children and young adults. It was first described in the medical literature in 1988. PFHT is considered a low-grade malignancy, meaning it has the potential to spread to other parts of the body, but this is uncommon.

Signs and Symptoms

Patients with PFHT typically present with a slow-growing, painless mass. The most common locations for these tumors are the upper extremities, followed by the head and neck region. However, they can occur anywhere in the body.

Diagnosis

The diagnosis of PFHT is made based on the findings of a biopsy of the tumor. The tumor is composed of a mixture of fibroblasts and histiocytes, arranged in a distinctive plexiform pattern. The cells are typically spindle-shaped and have a moderate amount of cytoplasm. There may also be areas of necrosis or hemorrhage within the tumor.

Treatment

The primary treatment for PFHT is surgical removal of the tumor. In some cases, chemotherapy or radiation therapy may be used as well.

Prognosis

The prognosis for patients with PFHT is generally good, with a high rate of long-term survival. However, the tumor can recur in up to one-third of cases, and metastasis can occur, although this is rare.

See Also

• Soft tissue tumor
• Fibroblast
• Histiocyte

References

Plexiform fibrohistiocytic tumor Resources
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