Mfh Tumors

This has become the most common malignant sarcoma of older adults. It tends to occur in the deep soft tissue of the extremities and the retroperitoneum. However, it has been described in nearly every organ of the body. Signs and symptoms relate directly to the location in which it arises. It is a diagnosis of exclusion; the burden of proof is upon the pathologist to do a battery of immunohistochemical studies to exclude other sarcomas such as rhabdomyosarcoma and liposarcoma. Several histologic variants are described (storiform-pleomorphic, myxoid, inflammatory, giant cell, and angiomatoid) In general, all of these tumors follow an aggressive course and will recur unless widely excised. Angiomatoid variants appear to be a distinct disease occurring in younger patients and having a more indolent course. Recently, many investigators have questioned the uniqueness of this tumor, indicating that many are actually pleomorphic tumor derived from muscle cells.

In typical cases, the tumor surface is smooth, multinodular and capsulated. The sectional property of the tumor is solid, and rarely shows mucoid pattern. When the tumor becomes large, cystic, necrotic and bloody changes occur. Regardless of those changes, there are no specific features for MFH that are detectable by ultrasonography, CT, MRI and angiography. Therefore, we must rely on histopathological findings discriminating it from other diseases.

Surgical excision is the first choice of treatment for MFH and itslocal recurrence. Because of its frequent metastases, it is important to perform wide resection of the tumor including the connective tissue,the fascia and the muscles around the tumor and regional lymph nodes. Hashimoto reports a survival rate over a period of five years of 87% in the cases treated with wide excision in comparison with that of 50% in those with simple extraction. When the tumor becomes large, without symptoms in the retroperitoneal cases, curative resection is much more difficult. Dalton states that the survival rate over a period of five years in those patients with tumor sizes of more than 5cm is 56% and 36% in those with infiltrating tumors. There is no relationship between the degrees of atypicality or extent of karyomitosis of tumor tissue and the degrees of biological malignancy of the tumor in MFH. It is assumed that the cases with large tumors and of retroperitoneal origin have poor prognosis with frequent hematogenic metastases. Remote metastases are