MFH: An Aggressive Cancer Rarely Seen in Head and Neck

Tumor size, location, and histology are the major determinants of outcome, but patients with less aggressive myxoid tumors do not require systemic therapy, and patients with nonmyxoid disease are at significant risk for metastases.

MFH is not as sensitive to radiotherapy and chemotherapy as some other cancers, but multidisciplinary therapy must be offered for the overwhelming majority of patients. Salvage following initial treatment failure is difficult.

Prognosis

As with all cancer, the most important prognostic factors for MFH are tumor grade, size, and extent of metastasis, as well as histiologic subtype and surgical margins. About 20% of all cases are associated with previous radiation treatment.

At the January 2009 meeting of the Triological Society, a 40-year retrospective review of 95 MFH patients at M. D. Anderson Cancer Center was presented. It revealed that:

• Twenty-eight percent of patients had surgery alone, 26% had surgery with radiation, 17% had surgery plus chemoradiation, 13% had surgery plus chemotherapy, and 16% did not have surgery.
• After 34 months of follow-up, 36% of patients had recurrence after initial therapy, about three-quarters of which was local.
• Overall five-year survival for patients with MFH was 51%; disease-free survival was 44%.
• Worse survival correlated with being treated before 1990, as well as with larger tumors, tumor site, and previous radiation treatment.

Others have reported that tumor characteristics also affect survival: Patients with low-, intermediate-, and high-grade tumors have 10-year survival rates of 90%, 60%, and 20%, respectively. Tumors smaller than 5 cm at diagnosis (stage 1) have survival of 79% to 82%, those of 5 to 10 cm (stage 2) have rates of 62% to 68%, and tumors larger than 10 cm (stage 3) result in only 41% to 51% survival.

In general, prognosis is poor if the disease has metastasized to the lungs, lymph, or bone; complete tumor removal is not possible; the patient is very old and/or the tumor is very large; and the tumor is deep, rather than superficial.

Sometimes MFH is a second malignant neoplasm. The prognostic factors here have not been well described, but one observational study looked at 50 patients who had had breast cancer, Hodgkin’s disease, lung cancer, and melanoma. The average time between the first malignancy and MFH was 10 years, and half of the cases of MFH developed in irradiated fields. Two-year survival was 33%, and five-year survival was 13%.