Clinical and pathological features of head and neck follicular dendritic cell sarcoma

Follicular dendritic cell sarcoma (FDCS) in the head and neck is uncommon. The etiology, pathogenesis, optimal treatment, and prognosis are not well understood. In this review, we investigated these features to deepen the understanding of the disease.

We reviewed FDCS in the head and neck in the English language literature through Medline. We analyzed the clinical characteristics, pathologic features, immunophenotypic profile, and treatments of FDCS.

Of 137 reported cases of FDCS in the head and neck region, 127 included data on age and gender. Among those, 64 were females and 63 were males. The mean age was 46 years (9-79 years). In the 106 cases with complete follow-up data, fourteen patients (13.2%) had local recurrence. Fourteen patients (13.2%) had distant metastasis. The metastatic site involved the lung, liver, adrenal, rib, vertebral body, and iliac bone. The overall 5-year survival rate was 80.0%. Compared to nodal FDCS and extranodal FDCS, the overall 5-year survival rates were 71.1 and 85.3%, respectively, with no significance (P = 0.42), 0.8% waived treatment, 46.6% received surgery alone, 30.5% received surgery combined with postoperative radiotherapy, 8.4% received surgery combined with postoperative radiotherapy and chemotherapy, 7.6% received surgery combined with postoperative chemotherapy, 3.1% received chemotherapy alone, 2.2% received chemotherapy combined with radiotherapy, 0.8% received radiotherapy alone.

FDCS in the head and neck is rare. There was no difference in the survival between nodal and extranodal FDCS. The optimal treatment was undetermined.