Follicular dendritic cell sarcoma of the head and neck: Case report, literature review, and pooled analysis of 97 cases

Abstract

Background: Follicular dendritic cell sarcoma (FDCS) is a rare lymphoid neoplasm presenting in the head and neck. There are no pooled analyses of head and neck FDCS cases in the English language literature.

Methods: A MEDLINE and PubMed review of cases from 1978 to February 2014 was performed. Demographics, clinicopathologic data, and outcomes were summarized.

Results: We presented 2 patients and analyzed 97 cases. The mean age was 42.7 years (SD = 16.3 years). Outcomes were available for 76 patients. Tumors ≤4 cm had better disease-free survival (63% vs 28% at 5 years; p = .0282). Locoregional recurrence was significantly less likely with surgery and radiation compared to surgery alone (15% vs 45%; p = .019) and in patients receiving a neck dissection (10% vs 43%; p = .046).

Conclusion: This pooled analysis provides the largest sample size of FDCS of the head and neck to date and suggests that radiation and neck dissection may be beneficial to locoregional oncologic control. © 2015 Wiley Periodicals, Inc. Head Neck 38: E2241-E2249, 2016.

Keywords: dendritic cell; follicular dendritic cell sarcoma (FDCS); head and neck; sarcoma.

FIGURE 1

MRI evaluation from case 1. (A) Demonstrates the right mandibular lesion from an axial view (white arrow; T1-weighted postcontrast). The tumor was enhancing, expansile, lobulated, and 2.5 × 1.3 × 4.0 cm in size. It was located in the body of the mandible extending to the right mandibular angle abutting the posterior molar dentition and partially extending through the buccal cortex. (B) Demonstrates the lesion with T2-weighting (white arrow).

FIGURE 2

Resected specimen from case 1. (A) Demonstrates a lateral view of the resected specimen from the right segmental mandibulectomy. (B) Demonstrates the surgical specimen from a medial view.

FIGURE 3

Histology from case 1. (A) On low magnification, the tumor contained spindled to ovoid cells arranged in a storiform pattern with scattered lymphocytes (hematoxylin-eosin stain; original magnification ×100). (B) On high power, the tumor cells had nuclei with mild atypia, prominent nucleoli, few mitoses, and a moderate amount of eosinophilic cytoplasm with indistinct cell borders (hematoxylin-eosin stain; original magnification ×400). Immunohistochemical stains for (C) CD21 and (D) CD23 showed strong membranous reactivity in the tumor cells, confirming a follicular dendritic cell origin (hematoxylin-eosin stain; original magnification ×400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

FIGURE 4

Clinical outcomes depicted as survival curves. On Kaplan–Meier analysis, 2-year and 5-year disease-free survival (DFS) was 64% and 34%, respectively (A). Two-year and 5-year overall survival (OS) was 91% and 81%, respectively (A). Tumors with a primary location (Loc) in the oropharynx had statistically significantly better DFS than tumors with a primary location outside of the oropharynx (p = .0445) (B). OS was 75% versus 61% at 2 years and DFS was 61% versus 26% at 5 years (B). Tumors ≤4 cm in size had statistically significantly better DFS than tumors >4 cm in size (p = .0282) (C). OS was 72% versus 37% at 2 years and DFS was 63% versus 28% at 5 years (C).