Follicular dendritic cell sarcoma of the right tonsil: A case report and literature review

Abstract

The current study presents a case of extranodal follicular dendritic cell sarcoma (FDCS) of the tonsil and reviews the relevant literature. In the present case, a 59-year-old male presented with a globus sensation in the right pharynx for 6 weeks. On clinical examination, a painless non-ulcerated enlarged right tonsil was identified; the tonsil was covered with a normal mucus membrane. A right tonsillectomy was performed under general anesthesia. The final pathological diagnosis was follicular dendritic cell sarcoma of the right tonsil. Postoperatively, the patient received radiotherapy. The patient remains alive without disease recurrence or metastasis 44 months after tonsillectomy. To the best of our knowledge, only 42 cases of FDCS of the tonsil have been reported to date. Of the 42 cases, 41 patients underwent surgery and one patient refused treatment. A total of 23 (54.7%) received surgery alone. Adjuvant treatment was administered for 18 patients (42.9%). Six patients (14.3%) experienced local recurrences and two patients (4.8%) succumbed to the disease 24 months after treatment. The three-, five-, and eight-year overall survival rates for the entire group were 86.5, 77.8 and 77.8%, respectively. Furthermore, a tumor diameter of ≥4 cm was prognostic upon univariate analysis (χ²=4.634; P=0.031; excluding incomplete data). Tonsillar FDCS is rare and is associated with high rates of recurrence and metastasis, therefore, adjuvant treatment should be prescribed.

Keywords: follicular dendritic cell sarcoma; prognosis; size of tumor; tonsil; treatment.

Figure 1

Computed tomography scans revealing a homogenously enlarged 4.6×2.5×2.5-cm right tonsil, which was well-circumscribed. (A) The initial enhancement value was 53 HU, and (B) slight continuing heterogeneous enhancement was evident following injection of contrast medium. R, right.

Figure 2

Pathological tests results showing a lesion containing large spindle-shaped heterogeneous cells forming solid or nested patterns, infiltrating the lymphoid stroma. Immunohistochemically, the cells were positive for (A) cluster of differentiation (CD)21 and (B) CD23.

Figure 3

Postoperatively, the patient received radiotherapy (6,000 cGy in 200-cGy fractions delivered over 30 days) to the oropharyngeal and corresponding neck lymphatic regions.

Figure 4

Univariate analysis results revealing that a tumor diameter of ≥4 cm was prognostic (χ²=4.634; P=0.031).