Acta Otorrinolaringol Esp.

2014;65(3):200---201

www.elsevier.es/otorrino

CASE STUDY

Follicular Dendritic Cell Sarcoma of the Tonsil

Sarcoma de clulas foliculares dendrticas de la amgdala
Leandro Ribeiro, Nuno Lima, Antnio Almeida, Artur Cond
Department of Otorhinolaryngology, Vila Nova de Gaia/Espinho Hospital, Vila Nova de Gaia, Portugal
Received 6 October 2012; accepted 4 November 2012

Case Report
A 80-year-old caucasian man presented in the otorhinolaryngology emergency with a 3-week long history of sore throat.
This patient reported heavy smoking in the past 20 years.
He denied fever, dysphagia or weight loss. On clinical examination a 2 cm exophytic violaceous mass involving the upper
pole of the left tonsil was found. The mucous membrane covering the tonsil was normal, without ulcerations. No other
masses or lymphadenopaties were noted.
Computed tomography of the head and neck conrmed
a tumour with 1.9 1.8 cm in the left tonsil (Fig. 1). After
tonsillectomy was performed, histological examination was
compatible with a Follicular Dentritic Cell Sarcoma (FDCS),
as the tumour cells expressed CD23, CD21 and Vimentine on
immunohistochemical study (Fig. 2). This tumour was classied as T1 N0 M0 according to TNM Classication System.
Positron emission tomography carried out after the tonsillectomy did not show any residual xation in the left tonsil
area or the lymph nodes. Because the margins of resection
were positive, partial pharingectomy was performed. The
new margins were tumour free.
The patient died 1 week after, at home, of cardiac arrest.

to capture and present antigens and immune complexes to

B-cells. The FDCS was rst described in 1986 by Monda et al.1
and is most frequently found within the axillary, mediastinal and cervical lymph nodes, but extranodal involvement
has been seen in approximately 30% of cases: tonsils, oral
cavity and abdominal organs are the most frequent.2---8
In the head and neck, the tonsil is the most frequently
affected structure, eventhough less than 20 cases have been
described.2
The aetiology of FDCS is poorly understood. To date,
Castleman disease and Epstein---Barr infection are the only
predisposing factors that have been identied.3,6,9

Discussion
Follicular dendritic cells are non-lymphoid, non-phagocytic
elements that fall under the generic designation of
accessory cells of the lymphoid system. Their primary role is

Please cite this article as: Ribeiro L, Lima N, Almeida A, Cond

A. Sarcoma de clulas foliculares dendrticas de la amgdala. Acta
Otorrinolaringol Esp. 2014;65(3):200---201.
Corresponding author.
E-mail address: leandro.ribeiro@live.com.pt (A. Cond).

Figure 1 Computed tomographic scan showing a mass in the

left tonsil (arrow).

2173-5735/$ see front matter 2012 Elsevier Espaa, S.L. All rights reserved.

Follicular dendritic cell sarcoma of the tonsil

201
In the current case none of hese prognostic factors were
reported --- the patient died, probably, due to conditions
unrelated to the tumour.
Complete surgical excision is the gold-standard treatment of tonsils FDCS2,6,8,9 ; some authors advocate neck
dissection in case of evidence of ganglionar metastasis.3 The
benet in survival of postoperative radiation or chemotherapy is unknown.7

Conclusion
Folicular dentritic cell sarcoma is a recently recognized
condition that must be considered in the differential diagnosis of unilateral tonsillar swelling, due to its potentially
aggressive behaviour with local recurrences and metastasis.
Figure 2 Photograph shows the FDCS immunohistochemically
stained for CD23 (original magnication 40).

FDCS are most common in adults of 30---50 years

of age (average 47 years old), with an equal gender
distribution.2,7,9
Most patients with FDCS present to the doctor with a painless, slow-growing and well-circumscribed mass.3,4,8 When
affecting the tonsils, it frequently presents as a lobulated,
well-demarcated and grey-white mass,2 like in the case presented (Fig. 1).
Histological examination and immunohistochemical
assays are mandatory for diagnosis. Histological examination commonly reveals eosinophilic oval or spindle cells
arranged in sheets and fascicles,8 which typically stain positively with CD21, CD23, CD35, CD31, KI-M4, Ki-M4p, S-100
protein and Vimentine immunohistochemical markers.1---8
As most of these markers are not routinely used in poorly
differentiated tumours, FDCS may be misdiagnosed in more
than 50% of cases.4,6
The differential diagnosis of FDCS includes undifferentiated carcinomas, squamous cell carcinoma and large cell
lymphoma, among others.2---10
FDCS is considered a low- or intermediate-grade malignant neoplasm.2,4,6---8 Metastasis may occur in 25% of the
cases3 (usually affecting the lungs, liver or lymph nodes)
and local recurrences are common (40%)4 ; locoregional
recurrence has been reported up to 15 years after initial
treatment.4,6 To date, no deaths have been reported with
FDCS of the tonsil.4
The prognosis is aggravated by tumour size (>5 cm---6 cm)
and the presence of nuclear pleomorphism, numerous mitosis (>5 per 10 high-power eld), coagulative necrosis or
signicant cellular atypias on histological examination.3,6,8

References
1. Monda L, Warnke R, Rosai J. A primary lymph node malignancy with features suggestive of dentritic reticulum cell
differentiation. A report of 4 cases. Am J Pathol. 1986;122:
562---72.
2. Vaideeswar P, George SM, Kane SV, Chaturvedi RA, Pandit SP.
Extranodal follicular dentritic cell sarcoma of the tonsil- case
report of an epithelioid cell variant with osteoclastic giant cells.
Pathol Res Pract. 2009;205:149---53.
3. Clement P, Saint-Blancard P, Minvielle F, le Page PC, et al.
Follicular dentritic cell sarcoma of the tonsil: a case
report. Am J Otolaryngol Head Neck Med Surg. 2006;27:
207---10.
4. Duan GJ, Wu F, Zhu J, Guo DY, Zhang R, Shen LL, et al. Extranodal follicular dentritic cell sarcoma of the pharyngeal region.
A potencial diagnostic Pitfall, with literature review. Am J Clin
Pathol. 2010;133:49---58.
5. Biddle DA, Ro JY, Yoon GS, Yong YW, Ayala AG, Ordonez NG, et al.
Extranodal follicular dentritic cell sarcoma of the head and neck
region: three new cases, with a review of the literature. Mod
Pathol. 2002;15:50---8.
6. Amiri-Kordestini L, Priebat D, Chia S. Follicular dentritic cell
sarcoma of the neck: case report and review of current
diagnostic and management strategies. Ear Nose Throat J.
2010;89:E14---7.
7. Choi B, Baek J, Shin Y, Kim J, Kim H, Lee S, et al. Follicular dentritic cell sarcoma: a case report and review of the literature.
Cancer Res Treat. 2010;42:121---4.
8. Eun Y, Kim S, Kwon K. Follicular dentritic cell sarcoma of the
tonsil. Yonsei Med J. 2010;51:602---4.
9. Tisch M, Hengstermann F, Kraft K, Hinuber G, Maier H. Follicular
dentritic cell sarcoma of the tonsil: report of a rare case. Ear
Nose Throat J. 2003;82:507---9.
10. Encabo RS, McHugh J, Carrau RL, Kassam A, Heron D. Follicular
dendritic cell sarcoma of the nasopharynx. Am J Otolaryngol.
2008;29:262---4.