7/27/2019 Nasopharynx Sarcoma

1/3

Follicular dendritic cell sarcoma of the nasopharynxRosala Souvirn Encabo, MDa, Jonathan McHugh, MDb, Ricardo L. Carrau, MDc,,

Amin Kassam, MDd, Dwight Heron, MDe

aDepartment of Otolaryngology and Head and Neck Surgery, Gregorio Maran University General Hospital, Madrid, SpainbDepartment of Pathology, University of Pittsburgh Medical Center, USA

cDepartment of Otolaryngology and Neurological Surgery, University of Pittsburgh School of Medicine, Eye and Ear Institute, Pittsburgh, PA, USA

dDepartment of Neurological Surgery, University of Pittsburgh Medical Center, USAeDepartment of Radiation Oncology, University of Pittsburgh Medical Center, USA

Received 16 August 2007

Abstract Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm that can develop in

the head and neck in both lymph nodes and extranodal sites. Only 4 cases of FDC tumor of the

nasopharynx have been published before. Because of its rarity, FDC sarcoma is not easily recognized

by clinicians or pathologists. We present the case of a FDC tumor of the nasopharynx in a 36-year-

old woman.

2008 Elsevier Inc. All rights reserved.

1. Introduction

Follicular dendritic cell (FDC) sarcoma is an uncommon

tumor comprised within the spectrum of histiocytic and

dendritic cell neoplasms [1]. Besides being rare, these classesof tumors are notoriously difficult to diagnose. Less than

40 cases of FDC sarcoma of the head and neck have been

reported in the literature [2]. Surgery is the mainstay of

treatment and should include diligent control of surgical

margins. The role of adjuvant therapy remains undefined [2].

2. Case report

A 36-year-old African American woman presented with

bilateral nasal congestion, sensation of blockage in her ears,

and paresthesia of the roof of mouth. She also reported

nocturnal postnasal drainage and recurrent sinus headaches.

Her social history was notable for smoking 1/2 pack per day

for the past 16 years.

A physical examination was unremarkable except for

what seemed to be marked adenoid hypertrophy that was

obstructing the posterior choana bilaterally, but more

prominent on the left than the right. Cranial nerves II to

XII were intact. A computed tomographic (CT) scan of theparanasal sinuses revealed air-fluid levels in the right

maxillary and right frontal sinuses with extensive mucosal

thickening of the inferior left maxillary sinus. A rounded soft

tissue mass centered in the left side of the nasopharynx was

also identified. A CT scan with contrast better evaluated and

defined the area showing a soft tissue mass in the

nasopharynx measuring approximately 2.3 2.3 cm without

infiltration of the longus colli muscle or the adjacent bone. A

magnetic resonance imaging corroborated the findings.

Multiple cervical lymph nodes, all less than 1 cm in size,

were noted in the CT. A left endoscopic maxillary

antrostomy and a biopsy of nasopharynx were performed.Tissue samples showed a malignant neoplasm with epithe-

lioid and spindle morphology with tumor necrosis and

increased mitotic activity. The patient was taken back to

the operating room for a completion adenoidectomy as a

repeat biopsy.

An initial diagnosis of nasopharyngeal teratocarcinosar-

coma was rendered by an outside institution and the patient

was referred to our center for further management. A review

of the case by our pathologists lead to a diagnosis of a FDC

Available online at www.sciencedirect.com

American Journal of OtolaryngologyHead and Neck Medicine and Surgery 29 (2008) 262264

www.elsevier.com/locate/amjoto

Corresponding author. Department of Otolaryngology and Neurolo-

gical Surgery, University of Pittsburgh School of Medicine, Eye and Ear

Institute, Pittsburgh, PA 15213, USA. Tel.: +1 412 647 8186; fax: +1 412

647 2080.

E-mail address: carraurl@upmc.edu (R.L. Carrau).

0196-0709/$ see front matter 2008 Elsevier Inc. All rights reserved.

doi:10.1016/j.amjoto.2007.08.005

mailto:carraurl@upmc.eduhttp://dx.doi.org/10.1016/j.amjoto.2007.08.005http://dx.doi.org/10.1016/j.amjoto.2007.08.005mailto:carraurl@upmc.edu

7/27/2019 Nasopharynx Sarcoma

2/3

sarcoma. Specifically, the tumor was composed of spindled

and epithelioid cells forming nodules and fascicles surround-

ing and displacing residual mucoserous glands. Areas of

whorling and focally myxoid stroma were present as well.

The tumor nodules contained scattered small lymphocytes

with areas of perivascular clustering (Fig. 1). The tumor cellswere strongly positive for both CD21 and CD23. In addition,

the cells were positive for vimentin, neuron-specific enolase,

CD68, epithelial membrane antigen, and were negative for

cytokeratins (AE1/AE3, 7, and 5/6), human melanoma black

(HMB)-45, chromogranin, synaptophysin, -fetoprotein,

CD99, CD33, desmin, myogenin, and smooth muscleand

muscle-specific actins. Leukocyte common antigen and S-

100 protein appeared to stain only background lymphoid and

dendritic cells. These findings excluded carcinoma, lym-

phoma, melanoma, rhabdomyosarcoma, epithelioid heman-

gioendothelioma, and olfactory neuroblastoma [3,4].

A postoperative magnetic resonance imaging of the brain,

skull base, and neck with and without contrast revealed a

remaining soft tissue mass in the right nasopharynx

obliterating the fossa of Rosenmller, without extension to

the skull base or adjacent soft tissues. Computed tomo-

graphic scans of the chest and abdomen failed to reveal anymetastatic disease. An endoscopic transnasal, transpterygoid

approach was performed for the resection of the tumor. The

surgical margins were free of tumor. She received external

radiotherapy postoperatively and remains disease-free at

27 months follow-up.

3. Discussion

Follicular dendritic cell sarcomas, also known as dendritic

reticulum cell tumor, is an extremely rare malignant

Fig. 1. (A) Follicular cell dendritic sarcoma composed of spindled and epithelioid cells forming infiltrative nodules surrounding nasopharyngeal mucoserous

glands with focal myxoid stroma (right side) and containing scattered small lymphocytes with areas of clustering in areas uninvolved by tumor (original

magnification 100). (B) Neoplastic FDCs composed of ovoid cells with eosinophilic cytoplasm and indistinct borders. Nuclei are vesicular with small nucleoli

and occasional mitotic figures (arrow). Characteristic of this tumor, the infiltrate contains scattered small nonneoplastic lymphocytes (original magnification

400). The tumor cells showed immunohistochemical positivity for the FDC markers CD21 (C) and CD23 (D) with characteristic strong cytoplasmic reactivity

(original magnification 200).

263R.S. Encabo et al. / American Journal of OtolaryngologyHead and Neck Medicine and Surgery 29 (2008) 262264

7/27/2019 Nasopharynx Sarcoma

3/3

neoplasm of recent description and characterization [2-6]. It

seems to arise from antigen-presenting cells in B-lymphoid

follicles of nodal and extranodal sites and can occur virtually

anywhere in the body [5,7]. These include multiple sites of

origin including both nodal and extranodal sites, including

the head and neck region [5]. Less than 40 cases in the head

and neck have been reported in the literature since it was first

described [2]. Approximately 30% of the cases were located

in extranodal sites [8], and less than 20 cases have been

reported in the pharyngeal region [4]. Follicular dendritic

cell sarcoma of the nasopharynx is extremely rare, and we

identified only 4 previously reported cases [3,5,6,9].

This tumor is often underdiagnosed because it is easily

confused with other entities [2]. Diagnosis by punch biopsy

has often led to erroneous diagnosis because of the variety

of histologic patterns [3]. Recognition of extranodal FDC

sarcoma requires a high index of suspicion, especially when

they occur in extranodal sites of the head and neck region.

Follicular dendritic cell sarcoma, however, has numerous

distinctive histologic features that should bring theneoplasm into the differential diagnosis [3]. Correct

characterization of this neoplasm is imperative given

its potential for recurrence and metastases [3,4]. Tumor

cells typically express CD21, CD23, CD35, Ki-M4p,

Ki-FDRC1p, and vimentin, with occasional positivity for

S-100 protein, muscle-specific actin, and epithelial mem-

brane antigen [2,3].

In adults, this neoplasm originally was considered to be a

low-grade malignancy; however, high recurrence rates

(36%) and metastases (28%) have been documented [2,8].

The most effective treatment is complete surgical excision.

Adjuvant radiotherapy or chemotherapy appears to be

indicated in cases having adverse pathologic features and

in recurrent or incompletely resected lesions [8].

References

[1] Desai S, Deshpande RB, Jambhekar N. Follicular dendritic cell tumor of

the parapharyngeal region. Head Neck 1999;21(2):164-7.

[2] Vargas H, Mouzakes J, Purdy SS, et al. Follicular dendritic cell tumor:

an aggressive head and neck tumor. Am J Otolaryngol 2002;23(2):93-8.

[3] Biddle DA, Ro JY, Yoon GS, et al. Extranodal follicular dendritic cell

sarcoma of the head and neck region: three new cases, with a review of

the literature. Mod Pathol 2002;15(1):50-8.

[4] Dominguez-Malagn H, Cano-Valdez AM, Mosqueda-Taylor A.

Follicular dendritic cell sarcoma of the pharyngeal region: histologic,

cytologic, inmunohistochemical and ultrastructural study of three cases.

Ann Diagn Pathol 2004;8(6):325-32.

[5] Nakashima T, Kuratomi Y, Shiratsuchi H, et al. Follicular dendritic cell

sarcoma of the neck; a case report and literature review. Auris NasusLarynx 2002;29(4):401-3.

[6] Chan ACL, Chan KW, Chan JKC, et al. Development of follicular

dendritic cell sarcoma in hyaline-vascular Castelman's disease of the

nasopharynx: tracing its evolution by sequential biopsies. Histopathol-

ogy 2001;38(6):510-8.

[7] Galati LT, Barnes EL, Myers EN. Dendritic cell sarcoma of the thyroid.

Head Neck 1999;21(3):273-5.

[8] Perez-ordonez B, Rosai J. Follicular dendritic cell tumor: review of the

entity. Semin Diagn Pathol 1998;15(2):144-54.

[9] Beham-Schmid C, Beham A, Jakse R, et al. Extranodal follicular

dendritic cell tumor of the nasopharynx. 1998;432(3): 293-8.

264 R.S. Encabo et al. / American Journal of OtolaryngologyHead and Neck Medicine and Surgery 29 (2008) 262264