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Follicular dendritic cell sarcoma of a cervical lymph node: case report and review of the literature.

Abstract

Follicular dendritic cell sarcoma is a rare tumor that occurs mainly in lymph nodes. We report a case of follicular dendritic cell sarcoma in a cervical lymph node that was initially diagnosed as a B-cell lymphoma by Tru-Cut biopsy. The correct diagnosis was established by excision biopsy and immunohistochemistry. This tumor is of interest to head and neck surgeons because its recurrence rate is significant and its metastatic potential has been underestimated because of its rarity and the difficulty in making the diagnosis. We discuss the salient clinical and pathologic features of this tumor, as well as its management protocol, and we review the literature.

Introduction

Dendritic cells are potent antigen-presenting cells that have the ability to initiate primary immune responses. Their main role is to capture and present antigen and immune complexes. (1) These cells are present in lymph nodes, in nonlymphoid organs (e.g., the liver), in the gastrointestinal tract, and on epithelial surfaces. Follicular dendritic cells are classified as cells of the accessory lymphoid system; they are also known as dendritic reticulum cells. (2) Proliferation of follicular dendritic cells occurs in a number of reactive and neoplastic conditions, including reactive follicular hyperplasia, follicular lymphoma, mantle cell lymphoma, nodular lymphocyte-predominant Hodgkin's lymphoma, and angioimmunoblastic T-cell lymphoma. Follicular dendritic cell sarcoma has been previously reported in the lymph nodes of the cervical and axillary regions (3) and extranodally in the mediastinum, (4) tonsil, (5) soft palate, (5) parapharyngeal region, (6) thyroid, (7) gastrointestinal tract, (8) and liver. (9)

Case report

A 74-year-old man presented with a 3-month history of a swelling over the left side of his neck. He was otherwise asymptomatic. Examination revealed the presence of a firm, mobile, 2 x 2-cm swelling in the anterior triangle on the left side. Findings on the remainder of the physical examination were normal. Analysis of a Tru-Cut biopsy revealed the presence of an infiltrate of medium- to large-sized lymphoid cells with irregular nuclei and nucleoli. Immunohistochemical staining was positive for CD79, and a moderate number of reactive T cells stained with CD3. These findings were considered to be suspicious for lymphoma, and a subsequent excision biopsy of the node was undertaken. The patient also underwent random biopsies of the draining region, a left tonsillectomy, and panendoscopy. Findings on computed tomography (CT) of the neck, which was undertaken after the histopathologic examination of the excised lymph node, were normal.

On gross inspection, the 4.0 x 3.5 x 2.5-cm specimen had a rubbery consistency and well-defined margins. Microscopic examination identified a follicular dendritic cell tumor with plump spindle cells that formed meningiomalike whorls and large vascular spaces that were intermingled with small lymphocytes (figure). Immunohistochemically, the spindle cells were positive for CD21 and vimentin and negative for S-100.

[FIGURE OMITTED]

The patient underwent regular clinic follow-up and did not require any adjuvant therapy. At the 8-month postoperative evaluation, his CT was normal. At 4 years and 6 months, he remained disease-free.

Discussion

Follicular dendritic cell sarcomas are finn tumors that vary greatly in their shape, color, and size; intraabdominal tumors tend to be larger than those at other sites. (10) Microscopically, nodal tumors of the head and neck tend to have a "pushing" rather than a permeating margin and are traversed by a sclerotic band or delicate fibrovascular septa. (11) The tumor cells can be spindly, ovoid, or polygonal, and they can exhibit a storiform, fascicular, whorled, diffuse, follicle-like, or trabecular pattern. Most tumors have more than one growth pattern. The cellular whorls are usually circular and centered on a blood vessel. In most cases, the tumor completely replaces the nodal architecture. (10)

The tumor cells exhibit a moderate amount of lightly eosinophilic cytoplasm and indistinct cell borders, giving rise to a syncytial appearance. The nuclei are oval to round, and their contours are usually smooth. Nucleoplasm can be clear or granular. Mitotic counts vary from case to case, ranging from 1 to 20 per high-power field (HPF). Coagulative necrosis is also seen in some cases. Another notable feature is the presence of a rich network of small blood vessels. A constant and highly characteristic feature is that the entire tumor is sprinkled throughout with small lymphocytes. Perivascular cuffing with lymphocytes is also noted in some cases. (11) In 1998, Fonseca et al published immunohistochemical reactivities in follicular dendritic cell sarcoma (table). (12)

Follicular dendritic cell sarcoma most commonly presents in a young adult with lymphadenopathy (64% of all cases). (11) Various parameters affect outcomes. Because most reported series have been retrospective in nature, there is a lack of uniformity of treatment reported in the literature. Greater morbidity and mortality appear to be associated with tumors larger than 6 cm, intraabdominal tumors, a lack of postoperative adjuvant therapy, and the presence of coagulative necrosis, a high mitotic count (>5/HPF), and moderate nuclear pleomorphism. (10,11) Perez-Ordonez and Rosai reported that the association between a poor outcome and an intraabdominal location and marked nuclear pleomorphism is statistically significant. (10) According to reports, the overall recurrence rate is 43%, the metastasis rate is 24%, the mortality rate is 17%, and the median time to recurrence is 12 months. (3,5,11)

From available studies, it is evident that follicular dendritic cell sarcoma is aggressive and should be regarded as a potentially recurrent and metastatic disease entity. Surgery appears to be the clear choice for operable tumors, and adjuvant chemotherapy or radiotherapy should be considered for tumors with marked nuclear pleomorphism and for incompletely resected tumors. Although follicular dendritic cell sarcomas are uncommon, they should be considered in the differential diagnosis of tumors in the head and neck region.
Table. Observed immunohistochemical reactivities (12) in follicular
dendritic cell sarcoma

Antigen No. tests No. (%) positive

CD20 25 1 (4.0)
CD21 44 41 (93.2)
CD35 44 39 (88.6)
Vimentin 23 14 (60.9)
EMA * 17 7 (41.2)
S-100 42 13 (31.0)
Ki-M4 17 16 (94.1)
Ki-FDC1p 8 7 (87.5)

* Epithelial membrane antigen.


References

(1.) Steinman RM. The dendritic cell system and its role in immunogenicity. Annu Rev Immunol 1991;9:271-96.

(2.) Foucar K, Foucar E. The mononuclear phagocyte and immunoregulatory effector (M-PIRE) system: Evolving concepts. Semin Diagn Pathol 1990;7:4-18.

(3.) Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: Report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996;20:944-55.

(4.) Fassina A, Marino F, Poletti A, et al. Follicular dendritic cell tumor of the mediastinum. Ann Diagn Pathol 2001;5:361-7.

(5.) Chan JK, Tsang WY, Ng CS, et al. Follicular dendritic cell tumors of the oral cavity. Am J Surg Pathol 1994;18:148-57.

(6.) Desai S, Deshpande RB, Jambhekar N. Follicular dendritic cell tumor of the parapharyngeal region. Head Neck 1999;21:164-7.

(7.) Galati LT, Barnes EL, Myers EN. Dendritic cell sarcoma of the thyroid. Head Neck 1999;21:273-5.

(8.) Hollowood K, Stamp G, Zouvani I, Fletcher CD. Extranodal follicular dendritic cell sarcoma of the gastrointestinal tract. Morphologic, immunohistochemical and ultrastructural analysis of two cases. Am J Clin Pathol 1995;103:90-7.

(9.) Shek TW, Ho FC, Ng IO, et al. Follicular dendritic cell tumor of the liver. Evidence for an Epstein-Barr virus-related clonal proliferation of follicular dendritic cells. Am J Surg Pathol 1996;20:313-24.

(10.) Perez-Ordonez B, Rosai J. Follicular dendritic cell tumor: Review of the entity. Semin Diagn Pathol 1998; 15:144-54.

(11.) Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer 1997;79:294-313.

(12.) Fonseca R, Yamakawa M, Nakamura S, et al. Follicular dendritic cell sarcoma and interdigitating reticulum cell sarcoma: A review. Am J Hematol 1998;59:161-7.

Kanagasabai Sahadevan, MRCSEd; Anirvan Banerjee, FRCS (Otol); Richard Wight, FRCS (Otol)

From the Department of Urology, Freeman Hospital, Newcastle upon Tyne, U.K. (Dr. Sahadevan), and the Department of Otolaryngology, James Cook University Hospital, Middlesbrough, U.K. (Dr. Banerjee and Dr. Wight).

Reprint requests: Kanagasabai Sahadevan, 29 Greenlee Dr., Haydon Grange, Newcastle upon Tyne NE7 7GA, UK. Phone: 44-191-266-0965; fax: 44-191-213-5688; e-mail: ksahadevan@doctors.org.uk
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Author:Wight, Richard
Publication:Ear, Nose and Throat Journal
Date:Dec 1, 2004
Words:1356
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