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Year : 2010  |  Volume : 53  |  Issue : 1  |  Page : 175-177
Extra nodal follicular dendritic cell tumor

Department of Pathology, JSS Medical College, Mysore, Karnataka, India

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Date of Web Publication19-Jan-2010

How to cite this article:
Suchitha S, Sheeladevi C S, Sunila R, Manjunath G V. Extra nodal follicular dendritic cell tumor. Indian J Pathol Microbiol 2010;53:175-7

How to cite this URL:
Suchitha S, Sheeladevi C S, Sunila R, Manjunath G V. Extra nodal follicular dendritic cell tumor. Indian J Pathol Microbiol [serial online] 2010 [cited 2023 May 30];53:175-7. Available from:


Follicular dendritic cells (FDC) are non-lymphoid, non-phagocytic accessory cells of the immune system. [1] Extra nodal FDC tumor is rare and can occur in a wide variety of sites. [2] Recognition of extra nodal FDC tumor requires a high index of suspicion. Correct characterization of this neoplasm is imperative given its potential for recurrence and metastasis. [3]

A 63-year-old male presented with dysphagia since six months and blood tinged expectoration of 20 days duration. His intra oral examination revealed an irregularly enlarged firm tonsil. A contrast computerized tomography (CT) scan of the neck revealed an 'enhancing mass lesion' measuring 3.1 x 3.0 cm involving the pharyngeal mucosal space in the region of oropharynx on the left side [Figure 1].

The patient underwent a wide excision of his left tonsil. Grossly, the specimen was an irregular gray white tissue mass, measuring 4.2x4x2cm. Cut section of the mass was gray white with areas of hemorrhage.

Microscopically, there was a tumor beneath the stratified squamous epithelium [Figure 2], composed of spindly to ovoid cells with moderate amount of eosinophilic cytoplasm having fibrillary character, oval to round vesicular nuclei with distinct nucleoli and indistinct cell borders, giving a syncytial appearance. Sprinkling of lymphocytes, rich network of blood vessels and perivascular cuffing of lymphocytes were conspicuous [Figure 3]. Moderate nuclear pleomorphism was seen. The tumor cells were arranged in fascicular, storiform and whorled pattern. Mitotic figures numbering five to eight / 10 high power field (hpf) and multinucleated giant cells were noted [Figure 4]. Differential diagnoses of spindle cell carcinoma, lymphoma and follicular dendritic cell tumor were considered. On immunohistochemistry, the tumor cells expressed CD21, CD35 and S100P. They were negative for HMB45, cytokeratin (CK) and epithelial membrane antigen (EMA). A diagnosis of follicular dendritic cell tumor was made.

The patient received post-operative radiotherapy and is asymptomatic after eight months of treatment. Follicular dendritic cell (FDC) tumor, first reported in 1986 in a series of four cases, is a rare neoplastic proliferation of FDCs. [4] The differential diagnoses of FDC tumor at extra nodal sites, particularly in the tonsil, are large cell lymphoma and undifferentiated carcinomas of nasopharyngeal type. These lack immunoreactivity for FDC specific markers. [5] Syncytial sheets of cytokeratin negative spindle cells having bland nuclei with small but distinct nucleoli, focal storiform areas arranged in centripetal whorls with sprinkling of mature lymphocytes and focal multinucleate giant cells should bring FDC sarcoma into consideration regardless of the site. [3],[5] Interpretation of FDC as undifferentiated carcinoma or lymphoma may lead to a completely different line of treatment with its attendant morbidity. [5] If the possibility of FDC is not considered, this diagnosis is not reached because FDC markers are not routinely used in the immunohistochemical evaluation of poorly differentiated neoplasms. [6]

CD21 and CD35 antibodies are the most widely used FDC markers. The immunophenotypic profile of FDC tumors is as follows: positive for FDC markers; frequently positive for EMA, vimentin, desmoplakin, HLA-DR; occasionally positive for S-100P, CD68, leucocyte common antigen. [2],[6]

FDC tumor should be considered to be at least of intermediate grade malignancy. [6] Complete surgical resection is the therapy of choice, with the possibility of adjuvant radiation or chemotherapy. The optimal combination treatment for FDC sarcoma has yet to be defined. [2]

The present case reiterates the need to consider FDC in the differential diagnoses of poorly differentiated spindle cell neoplasms. A suspicion of FDC can only be resolved by immunohistochemistry. Rarity of the lesion, role of immunohistochemistry and positive clinical outcome are the highlights of this report.

   Acknowledgment Top

We would like to express gratitude to Dr. Sangeeta Desai, Professor in Pathology, Tata Memorial Hospital, Mumbai, for help rendered in immunohistochemical diagnosis.

   References Top

1.Mcduffie C, Lian TS, Thibodeaux J. Follicular dendritic cell sarcoma of tonsil: A case report and literature review. Ear Nose Throat J 2007;86:234-5.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Youens KE, Waugh MS. Extranodal follicular dendritic cell sarcoma. Arch Pathol Lab Med 2008;132:1683-7.   Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Biddle DA, Ro JY, Yoon GS, Yong YW, Ayala AG, Ordonez NG, 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;15:50-8.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Monda L, Warnke R, Rosai J. A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation. A report of 4 cases. Am J Pathol 1986;122:562-72.   Back to cited text no. 4      
5.Bothra R, Pai PS, Chaturvedi P, Majeed TA, Singh C, Gujral S, et al. Follicular dendritic cell tumour of tonsil - is it an under-diagnosed entity?. Indian J Cancer 2005;42:211-4.  Back to cited text no. 5      
6.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.  Back to cited text no. 6      

Correspondence Address:
S Suchitha
#892, I Block, 1st cross, Ramakrishnanagar, Mysore-570 022
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.59224

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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