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Year : 2015  |  Volume : 61  |  Issue : 1  |  Page : 55-56

Extranodal follicular dendritic cell sarcoma: A frequently misdiagnosed entity

1 Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication15-Dec-2014

Correspondence Address:
Dr. V D Pai
Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0022-3859.147058

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How to cite this article:
Pai V D, Desai S, Desouza A, Saklani A P. Extranodal follicular dendritic cell sarcoma: A frequently misdiagnosed entity. J Postgrad Med 2015;61:55-6

How to cite this URL:
Pai V D, Desai S, Desouza A, Saklani A P. Extranodal follicular dendritic cell sarcoma: A frequently misdiagnosed entity. J Postgrad Med [serial online] 2015 [cited 2023 Jun 5];61:55-6. Available from:


Follicular dendritic cell (FDC) sarcomas are rare malignancies arising from follicular dendritic cells found in lymph nodes. Diagnosis is based on expression of CD21 and CD35. Extranodal follicular dendritic cell sarcomas are rare with the oral cavity, tonsil, gastrointestinal tract and liver being the sites reported in the literature. There is no consensus on its management since only anecdotal case reports and short case series exist in the literature. We are reporting a case of extranodal follicular dendritic cell sarcoma arising from the mesentery, which was misdiagnosed and treated as gastrointestinal stromal tumor (GIST).

A twenty-two year old male presented with pain in abdomen of 6 months duration. CECT abdomen revealed a mesenteric mass. The mass was excised and final histopathology was read as high-risk GIST. Hence, he was given adjuvant therapy with oral Imatinib for 2 years. Then he developed local recurrence. Since it was closely abutting the ileocolic artery, right hemicolectomy was performed. The patient was referred to our center for an opinion regarding further management. On presentation, the patient was largely asymptomatic. CECT abdomen and thorax revealed no residual tumor or lung metastasis. Histopathology was reviewed which showed follicular dendritic cell sarcoma [Figure 1]a. Mitotic rate was 10/10 hpf. On immunohistochemistry (IHC) it was positive for CD23 while negative for C kit, DOG1, AE1/AE3 and S100 [Figure 1]b. In view of high grade nature of the tumor the patient has been advised gemcitabine-Docetaxel-based chemotherapy.
Figure 1: (a) Histopathology showing follicular dendritic cell showing sarcomatous change. (b) On IHC, cells showing CD23 positivity

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FDC sarcoma was first described by Monda et al. in 1986. [1] Follicular dendritic cells are found in primary and secondary follicles. They play an important role in antigen presentation for the B-cell compartment, as well as regulation of the germinal center reaction. Hence, these tumors arise from the lymph nodes in various parts of the body. Extranodal FDC sarcomas are rare and have been reported to arise from various sites in the body including such as head and neck, liver, spleen, gastrointestinal tract, soft tissue, skin, lung and breast. [2] More than one third of these tumors are initially misdiagnosed since IHC markers essential for their diagnosis are not routinely used. On histology, these tumors were composed of spindle arranged in sheets focally exhibiting a storiform or whorled growth pattern. On immunohistochemical testing, they stain positive for CD21, CD35 and CD23. There is no consensus on its management in view of its rarity though majority of these are treated by wide local excision followed by adjuvant therapy in the form of radiotherapy or chemotherapy. [3] Prognosis wise these are considered as intermediate grade sarcoma with a tendency to recurrence and development of distant metastasis. [4]

We are reporting this case in view of the rarity of this malignancy and the importance of IHC in the diagnosis of gastrointestinal malignancy to avoid inappropriate treatment practices.

 :: References Top

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. 1
Li Z, Jin K, Yu X, Teng X, Zhou H, Wang Y, et al. Extranodal follicular dendritic cell sarcoma in mesentery: A case report. Oncol Lett 2011; 2:649-52.  Back to cited text no. 2
Youens KE, Waugh MS. Extranodal follicular dendritic cell sarcoma. Arch Pathol Lab Med 2008; 132:1683-7.  Back to cited text no. 3
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. 4


  [Figure 1]

This article has been cited by
1 Death within 9 months in patient with follicular dendritic cell sarcoma of spleen: A case report
Zhao-Yang Xing
Oncology Letters. 2022; 25(1)
[Pubmed] | [DOI]
2 Clinicopathological characteristics of extranodal follicular dendritic cell sarcoma: A report of two cases
Xing Zhao, Dayong Sun, Gang Zhang
Oncology Letters. 2021; 21(3)
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