Follicular dendritic cell sarcoma of small intestine with aberrant T-cell marker expression

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Follicular dendritic cell sarcoma (FDCS) is an uncommon neoplasia usually occurring in lymphoid tissue. Herein is present a case of FDCS of the small intestine with positivity for T-cell antigen, simulating T-cell lymphoma. An 82-year-old man consulted a doctor for epigastric pain of one-week duration. Imaging study revealed a mass in the small intestine. Malignant lymphoma was suspected because of high serum levels of soluble interleukin-2 receptor, and resection of the tumor was performed. Microscopically, the tumor was comprised of large pleomorphic cells with reactive small lymphocytes. Most of the nuclei of the tumor cells were round or ovoid-shaped, and some of the tumor cells also showed spindle-shaped nuclei. Although the tumor cells were diffusely positive for CD45RO and CD4 immunohistochemically, negativity for pan-T-cell markers and CD56 were unusual for T-cell lymphoma of intestinal origin. Additional immunohistochemical study demonstrated that the tumor cells were positive for follicular dendritic cell markers including CD23, CD35 and CAN.42, and diagnosis of FDCS was made. To our knowledge, this is the first case of FDCS aberrantly expressing CD45RO, and FDCS expressing T-cell markers can be a pitfall for diagnosis of FDCS.
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