Follicular dendritic cell sarcoma of the nasopharynx: a case report and literature review

Abstract

Follicular dendritic cell sarcoma (FDCS) of the nasopharynx is a rare malignant tumor that has been described in only a few case reports, and its differential diagnoses include diverse clinicopathologic entities. FDCS is often initially misdiagnosed, especially when examining small biopsy specimens. We herein report a case of FDCS arising in the nasopharynx that was initially misdiagnosed as a nerve sheath tumor. A 44-year-old woman presented with persistent obstruction of the left nasal cavity and underwent an excisional biopsy. The specimen demonstrated morphologic and immunohistochemical features of FDCS. In situ hybridization for Epstein–Barr virus-encoded RNA was negative. The patient was treated with chemotherapy and radiotherapy. The sarcoma recurred near the original site more than 3 years after the initial treatment and was completely resected. At the time of this writing, the patient had remained disease-free for 1 year after resection. This case is being reported to improve the clinical recognition of FDCS.

Fine needle aspiration cytology of follicular dendritic cell sarcoma of the stomach masquerading as gastrointestinal stromal tumor: A case report of a unique entity with emphasis on cytomorphology

“…16 The tumor cells are characteristically positive for CD21, CD35, and CD23, with positivity rates of 85%, 55%, and 31.7% respectively. 3 Therefore, a panel of these stains is recommended to help make the diagnosis. Other non-specific positive markers include CD68, S-100, CD1a, and D2-40.…”

“…1 Although FDCS predominantly occurs in lymph nodes, it has also been documented in a variety of extra-nodal sites. [2][3][4] The tumor arises from follicular dendritic cells, which are unique accessory immune cells that contribute to the regulation of humoral immunity. 5 Neoplasms derived from follicular dendritic cells were first reported in 1986 by Monda and Rosai, 6 and the term FDCS was later adopted by the World Health Organization to describe these entities.…”

“…Follicular dendritic cell sarcoma (FDCS) is a rare, low to intermediate‐grade malignancy representing 0.4% of soft tissue sarcomas 1 . Although FDCS predominantly occurs in lymph nodes, it has also been documented in a variety of extra‐nodal sites 2–4 . The tumor arises from follicular dendritic cells, which are unique accessory immune cells that contribute to the regulation of humoral immunity 5 .…”

CT features, Pathological findings and Clinical data of Follicular Dendritic Cell Sarcoma in the Mediastinum: Case Report and Literature Review

“…[1] Histologically, FDC sarcoma comprises neoplastic follicular dendritic cells and close-combined lymphoid cells. [2] Although FDC sarcoma affects lymphoid tissues, it accounts for less than 1% of all lymphoid neoplasms with an extranodal origin. This tumour is an extremely rare malignant neoplasm that mostly occurs in extra-nodal sites (79.4% of cases) and lymph nodes (15.1%); in about 7%-10% of cases, it is associated with hyalinevascular Castleman disease.…”

“…This tumour presents with lymph node disease in 31% of patients, extra-nodal disease in 58%, and both nodal and extranodal disease in 10%. [2] This report aims to alert radiologists about radiological features of the FDC sarcoma marker by multiple mediastinal lymph nodes lesion with necrosis. The CT features and pathological findings of a case of FDC sarcoma in the mediastinum were described.…”