title: Follicular dendritic cell sarcoma of the pharyngeal region.
abstract: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm arising most commonly from follicular dendritic cells in the lymph nodes. It is exceedingly rare in extranodal sites, particularly in the pharyngeal region. The present study reports 3 cases occurring in the pharyngeal region. Case 1 had tonsil and cervical lymph node involvement, while case 3 also had tonsil involvement. Cases 1 and 3 relapsed locally at 3 and 17 months after surgery, respectively. Case 2 was diagnosed with a tumor in the parapharyngeal space and the patient succumbed to the disease 5 months after treatment with combined surgery and chemotherapy. All 3 cases were misdiagnosed initially. Pathological biopsy examination, including histopathology and immunohistochemistry, was essential for diagnosis. The data for 52 cases, including cases from the literature and the present cases, were analyzed. The results indicated that 57% (26/46) of the initial diagnoses were inaccurate, while the recurrence, metastasis and mortality rates were 40, 16 and 10%, respectively. The statistics supported the theory that FDCS of the pharyngeal region is a low-grade sarcoma. Involvement of the tonsils (52%, 27/52) and parapharyngeal space (19%, 10/52) were observed most commonly, while FDCS at various sites showed different prognoses. The various survival rates were calculated in the present study. The large tumors (≥4 cm) had a poorer prognosis than the small tumors (<4 cm; P<0.05). Among the 50 cases with available follow-up data, 46% (23/50) were treated with surgery alone, 52% (26/50) with combination therapy (surgery followed by chemotherapy and/or radiotherapy) and 2% (1/50) with surveillance. There was no statistically significant evidence (P>0.05) that combination therapy improves survival rates, compared with surgery alone.
authors.affiliation: Lymphoma Diagnosis and Treatment Center, Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China ;
authors.forename: Tengpeng
authors.initials: T
authors.lastname: Hu
authors.forename: Xinhua
authors.initials: X
authors.lastname: Wang
authors.forename: Chang
authors.initials: C
authors.lastname: Yu
authors.forename: Jiaqin
authors.initials: J
authors.lastname: Yan
authors.forename: Xundong
authors.initials: X
authors.lastname: Zhang
authors.forename: Ling
authors.initials: L
authors.lastname: Li
authors.forename: Xin
authors.initials: X
authors.lastname: Li
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.forename: Jingjing
authors.initials: J
authors.lastname: Wu
authors.forename: Wang
authors.initials: W
authors.lastname: Ma
authors.forename: Wencai
authors.initials: W
authors.lastname: Li
authors.forename: Guannan
authors.initials: G
authors.lastname: Wang
authors.forename: Wugan
authors.initials: W
authors.lastname: Zhao
authors.forename: Xianzheng
authors.initials: X
authors.lastname: Gao
authors.forename: Dandan
authors.initials: D
authors.lastname: Zhang
authors.forename: Mingzhi
authors.initials: M
authors.lastname: Zhang
keywords: chemotherapy; follicular dendritic cell sarcoma; immunohistochemistry; pharyngeal; radiotherapy; surgery