Original Article
Copyright ©2010 Baishideng. All rights reserved.
World J Gastroenterol. May 28, 2010; 16(20): 2504-2519
Published online May 28, 2010. doi: 10.3748/wjg.v16.i20.2504
Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma
Lan Li, Yong-Hong Shi, Zhi-Juan Guo, Tian Qiu, Lei Guo, Hong-Ying Yang, Xun Zhang, Xin-Ming Zhao, Qin Su
Lan Li, Tian Qiu, Lei Guo, Hong-Ying Yang, Xun Zhang, Qin Su, Department of Pathology, Cancer Institute and Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Yong-Hong Shi, Departments of Pathology, the First Affiliated Hospital, Inner Mongolia Medical College, Huhehot 010059, Inner Mongolia Autonomous Region, China
Zhi-Juan Guo, Department of Pathology, Renmin Hospital, Inner Mongolia Medical College, Huhehot 010020, Inner Mongolia Autonomous Region, China
Xin-Ming Zhao, Department of Radiology, Cancer Institute and Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Author contributions: Su Q designed the research and performed pathologic re-examination; Li L, Qiu T, Guo L and Guo ZJ performed the research; Shi YH, Yang HY and Zhang X participated in pathologic re-examination; Zhao XM participated in re-evaluation of the radiological data; Su Q and Li L evaluated data and prepared the manuscript.
Supported by Grants from National Natural Science Foundation of China, No. 30171052, 30572125 and 30772508
Correspondence to: Qin Su, Professor, Department of Pathology, Cancer Institute and Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Panjiayuan Nanli 17, Beijing 100021, China. q.su@wjgnet.com
Telephone: +86-10-87787508 Fax: +86-10-67713359
Received: February 23, 2010
Revised: March 24, 2010
Accepted: March 31, 2010
Published online: May 28, 2010
Abstract

AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.

METHODS: Nine lesions were examined by routine and molecular approaches. Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.

RESULTS: The current lesions were found in five male and four female patients, located mainly in the head and neck area and averaging 7.2 cm in size. Six patients had recurrence or metastasis and three remained free of disease. The 106 patients (male/female ratio, 1.1:1) were aged from 9 to 82 years (median, 44 years). The tumor sizes ranged from 1.5 to 21 cm (mean, 7.4 cm). Abdominal/pelvic region was affected most frequently (43%). Surgical resection was performed in 100 patients, followed by radiation and/or chemotherapy in 35 of them. Follow-up data were available in 91 cases, covering a period of 3-324 mo (mean, 27 mo; median, 19 mo). Of the informative cases, 38 (42%) had recurrence or metastasis, and 12 (13%) died of the disease. These tumors were classified histologically into low- and high-grade lesions. A size ≥ 5 cm (P = 0.003), high-grade histology (P = 0.046) and a mitotic count ≥ 5/10 HPF (P = 0.013) were associated with tumor recurrence. The lesions were defined as low-, intermediate- and high-risk tumors, and their recurrence rates were 16%, 46% and 73%, and their mortality rates 0%, 4% and 45%, respectively.

CONCLUSION: Extranodal FDC tumors behave like soft tissue sarcomas. Their clinical outcomes are variable and can be evaluated according to their sizes and grades.

Keywords: Extranodal follicular dendritic cell sarcoma; Prognosis assessment; Histologic grade; Immunohistochemistry; In situ hybridization; Mutation detection