Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma.
OBJECTIVE
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 > or = 5 cm (P = 0.003), high-grade histology (P = 0.046) and a mitotic count > or = 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.


CONCLUSIONS
Extranodal FDC tumors behave like soft tissue sarcomas. Their clinical outcomes are variable and can be evaluated according to their sizes and grades.
Li
Lan
L
Department of Pathology, Cancer Institute and Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Panjiayuan Nanli 17, Beijing 100021, China.
Shi
Yong-Hong
YH
Guo
Zhi-Juan
ZJ
Qiu
Tian
T
Guo
Lei
L
Yang
Hong-Ying
HY
Zhang
Xun
X
Zhao
Xin-Ming
XM
Su
Qin
Q
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D054740:Dendritic Cell Sarcoma, Follicular / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D012196:Review Literature as Topic; D012509:Sarcoma / Q000473:pathology; D016896:Treatment Outcome; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism; D055815:Young Adult
D016159:Tumor Suppressor Protein p53