The indolent course and high incidence of t(14;18) in primary duodenal follicular lymphoma.
BACKGROUND
Information on the clinical behavior of the recently proposed primary duodenal follicular lymphoma (DFL) is limited.


METHODS
Demographic data, signs, symptoms, disease stage, and treatment of the patients diagnosed in National Cancer Center Hospital from 1999 to 2007 were collected and analyzed.


RESULTS
Twenty-seven patients were studied. Nineteen patients were asymptomatic at the time of diagnosis. Twenty patients had stage I disease. The histological grade was 1 or 2 in 26 patients. IgH/BCL2 fusion was shown in 20 of the examined 24 cases (83%). Fourteen patients received therapy upon diagnosis (local radiotherapy in 2 patients and chemotherapy in 12 including rituximab therapy), their response rate was 85%, and the estimated progression-free survival (PFS) rate at 3 years was 70%. One patient developed histological transformation. The other 13 patients were followed up; their estimated PFS rate at 3 years was 74%. Five among six cases responded to treatment even after progressive disease. All 27 patients have survived with a median follow-up time of 47.9 months.


CONCLUSIONS
The majority of primary DFL patients have a localized tumor of low-grade histology and are positive for t(14;18). Watchful waiting might be an alternative approach for its indolent course; however, further studies are warranted.
Mori
M
M
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Kobayashi
Y
Y
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan. Electronic address: ykkobaya@ncc.go.jp.
Maeshima
A M
AM
Division of Clinical Laboratory, National Cancer Center Hospital, Tokyo, Japan.
Gotoda
T
T
Division of Endoscopy, National Cancer Center Hospital, Tokyo, Japan.
Oda
I
I
Division of Endoscopy, National Cancer Center Hospital, Tokyo, Japan.
Kagami
Y
Y
Division of Radiation Oncology, National Cancer Center Hospital, Tokyo, Japan.
Bennett
S
S
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Nomoto
J
J
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Azuma
T
T
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Yokoyama
H
H
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Maruyama
D
D
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Kim
S-W
SW
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Watanabe
T
T
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
Matsuno
Y
Y
Division of Clinical Laboratory, National Cancer Center Hospital, Tokyo, Japan.
Tobinai
K
K
Division of Hematology, National Cancer Center Hospital, Tokyo, Japan.
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics*; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics*; D020732:Cytogenetic Analysis; D018450:Disease Progression; D004379:Duodenal Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008224:Lymphoma, Follicular / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate; D014178:Translocation, Genetic / Q000235:genetics*; D016896:Treatment Outcome