Goodlad J R, MacPherson S, Jackson R, Batstone P, White J
Department of Pathology, Highland Acute Hospitals NHS Trust, Raigmore Hospital, Inverness, UK.
Histopathology. 2004 Mar;44(3):268-76. doi: 10.1111/j.1365-2559.2004.01804.x.
Recent studies of primary cutaneous follicular lymphoma suggest that it represents a clinicopathological entity distinct from nodal follicular lymphoma (FL). The purpose of this study was to determine if FL arising at other extranodal sites is more closely related to FL occurring in the skin or in lymph nodes.
Fifteen cases of non-cutaneous extranodal follicular lymphoma (ENFL) were identified from the Scotland and Newcastle Lymphoma Group (SNLG) database. All were stage 1E at presentation and involved the tonsil (n = 3), palate (n = 3), skeletal muscle (n = 2), ileum (n = 2), duodenum (n = 1), stomach (n = 1), thyroid gland (n = 1), submandibular gland (n = 1) and fallopian tube (n = 1). Polymerase chain reaction for t(14;18) using primers to the major breakpoint cluster region was performed on 14 cases of ENFL and the incidence of the translocation compared with that found in 16 cases of stage 1 nodal FL. Clinical and follow-up data were obtained from the SNLG database for the 15 cases of ENFL and 87 cases of stage 1 nodal FL, and a comparison of outcomes made. Only 2/14 cases of ENFL had detectable t(14;18) compared with 9/16 stage 1 nodal FL (P < 0.01). Freedom from progression and disease-specific survival was similar for the 15 cases of ENFL and 87 cases of stage 1 nodal FL. However, 13/15 patients with ENFL were disease free at the end of follow-up compared with 49/87 stage 1 nodal FL (P < 0.02).
The low incidence of t(14;18) and favourable outcome encountered in ENFL in this study is similar to that previously found for primary cutaneous FL. These results support the concept of a subtype of FL lacking t(14;18) involving the major breakpoint cluster region, and with a propensity to arise at extranodal sites. Despite a high relapse rate, patients with ENFL are more likely to achieve complete remission and may ultimately have a more favourable long-term prognosis than those with equivalent nodal disease.
近期对原发性皮肤滤泡性淋巴瘤的研究表明，它是一种与淋巴结滤泡性淋巴瘤（FL）不同的临床病理实体。本研究的目的是确定发生于其他结外部位的FL与发生于皮肤或淋巴结的FL是否关系更为密切。
从苏格兰和纽卡斯尔淋巴瘤组（SNLG）数据库中识别出15例非皮肤结外滤泡性淋巴瘤（ENFL）病例。所有病例初诊时均为ⅠE期，累及扁桃体（3例）、腭部（3例）、骨骼肌（2例）、回肠（2例）、十二指肠（1例）、胃（1例）、甲状腺（1例）、颌下腺（1例）和输卵管（1例）。对14例ENFL病例进行了使用主要断裂点簇区域引物的t(14;18)聚合酶链反应，并将易位发生率与16例Ⅰ期淋巴结FL中的发生率进行比较。从SNLG数据库中获取了15例ENFL病例和87例Ⅰ期淋巴结FL病例的临床及随访数据，并对结果进行了比较。ENFL病例中仅有2/14可检测到t(14;18)，而Ⅰ期淋巴结FL病例中有9/16可检测到（P<0.01）。15例ENFL病例和87例Ⅰ期淋巴结FL病例的无进展生存期和疾病特异性生存期相似。然而，随访结束时，15例ENFL患者中有13例无疾病，而87例Ⅰ期淋巴结FL患者中有49例无疾病（P<0.02）。
本研究中ENFL病例中t(14;18)的低发生率和良好预后与先前原发性皮肤FL的情况相似。这些结果支持了一种缺乏涉及主要断裂点簇区域的t(14;18)且倾向于发生于结外部位的FL亚型的概念。尽管复发率较高，但ENFL患者比同等淋巴结疾病患者更有可能实现完全缓解，最终可能具有更良好的长期预后。
