Rosen P P, Holmes G, Lesser M L, Kinne D W, Beattie E J
Cancer. 1985 Mar 15;55(6):1345-52. doi: 10.1002/1097-0142(19850315)55:6<1345::aid-cncr2820550631>3.0.co;2-b.
Juvenile papillomatosis (JP) is a breast tumor of young women featuring atypical papillary duct hyperplasia and numerous cysts, first described as a clinicopathologic entity in 1980. The disease is of interest because of the youth of these patients and the fact that the pathologic elements resemble those considered to be precancerous in older women. This report summarizes the findings in 180 patients enrolled in the Juvenile Papillomatosis Registry. The mean age at diagnosis was 23 years (range, 12-48 years; median, 21 years; mode, 18 years). No relationship was found with parity, age of menarche, or use of birth control pills. Fifty patients (28%) reported that one or more relatives had had breast cancer. Twelve (7%) patients reported a first degree relative (mother, 10; sister, 2), whereas in 38 (21%) breast cancer was limited to one or more secondary relatives, most often maternal (23/39 or 59%). Persistent (or recurrent) JP was found in 25 patients who had a subsequent biopsy, and 8 patients had bilateral JP. Pathologic analysis revealed focal epithelial necrosis, a feature often associated with carcinoma, in 30 (17%) cases predominantly in those with the most severe cytologic atypia of hyperplastic duct lesions (77%). Positive family history was reported by 33% (10/30) of women with epithelial necrosis and 27% (40/150) without this finding. Seven patients (4%) had breast carcinoma diagnosed concurrent with JP (ipsilateral, 5 cases; contralateral, 2 cases). A previously unreported finding is the diagnosis of carcinoma subsequent to JP in two cases (contralateral intraductal and ipsilateral microinvasive duct, respectively) with intervals of 8 and 9 years, respectively. The nine patients with carcinoma and JP tended to be slightly older at the time JP was diagnosed (mean, 27 years) and had a higher frequency of positive family history (56%) than those without breast cancer (mean age, 23 years; positive family history, 26%). These findings confirm earlier evidence that JP is a marker for families at risk and now indicate that the patient with JP may herself be in jeopardy of developing breast cancer coincidentally or at a later date. Wide local excision is adequate to control the lesion in most cases. Careful clinical surveillance is indicated for any woman who has juvenile papillomatosis and for her female relatives. The early age of onset of this unusually florid proliferative disease, and family history association, suggest there may be an underlying hormonal disturbance or genetic abnormality.
青少年乳头状瘤病（JP）是年轻女性的一种乳腺肿瘤，其特征为非典型乳头状导管增生和大量囊肿，于1980年首次被描述为一种临床病理实体。由于这些患者较为年轻，且病理特征与中老年女性中被认为可能是癌前病变的特征相似，因此这种疾病备受关注。本报告总结了青少年乳头状瘤病登记处登记的180例患者的研究结果。诊断时的平均年龄为23岁（范围12 - 48岁；中位数21岁；众数18岁）。未发现与生育次数、初潮年龄或口服避孕药的使用有关。50例患者（28%）报告有一位或多位亲属患过乳腺癌。12例（7%）患者报告有一级亲属（母亲10例；姐妹2例），而38例（21%）患者的乳腺癌局限于一位或多位二级亲属，大多数是母系亲属（23/39或59%）。在25例接受后续活检的患者中发现了持续性（或复发性）JP，8例患者双侧患有JP。病理分析显示30例（17%）病例存在局灶性上皮坏死，这一特征常与癌相关，主要见于增生性导管病变中具有最严重细胞学非典型性的病例（77%）。有上皮坏死的女性中33%（10/30）报告有阳性家族史，无此发现的女性中27%（40/150）报告有阳性家族史。7例患者（4%）在诊断JP的同时被诊断出患有乳腺癌（同侧5例；对侧2例）。有两个病例（分别为对侧导管内癌和同侧微浸润性导管癌）在诊断JP后分别间隔8年和9年被诊断出患有癌，这是一个此前未报告过的发现。9例患有癌和JP的患者在诊断JP时年龄往往稍大（平均27岁），且阳性家族史的发生率（56%）高于无乳腺癌的患者（平均年龄23岁；阳性家族史26%）。这些发现证实了早期的证据，即JP是有风险家族的一个标志，现在表明患有JP的患者自身可能会在偶然情况下或日后患乳腺癌。在大多数情况下，广泛局部切除足以控制病变。对于任何患有青少年乳头状瘤病的女性及其女性亲属，都需要进行仔细的临床监测。这种异常活跃的增生性疾病发病年龄较早，且与家族史相关，提示可能存在潜在的激素紊乱或基因异常。
