神经纤维瘤病为家族性疾病。一般多在出生时发生,小儿时发病,青春后期加重,而年龄愈大病症愈重。神经纤维瘤来源于不成熟的神经鞘细胞,肿瘤为多发性,局限于皮下,无包膜。发生于乳房内巨大神经纤维瘤则罕见。患者,女,51岁,因右侧乳房进行性肿大二十余年,破溃十余年入院。患者自述出生后发现右侧乳房没有乳头,18岁时,该乳房变平,缺乏弹性,无包块,左侧乳房饱满。26岁分娩后,右侧乳房逐渐增大,但无乳汁分泌,全身出现大小不等的结节。40岁分娩以后,右侧乳房增大达小儿头大小,局部破溃流脓,有臭味,创面经过治疗后,愈合。但又反复发作。最近一年,患侧乳房生长较速,全身乏力,消瘦,走路时心慌, Neurofibromatosis is a familial disease. It usually occurs at birth, when it develops in children, it becomes more severe later in life, and the older the illness, the heavier the disease. Neurofibromas are derived from immature ensheathing cells, with multiple tumors, subcutaneous and no capsules. Occurrence of giant neurofibromas in the breast is rare. The patient, female, 51 years old, had suffered a progressive enlargement of the right breast for more than 20 years and was hospitalized for more than 10 years. After the patient reported to be born, he found that there was no nipple in the right breast. At the age of 18, the breast was flattened, lacking elasticity, no mass, and the left breast was full. After delivery at the age of 26, the right breast was gradually enlarged but no milk was secreted and nodules of various sizes appeared throughout the body. After the 40-year-old childbirth, the right breast enlargement reaches the size of the head of the child, which partially ruptures the pus and has an odor. The wound is healed after treatment. However, it recurred. In the most recent year, the ipsilateral breasts have developed rapidly, the body is weak, weight loss, and palpitation when walking.