Gordon M D, Weilert M, Ireland K
Department of Pathology, Oregon Health Sciences University, Portland, USA.
Obstet Gynecol. 1996 Oct;88(4 Pt 2):699-701. doi: 10.1016/0029-7844(96)00242-6.
Neurofibromatosis involving the female genital tract is unusual. The vulva appears to be most frequently affected, with only rare reports of vaginal, cervical, and ovarian involvement in patients with known von Recklinghausen's disease.
We present a case of diffuse neurofibromatosis involving the cervix, endometrium, myometrium, ovarian cortex, and serosa in a patient with uncontrollable pelvic pain, diagnosed initially as endometriosis. No gross pathology was appreciated, and the histologic features of the small lesions were consistent with plexiform neurofibromas.
The case presented is an example of diffuse involvement of the female genital tract by neurofibromatosis in a patient without a family history of von Recklinghausen's disease and without other clinical manifestations of the disease.
累及女性生殖道的神经纤维瘤病并不常见。外阴似乎是最常受累的部位,仅有罕见报道称患有冯雷克林霍增氏病的患者出现阴道、宫颈和卵巢受累。
我们报告一例弥漫性神经纤维瘤病累及宫颈、子宫内膜、子宫肌层、卵巢皮质和浆膜的病例,该患者有无法控制的盆腔疼痛,最初被诊断为子宫内膜异位症。未发现明显的病理改变,小病变的组织学特征与丛状神经纤维瘤一致。
本病例是一例无冯雷克林霍增氏病家族史且无该疾病其他临床表现的患者,神经纤维瘤病弥漫性累及女性生殖道的病例。