Campo-Paysaa A, Stagnara P, Fauchet R, Biot B, Nouvel A
Chir Pediatr. 1982;23(6):411-3.
The authors report a case of severe Recklinghausen's disease, revealed early in infancy, by spinal deformities and then by severe dislocations with kypho-scoliosis surgically treated by anterior and posterior fusion. Other localisations of neurofibromatosis were present, especially bones and skin. During the disease's course a wide sub-cutaneous tumor of the chest wall was removed. Two years later a large abdomino-pelvic tumor of the mesentery was discovered and also removed. Histologic examination showed it to be related to Recklinghausen's disease and malignant. The authors analyse the risk of malignant degeneration of this tumor with relation to previous surgical treatment.
作者报告了一例严重的冯雷克林霍增氏病，该病在婴儿期早期就因脊柱畸形而被发现，随后又出现严重脱位并伴有脊柱后凸-脊柱侧凸，通过前后融合术进行了手术治疗。还存在神经纤维瘤病的其他定位表现，尤其是骨骼和皮肤。在病程中，切除了胸壁一个广泛的皮下肿瘤。两年后，发现并切除了肠系膜一个巨大的腹盆腔肿瘤。组织学检查显示其与冯雷克林霍增氏病有关且为恶性。作者分析了该肿瘤恶性变的风险与既往手术治疗的关系。
