Murayama Y, Yamamoto Y, Shimojima N, Takahara T, Kikuchi K, Lida S, Kondo Y
Department of Surgery, Nerima General Hospital, Tokyo, Japan.
Breast Cancer. 1999;6(3):227-30. doi: 10.1007/BF02967173.
A rare case of breast cancer associated with von Recklinghausen's neurofibromatosis is reported. This case and review of the literature illustrate the problems of clinical diagnosis.A 66-year-old woman who had undergone sigmoidectomy for sigmoid colon cancer two years previously, was admitted to the hospital because of a left breast skin retraction in October, 1998. The patient had von Recklinghausen's disease (neurofibromatosis type 1). The TNM clinical staging was TlcNOMO. Modified radical mastectomy was performed. The histopathological diagnosis of the breast tumor was invasive ductal carcinoma and the skin tumor was neurofibroma. The pTNM pathological staging was pTlcNlaMO.Among patients similar to our case, almost all were staged higher than T2. This may be because multiple neurofibromas obscure breast mass at palpation, leading to delayed detection of the cancer. Systemic and careful exploration is essential for patients with von Recklinghausen's neurofibromatosis to detect breast cancer at an early stage.
本文报道了一例罕见的与冯雷克林霍增氏神经纤维瘤病相关的乳腺癌病例。该病例及文献回顾阐述了临床诊断中的问题。一名66岁女性，两年前因乙状结肠癌接受了乙状结肠切除术，1998年10月因左乳皮肤凹陷入院。该患者患有冯雷克林霍增氏病（1型神经纤维瘤病）。TNM临床分期为T1cN0M0。实施了改良根治性乳房切除术。乳腺肿瘤的组织病理学诊断为浸润性导管癌，皮肤肿瘤为神经纤维瘤。pTNM病理分期为pT1cN1aM0。在与我们病例相似的患者中，几乎所有患者的分期都高于T2期。这可能是因为多个神经纤维瘤在触诊时掩盖了乳腺肿块，导致癌症检测延迟。对于患有冯雷克林霍增氏神经纤维瘤病的患者，进行全面且仔细的检查对于早期发现乳腺癌至关重要。
