Shimizu H, Yoshihara T, Sakurai H, Nozaki M
Department of Otolaryngology, Tokyo Women's Medical College, Japan.
Auris Nasus Larynx. 1994;21(4):253-7. doi: 10.1016/s0385-8146(12)80090-3.
A case of a huge cervical neurofibroma associated with von Recklinghausen's disease is reported. The patient with von Recklinghausen's disease was a 48-year-old woman complaining of a rapidly growing mass around the neck for the last 6 months. In von Recklinghausen's disease, which corresponds to neurofibromatosis-I, gigantic tumors around the neck often occur. The present case was classified as a plexiform neurofibroma which is derived from the peripheral nervous system, and this type frequently shows malignant change in contrast to other types of neurofibroma. In general, it is difficult to determine the origin of the nerve in cervical neurofibromas. A total extirpation of the tumor was done in our case, and revealed the nerve of origin to be the vagal nerve. Pathologically, H-E stained specimens showed that the tumor was a benign neurofibroma. However, immunostaining of the majority of the tumor cells were positive for PCNA. These results suggest that the tumor of our case possessed a low malignant potential. To date, neither local recurrence nor distant metastasis has been found on subsequent examinations.
报告一例与冯雷克林霍增氏病相关的巨大颈部神经纤维瘤病例。该冯雷克林霍增氏病患者为一名48岁女性，主诉在过去6个月中颈部周围有一个迅速生长的肿块。在与神经纤维瘤病I型相对应的冯雷克林霍增氏病中，颈部周围常出现巨大肿瘤。本病例被归类为源自周围神经系统的丛状神经纤维瘤，与其他类型的神经纤维瘤相比，这种类型经常发生恶变。一般来说，很难确定颈部神经纤维瘤的神经起源。在我们的病例中对肿瘤进行了完整切除，发现起源神经为迷走神经。病理上，苏木精-伊红染色标本显示肿瘤为良性神经纤维瘤。然而，大多数肿瘤细胞的免疫染色对增殖细胞核抗原呈阳性。这些结果表明我们病例中的肿瘤具有低恶性潜能。迄今为止，在后续检查中未发现局部复发或远处转移。
