von Willebrand病を伴うvon Hippel-Lindau病の両側腎細胞癌症例

Abstract

42歳男.右腎下極の腫瘍が大きく, 左腎の腫瘍も多発散在していることから, 両側腎全摘出後に人工透析を行うべきとの考えもあった.しかし, 母親が人工透析を受けており両側腎全摘出を本人が希望しないこと, von Willebrand病があること等を考慮し, 両腎の保存的手術を二期的に行った.術後16ヵ月を経た現在, 再発転移を認めず社会復帰している

A 42-year-old man diagnosed with von Willebrand disease 7 years earlier visited his home doctor with a chief complaint of gross hematuria. Abdominal ultrasonography showed bilateral renal tumors. He was referred to our hospital on June 15, 1993. His mother also had von Hippel-Lindau disease and was treated by hemodialysis after bilateral nephrectomy. The examination and imaging revealed bilateral renal cell carcinoma of von Hippel-Lindau disease associated with von Willebrand disease. Partial nephrectomy and enucleation for a small tumor on the right side and enucleations for five tumors on the left side were performed. Histological diagnosis of all tumors were renal cell carcinoma, clear cell subtype, grade 1 approximately 2. The patient has been well without local recurrence or distant metastasis for 16 months since the operation. This is the first case of bilateral renal cell carcinoma of von Hippel-Lindau disease associated with von Willebrand disease in Japan.