A 65-year-old woman presented with gross hematuria in February 1997. Left renal tumor was revealed and radical nephrectomy was performed. Pathological examination revealed papillary renal cell carcinoma, pT3aN1M1 (ipsilateral adrenal gland). Interferon-alpha was administered for 1 year. Two years after the nephrectomy, metastasis to the left supraclavicular lymph node appeared. Seven years after the nephrectomy, the metastatic tumor invaded the brachiocephalic vein and extended to the superior vena cava (SVC), compatible with SVC syndrome. Although interferon-alpha and external-beam radiotherapy was performed, she died in February 2005. Autopsy revealed a left supraclavicular lymph node metastasis invading the thyroid gland, mediastinum, and brachiocephalic vein. The tumor thrombus descended via the SVC into the right atrium. The right lung artery was obstructed by tumor thrombus. There were no visceral metastases and no local recurrence.
Adrenocortical and ureteral carcinomas were observed in a 50-year-old Japanese woman with Werner syndrome (MIM No. 27770). The syndrome is an autosomal recessive disorder characterized by premature aging and an increased risk of rare cancers, which are often multiple. This is the first reported association of adrenocortical carcinoma in Werner syndrome.
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