虽然AA淀粉样变性主要是由炎症引起的,AA淀粉样变性和实体癌之间的关联偶尔被描述。在这里,我们报道了一例48岁的男性患者,该患者切除了伴有AA淀粉样蛋白沉积的增生性毛囊瘤,从而缓解了伴随的AA胃肠道淀粉样变性.一个快速增长的,巨人,带红色,在我们医院就诊时,在左上臂发现了16×13cm大小的溃疡肿瘤。同时通过结直肠黏膜活检诊断为胃肠道AA淀粉样变性,体重减轻和大量腹泻是临床上明显的。作为治疗,切除肿瘤,切缘为10mm.组织学上,肿瘤主要包括周围嗜碱性细胞的小叶增殖,充满了嗜酸性粒细胞,在嗜碱性细胞中观察到的角化物质和阴影细胞有丝分裂。标本显示嗜酸性粒细胞,肿瘤巢周围均匀的沉积物,通过刚果红染色阳性染色证实为淀粉样沉积物。这些沉积物在用抗血清淀粉样蛋白A抗体染色时免疫组织化学阳性。总的来说,诊断为增生性绒毛瘤伴AA淀粉样变性。肿瘤切除后,结直肠活检中慢性腹泻缓解,无明显淀粉样蛋白沉积.重要的是要记住,如果肿瘤中存在淀粉样蛋白沉积,侵袭性肿瘤切除可以缓解系统性淀粉样变性。
Although AA amyloidosis is primarily caused by inflammatory conditions, associations between AA amyloidosis and solid cancers have occasionally been described. Herein, we report the
case of a 48-year-old man in whom resection of a proliferating pilomatricoma with deposition of AA amyloid resulted in remission of concomitant AA gastrointestinal amyloidosis. A rapidly growing, giant, reddish, ulcerated tumor measuring 16 × 13 cm in size was identified on the upper left arm on a visit to our hospital. Gastrointestinal AA amyloidosis was diagnosed from colorectal mucosal biopsy at the same time, and weight loss and profuse diarrhea were clinically evident. As treatment, the tumor was resected with a 10-mm surgical margin. Histologically, the tumor predominantly comprised a lobular proliferation of basophilic cells peripherally, filled with eosinophilic, cornified material and shadow cells with mitoses observed in basophilic cells. Specimens revealed eosinophilic, homogeneous deposits around tumor nests, which were confirmed as amyloid deposits by positive staining with Congo red stain. These deposits were immunohistochemically positive on staining with anti-serum amyloid A antibody. Collectively, proliferating pilomatricoma with AA amyloidosis was diagnosed. After tumor resection, chronic diarrhea resolved and no amyloid deposition was apparent in colorectal biopsy. It is important to remember that if amyloid deposition is present in a tumor, aggressive tumor excision may alleviate systemic amyloidosis.