Salemis Nikolaos S, Pinialidis Dionisios, Tsiambas Evangelos, Gakis Christos, Nakos Georgios, Sambaziotis Dimitrios, Christofyllakis Charalambos
2nd Department of Surgery, Army General Hospital, Athens, Greece.
J Gastrointest Cancer. 2011 Sep;42(3):131-6. doi: 10.1007/s12029-010-9167-7.
BACKGROUND-PURPOSE: The risk of secondary malignancy development in patients with hairy cell leukemia has been evaluated in several studies with varying results. The aim of this study is to describe a case of synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia.
A 69-year-old man presented with rectal bleeding. Colonoscopy revealed a rectal tumor, whereas biopsy specimens revealed a poorly differentiated carcinoma. During the preoperative evaluation, pancytopenia was detected.
At laparotomy, a mass was detected 16 cm from the anal verge and an anterior resection of the rectum was performed. Detailed histological and immunohistochemical analyses revealed a poorly differentiated neuroendocrine carcinoma of the rectum. Postoperative evaluation of pancytopenia revealed hairy cell leukemia. The patient was initially treated with chemotherapy for hairy cell leukemia followed by chemotherapy for neuroendocrine colon carcinoma. Survival was 44 months.
To our knowledge, synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia has not been previously reported in the literature. Given the rare incidence of both entities in the general population, it is highly unlikely that they occurred together by chance. Further research is needed to determine what would be the optimal management options of patients with simultaneous hairy cell leukemia and a neuroendocrine colon cancer.
背景目的:多项研究评估了毛细胞白血病患者发生继发性恶性肿瘤的风险,结果各异。本研究旨在描述一例神经内分泌性结肠癌与毛细胞白血病同时发生的病例。
一名69岁男性因直肠出血就诊。结肠镜检查发现直肠肿瘤,而活检标本显示为低分化癌。术前评估时检测到全血细胞减少。
剖腹手术时,在距肛门边缘16厘米处发现一个肿块,遂行直肠前切除术。详细的组织学和免疫组化分析显示为低分化直肠神经内分泌癌。全血细胞减少的术后评估显示为毛细胞白血病。患者最初接受毛细胞白血病化疗,随后接受神经内分泌性结肠癌化疗。生存期为44个月。
据我们所知,神经内分泌性结肠癌与毛细胞白血病同时发生此前在文献中未见报道。鉴于这两种疾病在普通人群中发病率都很低,它们同时发生的可能性极小。需要进一步研究以确定同时患有毛细胞白血病和神经内分泌性结肠癌的患者的最佳治疗方案。