2017年8月
Primary gastrointestinal anaplastic large cell lymphoma
PATHOLOGY
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- 巻
- 49
- 号
- 5
- 開始ページ
- 479
- 終了ページ
- 485
- 記述言語
- 英語
- 掲載種別
- 研究論文(学術雑誌)
- DOI
- 10.1016/j.pathol.2017.05.007
- 出版者・発行元
- ELSEVIER SCIENCE BV
Primary gastrointestinal anaplastic large cell lymphoma (GI-ALCL) is rare. We report eight new cases. The median age was 61.5 years (range 10-88), most frequently involving the stomach (n = 3) and small intestine (n = 4). The neoplastic hallmark cells in all cases expressed CD30. Anaplastic lymphoma kinase (ALK) protein was expressed in two cases (25%). By in situ hybridisation, all cases were negative for Epstein-Barr virus and for DUSP22/IRF4 gene translocation. At a median follow-up time of 37.5 months, four patients died of disease, one was alive with disease, and three were disease-free. Our literature review showed that GI-ALCL occurred mainly in older patients and was characterised by a low rate of ALK expression, a high rate of T-cell lineage, and a frequent occurrence in the small intestine. Incorporating our two ALK(+) GI-ALCL cases together with the four cases in the literature, the median age was 34 years (range 10-56), with four (67%) cases in the small intestine. The six patients were all alive with a median follow-up of 21 months. The 5-year overall survival of our six patients with ALK(-) GI-ALCL was 40%, in contrast to 100% with ALK(+) GI-ALCL. The prognosis for ALK -GI-ALCL was poor, while that for the ALK+ counterparts was good.
- リンク情報
- ID情報
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- DOI : 10.1016/j.pathol.2017.05.007
- ISSN : 0031-3025
- eISSN : 1465-3931
- Web of Science ID : WOS:000405776000005