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DOI: 10.1055/s-0034-1399058
Langerhans-Zellhistiozytose des Magens mit BRAF-V600E-Mutation: Fallbericht und Literaturübersicht
Langerhans cell histiocytosis of the stomach with BRAF-V600E-mutation: case report and review of the literaturePublication History
27 November 2014
27 January 2015
Publication Date:
10 April 2015 (online)
Zusammenfassung
Langerhans-Zellhistiozytose ist eine Erkrankung mit sehr unterschiedlichen klinischen Präsentationen. Extrem selten kann die Langerhans-Zellhistiozytose den Gastrointestinaltrakt erwachsener Patienten betreffen. Ein Fall mit Magenschleimhautinfiltration durch eine Langerhans-Zellhistiozytose bei endoskopischem Normalbefund wird vorgestellt. Der neoplastische Charakter dieses Infiltrats wird durch den Nachweis einer BRAF-V600E-Mutation unterstrichen. Darüber hinaus wird eine Übersicht zu den 5 bislang in englischsprachiger Literatur publizierten Fällen mit isolierter Magenschleimhautinfiltration durch Langerhans-Zellhistiozytose gegeben.
Abstract
Langerhans cell histiocytosis is a disease with different clinical presentations and a wide spectrum of organ involvements. Rarely Langerhans cell histiocytosis can involve the gastrointestinal tract of adult patients. A case of infiltration of gastric mucosa by Langerhans cell histiocytosis is presented. The neoplastic nature of this infiltrate is underlined by the detection of a BRAF-V600E-mutation. Additionally, an overview of the so far 5 cases published in the English literature is provided. The published clinical experience indicates a benign curse of the disease.
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Literatur
- 1 Jaffe R, Weill LM, Facchetti F. Tumours derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL, et al. (Hrsg). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC; 2007: 358-360
- 2 Chang KL, Snyder DS. Langerhans cell histiocytosis. Cancer Treat Res 2008; 142: 383-398
- 3 The French Langerhans' Cell Histiocytosis Study Group. A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. Arch Dis Child 1996; 75 (01) 17-24
- 4 Vazquez JJ, Ayestaran JR. Eosinophilic granuloma of the stomach similar to that of bone: light and electron microscopic study. Virchows Arch A Pathol Anat Histol 1975; 366 (02) 107-111
- 5 Nihei K, Terashima K, Aoyama K et al. Benign histiocytosis X of stomach. Previously undescribed lesion. Acta Pathol Jpn 1983; 33 (03) 577-588
- 6 Iwafuchi M, Watanabe H, Shiratsuka M. Primary benign histiocytosis X of the stomach. A report of a case showing spontaneous remission after 5 1/2 years. Am J Surg Pathol 1990; 14 (05) 489-496
- 7 Wada R, Yagihashi S, Konta R et al. Gastric polyposis caused by multifocal histiocytosis X. Gut 1992; 33 (07) 994-996
- 8 Singhi AD, Montgomery EA. Gastrointestinal tract langerhans cell histiocytosis: A clinicopathologic study of 12 patients. Am J Surg Pathol 2011; 35 (02) 305-310
- 9 Behdad A, Owens SR. Langerhans cell histiocytosis involving the gastrointestinal trakt. Arch Pathol Lab Med 2014; 138: 1350-1352
- 10 Cao J, Luo H, Gao Z. Gastric eosinophilic granuloma in China: case series. Digestion 2005; 71 (03) 176-178
- 11 Hans SS, Hans B, Lee PT et al. Eosinophilic granulomas of the gastrointestinal tract. Am Surg 1977; 43 (08) 512-516
- 12 Yousem SA, Dacic S, Nikiforov YE et al. Pulmonary Langerhans cell histiocytosis: profiling of multifocal tumors using next-generation sequencing identifies concordant occurrence of BRAF V600E mutations. Chest 2013; 143 (06) 1679-1684
- 13 Berres ML, Lim KP, Peters T et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med 2014; 211 (04) 669-683