Primary gastrointestinal anaplastic large cell lymphoma.
Pathology
; 49(5): 479-485, 2017 Aug.
Article
em En
| MEDLINE
| ID: mdl-28693749
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma de Células T Periférico
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Receptores Proteína Tirosina Quinases
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Linfoma Anaplásico de Células Grandes
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Neoplasias Gastrointestinais
Tipo de estudo:
Diagnostic_studies
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Observational_studies
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Prognostic_studies
Limite:
Adolescent
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Adult
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Aged
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Aged80
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Child
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Humans
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Middle aged
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article