title: Malignant solid tumors associated with congenital immunodeficiency disorders.
abstract: This objective of this study was to evaluate patients with immunodeficiency syndromes who had developed malignant solid tumors and to examine survival rates and prognosis with respect to type of immunodeficiency disease. Twenty-two patients who were diagnosed with malignant solid tumors and immunodeficiency syndromes between January 1972 and February 2003 were analyzed retrospectively. There were 12 (55%) patients with non-Hodgkin lymphoma, 8 (37%) with Hodgkin disease, 1 (5%) with mucinous adenocarcinoma of the colon, and 1 (5%) with brain stem glioma. Fifteen (68%) patients had ataxia-telangiectasia, 3 (14%) had common variable immunodeficiency disease, 2 (9%) had Bloom syndrome, 1 (5%) had combined immunodeficiency, and 1 (5%) had selective immunoglobulin A deficiency. Out of the 15 patients with ataxia-telangiectasia 9 patients had non-Hodgkin lymphoma, 5 had Hodgkin disease, and 1 had brain stem glioma. Two patients with common variable immunodeficiency disease had non-Hodgkin lymphoma and 1 had Hodgkin disease. One of the patients with Bloom syndrome had Hodgkin disease and 1 had colon carcinoma. The overall survival for the whole group was 24%. Overall survival rates in non-Hodgkin lymphoma, Hodgkin disease, colon carcinoma, and brain stem glioma were 17, 44, 0, and 0% (p =.25), respectively. Overall survival in ataxia-telangiectasia patients was 20%. In this series, most of the patients had ataxia-telangiectasia (68%). The survival rates of the malignant diseases were very poor in immunodeficiency. Overall survival in non-Hodgkin lymphoma patients was relatively worse than Hodgkin disease patients.
authors.affiliation: Department of Pediatric Oncology, Hacettepe University, Institute of Oncology, Ankara, Turkey. avaran@hacettepe.edu.tr
authors.forename: Ali
authors.initials: A
authors.lastname: Varan
authors.forename: M
authors.initials: M
authors.lastname: Büyükpamukçu
authors.forename: Fügen
authors.initials: F
authors.lastname: Ersoy
authors.forename: Ozden
authors.initials: O
authors.lastname: Sanal
authors.forename: Canan
authors.initials: C
authors.lastname: Akyüz
authors.forename: Tezer
authors.initials: T
authors.lastname: Kutluk
authors.forename: Bilgehan
authors.initials: B
authors.lastname: Yalçin
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000151:congenital* / Q000453:epidemiology* / Q000276:immunology; D007223:Infant; D008297:Male; D009369:Neoplasms / Q000453:epidemiology* / Q000401:mortality; D012189:Retrospective Studies; D015996:Survival Rate